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<meta name="keywords" content="C0042384, angiitides, angiitis, disease or syndrome, inflammation of blood vessel, systemic vasculitis, vasculitides, vasculitis, autosomal dominant, autosomal recessive, birth defects, chromosomal disease, chromosome, clinical features, clinical findings, clinical genetics, clinical recommendations, clinvar, congenital chromosomal disease, consumer genetic resources, cytogenetic location, disease characteristics, disease definitions, disease descriptions, disease ontology, disease synonyms, disease vocabulary, dysmorphology, entrez, familial disease, gene, gene-disease relationship, genereviews, genetic disease, genetic disorder, genetic terminology, genetic testing registry, genetics home reference, genomic disease, gtr, hereditary disease, heritable disease, hpo, human phenotype ontology, inherited disease, management guidelines, maternal inheritance, medgen, medical genetics, medical subject headings, mesh, mitochondrial inheritance, mode of inheritance, national center for biotechnology information, national institutes of health, national library of medicine, ncbi, nih, nlm, omim, ordo, orphanet, paternal inheritance, phenome, position statements, professional practice guidelines, rare disease, reference sequence, refseq, snomed ct, syndrome, undiagnosed diseases, x-linked recessive" /><meta name="description" content="Inflammation of blood vessel." /><meta name="robots" content="index,nofollow,noarchive" />
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<!--
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||
UID=12054
|
||
ConceptID=C0042384
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-->
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<!--imgCountBooks = 0--><h1 class="medgenTitle"><div class="MedGenTitleText">Vasculitis</div></h1><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>12054</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information."><span class="highlight" style="background-color:">C0042384</span></a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div><table class="medgenTable"><tbody><tr><td>Synonyms:</td>
|
||
<td>Angiitides; Angiitis; Vasculitides</td></tr>
|
||
<tr><td><span class="bold">SNOMED CT: </span></td>
|
||
<td>Vasculitis (31996006); Angiitis (31996006)</td></tr>
|
||
<tr><td colspan="2" class="small"> </td></tr><tr><td>HPO:</td>
|
||
<td><a target="_blank" title="Human Phenotype Ontology" href="https://hpo.jax.org/app/browse/term/HP:0002633">HP:0002633</a></td></tr>
|
||
<tr><td>Monarch Initiative:</td>
|
||
<td><a href="https://monarchinitiative.org/disease/MONDO:0018882" target="_blank">MONDO:0018882</a></td></tr>
|
||
<tr><td>Orphanet:</td>
|
||
<td><a target="_blank" title="Orphanet: The portal for rare diseases and orphan drugs" href="http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=52759">ORPHA52759</a></td></tr></tbody></table></div><div class="rprt-body jig-ncbiinpagenav" data-jigconfig="smoothScroll: false, gotoTopLink: true, gotoTopLinkText: '', gotoTopLinkAttrs: {'title': 'Go to the top of the page'},allHeadingLevels: ['h1'], topOfPageTOC: true, tocId: 'my-toc'"><div id="rprt-tabs-1" class="rprt-tab"><div id="tb-termsProp-1"><div class="leftCol mgCol"><div>
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<div class="portlet mgSection" id="ID_100">
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<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Definition">Definition</h1><a sid="100" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
|
||
<div class="portlet_content ln">Inflammation of blood vessel. [from <a title="Human Phenotype Ontology" href="http://www.human-phenotype-ontology.org" class="defSource" target="_blank">HPO</a>]</div>
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</div>
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<div class="portlet mgSection" id="ID_118">
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<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Term_Hierarchy">Term Hierarchy</h1><a sid="118" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
|
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<div class="portlet_content ln HierarchyGTR"><div class="jig-ncbitabs"><ul><li><a href="#tabGTR">GTR</a></li><li><a href="#tabMGEN">MeSH</a></li></ul><div id="tabGTR"><div class="search_result"><div class="rprts"><div class="chiclet_legend"><span class="chiclet_list" style="position:static;"><span title="Clinical test" class="chiclet Ccolor round">C</span><span>Clinical test, </span><span title="Research test" class="chiclet Rcolor round">R</span><span>Research test, </span><span title="OMIM" class="chiclet Ocolor ">O</span><span>OMIM, </span><span title="GeneReview" class="chiclet Gcolor">G</span><span><em>GeneReviews</em>, </span><span title="ClinVar" class="chiclet Vcolor">V</span><span>ClinVar </span></span></div><div id="hierarchy" class="margin_t1"><div class="ds_tree"><ul><li class="matched_ds"><span class="chiclet_list"><span class="chiclet Ccolor round" title="Clinical test"><a target="_blank" href="/gtr/tests/?term=C0042384[DISCUI]&test_type=Clinical" ref="ncbi_uid=12054">C</a></span><span class="chiclet unavailable round" title="Research Tests">R</span><span class="chiclet unavailable" title="OMIM">O</span><span class="chiclet unavailable" title="GeneReviews">G</span><span class="chiclet Vcolor" title="ClinVar"><a target="_blank" href="/clinvar?LinkName=medgen_clinvar&from_uid=12054" ref="ncbi_uid=12054">V</a></span></span><span class="TLline">Vasculitis</span></li></ul></div></div></div></div></div><div id="tabMGEN"><div class="ds_tree"><ul><li><span class="TLline"><a href="/medgen/232130" ref="tree=MeSH" title="MedGen record for Disorder by Site">Disorder by Site</a></span><ul><li><span class="TLline"><a href="/medgen/2848" ref="tree=MeSH" title="MedGen record for Disorder of cardiovascular system">Disorder of cardiovascular system</a></span><ul><li><span class="TLline"><a href="/medgen/116727" ref="tree=MeSH" title="MedGen record for Abnormality of the cardiovascular system">Abnormality of the cardiovascular system</a></span><ul><li><span class="TLline"><a href="/medgen/892473" ref="tree=MeSH" title="MedGen record for Abnormal cardiovascular system morphology">Abnormal cardiovascular system morphology</a></span><ul><li><span class="TLline"><a href="/medgen/927608" ref="tree=MeSH" title="MedGen record for Abnormal vascular morphology">Abnormal vascular morphology</a></span><ul><li><span class="TLline"><a href="/medgen/1778737" ref="tree=MeSH" title="MedGen record for Abnormal blood vessel morphology">Abnormal blood vessel morphology</a></span><ul><li><span class="matched_ds">Vasculitis</span><ul><li><span class="TLline"><a href="/medgen/10118" ref="tree=MeSH" title="MedGen record for Acute febrile mucocutaneous lymph node syndrome">Acute febrile mucocutaneous lymph node syndrome</a></span></li><li><span class="TLline"><a href="/medgen/57718" ref="tree=MeSH" title="MedGen record for Allergic cutaneous vasculitis">Allergic cutaneous vasculitis</a></span></li><li><span class="TLline"><a href="/medgen/8154" ref="tree=MeSH" title="MedGen record for Aortitis">Aortitis</a></span></li><li><span class="TLline"><a href="/medgen/13916" ref="tree=MeSH" title="MedGen record for Arteritis">Arteritis</a></span><ul><li><span class="TLline"><a href="/medgen/156263" ref="tree=MeSH" title="MedGen record for Central nervous system AIDS arteritis">Central nervous system AIDS arteritis</a></span></li><li><span class="TLline"><a href="/medgen/41777" ref="tree=MeSH" title="MedGen record for Endarteritis">Endarteritis</a></span></li><li><span class="TLline"><a href="/medgen/21478" ref="tree=MeSH" title="MedGen record for Giant cell arteritis">Giant cell arteritis</a></span></li><li><span class="TLline"><a href="/medgen/9088" ref="tree=MeSH" title="MedGen record for Granulomatous angiitis">Granulomatous angiitis</a></span><ul><li><span class="TLline"><a href="/medgen/365495" ref="tree=MeSH" title="MedGen record for Temporal arteritis">Temporal arteritis</a></span></li></ul></li><li><span class="TLline"><a href="/medgen/870559" ref="tree=MeSH" title="MedGen record for Granulomatous coronary arteritis">Granulomatous coronary arteritis</a></span></li><li><span class="TLline"><a href="/medgen/308443" ref="tree=MeSH" title="MedGen record for Infectious Arteritis">Infectious Arteritis</a></span></li><li><span class="TLline"><a href="/medgen/389393" ref="tree=MeSH" title="MedGen record for Microscopic polyangiitis">Microscopic polyangiitis</a></span></li><li><span class="TLline"><a href="/medgen/154298" ref="tree=MeSH" title="MedGen record for Necrotizing arteritis">Necrotizing arteritis</a></span><ul><li><span class="TLline"><a href="/medgen/364158" ref="tree=MeSH" title="MedGen record for Overlap Necrotizing Arteritis">Overlap Necrotizing Arteritis</a></span></li></ul></li><li><span class="TLline"><a href="/medgen/14681" ref="tree=MeSH" title="MedGen record for Polyarteritis nodosa">Polyarteritis nodosa</a></span><ul><li><span class="TLline"><a href="/medgen/83358" ref="tree=MeSH" title="MedGen record for Cutaneous polyarteritis nodosa">Cutaneous polyarteritis nodosa</a></span></li><li><span class="TLline"><a href="/medgen/927526" ref="tree=MeSH" title="MedGen record for Deficiency of adenosine deaminase 2">Deficiency of adenosine deaminase 2</a></span></li><li><span class="TLline"><a href="/medgen/1842954" ref="tree=MeSH" title="MedGen record for Primary polyarteritis nodosa">Primary polyarteritis nodosa</a></span></li><li><span class="TLline"><a href="/medgen/1843373" ref="tree=MeSH" title="MedGen record for Secondary polyarteritis nodosa">Secondary polyarteritis nodosa</a></span></li></ul></li><li><span class="TLline"><a href="/medgen/21458" ref="tree=MeSH" title="MedGen record for Takayasu arteritis">Takayasu arteritis</a></span></li></ul></li><li><span class="TLline"><a href="/medgen/2568" ref="tree=MeSH" title="MedGen record for Behcet disease">Behcet disease</a></span></li><li><span class="TLline"><a href="/medgen/148408" ref="tree=MeSH" title="MedGen record for Central nervous system vasculitis">Central nervous system vasculitis</a></span><ul><li><span class="TLline"><a href="/medgen/156265" ref="tree=MeSH" title="MedGen record for Central nervous system lupus">Central nervous system lupus</a></span></li></ul></li><li><span class="TLline"><a href="/medgen/82871" ref="tree=MeSH" title="MedGen record for Cogan syndrome">Cogan syndrome</a></span></li><li><span class="TLline"><a href="/medgen/858719" ref="tree=MeSH" title="MedGen record for Fetal Chorionic Vasculitis">Fetal Chorionic Vasculitis</a></span></li><li><span class="TLline"><a href="/medgen/811223" ref="tree=MeSH" title="MedGen record for Granulomatosis with polyangiitis">Granulomatosis with polyangiitis</a></span></li><li><span class="TLline"><a href="/medgen/48265" ref="tree=MeSH" title="MedGen record for Immunoglobulin A vasculitis">Immunoglobulin A vasculitis</a></span></li><li><span class="TLline"><a href="/medgen/445500" ref="tree=MeSH" title="MedGen record for Leukocytoclastic vasculitis">Leukocytoclastic vasculitis</a></span></li><li><span class="TLline"><a href="/medgen/113138" ref="tree=MeSH" title="MedGen record for Malignant atrophic papulosis">Malignant atrophic papulosis</a></span></li><li><span class="TLline"><a href="/medgen/230813" ref="tree=MeSH" title="MedGen record for Necrotizing vasculitis">Necrotizing vasculitis</a></span><ul><li><span class="TLline"><a href="/medgen/474873" ref="tree=MeSH" title="MedGen record for Pseudomonas-Related Vasculitis">Pseudomonas-Related Vasculitis</a></span></li></ul></li><li><span class="TLline"><a href="/medgen/18425" ref="tree=MeSH" title="MedGen record for Phlebitis">Phlebitis</a></span><ul><li><span class="TLline"><a href="/medgen/206811" ref="tree=MeSH" title="MedGen record for Infective Phlebitis">Infective Phlebitis</a></span></li><li><span class="TLline"><a href="/medgen/46043" ref="tree=MeSH" title="MedGen record for Post-thrombotic syndrome">Post-thrombotic syndrome</a></span></li><li><span class="TLline"><a href="/medgen/11799" ref="tree=MeSH" title="MedGen record for Thrombophlebitis">Thrombophlebitis</a></span><ul><li><span class="TLline"><a href="/medgen/154229" ref="tree=MeSH" title="MedGen record for Injection Site Thrombophlebitis">Injection Site Thrombophlebitis</a></span></li><li><span class="TLline"><a href="/medgen/83364" ref="tree=MeSH" title="MedGen record for Lemierre syndrome">Lemierre syndrome</a></span></li><li><span class="TLline"><a href="/medgen/763064" ref="tree=MeSH" title="MedGen record for Recurrent thrombophlebitis">Recurrent thrombophlebitis</a></span></li><li><span class="TLline"><a href="/medgen/266934" ref="tree=MeSH" title="MedGen record for Superficial thrombophlebitis">Superficial thrombophlebitis</a></span></li></ul></li></ul></li><li><span class="TLline"><a href="/medgen/57503" ref="tree=MeSH" title="MedGen record for Retinal vasculitis">Retinal vasculitis</a></span><ul><li><span class="TLline"><a href="/medgen/124383" ref="tree=MeSH" title="MedGen record for Retinal arteritis">Retinal arteritis</a></span></li></ul></li><li><span class="TLline"><a href="/medgen/48656" ref="tree=MeSH" title="MedGen record for Shwartzman phenomenon">Shwartzman phenomenon</a></span></li><li><span class="TLline"><a href="/medgen/21531" ref="tree=MeSH" title="MedGen record for Thromboangiitis obliterans">Thromboangiitis obliterans</a></span></li><li><span class="TLline"><a href="/medgen/856842" ref="tree=MeSH" title="MedGen record for Umbilical Vasculitis">Umbilical Vasculitis</a></span></li><li><span class="TLline"><a href="/medgen/1842424" ref="tree=MeSH" title="MedGen record for Unclassified vasculitis">Unclassified vasculitis</a></span></li><li><span class="TLline"><a href="/medgen/1841768" ref="tree=MeSH" title="MedGen record for Vasculitis by anatomical site">Vasculitis by anatomical site</a></span><ul><li><span class="TLline"><a href="/medgen/870761" ref="tree=MeSH" title="MedGen record for Large vessel vasculitis">Large vessel vasculitis</a></span></li><li><span class="TLline"><a href="/medgen/1391820" ref="tree=MeSH" title="MedGen record for Medium vessel vasculitis">Medium vessel vasculitis</a></span></li><li><span class="TLline"><a href="/medgen/1841582" ref="tree=MeSH" title="MedGen record for Muscular vasculitis">Muscular vasculitis</a></span></li><li><span class="TLline"><a href="/medgen/1826226" ref="tree=MeSH" title="MedGen record for Pulmonary vasculitis">Pulmonary vasculitis</a></span></li><li><span class="TLline"><a href="/medgen/868710" ref="tree=MeSH" title="MedGen record for Small vessel vasculitis">Small vessel vasculitis</a></span></li><li><span class="TLline"><a href="/medgen/488809" ref="tree=MeSH" title="MedGen record for Vasculitis in the skin">Vasculitis in the skin</a></span><ul><li><span class="TLline"><a href="/medgen/811126" ref="tree=MeSH" title="MedGen record for Acute Hemorrhagic Edema of Infancy">Acute Hemorrhagic Edema of Infancy</a></span></li><li><span class="TLline"><a href="/medgen/881641" ref="tree=MeSH" title="MedGen record for Cutaneous leukocytoclastic angiitis">Cutaneous leukocytoclastic angiitis</a></span></li><li><span class="TLline"><a href="/medgen/817999" ref="tree=MeSH" title="MedGen record for Drug Induced Cutaneous Vasculitis">Drug Induced Cutaneous Vasculitis</a></span></li><li><span class="TLline"><a href="/medgen/479323" ref="tree=MeSH" title="MedGen record for Punctate vasculitis skin lesions">Punctate vasculitis skin lesions</a></span></li></ul></li></ul></li><li><span class="TLline"><a href="/medgen/1841577" ref="tree=MeSH" title="MedGen record for Vasculitis by histology">Vasculitis by histology</a></span><ul><li><span class="TLline"><a href="/medgen/870765" ref="tree=MeSH" title="MedGen record for Inflammatory arteriopathy">Inflammatory arteriopathy</a></span></li><li><span class="TLline"><a href="/medgen/870762" ref="tree=MeSH" title="MedGen record for Nodular inflammatory vasculitis">Nodular inflammatory vasculitis</a></span></li></ul></li><li><span class="TLline"><a href="/medgen/82688" ref="tree=MeSH" title="MedGen record for Vasculitis of systemic vasculature">Vasculitis of systemic vasculature</a></span><ul><li><span class="TLline"><a href="/medgen/403453" ref="tree=MeSH" title="MedGen record for Anti-neutrophil cytoplasmic antibody-associated vasculitis">Anti-neutrophil cytoplasmic antibody-associated vasculitis</a></span><ul><li><span class="TLline"><a href="/medgen/3088" ref="tree=MeSH" title="MedGen record for Eosinophilic granulomatosis with polyangiitis">Eosinophilic granulomatosis with polyangiitis</a></span></li></ul></li><li><span class="TLline"><a href="/medgen/69122" ref="tree=MeSH" title="MedGen record for Rheumatoid vasculitis">Rheumatoid vasculitis</a></span></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></div></div></div></div>
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<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Conditions_with_this_feature">Conditions with this feature</h1><a sid="112" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
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<div class="divPopper rprt" id="rdis_2078"><div><strong>Rheumatoid arthritis</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>2078</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information.">C0003873</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
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<div class="spaceAbove">Rheumatoid arthritis is an inflammatory disease, primarily of the joints, with autoimmune features and a complex genetic component.</div>
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<div class="spaceAbove nowrap">See: <a href="/medgen/2078">Condition Record</a></div></div>
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<div class="divPopper rprt" id="rdis_120634"><div><strong>Familial amyloid nephropathy with urticaria AND deafness</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>120634</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information.">C0268390</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
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<div class="spaceAbove">Muckle-Wells syndrome (MWS) is characterized by episodic skin rash, arthralgias, and fever associated with late-onset sensorineural deafness and renal amyloidosis (Dode et al., 2002).</div>
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<div class="spaceAbove nowrap">See: <a href="/medgen/120634">Condition Record</a></div></div>
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<div class="divPopper rprt" id="rdis_96073"><div><strong>Familial cutaneous collagenoma</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>96073</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information.">C0406817</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Neoplastic Process</dd></dl></div></div></div>
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<div class="spaceAbove">Familial cutaneous collagenoma is a connective tissue nevus characterized by multiple, flesh-colored asymptomatic nodules distributed symmetrically on the trunk and upper arms (mainly on the upper two-thirds of the back), manifesting around adolescence. The skin biopsy reveals an accumulation of collagen fibers with reduction in the number of elastic fibers. Cardiac anomalies may be observed. Familial cutaneous collagenoma follows an autosomal dominant mode of transmission.</div>
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<div class="spaceAbove nowrap">See: <a href="/medgen/96073">Condition Record</a></div></div>
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<div class="divPopper rprt" id="rdis_162912"><div><strong>Aicardi-Goutieres syndrome 1</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>162912</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information.">C0796126</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
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<div class="spaceAbove">Most characteristically, Aicardi-Goutières syndrome (AGS) manifests as an early-onset encephalopathy that usually, but not always, results in severe intellectual and physical disability. A subgroup of infants with AGS present at birth with abnormal neurologic findings, hepatosplenomegaly, elevated liver enzymes, and thrombocytopenia, a picture highly suggestive of congenital infection. Otherwise, most affected infants present at variable times after the first few weeks of life, frequently after a period of apparently normal development. Typically, they demonstrate the subacute onset of a severe encephalopathy characterized by extreme irritability, intermittent sterile pyrexias, loss of skills, and slowing of head growth. Over time, as many as 40% develop chilblain skin lesions on the fingers, toes, and ears. It is becoming apparent that atypical, sometimes milder, cases of AGS exist, and thus the true extent of the phenotype associated with pathogenic variants in the AGS-related genes is not yet known.</div>
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<div class="spaceAbove nowrap">See: <a href="/medgen/162912">Condition Record</a></div></div>
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<div class="divPopper rprt" id="rdis_231300"><div><strong>Autoimmune lymphoproliferative syndrome type 1</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>231300</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information.">C1328840</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
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<div class="spaceAbove">Autoimmune lymphoproliferative syndrome (ALPS), caused by defective lymphocyte homeostasis, is characterized by the following: Non-malignant lymphoproliferation (lymphadenopathy, hepatosplenomegaly with or without hypersplenism) that often improves with age. Autoimmune disease, mostly directed toward blood cells. Lifelong increased risk for both Hodgkin and non-Hodgkin lymphoma. In ALPS-FAS (the most common and best-characterized type of ALPS, associated with heterozygous germline pathogenic variants in FAS), non-malignant lymphoproliferation typically manifests in the first years of life, inexplicably waxes and wanes, and then often decreases without treatment in the second decade of life; in many affected individuals, however, neither splenomegaly nor the overall expansion of lymphocyte subsets in peripheral blood decreases. Although autoimmunity is often not present at the time of diagnosis or at the time of the most extensive lymphoproliferation, autoantibodies can be detected before autoimmune disease manifests clinically. In ALPS-FAS caused by homozygous or compound heterozygous (biallelic) pathogenic variants in FAS, severe lymphoproliferation occurs before, at, or shortly after birth, and usually results in death at an early age. ALPS-sFAS, resulting from somatic FAS pathogenic variants in selected cell populations, notably the alpha/beta double-negative T cells (a/ß-DNT cells), appears to be similar to ALPS-FAS resulting from heterozygous germline pathogenic variants in FAS, although lower incidence of splenectomy and lower lymphocyte counts have been reported in ALPS-sFAS and no cases of lymphoma have yet been published.</div>
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<div class="spaceAbove nowrap">See: <a href="/medgen/231300">Condition Record</a></div></div>
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<div class="divPopper rprt" id="rdis_349065"><div><strong>Autoimmune lymphoproliferative syndrome type 2A</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>349065</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information.">C1858968</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
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<div class="spaceAbove">Autoimmune lymphoproliferative syndrome (ALPS), caused by defective lymphocyte homeostasis, is characterized by the following: Non-malignant lymphoproliferation (lymphadenopathy, hepatosplenomegaly with or without hypersplenism) that often improves with age. Autoimmune disease, mostly directed toward blood cells. Lifelong increased risk for both Hodgkin and non-Hodgkin lymphoma. In ALPS-FAS (the most common and best-characterized type of ALPS, associated with heterozygous germline pathogenic variants in FAS), non-malignant lymphoproliferation typically manifests in the first years of life, inexplicably waxes and wanes, and then often decreases without treatment in the second decade of life; in many affected individuals, however, neither splenomegaly nor the overall expansion of lymphocyte subsets in peripheral blood decreases. Although autoimmunity is often not present at the time of diagnosis or at the time of the most extensive lymphoproliferation, autoantibodies can be detected before autoimmune disease manifests clinically. In ALPS-FAS caused by homozygous or compound heterozygous (biallelic) pathogenic variants in FAS, severe lymphoproliferation occurs before, at, or shortly after birth, and usually results in death at an early age. ALPS-sFAS, resulting from somatic FAS pathogenic variants in selected cell populations, notably the alpha/beta double-negative T cells (a/ß-DNT cells), appears to be similar to ALPS-FAS resulting from heterozygous germline pathogenic variants in FAS, although lower incidence of splenectomy and lower lymphocyte counts have been reported in ALPS-sFAS and no cases of lymphoma have yet been published.</div>
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<div class="spaceAbove nowrap">See: <a href="/medgen/349065">Condition Record</a></div></div>
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<div class="divPopper rprt" id="rdis_356415"><div><strong>Hyperzincemia with functional zinc depletion</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>356415</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information.">C1865986</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
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<div class="spaceAbove">Autoinflammatory syndrome with cytopenia, hyperzincemia, and hypercalprotectinemia (AICZC) is characterized by chronic systemic inflammation, prominent skin inflammation, arthralgias/arthritis, hepatosplenomegaly, pancytopenia, and failure to thrive. A hallmark of the disease is the extreme increase in serum concentrations of zinc and the proinflammatory alarmins MRP8 (S100A8; 123885) and MRP14 (S100A9; 123886), which make up the heterodimeric calcium- and zinc-binding protein complex calprotectin. Severe anemia and neutropenia are accompanied by thrombocytopenia in most patients, and bone marrow aspirates show dysgranulopoiesis and dyserythropoiesis. Intrafamilial variable expressivity and incomplete penetrance have been observed (summary by Holzinger et al., 2015).</div>
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<div class="spaceAbove nowrap">See: <a href="/medgen/356415">Condition Record</a></div></div>
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<div class="divPopper rprt" id="rdis_482272"><div><strong>Complement component 4a deficiency</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>482272</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information.">C3280642</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Finding</dd></dl></div></div></div>
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<div class="spaceAbove">Any classic complement early component deficiency in which the cause of the disease is a mutation in the C4A gene.</div>
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<div class="spaceAbove nowrap">See: <a href="/medgen/482272">Condition Record</a></div></div>
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<div class="divPopper rprt" id="rdis_483045"><div><strong>Factor I deficiency</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>483045</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information.">C3463916</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
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<div class="spaceAbove">C3 glomerulopathy (C3G) is a complex ultra-rare complement-mediated renal disease caused by uncontrolled activation of the complement alternative pathway (AP) in the fluid phase (as opposed to cell surface) that is rarely inherited in a simple mendelian fashion. C3G affects individuals of all ages, with a median age at diagnosis of 23 years. Individuals with C3G typically present with hematuria, proteinuria, hematuria and proteinuria, acute nephritic syndrome or nephrotic syndrome, and low levels of the complement component C3. Spontaneous remission of C3G is uncommon, and about half of affected individuals develop end-stage renal disease (ESRD) within ten years of diagnosis, occasionally developing the late comorbidity of impaired visual acuity.</div>
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<div class="spaceAbove nowrap">See: <a href="/medgen/483045">Condition Record</a></div></div>
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<div class="divPopper rprt" id="rdis_854497"><div><strong>Vasculitis due to ADA2 deficiency</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>854497</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information.">C3887654</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
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<div class="spaceAbove">Adenosine deaminase 2 deficiency (DADA2) is a complex systemic autoinflammatory disorder in which vasculopathy/vasculitis, dysregulated immune function, and/or hematologic abnormalities may predominate. Inflammatory features include intermittent fevers, rash (often livedo racemosa/reticularis), and musculoskeletal involvement (myalgia/arthralgia, arthritis, myositis). Vasculitis, which usually begins before age ten years, may manifest as early-onset ischemic (lacunar) and/or hemorrhagic strokes, or as cutaneous or systemic polyarteritis nodosa. Hypertension and hepatosplenomegaly are often found. More severe involvement may lead to progressive central neurologic deficits (dysarthria, ataxia, cranial nerve palsies, cognitive impairment) or to ischemic injury to the kidney, intestine, and/or digits. Dysregulation of immune function can lead to immunodeficiency or autoimmunity of varying severity; lymphadenopathy may be present and some affected individuals have had lymphoproliferative disease. Hematologic disorders may begin early in life or in late adulthood, and can include lymphopenia, neutropenia, pure red cell aplasia, thrombocytopenia, or pancytopenia. Of note, both interfamilial and intrafamilial phenotypic variability (e.g., in age of onset, frequency and severity of manifestations) can be observed; also, individuals with biallelic ADA2 pathogenic variants may remain asymptomatic until adulthood or may never develop clinical manifestations of DADA2.</div>
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<div class="spaceAbove nowrap">See: <a href="/medgen/854497">Condition Record</a></div></div>
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<div class="divPopper rprt" id="rdis_854829"><div><strong>Aicardi-Goutieres syndrome 7</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>854829</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information.">C3888244</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
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<div class="spaceAbove">Most characteristically, Aicardi-Goutières syndrome (AGS) manifests as an early-onset encephalopathy that usually, but not always, results in severe intellectual and physical disability. A subgroup of infants with AGS present at birth with abnormal neurologic findings, hepatosplenomegaly, elevated liver enzymes, and thrombocytopenia, a picture highly suggestive of congenital infection. Otherwise, most affected infants present at variable times after the first few weeks of life, frequently after a period of apparently normal development. Typically, they demonstrate the subacute onset of a severe encephalopathy characterized by extreme irritability, intermittent sterile pyrexias, loss of skills, and slowing of head growth. Over time, as many as 40% develop chilblain skin lesions on the fingers, toes, and ears. It is becoming apparent that atypical, sometimes milder, cases of AGS exist, and thus the true extent of the phenotype associated with pathogenic variants in the AGS-related genes is not yet known.</div>
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<div class="spaceAbove nowrap">See: <a href="/medgen/854829">Condition Record</a></div></div>
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<div class="divPopper rprt" id="rdis_934581"><div><strong>Infantile-onset periodic fever-panniculitis-dermatosis syndrome</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>934581</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information.">C4310614</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
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<div class="spaceAbove">Autosomal recessive autoinflammation, panniculitis, and dermatosis syndrome (AIPDSB) is an autoinflammatory disease characterized by neonatal onset of recurrent fever, erythematous rash with painful nodules, painful joints, and lipodystrophy. Additional features may include diarrhea, increased serum C-reactive protein (CRP; 123260), leukocytosis, and neutrophilia in the absence of any infection. Patients exhibit no overt primary immunodeficiency (Damgaard et al., 2016 and Zhou et al., 2016).</div>
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<div class="spaceAbove nowrap">See: <a href="/medgen/934581">Condition Record</a></div></div>
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<div class="divPopper rprt" id="rdis_1618052"><div><strong>Platelet abnormalities with eosinophilia and immune-mediated inflammatory disease</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1618052</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information.">C4540232</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
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<div class="spaceAbove">Immunodeficiency-71 with inflammatory disease and congenital thrombocytopenia (IMD71) is an autosomal recessive immunologic disorder characterized by the onset of recurrent infections and inflammatory features such as vasculitis and eczema in infancy or early childhood. Infectious agents include bacteria and viruses. Laboratory findings are variable, but usually show thrombocytopenia, sometimes with abnormal platelet morphology, increased serum IgE, IgA, or IgM, leukocytosis, decreased or increased T lymphocytes, and increased eosinophils. Detailed studies show impaired neutrophil and T-cell chemotaxis, as well as impaired T-cell activation due to defects in F-actin (see 102610) polymerization (summary by Brigida et al., 2018).</div>
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<div class="spaceAbove nowrap">See: <a href="/medgen/1618052">Condition Record</a></div></div>
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<div class="divPopper rprt" id="rdis_1770239"><div><strong>X-linked lymphoproliferative disease due to SH2D1A deficiency</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1770239</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information.">C5399825</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
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<div class="spaceAbove">X-linked lymphoproliferative disease (XLP) in general is characterized by an inappropriate immune response to Epstein-Barr virus (EBV) infection leading to hemophagocytic lymphohistiocytosis (HLH) or severe mononucleosis, dysgammaglobulinemia, and lymphoproliferative disease (malignant lymphoma). The condition primarily affects males. XLP has two recognizable subtypes, XLP1 (due to pathogenic variants in SH2D1A) and XLP2 (due to pathogenic variants in XIAP). HLH / fulminant infectious mononucleosis is the most common presentation regardless of subtype. HLH is characterized as an acute illness with prolonged and high fever, bi- or trilineage cytopenias, and hepatosplenomegaly, which is often severe or fatal. Death is generally secondary to liver failure or multisystem organ dysfunction. In those with XLP1, dys- or hypogammaglobulinemia can lead to varying degrees of humoral immune dysfunction associated with bronchiectasis and recurrent respiratory infections that, if untreated, may result in death. Lymphoproliferative disease (malignant lymphoma) and other lymphoproliferative diseases are specific to XLP1 and often develop in childhood, usually following EBV exposure. Rarer findings in those with XLP1 can include aplastic anemia, vasculitis, and lymphoid granulomatosis. Males with XLP2 are more likely to have HLH without EBV infection, recurrent episodes of HLH (which is not typically seen in those with XLP1), splenomegaly, and gastrointestinal disease, including enterocolitis and perirectal abscesses or fistulae. Rarely, individuals with XLP2 and inflammatory bowel disease have been reported to develop inflammatory liver disease, which can progress to fatal liver failure. Transient hypogammaglobulinemia has been rarely observed in those with XLP2. To date, neither lymphoproliferative disease nor common variable immunodeficiency has been reported in males with XLP2. Heterozygous females rarely have symptoms. There are, however, increasing numbers of reports of affected females with unfavorable (skewed) X-chromosome inactivation favoring the X chromosome with the pathogenic variant who develop HLH, inflammatory bowel disease, and erythema nodosum.</div>
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<div class="spaceAbove nowrap">See: <a href="/medgen/1770239">Condition Record</a></div></div>
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<div class="divPopper rprt" id="rdis_1855512"><div><strong>Autoinflammation with arthritis and vasculitis</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1855512</dd><dt><span class="dotprefix"> •</span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS) Click for more information.">C5935634</a></dd><dt><span class="dotprefix"> •</span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
|
||
<div class="spaceAbove">Autoinflammation with arthritis and vasculitis (AIARV) is an autosomal recessive complex immunologic disorder with onset of symptoms in infancy or early childhood. Affected individuals have recurrent fever, erythematous skin rashes, vasculitis, oral aphthous lesions, and polyarthritis. Laboratory studies are consistent with an inflammatory state. Although patients may have recurrent infections, the infections are not severe. Additional features may include poor overall growth, microcytic anemia, mildly impaired intellectual development, seizures, and variable brain imaging abnormalities. Treatment with TNF (191160) inhibitors may result in clinical improvement (Taft et al., 2021).</div>
|
||
<div class="spaceAbove nowrap">See: <a href="/medgen/1855512">Condition Record</a></div></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_162912" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Aicardi-Goutieres syndrome 1</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_854829" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Aicardi-Goutieres syndrome 7</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_231300" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Autoimmune lymphoproliferative syndrome type 1</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_349065" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Autoimmune lymphoproliferative syndrome type 2A</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_1855512" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Autoinflammation with arthritis and vasculitis</a></div><div class="jig-moreless" data-jigconfig="class: 'moveDown', moreText: 'See full list (15)', lessText: 'Show less', nodeBefore: 0"><span id="clinMore">
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_482272" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Complement component 4a deficiency</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_483045" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Factor I deficiency</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_120634" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Familial amyloid nephropathy with urticaria AND deafness</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_96073" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Familial cutaneous collagenoma</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_356415" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Hyperzincemia with functional zinc depletion</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_934581" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Infantile-onset periodic fever-panniculitis-dermatosis syndrome</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_1618052" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Platelet abnormalities with eosinophilia and immune-mediated inflammatory disease</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_2078" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Rheumatoid arthritis</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_854497" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Vasculitis due to ADA2 deficiency</a></div>
|
||
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_1770239" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">X-linked lymphoproliferative disease due to SH2D1A deficiency</a></div></span></div></div>
|
||
</div>
|
||
|
||
<div class="portlet mgSection" id="ID_105">
|
||
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Professional_guidelines">Professional guidelines</h1><a sid="105" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
|
||
<div class="portlet_content ln"><h3 class="subhead">PubMed<a class="help jig-ncbi-popper" data-jig="ncbipopper" href="#guidelinesHelpPM"><img class="pulldown" src="//static.pubmed.gov/portal/portal3rc.fcgi/4223267/img/4204968" /></a></h3>
|
||
<div class="nl"><a target="_blank" href="/pubmed/38368016">Diagnosis and management of ANCA-associated vasculitis.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Kronbichler A,
|
||
Bajema IM,
|
||
Bruchfeld A,
|
||
Mastroianni Kirsztajn G,
|
||
Stone JH</span><br />
|
||
<span class="medgenPMjournal">Lancet</span>
|
||
2024 Feb 17;403(10427):683-698.
|
||
doi: 10.1016/S0140-6736(23)01736-1.
|
||
<span class="bold">PMID: </span><a href="/pubmed/38368016" target="_blank">38368016</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/35215786">Herpes zoster: A Review of Clinical Manifestations and Management.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Patil A,
|
||
Goldust M,
|
||
Wollina U</span><br />
|
||
<span class="medgenPMjournal">Viruses</span>
|
||
2022 Jan 19;14(2)
|
||
doi: 10.3390/v14020192.
|
||
<span class="bold">PMID: </span><a href="/pubmed/35215786" target="_blank">35215786</a><a href="/pmc/articles/PMC8876683" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/28356445">Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">McCrindle BW,
|
||
Rowley AH,
|
||
Newburger JW,
|
||
Burns JC,
|
||
Bolger AF,
|
||
Gewitz M,
|
||
Baker AL,
|
||
Jackson MA,
|
||
Takahashi M,
|
||
Shah PB,
|
||
Kobayashi T,
|
||
Wu MH,
|
||
Saji TT,
|
||
Pahl E;
|
||
American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young; Council on Cardiovascular and Stroke Nursing; Council on Cardiovascular Surgery and Anesthesia; and Council on Epidemiology and Prevention</span><br />
|
||
<span class="medgenPMjournal">Circulation</span>
|
||
2017 Apr 25;135(17):e927-e999.
|
||
Epub 2017 Mar 29
|
||
doi: 10.1161/CIR.0000000000000484.
|
||
<span class="bold">PMID: </span><a href="/pubmed/28356445" target="_blank">28356445</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=(%22vasculitis%22%5Btiab%3A~0%5D)%20AND%20(%22english%20and%20humans%22%5BFilter%5D)%20AND%20(%20(%22practice%20guideline%22%5BFilter%5D)%20OR%20(practice*%5Btitl%5D%20AND%20(guideline%5Btitl%5D%20OR%20parameter%5Btitl%5D%20OR%20resource%5Btitl%5D%20OR%20bulletin%5Btitl%5D%20OR%20best%5Btitl%5D))%20OR%20(genetic*%5Btitl%5D%20AND%20(evaluation%5Btitl%5D%20OR%20counseling%5Btitl%5D%20OR%20screening%5Btitl%5D%20OR%20test*%5Btitl%5D))%20OR%20(clinical%5Btitl%5D%20AND%20((expert%5Btitl%5D%20AND%20consensus%5Btitl%5D)%20OR%20utility%5Btitl%5D%20OR%20guideline*%5Btitl%5D))%20OR%20(management%5Btitl%5D%20AND%20(clinical%5Btitl%5D%20OR%20diagnos*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20pain%5Btitl%5D%20OR%20surveillance%5Btitl%5D%20OR%20emergency%5Btitl%5D%20OR%20guideline*%5Btitl%5D%20OR%20therap*))%20OR%20(treatment%5Btitl%5D%20AND%20((evaluation%5Btitl%5D%20AND%20diagnosis%5Btitl%5D)%20OR%20(assessment%5Btitl%5D%20AND%20prevention%5Btitl%5D)%20OR%20therap*))%20OR%20(Diagnos*%5Btitl%5D%20AND%20(prenatal%5Btitl%5D%20OR%20treatment%5Btitl%5D%20OR%20follow-up%5Btitl%5D%20OR%20statement%5Btitl%5D%20OR%20criteria%5Btitl%5D%20OR%20newborn%5Btitl%5D%20OR%20differential%5Btitl%5D%20OR%20neonatal%5Btitl%5D%20OR%20neonate%5Btitl%5D))%20OR%20(guideline*%5Btitl%5D%20AND%20(pharmacogenetic*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20evidence-based%5Btitl%5D%20OR%20consensus%5Btitl%5D%20OR%20(technical%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20(molecular%5Btitl%5D%20AND%20testing%5Btitl%5D)))%20OR%20(risk%5Btitl%5D%20AND%20assessment%5Btitl%5D)%20OR%20(recommendation*%5Btitl%5D%20AND%20(statement%5Btitl%5D%20OR%20Evidence-based%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(care%20AND%20((Patient%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20primary%5Btitl%5D%20OR%20psychosocial%5Btitl%5D))%20OR%20(Health%5Btitl%5D%20AND%20supervision%5Btitl%5D)%20OR%20(statement%5Btitl%5D%20AND%20(policy%5Btitl%5D%20OR%20position%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(pharmacogenetics%5Btitl%5D%20AND%20(Dosing%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20genotype*%5Btitl%5D%20OR%20drug*%5Btitl%5D))%20OR%20(Chemotherapy%5Btitl%5D%20AND%20decision*%5Btitl%5D)%20OR%20(screening%5Btitl%5D%20AND%20(newborn%5Btitl%5D%20OR%20neonat*%5Btitl%5D%20OR%20detection%5Btitl%5D%20OR%20diagnos*%5Btitl%5D))%20OR%20(criteria%5Btitl%5D%20OR%20genotype*%5Btitl%5D)%20)%20NOT%20(%22Case%20reports%22%5BPublication%20type%5D%20OR%20%22clinical%20study%22%5BPublication%20Type%5D%20OR%20%22randomized%20controlled%20trial%22%5BPublication%20Type%5D)" title="PubMed search">See all (1526)</a></div></div>
|
||
</div>
|
||
<div class="display-none help-popup" id="guidelinesHelpPM">These guidelines are articles in PubMed that match specific search criteria developed by MedGen to capture the most relevant practice guidelines. This list may not be comprehensive and may include broader topics as well. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div><div class="display-none help-popup" id="guidelinesHelpCurated">These guidelines are manually curated by the MedGen team
|
||
to supplement articles available in PubMed. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div>
|
||
<div class="portlet mgSection" id="ID_103">
|
||
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Recent_clinical_studies">Recent clinical studies</h1><a sid="103" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
|
||
<div class="portlet_content ln"><h3 class="subhead">Etiology</h3>
|
||
<div class="nl"><a target="_blank" href="/pubmed/38683204">Systemic vasculitis: one year in review 2024.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Treppo E,
|
||
Monti S,
|
||
Delvino P,
|
||
Marvisi C,
|
||
Ricordi C,
|
||
La Rocca G,
|
||
Moretti M,
|
||
Italiano N,
|
||
Di Cianni F,
|
||
Ferro F,
|
||
Muratore F,
|
||
Baldini C,
|
||
Talarico R,
|
||
Quartuccio L,
|
||
Salvarani C</span><br />
|
||
<span class="medgenPMjournal">Clin Exp Rheumatol</span>
|
||
2024 Apr;42(4):771-781.
|
||
Epub 2024 Apr 29
|
||
doi: 10.55563/clinexprheumatol/gkve60.
|
||
<span class="bold">PMID: </span><a href="/pubmed/38683204" target="_blank">38683204</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/37423264">Annular vasculitic lesions.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Wang HY,
|
||
Robson DC,
|
||
Kim SJ</span><br />
|
||
<span class="medgenPMjournal">Clin Dermatol</span>
|
||
2023 May-Jun;41(3):326-339.
|
||
Epub 2023 Jul 8
|
||
doi: 10.1016/j.clindermatol.2023.07.002.
|
||
<span class="bold">PMID: </span><a href="/pubmed/37423264" target="_blank">37423264</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/28148583">ANCA-associated vasculitis.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Yates M,
|
||
Watts R</span><br />
|
||
<span class="medgenPMjournal">Clin Med (Lond)</span>
|
||
2017 Feb;17(1):60-64.
|
||
doi: 10.7861/clinmedicine.17-1-60.
|
||
<span class="bold">PMID: </span><a href="/pubmed/28148583" target="_blank">28148583</a><a href="/pmc/articles/PMC6297586" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/24331794">Vasculitic neuropathies.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Gwathmey KG,
|
||
Burns TM,
|
||
Collins MP,
|
||
Dyck PJ</span><br />
|
||
<span class="medgenPMjournal">Lancet Neurol</span>
|
||
2014 Jan;13(1):67-82.
|
||
doi: 10.1016/S1474-4422(13)70236-9.
|
||
<span class="bold">PMID: </span><a href="/pubmed/24331794" target="_blank">24331794</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/22271809">Drug-induced vasculitis: a clinical and pathological review.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Radić M,
|
||
Martinović Kaliterna D,
|
||
Radić J</span><br />
|
||
<span class="medgenPMjournal">Neth J Med</span>
|
||
2012 Jan;70(1):12-7.
|
||
<span class="bold">PMID: </span><a href="/pubmed/22271809" target="_blank">22271809</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Vasculitis%22%20AND%20Etiology%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (12048)</a></div><h3 class="subhead">Diagnosis</h3>
|
||
<div class="nl"><a target="_blank" href="/pubmed/37073639">Systemic vasculitis: one year in review 2023.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Moretti M,
|
||
Treppo E,
|
||
Monti S,
|
||
La Rocca G,
|
||
Del Frate G,
|
||
Delvino P,
|
||
Italiano N,
|
||
Di Cianni F,
|
||
D'Alessandro F,
|
||
Talarico R,
|
||
Ferro F,
|
||
Quartuccio L,
|
||
Baldini C</span><br />
|
||
<span class="medgenPMjournal">Clin Exp Rheumatol</span>
|
||
2023 Apr;41(4):765-773.
|
||
Epub 2023 Apr 18
|
||
doi: 10.55563/clinexprheumatol/zf4daj.
|
||
<span class="bold">PMID: </span><a href="/pubmed/37073639" target="_blank">37073639</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/33713282">Diagnosis and management of leukocytoclastic vasculitis.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Fraticelli P,
|
||
Benfaremo D,
|
||
Gabrielli A</span><br />
|
||
<span class="medgenPMjournal">Intern Emerg Med</span>
|
||
2021 Jun;16(4):831-841.
|
||
Epub 2021 Mar 13
|
||
doi: 10.1007/s11739-021-02688-x.
|
||
<span class="bold">PMID: </span><a href="/pubmed/33713282" target="_blank">33713282</a><a href="/pmc/articles/PMC8195763" target="_blank" class="PubMedFree">Free PMC Article</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/32348513">Childhood vasculitis.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Ozen S,
|
||
Sag E</span><br />
|
||
<span class="medgenPMjournal">Rheumatology (Oxford)</span>
|
||
2020 May 1;59(Suppl 3):iii95-iii100.
|
||
doi: 10.1093/rheumatology/kez599.
|
||
<span class="bold">PMID: </span><a href="/pubmed/32348513" target="_blank">32348513</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/27720000">Epidemiology of Neurovasculitis.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Younger DS</span><br />
|
||
<span class="medgenPMjournal">Neurol Clin</span>
|
||
2016 Nov;34(4):887-917.
|
||
doi: 10.1016/j.ncl.2016.06.006.
|
||
<span class="bold">PMID: </span><a href="/pubmed/27720000" target="_blank">27720000</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/24703534">Headaches and vasculitis.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Younger DS</span><br />
|
||
<span class="medgenPMjournal">Neurol Clin</span>
|
||
2014 May;32(2):321-62.
|
||
doi: 10.1016/j.ncl.2013.11.004.
|
||
<span class="bold">PMID: </span><a href="/pubmed/24703534" target="_blank">24703534</a></div>
|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Vasculitis%22%20AND%20Diagnosis%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (19344)</a></div><h3 class="subhead">Therapy</h3>
|
||
<div class="nl"><a target="_blank" href="/pubmed/33596356">Avacopan for the Treatment of ANCA-Associated Vasculitis.</a></div>
|
||
<div class="portlet_content ln"><span class="medgenPMauthor">Jayne DRW,
|
||
Merkel PA,
|
||
Schall TJ,
|
||
Bekker P;
|
||
ADVOCATE Study Group</span><br />
|
||
<span class="medgenPMjournal">N Engl J Med</span>
|
||
2021 Feb 18;384(7):599-609.
|
||
doi: 10.1056/NEJMoa2023386.
|
||
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<div class="nl"><a target="_blank" href="/pubmed/12628043">Granulomatous vasculitis.</a></div>
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<div class="nl"><a target="_blank" href="/pubmed/28515156">Anti-Glomerular Basement Membrane Disease.</a></div>
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<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Vasculitis%22%20AND%20Clinical%20prediction%20guides%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (7782)</a></div></div>
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</div>
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<div class="portlet mgSection" id="ID_104">
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<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Recent_systematic_reviews">Recent systematic reviews</h1><a sid="104" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
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<div class="portlet_content ln">
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Kronbichler A,
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Little MA,
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Luqmani RA,
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Mahr A,
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Merkel PA,
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Mohammad AJ,
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Monti S,
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Mukhtyar CB,
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Musial J,
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Price-Kuehne F,
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Segelmark M,
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Teng YKO,
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Terrier B,
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Tomasson G,
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<span class="bold">PMID: </span><a href="/pubmed/36927642" target="_blank">36927642</a></div>
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<div class="nl"><a target="_blank" href="/pubmed/34995763">Myocarditis in systemic immune-mediated diseases: Prevalence, characteristics and prognosis. A systematic review.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Cheng CY,
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Baritussio A,
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Giordani AS,
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Iliceto S,
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Caforio ALP</span><br />
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<span class="bold">PMID: </span><a href="/pubmed/34995763" target="_blank">34995763</a></div>
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||
<div class="nl"><a target="_blank" href="/pubmed/34235894">2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Chung SA,
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Langford CA,
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Maz M,
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Abril A,
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Gorelik M,
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Guyatt G,
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Archer AM,
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Conn DL,
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Full KA,
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Grayson PC,
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Ibarra MF,
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Imundo LF,
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Kim S,
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Merkel PA,
|
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Rhee RL,
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Seo P,
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Stone JH,
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Sule S,
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Sundel RP,
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Vitobaldi OI,
|
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Warner A,
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Byram K,
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Dua AB,
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Husainat N,
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James KE,
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Kalot MA,
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Lin YC,
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Springer JM,
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Turgunbaev M,
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Villa-Forte A,
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Turner AS,
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Mustafa RA</span><br />
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<span class="medgenPMjournal">Arthritis Rheumatol</span>
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<span class="bold">PMID: </span><a href="/pubmed/34235894" target="_blank">34235894</a></div>
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|
||
<div class="nl"><a target="_blank" href="/pubmed/34235884">2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis.</a></div>
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<div class="portlet_content ln"><span class="medgenPMauthor">Maz M,
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Chung SA,
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Abril A,
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Langford CA,
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Gorelik M,
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Guyatt G,
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Archer AM,
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Conn DL,
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Full KA,
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Grayson PC,
|
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Ibarra MF,
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Imundo LF,
|
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Kim S,
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Merkel PA,
|
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Rhee RL,
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Seo P,
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Stone JH,
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Sule S,
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||
Sundel RP,
|
||
Vitobaldi OI,
|
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Warner A,
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Byram K,
|
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Dua AB,
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Husainat N,
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James KE,
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Kalot MA,
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Lin YC,
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Springer JM,
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Turgunbaev M,
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Villa-Forte A,
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Turner AS,
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Mustafa RA</span><br />
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<span class="medgenPMjournal">Arthritis Rheumatol</span>
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2021 Aug;73(8):1349-1365.
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Epub 2021 Jul 8
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doi: 10.1002/art.41774.
|
||
<span class="bold">PMID: </span><a href="/pubmed/34235884" target="_blank">34235884</a></div>
|
||
|
||
<div class="nl"><a target="_blank" href="/pubmed/27373108">COGAN'S SYNDROME.</a></div>
|
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<div class="portlet_content ln"><span class="medgenPMauthor">Iliescu DA,
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Timaru CM,
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Batras M,
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De Simone A,
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|
||
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Vasculitis%22%20AND%20systematic%5Bsb%5D%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (444)</a></div></div>
|
||
</div>
|
||
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|
||
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<div class="supplemental col three_col last">
|
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<h2 class="offscreen_noflow">Supplemental Content</h2>
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<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Table_of_contents">Table of contents</h1><a sid="113" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
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<div class="portlet_content ln"><ul><li><a href="/gtr/tests?term=C0042384%5bDISCUI%5d&filter=method%3A2%5F8" target="_blank">Deletion/duplication analysis (9)</a></li>
|
||
<li><a href="/gtr/tests?term=C0042384%5bDISCUI%5d&filter=method%3A2%5F7" target="_blank">Sequence analysis of the entire coding region (9)</a></li>
|
||
<li class="portletSeeAll portletSeeAllPad"><total><a href="/gtr/tests?term=C0042384%5bDISCUI%5d" target="_blank">See all (9)</a></total></li>
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<div class="portlet_content ln"><ul class="a_poppers"><li><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=(%22vasculitis%22%5Btiab%3A~0%5D)%20AND%20(%22english%20and%20humans%22%5BFilter%5D)%20AND%20(%20(%22practice%20guideline%22%5BFilter%5D)%20OR%20(practice*%5Btitl%5D%20AND%20(guideline%5Btitl%5D%20OR%20parameter%5Btitl%5D%20OR%20resource%5Btitl%5D%20OR%20bulletin%5Btitl%5D%20OR%20best%5Btitl%5D))%20OR%20(genetic*%5Btitl%5D%20AND%20(evaluation%5Btitl%5D%20OR%20counseling%5Btitl%5D%20OR%20screening%5Btitl%5D%20OR%20test*%5Btitl%5D))%20OR%20(clinical%5Btitl%5D%20AND%20((expert%5Btitl%5D%20AND%20consensus%5Btitl%5D)%20OR%20utility%5Btitl%5D%20OR%20guideline*%5Btitl%5D))%20OR%20(management%5Btitl%5D%20AND%20(clinical%5Btitl%5D%20OR%20diagnos*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20pain%5Btitl%5D%20OR%20surveillance%5Btitl%5D%20OR%20emergency%5Btitl%5D%20OR%20guideline*%5Btitl%5D%20OR%20therap*))%20OR%20(treatment%5Btitl%5D%20AND%20((evaluation%5Btitl%5D%20AND%20diagnosis%5Btitl%5D)%20OR%20(assessment%5Btitl%5D%20AND%20prevention%5Btitl%5D)%20OR%20therap*))%20OR%20(Diagnos*%5Btitl%5D%20AND%20(prenatal%5Btitl%5D%20OR%20treatment%5Btitl%5D%20OR%20follow-up%5Btitl%5D%20OR%20statement%5Btitl%5D%20OR%20criteria%5Btitl%5D%20OR%20newborn%5Btitl%5D%20OR%20differential%5Btitl%5D%20OR%20neonatal%5Btitl%5D%20OR%20neonate%5Btitl%5D))%20OR%20(guideline*%5Btitl%5D%20AND%20(pharmacogenetic*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20evidence-based%5Btitl%5D%20OR%20consensus%5Btitl%5D%20OR%20(technical%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20(molecular%5Btitl%5D%20AND%20testing%5Btitl%5D)))%20OR%20(risk%5Btitl%5D%20AND%20assessment%5Btitl%5D)%20OR%20(recommendation*%5Btitl%5D%20AND%20(statement%5Btitl%5D%20OR%20Evidence-based%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(care%20AND%20((Patient%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20primary%5Btitl%5D%20OR%20psychosocial%5Btitl%5D))%20OR%20(Health%5Btitl%5D%20AND%20supervision%5Btitl%5D)%20OR%20(statement%5Btitl%5D%20AND%20(policy%5Btitl%5D%20OR%20position%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(pharmacogenetics%5Btitl%5D%20AND%20(Dosing%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20genotype*%5Btitl%5D%20OR%20drug*%5Btitl%5D))%20OR%20(Chemotherapy%5Btitl%5D%20AND%20decision*%5Btitl%5D)%20OR%20(screening%5Btitl%5D%20AND%20(newborn%5Btitl%5D%20OR%20neonat*%5Btitl%5D%20OR%20detection%5Btitl%5D%20OR%20diagnos*%5Btitl%5D))%20OR%20(criteria%5Btitl%5D%20OR%20genotype*%5Btitl%5D)%20)%20NOT%20(%22Case%20reports%22%5BPublication%20type%5D%20OR%20%22clinical%20study%22%5BPublication%20Type%5D%20OR%20%22randomized%20controlled%20trial%22%5BPublication%20Type%5D)" title="PubMed search">PubMed</a><div class="help-popup">See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div></li><li><a target="_blank" href="/books/?term=((%22clinical%20guidelines%22%5BResource%20Type%5D)%20OR%20%22practice%20guideline%22%5BPublication%20Type%5D)%20AND%20(%22Vasculitis%22)">Bookshelf</a><div class="help-popup">See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div></li></ul></div>
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