Fri0338 anti-Mda5 Idiopathic Inflammatory Myositis (Iim) Confers Poor Prognosis but Negative Myositis Specific Antibody (Msa) Is Not Benign Either

Abstract

BackgroundMSA test is useful to diagnose IIM and subcategorize patients by disease phenotypes.ObjectivesThe study aims to evaluate the survival of IIM patients of different MSA patterns.MethodsAn 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 < 18 at disease onset. Immunoblot EUROLINE autoimmune inflammatory myopathies 16 antigens strip (EUROIMMUNE AG, Lubeck, Germany) was used. Information including baseline demographic data, disease manifestations, MSA results, co-existing malignancy, duration of survival and causes of death were collected. IIM patients were divided into seven groups, which included 1) anti-aminoacyl tRNA synthetase (ARS) 2) anti-MDA5, 3) anti-TIF1γ/anti-NXP2, 4) double positive MSA, 5) other MSAs, 6) negative MSA/MAA (myositis associated antibodies) and 7) positive MAA only. Survival probabilities were compared among different MSA groups by using the Kaplan-Meier method and log-rank test. A two-tailed probability value (p) < 0.05 was considered significant. The study was approved by Kowloon Central Cluster Ethic Committee (ref.: KC/KE-17-0177/ER-3)ResultsAmong 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<0.001), anti-MDA5 group had the worst survival, with 1-year and 5-year survival both at 43%, compared to above 80% in all other groups (log-rank test p<0.001) (Table 1). There were nine patients with double positive MSA and 28 had negative MSA/MAA. Analysis between MSA sub-groups found that the double positive MSA, MAA only and other MSAs group had no mortality during study period while the negative MSA/MAA group had the second highest mortality following the anti-MDA5 group (Figure 1). Infection and malignancy were the two major causes of death in the MSA/MAA negative group.ConclusionAnti-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.Figure 1 Kaplan-Meier survival of IIMTable 1 Survival rates in different MSA groups Survival 1 year 5 years Anti-ARS100%92%Anti-MDA543%43%Double positive100%100%Negative MSA/MAA89%82%Only positive MAA100%100%Anti-TIF1γ/anti-NXP2100%88%Other MSAs100%100%References[1] Lundberg IE, et al.. ARD2017;76(12):1955-1964.Disclosure of InterestsNone de...

Cyclophosphamide Versus Obinutuzumab for the Treatment of Anti-MDA5 Positive Inflammatory Myopathy with Interstitial Lung Disease: A Study Protocol and Literature Review

“…In a Japanese cohort of DM patients tested positive for anti-MDA5, 46% died within 6 months from disease onset due to respiratory failure [15]. Our local experience indicated that the one-year survival of IIM patients with the anti-MDA5 was 43% [16].…”