A case of systemic lupus erythematosus-progressive systemic sclerosis overlap syndrome with a nephrotic syndrome

Abstract

This patient was a 16-year-old girl who was diagnosed as a systemic lupus erythematosus (SLE)/progressive systemic sclerosis (PSS) overlap syndrome with 8 year history.She was in good health until 1970, when she noticed subcutaneous bleeding. The diagnosis of idiopathic thrombocytopenic purpura was made and she was treated with corticostreoids. In 1973, butterfly rash, polyarthritis, hemolytic anemia and LE cells phenomenon developed and she was diagnosed as SLE. In 1975, sclerodermatous skin changes with Raynaud's phenomenon developed. The area of sclerosis was rapidly spread from her fingers to the trunk and she was diagnosed as PSS besides SLE. In 1979, she admitted to Keio university hospital because proteiuria, which has appeared since 1977, developed to nephrotic syndrome. On the labolatory examinations, a skin biopsy showed an atrophy of the epidermis and a proliferation of collagen fibers in the dermis. The deposition of IgM at the dermal-epidermal junction was found. A renal biopsy demonstrated wire-loop lesions. In the serum from this patient, many autoantibodies, anti-RNP, anti-Sm, anti-Og (Scl70) and anti-ds DNA antibodies, were found. circulating immune complexes were also detected in the serum by Raji cell assay.In summary, this patient had a typical SLE/PSS overlap syndrome and had autoantibodies specific for both SLE and PSS. Circulating immune complexes and immune complex nephritis were present in this rare case.