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Objectives Mortality of dermatomyositis patients positive with anti-melanoma differentiation-related gene 5 antibody (anti-MDA5-DM) is alarming, especially during the first several months. Infection is an important cause of early death. As there are no reports regarding the effect of prophylactic use of compounded sulfamethoxazole (coSMZ; each tablet contains 400 mg of sulfamethoxazole and 80 mg of trimethoprim) in anti-MDA5-DM patients, we conducted this study to evaluate the efficacy of coSMZ in reducing the incidence of Pneumocystis jirovecii pneumonia (PJP). Methods Consecutive patients with new-onset anti-MDA5-DM from June 2018 to October 2021 in our centre were retrospectively reviewed for >12 months. They were divided into two groups—coSMZ and non-coSMZ—based on the initial use of prophylactic coSMZ. Mortality and the incidence of severe infection within 12 months were compared between two groups. Results Compared with the non-coSMZ group (n  = 93), the coSMZ group (n  = 121) had lower mortality (18.8% vs 51.1%; P   <  0.001) and a lower incidence of PJP (6.8% vs 15.2%; P  = 0.040) and fatal infection (16.1% vs 3.3%; P  = 0.001) during the first 12 months from diagnosis. After adjusting for age, gender, disease duration, peripheral blood lymphocyte count, anti-MDA5 antibody titres, ground-glass opacity scores and treatments, an inverse association was revealed between the prophylactic use of coSMZ and incidence of PJP [adjusted odds ratio 0.299 (95% CI 0.102–0.878), P  = 0.028]. Conclusion Prophylactic use of coSMZ is an effective and safe way to improve the prognosis of anti-MDA5-DM patients by preventing the incidence of PJP. [ABSTRACT FROM AUTHOR]

Objective: To analyze the clinical features and outcomes of the patients with anti-glycyl tRNA synthetase (anti-EJ) syndrome in a large Chinese cohort. Methods: The medical records, imaging, and serologic data of patients with anti-EJ antibodies from the China-Japan Friendship Hospital database were retrospectively investigated. Anti-EJ antibodies were identified using immunoblot assay. Long-term follow-up was conducted. Results: Anti-EJ antibodies were present in 46 (19.7%) of the 234 patients with antisynthetase syndrome (ASS), preceded by anti-Jo-1 and anti-PL-7 antibodies. The mean age of disease onset was 51.2 ± 15.9 years, and 69.6% of these patients were female. The most prevalent clinical feature was interstitial lung disease (ILD) (96.7%), which was also the most common initial manifestation, followed by fever (60.9%), mechanic's hands (56.5%), muscle involvement (50%), and Raynaud phenomenon (8.7%). Ten (21.7%) patients developed rapidly progressive ILD (RP-ILD). Organizing pneumonia (OP) on high-resolution computed tomography (HRCT) scans (OR = 37.5, p = 0.006) and a higher C-reactive protein-to-albumin ratio (CAR) (OR = 28.3, p = 0.01) were independent risk factors for RP-ILD. Muscular pathological features were heterogeneous. Concomitant infection was noted in 63.0% of the patients during the disease course. Hypoalbuminemia (OR = 0.759, p = 0.002) was an independent risk factor for concomitant infection. Patients responded well to glucocorticoid therapy. The 5- and 10-year survival rates of the patients with anti-EJ were 97.8% and 88%, respectively. Conclusion: Anti-EJ syndrome was found to be a relatively common ASS subtype, with a favorable outcome. A notable proportion of the patients experienced RP-ILD, which was prone to OP on HRCT and a higher CAR, and needed aggressive management. Key Points • ILD was the most common initial manifestation of anti-glycyl tRNA synthetase syndrome. • RP-ILD was notable in anti-EJ positive patients. • Anti-EJ positive patients possessed a favorable long-term prognosis, but easily relapsed. [ABSTRACT FROM AUTHOR]