전신성 경화증의 임상상에 관한 연구

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 scler...

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Veröffentlicht in:The Korean journal of medicine 1999-12, Vol.57 (6), p.979
Hauptverfasser: 강성욱, Seong Wook Kang, 이윤종, Yun Jong Lee, 차훈석, Hoon Seok Cha, 김현아, Hyun Ah Kim, 박명희, Myung Hee Park, 오명돈, Myoung Don Oh, 송영욱, Yeung Wook Song, 최강원, Kang Won Choi, 이은봉, Eun Bong Lee, 한창완, Chang Wan Han, 백한주, Han Joo Baek
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container_issue 6
container_start_page 979
container_title The Korean journal of medicine
container_volume 57
creator 강성욱
Seong Wook Kang
이윤종
Yun Jong Lee
차훈석
Hoon Seok Cha
김현아
Hyun Ah Kim
박명희
Myung Hee Park
오명돈
Myoung Don Oh
송영욱
Yeung Wook Song
최강원
Kang Won Choi
이은봉
Eun Bong Lee
한창완
Chang Wan Han
백한주
Han Joo Baek
description 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
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fullrecord <record><control><sourceid>kiss</sourceid><recordid>TN_cdi_kiss_primary_1884318</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><kiss_id>1884318</kiss_id><sourcerecordid>1884318</sourcerecordid><originalsourceid>FETCH-LOGICAL-k421-b82f36a5a57ec7bd5e8621b02047fa5e73c7e299cdd4285c6a6cafc0f6a35fee3</originalsourceid><addsrcrecordid>eNpjYeA0NDe20LU0NjPhYOAtLs5MMjA2MLQ0MTEy42QwfLOg5U33gjctGxVebdr7duaUN8vmvpk7Q-HNvJY3zY0gNH2CwqstDW-nzlF4M33Dq61reBhY0xJzilN5oTQ3Q4iba4izh66Pv7uns6OPbraJkaFukoVRmrFZommiqXlqsnlSimmqhZmRYZKBkYGJeVqiaaq5cbJ5qpGlZXJKiomRhWmyWaJZcmJaskGaWaKxaVpqqjE3gzTE2OzM4uL4gqLM3MSiynhDCwsTY0MLYwCpWUsI</addsrcrecordid><sourcetype>Publisher</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype></control><display><type>article</type><title>전신성 경화증의 임상상에 관한 연구</title><source>KoreaMed Open Access</source><creator>강성욱 ; Seong Wook Kang ; 이윤종 ; Yun Jong Lee ; 차훈석 ; Hoon Seok Cha ; 김현아 ; Hyun Ah Kim ; 박명희 ; Myung Hee Park ; 오명돈 ; Myoung Don Oh ; 송영욱 ; Yeung Wook Song ; 최강원 ; Kang Won Choi ; 이은봉 ; Eun Bong Lee ; 한창완 ; Chang Wan Han ; 백한주 ; Han Joo Baek</creator><creatorcontrib>강성욱 ; Seong Wook Kang ; 이윤종 ; Yun Jong Lee ; 차훈석 ; Hoon Seok Cha ; 김현아 ; Hyun Ah Kim ; 박명희 ; Myung Hee Park ; 오명돈 ; Myoung Don Oh ; 송영욱 ; Yeung Wook Song ; 최강원 ; Kang Won Choi ; 이은봉 ; Eun Bong Lee ; 한창완 ; Chang Wan Han ; 백한주 ; Han Joo Baek</creatorcontrib><description>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&lt;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. (Korean J Med 57:979-987, 1999)</description><identifier>ISSN: 1738-9364</identifier><language>kor</language><publisher>대한내과학회</publisher><subject>Clinical characteristics ; Systemic sclerosis</subject><ispartof>The Korean journal of medicine, 1999-12, Vol.57 (6), p.979</ispartof><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,777,781</link.rule.ids></links><search><creatorcontrib>강성욱</creatorcontrib><creatorcontrib>Seong Wook Kang</creatorcontrib><creatorcontrib>이윤종</creatorcontrib><creatorcontrib>Yun Jong Lee</creatorcontrib><creatorcontrib>차훈석</creatorcontrib><creatorcontrib>Hoon Seok Cha</creatorcontrib><creatorcontrib>김현아</creatorcontrib><creatorcontrib>Hyun Ah Kim</creatorcontrib><creatorcontrib>박명희</creatorcontrib><creatorcontrib>Myung Hee Park</creatorcontrib><creatorcontrib>오명돈</creatorcontrib><creatorcontrib>Myoung Don Oh</creatorcontrib><creatorcontrib>송영욱</creatorcontrib><creatorcontrib>Yeung Wook Song</creatorcontrib><creatorcontrib>최강원</creatorcontrib><creatorcontrib>Kang Won Choi</creatorcontrib><creatorcontrib>이은봉</creatorcontrib><creatorcontrib>Eun Bong Lee</creatorcontrib><creatorcontrib>한창완</creatorcontrib><creatorcontrib>Chang Wan Han</creatorcontrib><creatorcontrib>백한주</creatorcontrib><creatorcontrib>Han Joo Baek</creatorcontrib><title>전신성 경화증의 임상상에 관한 연구</title><title>The Korean journal of medicine</title><addtitle>대한내과학회지 (Korean J Med)</addtitle><description>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&lt;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. 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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&lt;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. (Korean J Med 57:979-987, 1999)</abstract><pub>대한내과학회</pub><tpages>9</tpages></addata></record>
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subjects Clinical characteristics
Systemic sclerosis
title 전신성 경화증의 임상상에 관한 연구
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