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Scleroderma-polymyositis overlap syndrome increases risks for cardiac disease, death

Patients with scleroderma-polymyositis overlap more often presented with pulmonary fibrosis and cardiac disease as the causes of death compared with patients with other conditions, according to a study.

Researchers performed a three-way comparison of clinical and myopthological features and causes of death among patients with scleroderma-polymyositis (SSc-PM) overlap (n = 25), systemic sclerosis (SSc; n = 397) and polymyositis (PM; n = 40). They scored the patients’ biopsies for the presence of inflammation, necrotic muscle fibers, immunohistochemical staining and rimmed vacuoles.

Patients with SSc-PM had a significantly higher incidence (83%) of pulmonary fibrosis compared with 49% in the SSc and 53% in PM cohorts (P = .04).

Eight of 25 SSc-PM patients died, compared with 22 in the SSC cohort and two in the PM cohort, and half of the deaths were cardiac-related; however, this difference was not statistically significant, according to the researchers.

The researchers also found that in 96% of SSc-PM patients’ biopsies, necrotic muscle fibers were present, which demonstrated a significant increase compared with patients in the PM cohort (P = .02).

Disclosure: The authors have no relevant disclosures.