Systemic Scleroderma.Clinical and Laboratory Characteristics of Baseline Dataof the National Register of Rheumatic Diseases

Overview

Introduction .
Systemic scleroderma (SSc)is a generalized disease of connective tissue manifestedclinically 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 theextent and progression of the disease incl.affection of the visceral organs,cumulation of theextracellular components of the matrix in some organs and specific immunological deviations aretypical.Objectives .The objective of the study was to analyze data on the group of patients with SScfrom different aspects – demographic,professional,anamnestic data,some clinical and laboratorydata.Another intention was to seek possible clinical-laboratory associations which might be ofprognostic importance.Methods .The patients were included in the register after completing theregistration sheet of baseline data.These questionnaires were completedby workers of some clinicaldepartments 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 65patients with SSc were subjected to statistical analysis.This group included 38.5 %with the diffuseform,49.2 %with the limited form,3.1 %scleroderma sine scleroderma,3.1 %with the overlapsyndrome and 6.2 %with un-differentiated connective tissue disease.The group comprised 9 menand 56 women at the time of evaluation with a mean age of 53.3 ±12.6 years.As regards occupationthe majority had full invalid pensions or old age pensions (both 35.4 %).SSc was recorded in parentsin one case,it was not observed in siblings.The incidence of pulmonary and gastrointestinalsymptoms and objective findings had a progressing trend with time,the development of changes onthe 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.Inanti-Scl-70 no statistical difference was found between groups,while in ACA and in nRNP thenumber of observations was too small.When the authors correlated the presence of the mentionedautoantibodies and synovitis and dysphagia and also basal fibrosis and rales at the bases nostatistically significant relationship was found.Conclusion .Although it was not possible to createa nationwide register in the true sense of the word which would include all subjects suffering fromSSc 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 isbeyond 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