The Clinical Features and Autoantibody Profile of Progressive Systemic Sclerosis in Korea

Park, Suk Kyu; Kim, Tae Hwan; Jun, Jae Bum; Jung, Sung Soo; Bae, Sang Cheol; Kim, Think You; Kim, Seong Yoon; Yoo, Dae Hyun

Abstract

OBJECTIVE
The aim of present study was to determine the differences in clinical features,laboratory data and autoantibody profile between limited and diffuse subsets of progressive systemic sclerosis in Korea,and to see differences from foreign studies on the same disease.
METHODS
Fifty-six patients with systemic sclerosis at the Hospital for Rheumatic Diseases were studied for age at diagnosis,subtype,clinical features, laboratory data,systemic involvement and pattern of autoantibodies by careful review of medical records and interviews.
RESULTS
The disease was relatively more prevalent in female groups of third,forth and fifth decade (male:female=1:17.7,mean age:39.5,range:11-68 years).The patients consisted of 29 diffuse and 27 limited subsets. Clinical features were as follows: sclerodactyly (92.9%),Raynaud 's phenomenon (92.9%), dyspnea (71.4%),arthralgia/arthritis (64.3%),dry cough (58.9%),myalgia(51.8%),rale (48.2%),esophageal symptoms (41.1%),digital pitting scar(41.1%),telangiectasia (28.6%).Antinuclear antibody was positive in all 54 cases (100%)which is tested.The respiratory system involvement was statistically higher in diffuse subset than in limited subset (77.4 vs.52.0%,p=0.046). The anti-centromere antibody was detected in 1 case with limited subset and the anti-Scl-70 antibody in 6 cases (11.3%)with diffuse subset.
CONCLUSION
Interstitial lung disease was more prevalent in patients of this study than patients of other countries.Investigation of pulmonary system is needed,especially in earlier stage and diffuse type.Systemic sclerosis-related autoantibodies (anti-Scl 70 antibody,anti-centromere antibody)are less prevalent than the results of other studies.