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Lupus
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Renal crisis in asclerodermic scleroderma—lupus overlap syndrome

H C Horn

Department of Internal Medicine C, Section of Rheumatology, Odense University Hospital, Odense, Denmark, Department of Nephrology Y, Odense University Hospital, DK-5000 Odense-C, Denmark

P Ottosen

Department of Pathology, Odense University Hospital, Odense, Denmark

P Junker

Department of Internal Medicine C, Section of Rheumatology, Odense University Hospital, Odense, Denmark

A 24-year-old woman with overlappingfeatures of sclerodermia sine scleroderma and systemic lupus erythematosus (SLE) presented with rapidly acceleratinghypertension accompanied by neuropsychological deficits and tonic—clonic seizures. Kidney biopsy showed severe intimal hyperplasia of small renal arteries but no glomerulonephritis. Following treatment with ACE inhibitor, prednisolone and cyclophosphamide complete remission was achieved with minimal brain damage and normal kidney function. Anti-RNA polymerase I, II and III antibodies have remained positive during follow-up for 2 years, suggesting a linkage with the underlying pathogenetic pathway.

Key Words: systemic sclerosis • scleroderma renal crisis • systemic lupus erythematosus • overlap syndrome • anti-RNA polymerase I, II and III antibodies

Lupus, Vol. 10, No. 12, 886-888 (2001)
DOI: 10.1191/096120301701548382
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