Abstract
Background: To describe the long term clinical outcome and prognostic factors associated with outcome in a longitudinal cohort of idiopathic inflammatory myositis (IIM). Methods: In this retrospective cohort study, IIM patients were classified as per Bohan and Peter criteria. In those with ≥ 24 months of follow-up; treatment response, functional outcomes [health assessment questionnaire-disability index(HAQ-DI) and modified Rankin score(MRS)], Myositis damage index(MDI) at last follow-up was recorded. Results: The cohort consists of 175 patients, with a mean age of 40.9(+12.6) years, M:F 1:3.3; the IIM subsets were dermatomyositis(DM) 78(44.6%), overlap myositis(OM) 45(25.7%), antisynthetase syndrome(ASS) 11(6.3%), polymyositis(PM) 25(14.3%) and juvenile DM/OM in 15(8.6%) patients. Mortality rate was 13.4% and disease related deaths was 9.1%. Ninety-four patients have followed up for >24 months, the median (IQR) of 65(35,100.7) months. At last follow-up, 13.8% were in treatment free remission, 73.4%, 11.7% had complete clinical response and partial clinical response with treatment respectively. HAQ-DI and MRS were favourable in > 90% of patients. At last follow-up, one-third were off-steroids. Discontinuation of steroids was associated with HAQ-DI of 0 and lower MRS. Complete clinical response on/off medication at last follow-up was associated with HAQ-DI of 0[OR10.9; 95%CI(3.3,160)], better MRS[OR 3.2; 95%CI(1.4,7.3)] and lesser MDI[OR 1.7; 95% CI(1.1,2.7)] at the last follow-up as compared to partial response. Baseline parameters and IIM subsets did not significantly influence outcome.Conclusion: Our longitudinal cohort of IIM had a good outcome in all major myositis subsets. Partial clinical response on treatment is associated with worse functional outcomes and damage accrual.