Introduction .Systemic scleroderma (SSc)is a generalized disease of connective tissue manifested clinically by thickening and fibrosis of the skin (scleroderma)and vascular affection and fibrotiza- tion of the internal organs – heart,,lungs,kidneys and gastrointestinal tract.The variability of the extent and progression of the disease incl.affection of the visceral organs,cumulation of the extracellular components of the matrix in some organs and specific immunological deviations are typical.Objectives .The objective of the study was to analyze data on the group of patients with SSc from different aspects – demographic,professional,anamnestic data,some clinical and laboratory data.Another intention was to seek possible clinical-laboratory associations which might be of prognostic importance.Methods .The patients were included in the register after completing the registration sheet of baseline data.These questionnaires were completedby workers of some clinical departments and non-state health institutions in the entire Czech Republic.After obtaining infor- med consent blood samples were taken for creating a serum and DNA bank.Results .Data of 65 patients with SSc were subjected to statistical analysis.This group included 38.5 %with the diffuse form,49.2 %with the limited form,3.1 %scleroderma sine scleroderma,3.1 %with the overlap syndrome and 6.2 %with un-differentiated connective tissue disease.The group comprised 9 men and 56 women at the time of evaluation with a mean age of 53.3 ±12.6 years.As regards occupation the majority had full invalid pensions or old age pensions (both 35.4 %).SSc was recorded in parents in one case,it was not observed in siblings.The incidence of pulmonary and gastrointestinal symptoms and objective findings had a progressing trend with time,the development of changes on the hands as a result of damage was not asequivocal.The authors tested also differences between subgroups according to the following classification:limited –sclerodactylia,limited –acrosclerosis, diffuse and others from the aspect of the presence of autoantibodies anti-Scl-70,ACA and nRNP.In anti-Scl-70 no statistical difference was found between groups,while in ACA and in nRNP the number of observations was too small.When the authors correlated the presence of the mentioned autoantibodies and synovitis and dysphagia and also basal fibrosis and rales at the bases no statistically significant relationship was found.Conclusion .Although it was not possible to create a nationwide register in the true sense of the word which would include all subjects suffering from SSc a unique group with a rare disease was assembled which was not available so far in this country. The limited information provided by some statistical tests when investigating some correlations is beyond doubt due to the small number of subjects in different subgroups of SSc.

        Key words: systemic scleroderma,national register,acroclerosis,sclerodactylia,anti-Scl-70, anticentromere antibodies