Survival and causes of death in 366 Hungarian patients with systemic sclerosis.

Abstract

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.

• Authors: L Czirják, G Kumánovics, C Varjú, Z Nagy, A Pákozdi, Z Szekanecz, G Szucs
• Journal: Annals of the rheumatic diseases (Ann Rheum Dis) Vol. 67 Issue 1 Pg. 59-63 (Jan 2008) ISSN: 1468-2060 [Electronic] England
• PMID: 17519276 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Autoantibodies

Topics

• Adult
• Age Factors
• Aged
• Autoantibodies (blood)
• Blood Sedimentation
• Cause of Death
• Centromere (immunology)
• Female
• Heart Diseases (complications, mortality)
• Humans
• Kidney Diseases (complications, mortality)
• Male
• Middle Aged
• Neoplasms (complications, mortality)
• Prognosis
• Proportional Hazards Models
• Prospective Studies
• Scleroderma, Diffuse (complications, mortality)
• Scleroderma, Systemic (complications, immunology, mortality)
• Survival Analysis