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Background MSA test is useful to diagnose IIM and subcategorize patients by disease phenotypes. Objectives The study aims to evaluate the survival of IIM patients of different MSA patterns. Methods An IIM registry had been set up in a tertiary referral centre since 2014 by recruiting prevalent and incident cases. Patients were followed-up prospectively. This study included patients fulfilling the 2017 EULAR/ACR classification criteria for IIM1 and excluded those aged Results Among 112 IIM patients, 79 (70.5%) were female, and the median age of onset was 55 (18-90) years old; 63.4% were dermatomyositis (DM), 17.9% polymyositis (PM) and 18.8% clinically amyotrophic DM (CADM). Co-existing interstitial lung disease (ILD) was common and found in 65 (58%) patients; 16 (14.3%) had rapidly progressive interstitial lung disease (RPILD), and 16 (14.3%) died within the observed period. Overall, the commonest cause of death was RPILD, followed by infection and malignancy. While anti-MDA5 was strongly associated with RPILD (odds ratio = 33.0 [95% CI: 7.2-151.8], p Conclusion Anti-MDA5 associated RPILD was the leading cause of mortality in IIM. However, those tested negative for both MSA and MAA by current immunoblot technique also had guarded prognosis related to the risk of infection and malignancy. References [1] Lundberg IE, et al.. ARD2017;76(12):1955-1964. Disclosure of Interests None declared |
