Contributed by Lina Perry, M.D. and Sheldon Bastacky, M.D.
Published on line in May 1996
PATIENT HISTORY :
The patient is nomotensive 74 year old male referred for evaluation of renal failure complicating scleroderma, the latter diagnosed in 10/94. The patient's scleroderma initially presented with cutaneous changes and a mild (abortive) renal "crisis" (creatinine 1.2 mg%, positive ANA 1:80) treated with captopril (later switched to enalopril and then back to captopril), penicillamine, and later azathioprine. In 6/95, protein excretion was 8.9 gm/24 hrs which was presumptively attributed to the penicillamine therapy. Around this time exudative pleural effusions (and later ascites) were noted which were treated with repeated thoracenteses. In early 11/95, urine protein had decreased to 3.9 gm/24hrs and serum creatinine was 1.7 mg%. By late 2/96, BUN was 104 mg% and was creatinine 4.3 mg%; a 24 hour urine collection was remarkable for a creatinine clearance 9.6 ml/min and 1.9 gm protein. Serum C3 and C4 were 70 and 20 mg/dl respectively. Blood studies were ANA-positive, speckled (1:800), ANCA- negative, anti-GBM-negative, hepatitis B/C serologies-negative, and anti-dsDNA-negative. Urinalysis showed 4+ protein and 3+ blood, with urine sediment revealing many red blood cells and white blood cells, scattered white blood cell casts, hyalin casts and an occasional waxy and hemoglobin cast. Serum and urine protein electrophoretic studies were negative for a monoclonal protein and a cryoglobulin screen was also negative.