Sclerosing panniculitis is associated with pulmonary hypertension in systemic sclerosis

Background There have been no reports of patients with sclerosing panniculitis and systemic sclerosis (SSc). Objectives To evaluate the incidence of sclerosing panniculitis in patients with SSc, and to investigate the clinical features of such cases. Methods In total, 128 patients with SSc treated at our clinic were investigated retrospectively. Results SSc patients with sclerosing panniculitis had pulmonary hypertension (PH), especially isolated PH, at a significantly higher incidence than those without. Among the SSc patients with PH, those with sclerosing panniculitis had pulmonary infarctions at a higher incidence than those without. Conclusions Our results suggest that thrombosis caused by venous hypertension of the leg may be the main cause of PH in patients with SSc and sclerosing panniculitis. Sclerosing panniculitis may be a useful marker of PH in patients with SSc.