Disease severity of 100 patients with systemic sclerosis over a period of 14 years: using a modified Medsger scale
Open Access
- 1 December 2001
- journal article
- other
- Published by BMJ in Annals Of The Rheumatic Diseases
- Vol. 60 (12), 1117-1122
- https://doi.org/10.1136/ard.60.12.1117
Abstract
OBJECTIVE To assess organ involvement according to a modified Medsger severity scale and its relation to outcome and prognosis in patients with systemic sclerosis. METHODS One hundred consecutive patients observed in Lund with systemic sclerosis were followed up for a period of 14 years. The mean follow up time was 7.7 years. Initial assessment and an annual evaluation was performed for each patient, with a mean visit frequency of 5.6 per patient. RESULTS Age at referral, high total skin score, truncal skin involvement, low vital capacity, low static lung compliance, low Cr-EDTA clearance, and ECG abnormalities at the initial assessment predict poor outcome. A severity scoring system for five organ systems indicates a slow progression of organ dysfunction after recruitment into the study. The female: male ratio was 2:1, the mean age at onset of symptoms was 42.3 (range 3–82), and the mean age at recruitment was 47.2 years (range 17–82). Thirty patients died during the follow up period at the mean age of 61.3 years (range 33–85). The causes of death were directly related to systemic sclerosis in at least 10 patients, infections in six, cancers in nine, and other causes in four patients. The standardised mortality ratio was 3.5 and 3.7 for men and women, respectively. CONCLUSION A high severity score for function of vital organs was shown to predict shortened survival. In this study a severity score based on simple clinical assessment variables was able to predict poor outcome from extensive skin changes, ECG changes, and compromised lung and renal function. Organ dysfunction mainly became manifest during the first five years of the disease, whereafter organ function remained largely stable.Keywords
This publication has 26 references indexed in Scilit:
- SURVIVAL FOLLOWING THE ONSET OF SCLERODERMA: RESULTS FROM A RETROSPECTIVE INCEPTION COHORT STUDY OF THE UK PATIENT POPULATIONRheumatology, 1995
- Severe restrictive lung disease in systemic sclerosisArthritis & Rheumatism, 1994
- Improved pulmonary function in systemic sclerosis after treatment with cyclophosphamideArthritis & Rheumatism, 1994
- ORGAN MANIFESTATIONS IN 100 PATIENTS WITH PROGRESSIVE SYSTEMIC SCLEROSIS: A COMPARISON BETWEEN THE CREST SYNDROME AND DIFFUSE SCLERODERMARheumatology, 1989
- Nifedipine and Thallium-201 Myocardial Perfusion in Progressive Systemic SclerosisNew England Journal of Medicine, 1986
- Physiologic Abnormalities of Cardiac Function in Progressive Systemic Sclerosis with Diffuse SclerodermaNew England Journal of Medicine, 1984
- Preliminary criteria for the classification of systemic sclerosis (scleroderma)Arthritis & Rheumatism, 1980
- Survival in scleroderma.Annals Of The Rheumatic Diseases, 1971
- A Method for Determination of Pulmonary Elastic Recoil and Resistance at a Regulated Flow RateScandinavian Journal of Clinical and Laboratory Investigation, 1969
- Prognostic Significance of Raynaud's Phenomenon and Other Clinical Characteristics of Systemic SclerodermaCell Metabolism, 1960