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Volume 34, Issue 4, December 2023



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Mediterr J Rheumatol 2023;34(4):513-24
Long Term Outcomes in Idiopathic Inflammatory Myositis: An Observational Epidemiologic Study over 15 Years
Authors Information

1Department of Clinical Immunology and Rheumatology, St. John's Medical College Hospital, Bengaluru, Karnataka, India

2Department of Biostatistics, St. John's Medical College Hospital, Bengaluru, Karnataka, India

3Department of Neurology, St. John's Medical College Hospital, Bengaluru, Karnataka, India

4Department of Pathology, NIMHANS, Bengaluru, Karnataka, India

Abstract

Background: We report a longitudinal observational cohort of idiopathic inflammatory myositis (IIM) focusing on the long-term clinical outcome and associated parameters.

Methods: IIM patients were classified as per Bohan and Peter criteria. In those with ≥ 24 months of follow-up; the treatment response, functional outcomes, and damage at last follow-up were recorded. Complete clinical response and clinical remission as defined by Oddis et al., was used to define outcomes at last follow-up. Results: The cohort consists of 175 patients, mean age 40.9 (+12.6) years, M:F 1:3.3; and the major subsets were dermatomyositis (44.6%), overlap myositis (25.7%), antisynthetase syndrome (6.3%), polymyositis (14.3%), and juvenile DM/OM (8.6%). Ninety-four patients have followed up for 24 months or more, with the median (IQR) of 65(35,100.7) months. Of them, 74.1% and 11.8% had complete and partial clinical responses respectively at the last follow-up.  In our cohort 40.2% were off-steroids and 13.8% were in clinical remission at the last follow-up. Complete clinical response was associated with better functional outcomes and lesser damage as determined by HAQ-DI of 0[OR10.9; 95%CI (3.3,160)], MRS [OR 3.2; 95%CI (1.4,7.3)] and lesser MDI [OR 1.7; 95% CI (1.1,2.7)] respectively as compared to partial response (unadjusted analysis). Baseline parameters and IIM subsets did not significantly influence the functional outcome and damage. The mortality rate in our cohort is 24/175 (13.7%), the disease-specific mortality rate being 9.1%. Large majority of deaths were early, associated with active disease. Conclusion: We report good long-term outcomes in all major myositis subsets. Partial clinical response to treatment is associated with worse functional outcomes and damage accrual. Death occurs early in association with active disease.


Cite this article as: Janardana R, Sangeetha KN, Bhat V, Balakrishnan D, Raj JM, Pinto B, Chanakya K, Nadig R, Mahadevan A, Shobha V. Long Term Outcomes in Idiopathic Inflammatory Myositis: An Observational Epidemiologic Study over 15 Years. Mediterr J Rheumatol 2023;34(4):513-24

Article Submitted: 08 Mar 2023; Revised Form: 23 May 2023; Article Accepted: 25 May 2023; Available Online: 28 Aug 2023

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

©2023 The Author(s).

https://doi.org/10.31138/mjr.280823.lto