Clinical Features of Anti-Synthetase Syndrome Associated with Prognosis in Patients with Dermatomyositis and Polymyositis

Abstract

We evaluated whether the clinical features of anti-synthetase syndrome (ASA)—myositis, fever, arthritis, mechanic’s hand, Raynaud’s phenomenon and interstitial lung disease—are relevant to prognosis in patients with dermatomyositis/polymyositis (DM/PM). A retrospective analysis was performed to identify patients diagnosed with DM/PM according to Bohan and Peter criteria. Clinical information, laboratory data and the presence of ASA clinical features at disease diagnosis were searched, and the outcomes of all-cause mortality, intensive care unit admission and disease remission at 1 year were assessed. Among the 86 patients included, fever (36.0%) and interstitial lung disease (26.7%) were the most common ASA clinical features. During the follow-up, 12 patients experienced death, and 7 of the 12 deaths (58.3%) occurred within 3 months of DM/PM diagnosis. Mortality was more frequently observed in those presenting with fever than in those without (25.8% versus 7.3%, p = 0.024). Multivariable Cox proportional analysis revealed that male sex (hazard ratio [HR] 5.53, 95% confidence interval [CI] 1.65, 18.49, p < 0.01) and fever (HR 4.20, 95% CI 1.26, 14.01, p = 0.02) independently predicted mortality. The clinical impact of fever was consistent in both sexes. Fever could be a warning signal heralding the poor outcome of mortality in patients with DM/PM, especially in early disease phases.

Clinical Characteristics of Anti-Synthetase Syndrome and Variables Associated with Interstitial Lung Disease and Mortality: A Retrospective Cohort Study

“…The presence of ILD is associated with a worse prognosis in ASS [6]. Other poor prognostic factors include older age, male gender, and the presence of cardiac or renal involvement [9].…”

The Clinical Value of the Neutrophil-to-Lymphocyte Ratio, the C-Reactive Protein-to-Albumin Ratio, the Systemic Inflammatory Index, and the Systemic Inflammatory Response Index in Patients with the Anti-Synthetase Syndrome

“…One article reported a significant correlation between fever and poor prognosis in DM/PM. 46 Taken together, NLR, CAR, SII, and SIRI help to identify patients with ASS with concomitant ILD, fever, and infection, which may improve patient survival.…”