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.
Antibodies, Antinuclear ; Azotemia ; Calcinosis ; Cicatrix ; Connective Tissue ; Diagnosis ; Esophagus ; Fibrosis ; Heart Failure ; Humans ; Hypertension, Pulmonary ; Lung ; Lung Diseases, Interstitial ; Musculoskeletal System ; Myalgia ; Pericardial Effusion ; Rheumatology ; Scleroderma, Diffuse ; Scleroderma, Limited ; Scleroderma, Systemic* ; Seoul ; Skin ; Vital Capacity

Scleroderma is an incidious and chronic disease of unknown cause. The clinical picute of scleroderma is characterized by the apperance of circumscribed of diffuse, hard, smooth, ivory colored areas that are immobile upon upon the underlying tissues. It may be classified to as a localized form (morphea) and a systemic scleroderma or progressive systemic sclerosis (PSS) which is characterized by diffuse involvment of the connective tissue of the skin and certain intemal organs. The authors observed 3 cases of PSS and 6 cases of morphea. They have complained of marked sclerosis or thickening of skin or slowly developing dyspneadue to interstitial pulmonary fibrosis which were confirmed by chest X-ray. Recently, penicillamine-D has been advocated as a beneficial agent in scleroderma by Asboe-Hansen. therefore, penicillamine-D was administered to the above patients. Two of PSS were markedly improved with normal skin softness. Two of morphea were also moderately improved. Other patients remained stationary or no effect.
Chronic Disease ; Connective Tissue ; Humans ; Pulmonary Fibrosis ; Scleroderma, Diffuse ; Scleroderma, Localized ; Scleroderma, Systemic ; Sclerosis ; Skin ; Thorax

Systemic sclerosis is an intractable clinical subset characterized by diffuse fibrosis, vasculopathy, and immune abnormalities. Diffuse cutaneous systemic sclerosis has a variety of symptoms depending on the internal organs involved, such as the lungs, heart, kidneys, and gastrointestinal system. Gastrointestinal involvement is frequent and one of the major cause of morbidity and mortality. There is no sustained, effective therapy for treating the active gastrointestinal involvement of systemic sclerosis. Although immunoglobulin is commonly used in the treatment of other autoimmune diseases, its effects in the treatment of the gastrointestinal involvement in systemic sclerosis are unclear. Here, we report a patient with a severe cough caused by uncontrolled gastrointestinal involvement in diffuse cutaneous systemic sclerosis treated with immunoglobulin.
Autoimmune Diseases ; Cough ; Fibrosis ; Heart ; Humans ; Immunoglobulins* ; Kidney ; Lung ; Mortality ; Scleroderma, Diffuse ; Scleroderma, Systemic*

The antiphospholipid syndrome is characterized by arterial thrombosis, venous thrombosis, pregnancy wastage, and thrombocytopenia associated with a persis tently positive lupus anticoagulant and/or moderate to high positive anticardiolipin antibodies(IgG or IgM). The antiphospholipid antibodies have been detected in many medical conditions, but the antiphospholipid syndrome (APS) has mainly been restricted to the primary antiphospholipid syndrome and APS associated with systemic lupus erythematosus. Rarely, the APS has been reported in other autoimmune disorders in the literature. We describe a woman with a limited form of scleroderma and the APS manifested by complete occlusion of left axillary artery with probable thrombotic occlusive nature, thrombocytope nia, prolonged aPTT, and persistently positive lupus anticoagulant.
Antibodies, Antiphospholipid ; Antiphospholipid Syndrome* ; Axillary Artery ; Female ; Humans ; Lupus Coagulation Inhibitor ; Lupus Erythematosus, Systemic ; Pregnancy ; Scleroderma, Limited* ; Thrombocytopenia ; Thrombosis ; Venous Thrombosis

Systemic sclerosis is a connective tissue disease characterized by cutaneous and visceral fibrosis, as well as vascular disease involving arterioles, small and medium arteries of the peripheral circulation. Digital ulcers, defined as necrotic lesions that occur either at distal aspects of digits or over bony prominence, occur in up to 50% of patients with limited or diffuse systemic sclerosis. These lesions are exquisitely painful, heal slowly, and lead to substantial functional disability. We describe a 59-year-old woman with systemic sclerosis, suffering from a painful, non-healing digital ulcer despite conventional therapies, who demonstrated dramatic improvement with oral sildenafil treatment.
Arteries ; Arterioles ; Connective Tissue Diseases ; Female ; Fibrosis ; Humans ; Middle Aged ; Scleroderma, Diffuse ; Scleroderma, Systemic* ; Sildenafil Citrate ; Ulcer* ; Vascular Diseases

Extensive tumoral calcinosis affecting a large joint is uncommon in patients with systemic sclerosis. We report the case of a 52-year-old female patient referred for a growing calcified mass in the shoulder. She was diagnosed with interstitial lung disease and progressive systemic sclerosis. Although the pain and disability associated with the affected joint was not severe, the patient underwent surgical excision because the mass continued to grow and was likely to produce shoulder dysfunction and skin ulceration. The patient appeared well 10 months after surgery with no signs of recurrence. This report highlights the timing and indication of surgical excision in similar cases.
Calcinosis* ; Female ; Humans ; Joints ; Lung Diseases, Interstitial ; Middle Aged ; Quality of Life ; Recurrence ; Scleroderma, Diffuse ; Scleroderma, Systemic* ; Shoulder ; Skin Ulcer

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.
Autoantibodies ; Cough ; Dyspnea ; Female ; Humans ; Korea* ; Lung Diseases ; Medical Records ; Respiratory System ; Rheumatic Diseases ; Scleroderma, Diffuse* ; Scleroderma, Systemic

Sjogren's syndrome(SS) is a chronic inflammatory disorder characterized by lymphocytic infiltration of lacrimal and sailvary glands, which results in dry eyes and dry mouth. SS may exist as a secondary condition or as a secondary condition in association with connective tissue disease such as rheumatoid arthritis, systemic lupus erythematosus, or progressive systemic sclerosis. We experienced a patient with primary SS who developed multiple bullae, nodular type of pulmonary amyloidosis and lymphocytic interstitial peumonitis. We believe this to be the first reported case of SS acompanied by these three types pulmonary manifestations at the same time.
Amyloidosis* ; Arthritis, Rheumatoid ; Bronchioles* ; Connective Tissue Diseases ; Humans ; Lupus Erythematosus, Systemic ; Mouth ; Scleroderma, Diffuse ; Sjogren's Syndrome*

No abstract available.
Brain ; Scleroderma, Localized ; Scleroderma, Systemic

Systemic sclerosis is an autoimmune disease, characterized by inflammation, microangiopathy and fibrosis in the skin and various intestinal organs. Gastrointestinal involvement is one of the major causes of serious morbidity, and next to the skin, the gastrointestinal tract is the most commonly involved organ. While esophageal involvement is most common gastrointestinal manifestation, the involvement of the small intestine and colon is less common but may lead to life-threatening complications like chronic pesudoobstruction or pneumatosis cystoids intestinalis. Here, we describe a case of progressive systemic sclerosis associated with intestinal pseudoobstruction. 28 year-old male presented abdominal pain and vomiting and he was diagnosed as having intestinal pseudoobstruction. His symptoms were well managed using the combination of octreotide, a long-acting somatostatin analogue, and erythromycin.
Abdominal Pain ; Adult ; Autoimmune Diseases ; Colon ; Erythromycin* ; Fibrosis ; Gastrointestinal Tract ; Humans ; Inflammation ; Intestinal Pseudo-Obstruction* ; Intestine, Small ; Male ; Octreotide* ; Scleroderma, Diffuse ; Scleroderma, Systemic* ; Skin ; Somatostatin ; Vomiting