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Volume 33, Issue 4, December 2022



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Mediterr J Rheumatol 2022;33(4):437-43
Anti-Jo1 Syndrome: Understanding a Rare Cause of Interstitial Lung Disease
Authors Information
  1. Department of Respiratory Medicine, AICTS, Pune, India
  2. Department of Rheumatology, Command Hospital, Lucknow, India
  3. Department of Radiology, AICTS, Pune, India

 A Hegde, V Marwah, Shrinath V, R Choudhary, V Malik

Abstract

Background: Anti-Jo1 syndrome is one of the most common amongst the various anti synthetase syndromes (ASS), which forms a subgroup of the idiopathic inflammatory myositis (IIM). It is characterised by myositis, interstitial lung disease (ILD), fever, Raynaud’s phenomenon, and mechanic’s hands; associated with the presence of anti-Jo1 antibodies in serum. Being an orphan disease, the clinical diagnosis is often delayed. Materials and methods: In this retrospective study, all patients diagnosed as Anti-Jo1 syndrome, from two tertiary care hospitals in Western Maharashtra, between 01 January 2019 – 31 December 2020, were enrolled. The parameters studied included demographic data, clinical features at presentation, laboratory parameters, spirometry, and radiographic findings, along with treatment instituted. Result: A total of 17 patients (8 males, 9 females) qualified for inclusion in the study. The mean age of diagnosis was 40 (±13) years with mean time to diagnosis being 2 years (± 0.6 years), from first clinical presentation. The most common presenting symptoms encountered were arthritis (n = 12, 70.5%), fever (n = 16, 70.5%), myositis (n=11, 64.7%) and breathlessness (n=10, 58.8%).10 patients had ILD at presentation on high resolution computerised tomography of chest (n=10, 58.8%) with restrictive lung defect on spirometry. Six patients required induction of immunosuppression using pulse methylprednisolone (n=6) and Rituximab (n=6), while 11 were managed with oral steroids. Mycophenolate mofetil (n=10) and Azathioprine (n=7) were used as maintenance immunosuppression. Conclusion: Anti-Jo1 syndrome is a myositis syndrome, presenting with a multitude of clinical features. Steroids and disease modifying anti rheumatic drugs form mainstay of therapy.

Cite this article as: Hegde A, Marwah V, Shrinath V, Choudhary R, Malik V. Anti-Jo1 Syndrome: Understanding a Rare Cause of Interstitial Lung Disease. Mediterr J Rheumatol 2022;33(4):437-43.

Article Submitted: 3 Dec 2021; Revised Form: 14 Jun 2022; Article Accepted: 30 Jun 2022; Available Online: 31 Dec 2022

https://doi.org/10.31138/mjr.33.4.437

This work is licensed under a Creative Commons Attribution 4.0 International License.

©2022 The Author(s).