Abstract
Background:Idiopathic inflammatory myopathies (IIM) was associated with a significantly higher risk of opportunistic infections that including Pneumocystis jiroveci pneumonia(PJP) which is potentially fatal opportunistic infection. However, no prior studies have evaluated the PJP infection in subtypes of IIM.Objectives:To investigate the incidence rate and mortality rate of PJP infection in subgroups of IIM patients according to myopathy specific antibodies.Methods:In the first part, we reviewed 463 consecutive patients with IIM retrospectively to analyze incidence of PJP infection. In the next part, we enrolled 30 consecutive PJP infection patients with any rheumatic disease was to identify the mortality rate and risk factors. Kaplan-Meier curve with log rank test was used to access differences in survival. Univariate and multivariate analyses were performed to identify prognostic factors using Cox regression.Results:We found that 12(7.5%) PJP cases occurred in 160 anti-MDA5-ab-positive DM patients, while only two (0.7%) PJP cases were found in 303 anti-MDA5-ab-negtive DM/PM patients(P < 0.05). PJP infection typically happened in the first two months of the treatment for anti-MDA5-ab-positive DM patients who have a significant decrease in the CD4+ T cell counts and Lymphocyte counts (P < 0.05). Only two (16.7%) anti-MDA5-ab-positive DM patients recover from PJP, with lethally higher mortality than those PJP infection with other rheumatic diseases (83.3% vs. 38.9%, P < 0.05). We found no association between the time to anti-PJP treatment and treatment outcomes in anti-MDA5-ab-positive DM; yet we confirmed in PJP infection with other rheumatic diseases that anti-PJP treatment within 6 days crucially increased the survival (P < 0.05).Conclusion:PJP infection has alarming high incidence and mortality in anti-MDA5-ab-positive DM patients. Unlike PJP infection with other rheumatic diseases, timely treatment for PJP doesn’t improve the prognosis of this particular subtype. Therefore, the necessity of further study of PJP prophylaxis treatment in anti-MDA5-ab-positive DM patients is verified.
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