Survival and causes of death in 366 Hungarian patients with systemic sclerosis.
OBJECTIVE
Survival analysis of a series of 366 consecutive patients with systemic sclerosis (SSc).


METHODS
Clinical and laboratory data were evaluated from 1983 until 2005 using a standard protocol. The female/male ratio was 315/51. The mean (SD) age of the patients was 56.8 (12.2) years. The duration of disease was 12 (5-19) years with a median follow-up of 6 (3-12) years.


RESULTS
Kaplan-Meier univariate analysis showed that renal, cardiac involvement, pigmentation disturbances, malabsorption, a forced vital capacity <50%, diffuse scleroderma, presence of early malignancy, anaemia, and increased erythrocyte sedimentation rate (ESR) were signs of unfavourable prognosis, whereas anti-centromere antibodies were indicators of a good survival. In the multivariate Cox proportional hazards model the presence of diffuse scleroderma, renal involvement, coexistence of a malignant disease, and increased ESR were poor independent prognostic signs. Elderly age at the onset of disease also caused an unfavourable outcome. A total of 86 SSc-related deaths were recorded during the follow-up. Of them, 65% were attributed to cardiorespiratory manifestation of disease. Tumour associated early death was found in 12 cases (14%).


CONCLUSIONS
In addition to the well-known factors influencing the outcome (diffuse subset, internal organ involvements, and inflammatory signs), the coexistence of scleroderma with a malignancy also causes a poor outcome.
Czirják
L
L
University of Pécs, Medical School, Department of Immunology and Rheumatology, H-7621 Pécs, Hungary. laszlo.czirjak@aok.pte.hu
Kumánovics
G
G
Varjú
C
C
Nagy
Z
Z
Pákozdi
A
A
Szekanecz
Z
Z
Szucs
G
G
D000328:Adult; D000367:Age Factors; D000368:Aged; D001323:Autoantibodies / Q000097:blood; D001799:Blood Sedimentation; D002423:Cause of Death; D002503:Centromere / Q000276:immunology; D005260:Female; D006331:Heart Diseases / Q000150:complications / Q000401:mortality; D006801:Humans; D007674:Kidney Diseases / Q000150:complications / Q000401:mortality; D008297:Male; D008875:Middle Aged; D009369:Neoplasms / Q000150:complications / Q000401:mortality; D011379:Prognosis; D016016:Proportional Hazards Models; D011446:Prospective Studies; D045743:Scleroderma, Diffuse / Q000150:complications / Q000401:mortality; D012595:Scleroderma, Systemic / Q000150:complications / Q000276:immunology / Q000401:mortality*; D016019:Survival Analysis
D001323:Autoantibodies