Scleroderma is a systemic disorder with multiorgan involvement. About 90% of patients with scleroderma has Raynaud's phenomenon and microvascular involvement is well recognized in scleroderma, but macrovascular involvement is not recognized. We experienced 2 cases of scleroderma with peripheral vascular occlusions that had been diagnosed by fernoral angiography. The one patient with limited scleroderma had anticentromere antibody and angiography of both fernoral arteries showed nonvisualization of posterior tibial artery. The other patient with diffuse scleroderma had anti-Scl-70 antibody and angiography of right fernoral artery showed occlusion of both anterior and posterior tibial arteries and stenosis of distal portion of peronial artery. Both patients didn't have the risk factors of atherosclerosis such as hypertension, hypercholesterolemia, obesity, and smoking. In recent years, there have been reports that scleroderma is associated with macrovascular disease. In the future, the study on the freguency, prevention and treatment of macrovasular disease in scleroderma is necessary. We report 2 cases of scleroderma with peripheral vascular occlusions that had been diagnosed by fernoral angiography with review of literatures.
Angiography ; Arteries ; Atherosclerosis ; Constriction, Pathologic ; Humans ; Hypercholesterolemia ; Hypertension ; Obesity ; Peripheral Vascular Diseases* ; Risk Factors ; Scleroderma, Diffuse ; Scleroderma, Limited ; Smoke ; Smoking ; Tibial Arteries

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

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