Rheumatology (Feb 2010)
Rapidly progressive systemic sclerosis with a fatal outcome in male patients
Abstract
Objectives: Retrospective analysis of clinical outcomes of malepatients with particularly severe and rapidly progressive diffusesystemic sclerosis (SSc) with a fatal outcome with emphasis onorgan involvement and results of diagnostic tests, and tentativedistinction of a subgroup of especially progressive SSc. Material and methods: In the last few years among patients withSSc hospitalized in our centres, five patients with particularlyrapidly progressive disease were distinguished. Despiteaggressive treatment, the disease led to a fatal outcome ina short time. Their clinical history and results of diagnostic testswere evaluated. Results: All of them were smokers and three of them did not stopsmoking after the diagnosis. Laboratory findings revealed hightitres of Scl70 antibodies and enhanced erythrocytesedimentation rate (ESR) in all of the patients. Most of them hadincreased serum creatine kinase (CK) values. During the diseaserapidly progressive severe organ involvement was observed(pulmonary fibrosis, renal failure, cardiac failure, pulmonaryarterial hypertension). The skin thickening increased rapidly andthey died within 12-24 months after the first signs of skinthickening. Acute cardiac failure was the cause of death. Conclusions: The described cases suggest possible distinction ofa subset of a subgroup of patients with a particularly severe and rapidly progressive disease. It might be a population of patientswith the following characteristics: males over 40 years of agewith high titres of anti-Scl70 antibodies and elevated serum CKlevels. This is consistent with the presently published data onfactors associated with fatal prognosis in patients with SSc.
