Study on the clinical characteristics of systemic sclerosis

Kang, Seong Wook; Lee, Yun Jong; Cha, Hoon Seok; Kim, Hyun Ah; Park, Myung Hee; Oh, Myoung Don; Song, Yeung Wook; Choi, Kang Won; Lee, Eun Bong; Han, Chang Wan; Baek, Han Joo

Abstract

BACKGROUND: Systemic sclerosis (scleroderma) is a connective tissue disorder of unknown etiology characterized by fibrosis of the skin and internal organs. It is remarkably heterogeneous in initial presentations and internal organ involvement. Limited and diffuse cutaneous subsets of systemic sclerosis (SSc) are known to be different in clinical and laboratory features. The aim of the present study was to determine the clinical characteristics of systemic sclerosis in Koreans.
METHODS
Fifty-six patients with systemic sclerosis at the Rheumatology Clinic of Seoul National University Hospital were studied for age, sex, symptoms, signs, and laboratory results. The differences in clinical and laboratory features between limited and diffuse cutaneous subsets were investigated.
RESULTS
The mean age at diagnosis of 56 patients (male:female=1:4.6) was 42.4 years (range 11-72 years). The patients consisted of 30 limited and 26 diffuse cutaneous SSc. Cutaneous involvement was as follows: sclerodactyly (100%), Raynaud's phenomenon (94.6%), digital pitting scar (66.1%), subcutaneous calcinosis (1.8%). In musculoskeletal system, 25 cases (46.3%) developed arthralgia/arthritis, 14 cases (25.9%) myalgia. In gastrointestinal system, esophagus was affected in 11 cases (20.7%). Respiratory involvement consisted of interstitial lung disease (24 cases, 43.7%) and pulmonary hypertension (2 cases, 3.6%). Total skin score and functional vital capacity showed significant negative correlation (p<0.05). Cardiovascular involvement consisted of congestive heart failure (3 cases, 5.5%) and pericardial effusion (1 case, 1.8%). Azotemia was found in one patient (1.8%). Antinuclear antibody was positive in 53 cases (94.6%) and anticentromere antibody 2 cases (3.6%). Anti-Scl 70 antibody was positive in 46.4% of all patients, 40.0% of limited scleroderma and 53.8% of diffuse scleroderma. When comparing clinical features between limited and diffuse cutaneous subsets, musculoskeletal involvement was more common in limited scleroderma.
CONCLUSION
Systemic sclerosis in Koreans showed various systemic and organ involvement, musculoskeletal system, lung and esophagus being commonly affected. There was no significant difference between limited and diffuse scleroderma in clinical features except musculoskeletal involvement. Investigation of major internal organs, especially lung and esophagus, is needed, regardless of cutaneous subsets in systemic sclerosis.