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<meta name="keywords" content="C5561926, cfa, cryptogenic fibrosing alveolitis, disease or syndrome, familial idiopathic pulmonary fibrosis, familial pulmonary fibrosis, fibrocystic pulmonary dysplasia, fibrosing alveolitis, fibrosing alveolitis, cryptogenic, idiopathic pulmonary fibrosis, idiopathic pulmonary fibrosis, familial, ild2, interstitial lung disease 2, interstitial pneumonitis, usual, ipf, muc5b, pulmonary fibrosis, idiopathic, sftpa2, sftpc, tert, uip, usual interstitial pneumonia, 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="Interstitial lung disease (ILD) comprises a heterogeneous group of rare diseases affecting the distal part of the lung and characterized by a progressive remodeling of the alveolar interstitium. The manifestations form a spectrum ranging from idiopathic interstitial pneumonia (IIP) or pneumonitis to the more severe idiopathic pulmonary fibrosis (IPF). IPF is associated with an increased risk of developing lung cancer, which occurs in a subset of patients with ILD. Clinical features of ILD include dyspnea, clubbing of the fingers, and restrictive lung capacity. Imaging typically shows ground glass opacities and inter- and intraseptal thickening, while histologic studies usually show a pattern consistent with 'usual interstitial pneumonia' (UIP) (review by Gross and Hunninghake, 2001; summary by Legendre et al., 2020).&#13; Idiopathic pulmonary fibrosis is one of a family of idiopathic pneumonias sharing clinical features of shortness of breath, radiographically evident diffuse pulmonary infiltrates, and varying degrees in inflammation, fibrosis, or both on lung biopsy. In some cases, the disorder can be rapidly progressive and characterized by sequential acute lung injury with subsequent scarring and end-stage lung disease. Although older studies included several forms of interstitial pneumonia under the term 'idiopathic pulmonary fibrosis,' the clinical label of 'idiopathic pulmonary fibrosis' should be reserved for patients with a specific form of fibrosing interstitial pneumonia referred to as usual interstitial pneumonia (Gross and Hunninghake, 2001). It is estimated that 0.5 to 2.2% of cases of idiopathic pulmonary fibrosis are familial (Marshall et al., 2000). Gross and Hunninghake (2001) reviewed idiopathic pulmonary fibrosis, emphasizing definition, pathogenesis, diagnosis, natural history, and therapy. Antoniou et al. (2004) provided a 'top ten list' of references pertaining to etiopathogenesis, prognosis, diagnosis, therapy, and other aspects of idiopathic pulmonary fibrosis.&#13; For a discussion of genetic heterogeneity of ILD, see ILD1 (619611).&#13; Pulmonary fibrosis can also be a feature in patients with mutations in the TERT (187270) or the TERC (602322) gene; see PFBMFT1 (614742) and PFBMFT2 (614743).&#13; Some patients with surfactant protein C deficiency (610913) who survive to adulthood manifest features of pulmonary fibrosis." /><meta name="robots" content="index,nofollow,noarchive" />
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<!--
UID=1794136
ConceptID=C5561926
-->
<!--imgCountBooks = 0--><h1 class="medgenTitle"><div class="MedGenTitleText">Interstitial lung disease 2<span class="h1sub">(ILD2)</span></div></h1><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1794136</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)&#10;Click for more information.">C5561926</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>Familial idiopathic pulmonary fibrosis; Familial Pulmonary Fibrosis; FIBROCYSTIC PULMONARY DYSPLASIA; FIBROSING ALVEOLITIS, CRYPTOGENIC; ILD2</td></tr>
<tr><td>Modes of inheritance:</td>
<td>
<div class="divPopper rprt" id="moi_109109"><div><strong>Non-Mendelian inheritance</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>109109</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)&#10;Click for more information.">C0600599</a></dd><dt><span class="dotprefix"></span></dt><dd>Genetic Function</dd></dl></div></div></div>
<div class="spaceAbove">Source: Orphanet</div>
<div class="spaceAbove">A mode of inheritance that depends on genetic determinants in more than one gene.</div></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#moi_109109" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Non-Mendelian inheritance</a><span> (Orphanet)</span></div></td></tr>
<tr><td colspan="2" class="small"> </td></tr><tr><td><a class="help jig-ncbi-popper" data-jig="ncbipopper" href="#target-gene-loc">Genes (locations):<img class="pulldown" src="//static.pubmed.gov/portal/portal3rc.fcgi/4223267/img/4204968" /></a><div class="display-none" id="target-gene-loc">
Gene(s) directly associated with<br />
this condition or phenotype.</div></td>
<td><a target="_blank" title="MUC5B - ID: 727897 - NCBI Gene" href="/gene/727897" class="medgenPMinfo">MUC5B</a> (11p15.5); <a target="_blank" title="SFTPA2 - ID: 729238 - NCBI Gene" href="/gene/729238" class="medgenPMinfo">SFTPA2</a> (10q22.3); <a target="_blank" title="SFTPC - ID: 6440 - NCBI Gene" href="/gene/6440" class="medgenPMinfo">SFTPC</a> (8p21.3); <a target="_blank" title="TERT - ID: 7015 - NCBI Gene" href="/gene/7015" class="medgenPMinfo">TERT</a> (5p15.33)</td></tr>
<tr><td colspan="2" class="small"> </td></tr><tr><td>Monarch Initiative:</td>
<td><a href="https://monarchinitiative.org/disease/MONDO:0800029" target="_blank">MONDO:0800029</a></td></tr>
<tr><td>OMIM<span class="superscript">®</span>:</td>
<td><a href="https://omim.org/entry/178500" target="_blank">178500</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&amp;Expert=2032">ORPHA2032</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>
<div class="portlet mgSection" id="ID_100">
<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">Interstitial lung disease (ILD) comprises a heterogeneous group of rare diseases affecting the distal part of the lung and characterized by a progressive remodeling of the alveolar interstitium. The manifestations form a spectrum ranging from idiopathic interstitial pneumonia (IIP) or pneumonitis to the more severe idiopathic pulmonary fibrosis (IPF). IPF is associated with an increased risk of developing lung cancer, which occurs in a subset of patients with ILD. Clinical features of ILD include dyspnea, clubbing of the fingers, and restrictive lung capacity. Imaging typically shows ground glass opacities and inter- and intraseptal thickening, while histologic studies usually show a pattern consistent with 'usual interstitial pneumonia' (UIP) (review by Gross and Hunninghake, 2001; summary by Legendre et al., 2020).&#13; Idiopathic pulmonary fibrosis is one of a family of idiopathic pneumonias sharing clinical features of shortness of breath, radiographically evident diffuse pulmonary infiltrates, and varying degrees in inflammation, fibrosis, or both on lung biopsy. In some cases, the disorder can be rapidly progressive and characterized by sequential acute lung injury with subsequent scarring and end-stage lung disease. Although older studies included several forms of interstitial pneumonia under the term 'idiopathic pulmonary fibrosis,' the clinical label of 'idiopathic pulmonary fibrosis' should be reserved for patients with a specific form of fibrosing interstitial pneumonia referred to as usual interstitial pneumonia (Gross and Hunninghake, 2001). It is estimated that 0.5 to 2.2% of cases of idiopathic pulmonary fibrosis are familial (Marshall et al., 2000). Gross and Hunninghake (2001) reviewed idiopathic pulmonary fibrosis, emphasizing definition, pathogenesis, diagnosis, natural history, and therapy. Antoniou et al. (2004) provided a 'top ten list' of references pertaining to etiopathogenesis, prognosis, diagnosis, therapy, and other aspects of idiopathic pulmonary fibrosis.&#13; For a discussion of genetic heterogeneity of ILD, see ILD1 (619611).&#13; Pulmonary fibrosis can also be a feature in patients with mutations in the TERT (187270) or the TERC (602322) gene; see PFBMFT1 (614742) and PFBMFT2 (614743).&#13; Some patients with surfactant protein C deficiency (610913) who survive to adulthood manifest features of pulmonary fibrosis. [from <a title="Online Mendelian Inheritance in Man" href="http://www.omim.org" class="defSource" target="_blank">OMIM</a>]</div>
</div>
<div class="portlet mgSection" id="ID_102">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Clinical_features">Clinical features</h1><a sid="102" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln clinfeat"><strong>From HPO</strong><br />
<div class="divPopper rprt" id="clin_2871"><div><strong>Minimally invasive lung adenocarcinoma</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>2871</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)&#10;Click for more information.">C0007120</a></dd><dt><span class="dotprefix"></span></dt><dd>Neoplastic Process</dd></dl></div></div></div>
<div class="spaceAbove">Adenocarcinoma of the Bronchus.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/2871">Feature record</a> | <a href="/medgen?term=%22Minimally%20invasive%20lung%20adenocarcinoma%22%5BClinical%20Features%5D%20OR%202871%5Buid%5D">Search on this feature</a></div></div>
<div class="divPopper rprt" id="clin_3129"><div><strong>Clubbing of fingers</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>3129</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)&#10;Click for more information.">C0009080</a></dd><dt><span class="dotprefix"></span></dt><dd>Finding</dd></dl></div></div></div>
<div class="spaceAbove">Terminal broadening of the fingers (distal phalanges of the fingers).</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/3129">Feature record</a> | <a href="/medgen?term=%22Clubbing%20of%20fingers%22%5BClinical%20Features%5D%20OR%203129%5Buid%5D">Search on this feature</a></div></div>
<div class="divPopper rprt" id="clin_425404"><div><strong>Pulmonary arterial hypertension</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>425404</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)&#10;Click for more information.">C2973725</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Pulmonary hypertension is defined mean pulmonary artery pressure of 25mmHg or more and pulmonary capillary wedge pressure of 15mmHg or less when measured by right heart catheterisation at rest and in a supine position.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/425404">Feature record</a> | <a href="/medgen?term=%22Pulmonary%20arterial%20hypertension%22%5BClinical%20Features%5D%20OR%20425404%5Buid%5D">Search on this feature</a></div></div>
<div class="divPopper rprt" id="clin_7368"><div><strong>Cirrhosis of liver</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>7368</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)&#10;Click for more information.">C0023890</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">A chronic disorder of the liver in which liver tissue becomes scarred and is partially replaced by regenerative nodules and fibrotic tissue resulting in loss of liver function.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/7368">Feature record</a> | <a href="/medgen?term=%22Cirrhosis%20of%20liver%22%5BClinical%20Features%5D%20OR%207368%5Buid%5D">Search on this feature</a></div></div>
<div class="divPopper rprt" id="clin_41325"><div><strong>Cough</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>41325</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)&#10;Click for more information.">C0010200</a></dd><dt><span class="dotprefix"></span></dt><dd>Sign or Symptom</dd></dl></div></div></div>
<div class="spaceAbove">A sudden, audible expulsion of air from the lungs through a partially closed glottis, preceded by inhalation.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/41325">Feature record</a> | <a href="/medgen?term=%22Cough%22%5BClinical%20Features%5D%20OR%2041325%5Buid%5D">Search on this feature</a></div></div>
<div class="divPopper rprt" id="clin_3938"><div><strong>Dyspnea</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>3938</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)&#10;Click for more information.">C0013404</a></dd><dt><span class="dotprefix"></span></dt><dd>Sign or Symptom</dd></dl></div></div></div>
<div class="spaceAbove">Difficult or labored breathing. Dyspnea is a subjective feeling only the patient can rate, e.g., on a Borg scale.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/3938">Feature record</a> | <a href="/medgen?term=%22Dyspnea%22%5BClinical%20Features%5D%20OR%203938%5Buid%5D">Search on this feature</a></div></div>
<div class="divPopper rprt" id="clin_11028"><div><strong>Pulmonary fibrosis</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>11028</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)&#10;Click for more information.">C0034069</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Replacement of normal lung tissues by fibroblasts and collagen.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/11028">Feature record</a> | <a href="/medgen?term=%22Pulmonary%20fibrosis%22%5BClinical%20Features%5D%20OR%2011028%5Buid%5D">Search on this feature</a></div></div>
<div class="divPopper rprt" id="clin_68549"><div><strong>Exertional dyspnea</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>68549</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)&#10;Click for more information.">C0231807</a></dd><dt><span class="dotprefix"></span></dt><dd>Sign or Symptom</dd></dl></div></div></div>
<div class="spaceAbove">Perceived difficulty to breathe that occurs with exercise or exertion and improves with rest.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/68549">Feature record</a> | <a href="/medgen?term=%22Exertional%20dyspnea%22%5BClinical%20Features%5D%20OR%2068549%5Buid%5D">Search on this feature</a></div></div>
<div class="divPopper rprt" id="clin_892993"><div><strong>Decreased DLCO</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>892993</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)&#10;Click for more information.">C4073175</a></dd><dt><span class="dotprefix"></span></dt><dd>Finding</dd></dl></div></div></div>
<div class="spaceAbove">Reduced ability of the lungs to transfer gas from inspired air to the bloodstream as measured by the diffusing capacity of the lungs for carbon monoxide (DLCO) test.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/892993">Feature record</a> | <a href="/medgen?term=%22Decreased%20DLCO%22%5BClinical%20Features%5D%20OR%20892993%5Buid%5D">Search on this feature</a></div></div>
<div class="divPopper rprt" id="clin_1662563"><div><strong>Usual interstitial pneumonia</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1662563</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)&#10;Click for more information.">C4721509</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Temporal and spatial heterogeneity in lungs based on presence of fibrosis and honeycombing.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/1662563">Feature record</a> | <a href="/medgen?term=%22Usual%20interstitial%20pneumonia%22%5BClinical%20Features%5D%20OR%201662563%5Buid%5D">Search on this feature</a></div></div>
<div class="divPopper rprt" id="clin_1710230"><div><strong>Elevated bronchoalveolar lavage fluid neutrophil proportion</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1710230</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)&#10;Click for more information.">C5397969</a></dd><dt><span class="dotprefix"></span></dt><dd>Finding</dd></dl></div></div></div>
<div class="spaceAbove">Usually, Neutrophils make up less than 3% of all cells found in the broncho-alveloar lavage fluid. In children, standard value of neutrophils is higher depending on their age (children under the age of 5 show a maximum value of 10%). This elevated cell proportion is a sign for acute and chronic infections (HP:0012387, HP:0006538) and can be associated to specific diseases.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/1710230">Feature record</a> | <a href="/medgen?term=%22Elevated%20bronchoalveolar%20lavage%20fluid%20neutrophil%20proportion%22%5BClinical%20Features%5D%20OR%201710230%5Buid%5D">Search on this feature</a></div></div>
<div class="divPopper rprt" id="clin_1713383"><div><strong>Increased circulating antibody concentration</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>1713383</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)&#10;Click for more information.">C5397581</a></dd><dt><span class="dotprefix"></span></dt><dd>Finding</dd></dl></div></div></div>
<div class="spaceAbove">An increased level of gamma globulin (immunoglobulin) in the blood.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/1713383">Feature record</a> | <a href="/medgen?term=%22Increased%20circulating%20antibody%20concentration%22%5BClinical%20Features%5D%20OR%201713383%5Buid%5D">Search on this feature</a></div></div><div class="TreeLite" data-jigconfig="closed: 1"><div class="concept-def"><a class="small" href="#" onclick="jQuery(&quot;.TreeLite&quot;,&quot;#ID_102&quot;).TreeLite().openAll(); return false;">Show all</a><a class="small" href="#" onclick="jQuery(&quot;.TreeLite&quot;,&quot;#ID_102&quot;).TreeLite().closeAll(); return false;">Hide all</a></div><ul><li><span class="TLline">Abnormality of limbs</span><ul><li class="TLline">
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_3129" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Clubbing of fingers</a></span></li></ul></li><li><span class="TLline">Abnormality of the cardiovascular system</span><ul><li class="TLline">
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_425404" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Pulmonary arterial hypertension</a></span></li></ul></li><li><span class="TLline">Abnormality of the digestive system</span><ul><li class="TLline">
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_7368" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Cirrhosis of liver</a></span></li></ul></li><li><span class="TLline">Abnormality of the immune system</span><ul><li class="TLline">
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_1713383" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Increased circulating antibody concentration</a></span></li></ul></li><li><span class="TLline">Abnormality of the respiratory system</span><ul><li class="TLline">
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_41325" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Cough</a></span></li><li class="TLline">
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_892993" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Decreased DLCO</a></span></li><li class="TLline">
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_3938" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Dyspnea</a></span></li><li class="TLline">
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_1710230" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Elevated bronchoalveolar lavage fluid neutrophil proportion</a></span></li><li class="TLline">
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_68549" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Exertional dyspnea</a></span></li><li class="TLline">
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_11028" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Pulmonary fibrosis</a></span></li><li class="TLline">
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_1662563" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Usual interstitial pneumonia</a></span></li></ul></li><li><span class="TLline">Neoplasm</span><ul><li class="TLline">
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_2871" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Minimally invasive lung adenocarcinoma</a></span></li></ul></li></ul></div></div>
</div>
<div class="portlet mgSection" id="ID_118">
<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>
<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="TLclosed"><span class="chiclet_list"><span class="chiclet Ccolor round" title="Clinical test"><a target="_blank" href="/gtr/tests/?term=C4721507[DISCUI]&amp;test_type=Clinical&amp;redirect=true" ref="ncbi_uid=1658997">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&amp;from_uid=1658997" ref="ncbi_uid=1658997">V</a></span></span><span class="TLline"><a href="/medgen/1658997" ref="tree=GTR&amp;ncbi_uid=1658997&amp;link_uid=1658997" title="View MedGen record for 'Diffuse interstitial pulmonary fibrosis'">Diffuse interstitial pulmonary fibrosis</a></span><ul><li class="matched_ds"><span class="chiclet_list"><span class="chiclet Ccolor round" title="Clinical test"><a target="_blank" href="/gtr/tests/?term=C5561926[DISCUI]&amp;test_type=Clinical" ref="ncbi_uid=1794136">C</a></span><span class="chiclet unavailable round" title="Research Tests">R</span><span class="chiclet Ocolor" title="OMIM"><a ref="ncbi_uid=1794136" target="_blank" href="/omim/178500">O</a></span><span class="chiclet Gcolor" title="GeneReviews"><a target="_blank" href="/books/NBK1230/" ref="ncbi_uid=1794136">G</a></span><span class="chiclet Vcolor" title="ClinVar"><a target="_blank" href="/clinvar?LinkName=medgen_clinvar&amp;from_uid=1794136" ref="ncbi_uid=1794136">V</a></span></span><span class="TLline">Interstitial lung disease 2</span><ul><li class="TLclosed"><span class="chiclet_list"><span class="chiclet unavailable round" title="Clinical test">C</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 unavailable" title="ClinVar">V</span></span><span class="TLline"><a href="/medgen/39340" ref="tree=GTR&amp;ncbi_uid=39340&amp;link_uid=39340" title="View MedGen record for 'Acute interstitial pneumonia'">Acute interstitial pneumonia</a></span></li></ul></li><li class="TLclosed"><span class="chiclet_list"><span class="chiclet Ccolor round" title="Clinical test"><a target="_blank" href="/gtr/tests/?term=C3553617[DISCUI]&amp;test_type=Clinical" ref="ncbi_uid=766531">C</a></span><span class="chiclet unavailable round" title="Research Tests">R</span><span class="chiclet Ocolor" title="OMIM"><a ref="ncbi_uid=766531" target="_blank" href="/omim/187270">O</a></span><span class="chiclet Gcolor" title="GeneReviews"><a target="_blank" href="/books/?term=(NBK1230%20OR%20NBK22301)%20AND%20gene%5Bbook%5D&amp;showtype=onebook" ref="ncbi_uid=766531">G</a></span><span class="chiclet Vcolor" title="ClinVar"><a target="_blank" href="/clinvar?LinkName=medgen_clinvar&amp;from_uid=766531" ref="ncbi_uid=766531">V</a></span></span><span class="TLline"><a href="/medgen/766531" ref="tree=GTR&amp;ncbi_uid=766531&amp;link_uid=766531" title="View MedGen record for 'Pulmonary fibrosis and/or bone marrow failure, Telomere-related, 1'">Pulmonary fibrosis and/or bone marrow failure, Telomere-related, 1</a></span></li><li class="TLclosed"><span class="chiclet_list"><span class="chiclet Ccolor round" title="Clinical test"><a target="_blank" href="/gtr/tests/?term=C4225346[DISCUI]&amp;test_type=Clinical" ref="ncbi_uid=901644">C</a></span><span class="chiclet unavailable round" title="Research Tests">R</span><span class="chiclet Ocolor" title="OMIM"><a ref="ncbi_uid=901644" target="_blank" href="/omim/608833">O</a></span><span class="chiclet Gcolor" title="GeneReviews"><a target="_blank" href="/books/?term=(NBK1230%20OR%20NBK22301)%20AND%20gene%5Bbook%5D&amp;showtype=onebook" ref="ncbi_uid=901644">G</a></span><span class="chiclet Vcolor" title="ClinVar"><a target="_blank" href="/clinvar?LinkName=medgen_clinvar&amp;from_uid=901644" ref="ncbi_uid=901644">V</a></span></span><span class="TLline"><a href="/medgen/901644" ref="tree=GTR&amp;ncbi_uid=901644&amp;link_uid=901644" title="View MedGen record for 'Pulmonary fibrosis and/or bone marrow failure, Telomere-related, 3'">Pulmonary fibrosis and/or bone marrow failure, Telomere-related, 3</a></span></li><li class="TLclosed"><span class="chiclet_list"><span class="chiclet Ccolor round" title="Clinical test"><a target="_blank" href="/gtr/tests/?term=C4225347[DISCUI]&amp;test_type=Clinical" ref="ncbi_uid=903928">C</a></span><span class="chiclet unavailable round" title="Research Tests">R</span><span class="chiclet Ocolor" title="OMIM"><a ref="ncbi_uid=903928" target="_blank" href="/omim/604212">O</a></span><span class="chiclet Gcolor" title="GeneReviews"><a target="_blank" href="/books/?term=(NBK1230%20OR%20NBK22301)%20AND%20gene%5Bbook%5D&amp;showtype=onebook" ref="ncbi_uid=903928">G</a></span><span class="chiclet Vcolor" title="ClinVar"><a target="_blank" href="/clinvar?LinkName=medgen_clinvar&amp;from_uid=903928" ref="ncbi_uid=903928">V</a></span></span><span class="TLline"><a href="/medgen/903928" ref="tree=GTR&amp;ncbi_uid=903928&amp;link_uid=903928" title="View MedGen record for 'Pulmonary fibrosis and/or bone marrow failure, Telomere-related, 4'">Pulmonary fibrosis and/or bone marrow failure, Telomere-related, 4</a></span></li></ul></li></ul></div></div></div></div></div><div id="tabMGEN"><div class="ds_tree"><ul><li><span class="TLline"><a href="/medgen/1658997" ref="tree=MeSH" title="MedGen record for Diffuse interstitial pulmonary fibrosis">Diffuse interstitial pulmonary fibrosis</a></span><ul><li><span class="matched_ds">Interstitial lung disease 2</span><ul><li><span class="TLline"><a href="/medgen/39340" ref="tree=MeSH" title="MedGen record for Acute interstitial pneumonia">Acute interstitial pneumonia</a></span></li></ul></li></ul></li></ul></div></div></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/35430509">Multidisciplinary clinical guidance on trastuzumab deruxtecan (T-DXd)-related interstitial lung disease/pneumonitis-Focus on proactive monitoring, diagnosis, and management.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Swain SM,
Nishino M,
Lancaster LH,
Li BT,
Nicholson AG,
Bartholmai BJ,
Naidoo J,
Schumacher-Wulf E,
Shitara K,
Tsurutani J,
Conte P,
Kato T,
Andre F,
Powell CA</span><br />
<span class="medgenPMjournal">Cancer Treat Rev</span>
2022 May;106:102378.
Epub 2022 Mar 12
doi: 10.1016/j.ctrv.2022.102378.
<span class="bold">PMID: </span><a href="/pubmed/35430509" target="_blank">35430509</a></div>
<div class="nl"><a target="_blank" href="/pubmed/33812494">Association between pre-existing respiratory disease and its treatment, and severe COVID-19: a population cohort study.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Aveyard P,
Gao M,
Lindson N,
Hartmann-Boyce J,
Watkinson P,
Young D,
Coupland CAC,
Tan PS,
Clift AK,
Harrison D,
Gould DW,
Pavord ID,
Hippisley-Cox J</span><br />
<span class="medgenPMjournal">Lancet Respir Med</span>
2021 Aug;9(8):909-923.
Epub 2021 Apr 1
doi: 10.1016/S2213-2600(21)00095-3.
<span class="bold">PMID: </span><a href="/pubmed/33812494" target="_blank">33812494</a><a href="/pmc/articles/PMC8016404" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/21709948">Interstitial lung disease and gastroesophageal reflux disease: key role of esophageal function tests in the diagnosis and treatment.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Soares RV,
Forsythe A,
Hogarth K,
Sweiss NJ,
Noth I,
Patti MG</span><br />
<span class="medgenPMjournal">Arq Gastroenterol</span>
2011 Apr-Jun;48(2):91-7.
doi: 10.1590/s0004-28032011000200002.
<span class="bold">PMID: </span><a href="/pubmed/21709948" target="_blank">21709948</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=(%22interstitial%20lung%20disease%202%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 (4)</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/38648021">Interstitial Lung Disease: A Review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Maher TM</span><br />
<span class="medgenPMjournal">JAMA</span>
2024 May 21;331(19):1655-1665.
doi: 10.1001/jama.2024.3669.
<span class="bold">PMID: </span><a href="/pubmed/38648021" target="_blank">38648021</a></div>
<div class="nl"><a target="_blank" href="/pubmed/35219244">Drug-induced interstitial lung disease during cancer therapies: expert opinion on diagnosis and treatment.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Conte P,
Ascierto PA,
Patelli G,
Danesi R,
Vanzulli A,
Sandomenico F,
Tarsia P,
Cattelan A,
Comes A,
De Laurentiis M,
Falcone A,
Regge D,
Richeldi L,
Siena S</span><br />
<span class="medgenPMjournal">ESMO Open</span>
2022 Apr;7(2):100404.
Epub 2022 Feb 24
doi: 10.1016/j.esmoop.2022.100404.
<span class="bold">PMID: </span><a href="/pubmed/35219244" target="_blank">35219244</a><a href="/pmc/articles/PMC8881716" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/34876670">Acute exacerbation of interstitial lung disease associated with rheumatic disease.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Luppi F,
Sebastiani M,
Salvarani C,
Bendstrup E,
Manfredi A</span><br />
<span class="medgenPMjournal">Nat Rev Rheumatol</span>
2022 Feb;18(2):85-96.
Epub 2021 Dec 7
doi: 10.1038/s41584-021-00721-z.
<span class="bold">PMID: </span><a href="/pubmed/34876670" target="_blank">34876670</a></div>
<div class="nl"><a target="_blank" href="/pubmed/33741803">Treatment for systemic sclerosis-associated interstitial lung disease.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Roofeh D,
Lescoat A,
Khanna D</span><br />
<span class="medgenPMjournal">Curr Opin Rheumatol</span>
2021 May 1;33(3):240-248.
doi: 10.1097/BOR.0000000000000795.
<span class="bold">PMID: </span><a href="/pubmed/33741803" target="_blank">33741803</a><a href="/pmc/articles/PMC8021460" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/24591661">Interstitial lung disease.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Antoniou KM,
Margaritopoulos GA,
Tomassetti S,
Bonella F,
Costabel U,
Poletti V</span><br />
<span class="medgenPMjournal">Eur Respir Rev</span>
2014 Mar 1;23(131):40-54.
doi: 10.1183/09059180.00009113.
<span class="bold">PMID: </span><a href="/pubmed/24591661" target="_blank">24591661</a><a href="/pmc/articles/PMC9487254" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Interstitial%20lung%20disease%202%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 (11467)</a></div><h3 class="subhead">Diagnosis</h3>
<div class="nl"><a target="_blank" href="/pubmed/38648021">Interstitial Lung Disease: A Review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Maher TM</span><br />
<span class="medgenPMjournal">JAMA</span>
2024 May 21;331(19):1655-1665.
doi: 10.1001/jama.2024.3669.
<span class="bold">PMID: </span><a href="/pubmed/38648021" target="_blank">38648021</a></div>
<div class="nl"><a target="_blank" href="/pubmed/35430509">Multidisciplinary clinical guidance on trastuzumab deruxtecan (T-DXd)-related interstitial lung disease/pneumonitis-Focus on proactive monitoring, diagnosis, and management.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Swain SM,
Nishino M,
Lancaster LH,
Li BT,
Nicholson AG,
Bartholmai BJ,
Naidoo J,
Schumacher-Wulf E,
Shitara K,
Tsurutani J,
Conte P,
Kato T,
Andre F,
Powell CA</span><br />
<span class="medgenPMjournal">Cancer Treat Rev</span>
2022 May;106:102378.
Epub 2022 Mar 12
doi: 10.1016/j.ctrv.2022.102378.
<span class="bold">PMID: </span><a href="/pubmed/35430509" target="_blank">35430509</a></div>
<div class="nl"><a target="_blank" href="/pubmed/35219244">Drug-induced interstitial lung disease during cancer therapies: expert opinion on diagnosis and treatment.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Conte P,
Ascierto PA,
Patelli G,
Danesi R,
Vanzulli A,
Sandomenico F,
Tarsia P,
Cattelan A,
Comes A,
De Laurentiis M,
Falcone A,
Regge D,
Richeldi L,
Siena S</span><br />
<span class="medgenPMjournal">ESMO Open</span>
2022 Apr;7(2):100404.
Epub 2022 Feb 24
doi: 10.1016/j.esmoop.2022.100404.
<span class="bold">PMID: </span><a href="/pubmed/35219244" target="_blank">35219244</a><a href="/pmc/articles/PMC8881716" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/30695649">Interstitial Pneumonia with Autoimmune Features.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Graney BA,
Fischer A</span><br />
<span class="medgenPMjournal">Ann Am Thorac Soc</span>
2019 May;16(5):525-533.
doi: 10.1513/AnnalsATS.201808-565CME.
<span class="bold">PMID: </span><a href="/pubmed/30695649" target="_blank">30695649</a><a href="/pmc/articles/PMC6850782" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/24591661">Interstitial lung disease.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Antoniou KM,
Margaritopoulos GA,
Tomassetti S,
Bonella F,
Costabel U,
Poletti V</span><br />
<span class="medgenPMjournal">Eur Respir Rev</span>
2014 Mar 1;23(131):40-54.
doi: 10.1183/09059180.00009113.
<span class="bold">PMID: </span><a href="/pubmed/24591661" target="_blank">24591661</a><a href="/pmc/articles/PMC9487254" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Interstitial%20lung%20disease%202%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 (10489)</a></div><h3 class="subhead">Therapy</h3>
<div class="nl"><a target="_blank" href="/pubmed/38648021">Interstitial Lung Disease: A Review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Maher TM</span><br />
<span class="medgenPMjournal">JAMA</span>
2024 May 21;331(19):1655-1665.
doi: 10.1001/jama.2024.3669.
<span class="bold">PMID: </span><a href="/pubmed/38648021" target="_blank">38648021</a></div>
<div class="nl"><a target="_blank" href="/pubmed/37055089">Smoking-Related Interstitial Lung Diseases.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Alarcon-Calderon A,
Vassallo R,
Yi ES,
Ryu JH</span><br />
<span class="medgenPMjournal">Immunol Allergy Clin North Am</span>
2023 May;43(2):273-287.
Epub 2023 Mar 1
doi: 10.1016/j.iac.2023.01.007.
<span class="bold">PMID: </span><a href="/pubmed/37055089" target="_blank">37055089</a></div>
<div class="nl"><a target="_blank" href="/pubmed/35430509">Multidisciplinary clinical guidance on trastuzumab deruxtecan (T-DXd)-related interstitial lung disease/pneumonitis-Focus on proactive monitoring, diagnosis, and management.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Swain SM,
Nishino M,
Lancaster LH,
Li BT,
Nicholson AG,
Bartholmai BJ,
Naidoo J,
Schumacher-Wulf E,
Shitara K,
Tsurutani J,
Conte P,
Kato T,
Andre F,
Powell CA</span><br />
<span class="medgenPMjournal">Cancer Treat Rev</span>
2022 May;106:102378.
Epub 2022 Mar 12
doi: 10.1016/j.ctrv.2022.102378.
<span class="bold">PMID: </span><a href="/pubmed/35430509" target="_blank">35430509</a></div>
<div class="nl"><a target="_blank" href="/pubmed/33741803">Treatment for systemic sclerosis-associated interstitial lung disease.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Roofeh D,
Lescoat A,
Khanna D</span><br />
<span class="medgenPMjournal">Curr Opin Rheumatol</span>
2021 May 1;33(3):240-248.
doi: 10.1097/BOR.0000000000000795.
<span class="bold">PMID: </span><a href="/pubmed/33741803" target="_blank">33741803</a><a href="/pmc/articles/PMC8021460" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/30695649">Interstitial Pneumonia with Autoimmune Features.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Graney BA,
Fischer A</span><br />
<span class="medgenPMjournal">Ann Am Thorac Soc</span>
2019 May;16(5):525-533.
doi: 10.1513/AnnalsATS.201808-565CME.
<span class="bold">PMID: </span><a href="/pubmed/30695649" target="_blank">30695649</a><a href="/pmc/articles/PMC6850782" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Interstitial%20lung%20disease%202%22%20AND%20Therapy%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 (8396)</a></div><h3 class="subhead">Prognosis</h3>
<div class="nl"><a target="_blank" href="/pubmed/35513341">Contemporary Concise Review 2021: Interstitial lung disease.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">McCarthy C,
Keane MP</span><br />
<span class="medgenPMjournal">Respirology</span>
2022 Jul;27(7):539-548.
Epub 2022 May 5
doi: 10.1111/resp.14278.
<span class="bold">PMID: </span><a href="/pubmed/35513341" target="_blank">35513341</a><a href="/pmc/articles/PMC9320947" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/35219244">Drug-induced interstitial lung disease during cancer therapies: expert opinion on diagnosis and treatment.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Conte P,
Ascierto PA,
Patelli G,
Danesi R,
Vanzulli A,
Sandomenico F,
Tarsia P,
Cattelan A,
Comes A,
De Laurentiis M,
Falcone A,
Regge D,
Richeldi L,
Siena S</span><br />
<span class="medgenPMjournal">ESMO Open</span>
2022 Apr;7(2):100404.
Epub 2022 Feb 24
doi: 10.1016/j.esmoop.2022.100404.
<span class="bold">PMID: </span><a href="/pubmed/35219244" target="_blank">35219244</a><a href="/pmc/articles/PMC8881716" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/32437842">Adult interstitial lung diseases and their epidemiology.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Sesé L,
Khamis W,
Jeny F,
Uzunhan Y,
Duchemann B,
Valeyre D,
Annesi-Maesano I,
Nunes H</span><br />
<span class="medgenPMjournal">Presse Med</span>
2020 Jun;49(2):104023.
Epub 2020 May 11
doi: 10.1016/j.lpm.2020.104023.
<span class="bold">PMID: </span><a href="/pubmed/32437842" target="_blank">32437842</a></div>
<div class="nl"><a target="_blank" href="/pubmed/30741505">Interstitial Lung Diseases in Developing Countries.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Rivera-Ortega P,
Molina-Molina M</span><br />
<span class="medgenPMjournal">Ann Glob Health</span>
2019 Jan 22;85(1)
doi: 10.5334/aogh.2414.
<span class="bold">PMID: </span><a href="/pubmed/30741505" target="_blank">30741505</a><a href="/pmc/articles/PMC7052338" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/24591661">Interstitial lung disease.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Antoniou KM,
Margaritopoulos GA,
Tomassetti S,
Bonella F,
Costabel U,
Poletti V</span><br />
<span class="medgenPMjournal">Eur Respir Rev</span>
2014 Mar 1;23(131):40-54.
doi: 10.1183/09059180.00009113.
<span class="bold">PMID: </span><a href="/pubmed/24591661" target="_blank">24591661</a><a href="/pmc/articles/PMC9487254" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Interstitial%20lung%20disease%202%22%20AND%20Prognosis%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 (8107)</a></div><h3 class="subhead">Clinical prediction guides</h3>
<div class="nl"><a target="_blank" href="/pubmed/38648021">Interstitial Lung Disease: A Review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Maher TM</span><br />
<span class="medgenPMjournal">JAMA</span>
2024 May 21;331(19):1655-1665.
doi: 10.1001/jama.2024.3669.
<span class="bold">PMID: </span><a href="/pubmed/38648021" target="_blank">38648021</a></div>
<div class="nl"><a target="_blank" href="/pubmed/37534937">Prevalence, Risk Factors, and Outcomes of Adult Interstitial Lung Abnormalities: A Systematic Review and Meta-Analysis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Grant-Orser A,
Min B,
Elmrayed S,
Podolanczuk AJ,
Johannson KA</span><br />
<span class="medgenPMjournal">Am J Respir Crit Care Med</span>
2023 Sep 15;208(6):695-708.
doi: 10.1164/rccm.202302-0271OC.
<span class="bold">PMID: </span><a href="/pubmed/37534937" target="_blank">37534937</a><a href="/pmc/articles/PMC10515575" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/32943410">Progressive fibrosing interstitial lung disease: a clinical cohort (the PROGRESS study).</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Nasser M,
Larrieu S,
Si-Mohamed S,
Ahmad K,
Boussel L,
Brevet M,
Chalabreysse L,
Fabre C,
Marque S,
Revel D,
Thivolet-Bejui F,
Traclet J,
Zeghmar S,
Maucort-Boulch D,
Cottin V</span><br />
<span class="medgenPMjournal">Eur Respir J</span>
2021 Feb;57(2)
Epub 2021 Feb 11
doi: 10.1183/13993003.02718-2020.
<span class="bold">PMID: </span><a href="/pubmed/32943410" target="_blank">32943410</a><a href="/pmc/articles/PMC8411897" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/24591661">Interstitial lung disease.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Antoniou KM,
Margaritopoulos GA,
Tomassetti S,
Bonella F,
Costabel U,
Poletti V</span><br />
<span class="medgenPMjournal">Eur Respir Rev</span>
2014 Mar 1;23(131):40-54.
doi: 10.1183/09059180.00009113.
<span class="bold">PMID: </span><a href="/pubmed/24591661" target="_blank">24591661</a><a href="/pmc/articles/PMC9487254" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/18369202">Interstitial lung disease in systemic sclerosis: a simple staging system.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Goh NS,
Desai SR,
Veeraraghavan S,
Hansell DM,
Copley SJ,
Maher TM,
Corte TJ,
Sander CR,
Ratoff J,
Devaraj A,
Bozovic G,
Denton CP,
Black CM,
du Bois RM,
Wells AU</span><br />
<span class="medgenPMjournal">Am J Respir Crit Care Med</span>
2008 Jun 1;177(11):1248-54.
Epub 2008 Mar 27
doi: 10.1164/rccm.200706-877OC.
<span class="bold">PMID: </span><a href="/pubmed/18369202" target="_blank">18369202</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Interstitial%20lung%20disease%202%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 (7997)</a></div></div>
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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="nl"><a target="_blank" href="/pubmed/38355151">Lung cancer risk and occupational pulmonary fibrosis: systematic review and meta-analysis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Krabbe J,
Steffens KM,
Drießen S,
Kraus T</span><br />
<span class="medgenPMjournal">Eur Respir Rev</span>
2024 Jan 31;33(171)
Epub 2024 Feb 14
doi: 10.1183/16000617.0224-2023.
<span class="bold">PMID: </span><a href="/pubmed/38355151" target="_blank">38355151</a><a href="/pmc/articles/PMC10865097" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/36927642">EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Hellmich B,
Sanchez-Alamo B,
Schirmer JH,
Berti A,
Blockmans D,
Cid MC,
Holle JU,
Hollinger N,
Karadag O,
Kronbichler A,
Little MA,
Luqmani RA,
Mahr A,
Merkel PA,
Mohammad AJ,
Monti S,
Mukhtyar CB,
Musial J,
Price-Kuehne F,
Segelmark M,
Teng YKO,
Terrier B,
Tomasson G,
Vaglio A,
Vassilopoulos D,
Verhoeven P,
Jayne D</span><br />
<span class="medgenPMjournal">Ann Rheum Dis</span>
2024 Jan 2;83(1):30-47.
doi: 10.1136/ard-2022-223764.
<span class="bold">PMID: </span><a href="/pubmed/36927642" target="_blank">36927642</a></div>
<div class="nl"><a target="_blank" href="/pubmed/37775347">Efficacy and Safety of Trastuzumab Deruxtecan in Breast Cancer: A Systematic Review and Meta-Analysis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Dowling GP,
Daly GR,
Keelan S,
Boland F,
Toomey S,
Hill ADK,
Hennessy BT</span><br />
<span class="medgenPMjournal">Clin Breast Cancer</span>
2023 Dec;23(8):847-855.e2.
Epub 2023 Sep 11
doi: 10.1016/j.clbc.2023.09.005.
<span class="bold">PMID: </span><a href="/pubmed/37775347" target="_blank">37775347</a></div>
<div class="nl"><a target="_blank" href="/pubmed/34559419">Pulmonary rehabilitation for interstitial lung disease.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Dowman L,
Hill CJ,
May A,
Holland AE</span><br />
<span class="medgenPMjournal">Cochrane Database Syst Rev</span>
2021 Feb 1;2(2):CD006322.
doi: 10.1002/14651858.CD006322.pub4.
<span class="bold">PMID: </span><a href="/pubmed/34559419" target="_blank">34559419</a><a href="/pmc/articles/PMC8094410" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/32442559">Vaping-Related Acute Parenchymal Lung Injury: A Systematic Review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Jonas AM,
Raj R</span><br />
<span class="medgenPMjournal">Chest</span>
2020 Oct;158(4):1555-1565.
Epub 2020 May 19
doi: 10.1016/j.chest.2020.03.085.
<span class="bold">PMID: </span><a href="/pubmed/32442559" target="_blank">32442559</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Interstitial%20lung%20disease%202%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 (386)</a></div></div>
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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>
<div class="portlet_content ln"><ul id="my-toc"></ul></div>
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<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Genetic_Testing_Registry">Genetic Testing Registry</h1><a sid="106" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln"><ul><li><a href="/gtr/tests?term=C5561926%5bDISCUI%5d&amp;filter=method%3A2%5F8" target="_blank">Deletion/duplication analysis (48)</a></li>
<li><a href="/gtr/tests?term=C5561926%5bDISCUI%5d&amp;filter=method%3A2%5F7" target="_blank">Sequence analysis of the entire coding region (49)</a></li>
<li><a href="/gtr/tests?term=C5561926%5bDISCUI%5d&amp;filter=method%3A2%5F19" target="_blank">Targeted variant analysis (4)</a></li>
<li class="portletSeeAll portletSeeAllPad"><total><a href="/gtr/tests?term=C5561926%5bDISCUI%5d" target="_blank">See all (57)</a></total></li>
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<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Clinical_resources">Clinical resources</h1><a sid="119" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln"><ul><li><a href="https://www.omim.org/search?index=entry&amp;start=1&amp;limit=10&amp;sort=score%20desc&amp;field=number&amp;search=178500" target="_blank">OMIM</a></li><li><a href="http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Expert=2032" target="_blank">Orphanet</a></li><li><a href="https://clinicaltrials.gov/search?cond=Interstitial%20lung%20disease%202" target="_blank">ClinicalTrials.gov</a></li></ul></div>
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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=(%22interstitial%20lung%20disease%202%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(%22Interstitial%20lung%20disease%202%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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<div class="portlet_content ln"><ul><li><a href="http://www.omim.org/search?index=entry&amp;start=1&amp;limit=10&amp;sort=score%20desc&amp;field=number&amp;search=178620%20178642%20187270%20600770" target="_blank">OMIM</a></li><li><a href="/clinvar/?term=6440[geneid]" target="_blank">View SFTPC variations in ClinVar</a></li><li><a href="/clinvar/?term=7015[geneid]" target="_blank">View TERT variations in ClinVar</a></li><li><a href="/clinvar/?term=727897[geneid]" target="_blank">View MUC5B variations in ClinVar</a></li><li><a href="/clinvar/?term=729238[geneid]" target="_blank">View SFTPA2 variations in ClinVar</a></li><li><a href="/nuccore/219801765,260656017,293597502" target="_blank">RefSeqGene</a></li><li><a href="https://catalog.coriell.org/Search?q=178500" target="_blank">Coriell Institute for Medical Research</a></li></ul></div>
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