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Antisynthetase syndrome

MedGen UID:
388567
•Concept ID:
C2609059
•
Disease or Syndrome
Synonyms: Anti-Jo1 syndrome; anti-Jo1 syndrome; antisynthetase syndrome; AS syndrome
SNOMED CT: Antisynthetase syndrome (445187004)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0019344
Orphanet: ORPHA81

Definition

A rare idiopathic inflammatory myopathy (IIM) characterized principally by myositis, generally symmetrical arthritis and interstitial lung disease (ILD) in association with serum autoantibodies to aminoacyl-transfer RNA synthetases (anti-ARS). More variable features include arthralgia, Raynaud phenomenon, heliotrophic rash, distal esophageal dysmotility and mechanic's hands. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Antisynthetase syndrome
Follow this link to review classifications for Antisynthetase syndrome in Orphanet.

Professional guidelines

PubMed

Diagnosis and Management of Myositis-Associated Lung Disease.
Hallowell RW, Danoff SK
Chest 2023 Jun;163(6):1476-1491. Epub 2023 Feb 9 doi: 10.1016/j.chest.2023.01.031. PMID: 36764512
Clinical manifestations and treatment of antisynthetase syndrome.
Marco JL, Collins BF
Best Pract Res Clin Rheumatol 2020 Aug;34(4):101503. Epub 2020 Apr 11 doi: 10.1016/j.berh.2020.101503. PMID: 32284267
Classification and management of adult inflammatory myopathies.
Selva-O'Callaghan A, Pinal-Fernandez I, Trallero-Araguás E, Milisenda JC, Grau-Junyent JM, Mammen AL
Lancet Neurol 2018 Sep;17(9):816-828. doi: 10.1016/S1474-4422(18)30254-0. PMID: 30129477Free PMC Article

Recent clinical studies

Etiology

Recent Updates on the Pathogenesis of Inflammatory Myopathies.
Musai J, Mammen AL, Pinal-Fernandez I
Curr Rheumatol Rep 2024 Dec;26(12):421-430. Epub 2024 Sep 24 doi: 10.1007/s11926-024-01164-7. PMID: 39316320Free PMC Article
Paraneoplastic myopathies.
Mammen AL
Handb Clin Neurol 2024;200:327-332. doi: 10.1016/B978-0-12-823912-4.00022-0. PMID: 38494286
Diagnosis and Management of Myositis-Associated Lung Disease.
Hallowell RW, Danoff SK
Chest 2023 Jun;163(6):1476-1491. Epub 2023 Feb 9 doi: 10.1016/j.chest.2023.01.031. PMID: 36764512
Idiopathic inflammatory myopathies.
Lundberg IE, Fujimoto M, Vencovsky J, Aggarwal R, Holmqvist M, Christopher-Stine L, Mammen AL, Miller FW
Nat Rev Dis Primers 2021 Dec 2;7(1):86. doi: 10.1038/s41572-021-00321-x. PMID: 34857798
Inflammatory Myopathies.
Atluri RB
Mo Med 2016 Mar-Apr;113(2):127-30. PMID: 27311223Free PMC Article

Diagnosis

Diagnosis and Management of Myositis-Associated Lung Disease.
Hallowell RW, Danoff SK
Chest 2023 Jun;163(6):1476-1491. Epub 2023 Feb 9 doi: 10.1016/j.chest.2023.01.031. PMID: 36764512
Idiopathic inflammatory myopathies.
Lundberg IE, Fujimoto M, Vencovsky J, Aggarwal R, Holmqvist M, Christopher-Stine L, Mammen AL, Miller FW
Nat Rev Dis Primers 2021 Dec 2;7(1):86. doi: 10.1038/s41572-021-00321-x. PMID: 34857798
Clinical manifestations and treatment of antisynthetase syndrome.
Marco JL, Collins BF
Best Pract Res Clin Rheumatol 2020 Aug;34(4):101503. Epub 2020 Apr 11 doi: 10.1016/j.berh.2020.101503. PMID: 32284267
Classification of idiopathic inflammatory myopathies: pathology perspectives.
Tanboon J, Nishino I
Curr Opin Neurol 2019 Oct;32(5):704-714. doi: 10.1097/WCO.0000000000000740. PMID: 31369423
Inflammatory Myopathies.
Atluri RB
Mo Med 2016 Mar-Apr;113(2):127-30. PMID: 27311223Free PMC Article

Therapy

CAR T cells for treating autoimmune diseases.
Blache U, Tretbar S, Koehl U, Mougiakakos D, Fricke S
RMD Open 2023 Nov 23;9(4) doi: 10.1136/rmdopen-2022-002907. PMID: 37996128Free PMC Article
CD19-Targeting CAR T Cells for Myositis and Interstitial Lung Disease Associated With Antisynthetase Syndrome.
Pecher AC, Hensen L, Klein R, Schairer R, Lutz K, Atar D, Seitz C, Stanger A, Schneider J, Braun C, Schmidt M, Horger M, Bornemann A, Faul C, Bethge W, Henes J, Lengerke C
JAMA 2023 Jun 27;329(24):2154-2162. doi: 10.1001/jama.2023.8753. PMID: 37367976Free PMC Article
From bench to bedside: the history and progress of CAR T cell therapy.
Mitra A, Barua A, Huang L, Ganguly S, Feng Q, He B
Front Immunol 2023;14:1188049. Epub 2023 May 15 doi: 10.3389/fimmu.2023.1188049. PMID: 37256141Free PMC Article
Antisynthetase syndrome - much more than just a myopathy.
Opinc AH, Makowska JS
Semin Arthritis Rheum 2021 Feb;51(1):72-83. Epub 2020 Dec 22 doi: 10.1016/j.semarthrit.2020.09.020. PMID: 33360231
Immune-Mediated Myopathies.
Goyal NA
Continuum (Minneap Minn) 2019 Dec;25(6):1564-1585. doi: 10.1212/CON.0000000000000789. PMID: 31794460

Prognosis

Autoimmune inflammatory myopathies.
Dalakas MC
Handb Clin Neurol 2023;195:425-460. doi: 10.1016/B978-0-323-98818-6.00023-6. PMID: 37562881
Myositis-associated interstitial lung disease.
Vu TTT, Brown KK, Solomon JJ
Curr Opin Pulm Med 2023 Sep 1;29(5):427-435. Epub 2023 Jul 11 doi: 10.1097/MCP.0000000000001000. PMID: 37435671
Myositis Antibodies and Interstitial Lung Disease.
Basuita M, Fidler LM
J Appl Lab Med 2022 Jan 5;7(1):240-258. doi: 10.1093/jalm/jfab108. PMID: 34996093
Idiopathic inflammatory myopathies.
Lundberg IE, Fujimoto M, Vencovsky J, Aggarwal R, Holmqvist M, Christopher-Stine L, Mammen AL, Miller FW
Nat Rev Dis Primers 2021 Dec 2;7(1):86. doi: 10.1038/s41572-021-00321-x. PMID: 34857798
Clinical manifestations and treatment of antisynthetase syndrome.
Marco JL, Collins BF
Best Pract Res Clin Rheumatol 2020 Aug;34(4):101503. Epub 2020 Apr 11 doi: 10.1016/j.berh.2020.101503. PMID: 32284267

Clinical prediction guides

Myositis-associated interstitial lung disease.
Vu TTT, Brown KK, Solomon JJ
Curr Opin Pulm Med 2023 Sep 1;29(5):427-435. Epub 2023 Jul 11 doi: 10.1097/MCP.0000000000001000. PMID: 37435671
CD19-Targeting CAR T Cells for Myositis and Interstitial Lung Disease Associated With Antisynthetase Syndrome.
Pecher AC, Hensen L, Klein R, Schairer R, Lutz K, Atar D, Seitz C, Stanger A, Schneider J, Braun C, Schmidt M, Horger M, Bornemann A, Faul C, Bethge W, Henes J, Lengerke C
JAMA 2023 Jun 27;329(24):2154-2162. doi: 10.1001/jama.2023.8753. PMID: 37367976Free PMC Article
From bench to bedside: the history and progress of CAR T cell therapy.
Mitra A, Barua A, Huang L, Ganguly S, Feng Q, He B
Front Immunol 2023;14:1188049. Epub 2023 May 15 doi: 10.3389/fimmu.2023.1188049. PMID: 37256141Free PMC Article
Antisynthetase syndrome - much more than just a myopathy.
Opinc AH, Makowska JS
Semin Arthritis Rheum 2021 Feb;51(1):72-83. Epub 2020 Dec 22 doi: 10.1016/j.semarthrit.2020.09.020. PMID: 33360231
Development of a New Classification System for Idiopathic Inflammatory Myopathies Based on Clinical Manifestations and Myositis-Specific Autoantibodies.
Mariampillai K, Granger B, Amelin D, Guiguet M, Hachulla E, Maurier F, Meyer A, Tohmé A, Charuel JL, Musset L, Allenbach Y, Benveniste O
JAMA Neurol 2018 Dec 1;75(12):1528-1537. doi: 10.1001/jamaneurol.2018.2598. PMID: 30208379Free PMC Article

Recent systematic reviews

Rituximab Treatment in Adult Patients With Idiopathic Inflammatory Myositis: A Systematic Review and Meta-analysis.
Rojas LO, Ramsubeik K, Sanchez-Ramos L, Motilal S, Singh JA, Kaeley GS
J Clin Rheumatol 2025 Jan 1;31(1):33-39. Epub 2024 Nov 11 doi: 10.1097/RHU.0000000000002151. PMID: 39527803
Management and outcomes of interstitial lung disease associated with anti-synthetase syndrome: a systematic literature review.
Kouranloo K, Dey M, Elwell H, Yioe V, Spencer LG, Cotton CV
Rheumatology (Oxford) 2025 Jan 1;64(1):45-55. doi: 10.1093/rheumatology/keae403. PMID: 39083028
One-year survival benefit of plasma exchange in idiopathic inflammatory myositis patients with progressive interstitial lung disease-a systemic review and meta-analysis.
Tangborwornweerakul B, Phutthinart N, Disayabutr S, Katchamart W
Semin Arthritis Rheum 2024 Dec;69:152564. Epub 2024 Oct 11 doi: 10.1016/j.semarthrit.2024.152564. PMID: 39423699
Association of common variable immunodeficiency and rare and complex connective tissue and musculoskeletal diseases. A systematic literature review.
Mucke J, Cornet A, Witte T, Schneider M
Clin Exp Rheumatol 2022 May;40 Suppl 134(5):40-45. Epub 2022 Mar 28 doi: 10.55563/clinexprheumatol/bbuvih. PMID: 35349404
Treatment of idiopathic inflammatory myositis associated interstitial lung disease: A systematic review and meta-analysis.
Barba T, Fort R, Cottin V, Provencher S, Durieu I, Jardel S, Hot A, Reynaud Q, Lega JC
Autoimmun Rev 2019 Feb;18(2):113-122. Epub 2018 Dec 17 doi: 10.1016/j.autrev.2018.07.013. PMID: 30572131

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