Rapidly progressive systemic sclerosis with a fatal outcome in male patients

Objectives: Retrospective analysis of clinical outcomes of male patients with particularly severe and rapidly progressive diffuse systemic sclerosis (SSc) with a fatal outcome with emphasis on organ involvement and results of diagnostic tests, and tentative distinction of a subgroup of especially progressive SSc.
Material and methods: In the last few years among patients with SSc hospitalized in our centres, five patients with particularly rapidly progressive disease were distinguished. Despite aggressive treatment, the disease led to a fatal outcome in a short time. Their clinical history and results of diagnostic tests were evaluated.
Results: All of them were smokers and three of them did not stop smoking after the diagnosis. Laboratory findings revealed high titres of Scl70 antibodies and enhanced erythrocyte sedimentation rate (ESR) in all of the patients. Most of them had increased serum creatine kinase (CK) values. During the disease rapidly progressive severe organ involvement was observed (pulmonary fibrosis, renal failure, cardiac failure, pulmonary arterial hypertension). The skin thickening increased rapidly and they died within 12-24 months after the first signs of skin thickening. Acute cardiac failure was the cause of death.
Conclusions: The described cases suggest possible distinction of a subset of a subgroup of patients with a particularly severe and rapidly progressive disease. It might be a population of patients with the following characteristics: males over 40 years of age with high titres of anti-Scl70 antibodies and elevated serum CK levels. This is consistent with the presently published data on factors associated with fatal prognosis in patients with SSc.