Systemic sclerosis (SSc) is a chronic, systemic connective tissue disease, characterized by progressive skin fibrosis, internal organs and disfunction blood vessels.
The aim: of the study was o analyze death causes in patients with SSc and the assessment of relationship between clinical status, immunologic test results and survival/mortality of patients with SSc.
Material and methods: Case histories of all patients with SSc, hospitalized for six years (since 1'" October 1999 to 1It October 2005) in Department of Rheumatology and Internal Diseases, Medical University of Bialystok, were retrospectively analyzed. Current patients status at the end-point of study was estimated during control examination and phone contact in selected cases. The time and cause of death was based on autopsy results.
Results: In study group of 76 patients with SSc, 13 deaths were found (17,1%). Death cause analyses revealed that: interstitial lung disease was the main cause of death (4/13 - 30%). As a secondary cause of death in these patients neoplasms were recognized (3/13 - 23,1%). In all patients with neoplasms exudation in pleura was diagnosed.
Conclusions: Pulmonary complications and neoplasms are predominant causes of death in patients with SSc. Risk factors of death are: pulmonary hypertension and myositis. The presence of exudation in pleura in patients with SSc is a bad prognostic symptom and a possible sign of neoplastic disease.