Abstract
A 62-year-old woman with no contributory medical history transferred to our hospital. Three years before presentation, she had complained of sausage-like fingers, Raynaud's phenomenon, digital-tip ulcers and decreased wrinkles around her mouth. One year before presentation, she had dyspnea on effort. Though Chinese herbal medicine was administered, these symptoms did not improve. Three days before presentation, she had been admitted to a local hospital because of orthopnea. On admission, hypertension with papilledema, massive pericardial effusion, congestive heart failure, pulmonary fibrosis, red cell fragmentation on peripheral blood smear and renal impairment were present. On anti-hypertensive therapy, blood pressure decreased to 150/80 mmHg and red cell fragmentation on peripheral blood smear disappeared. However, renal dysfunction and edema of extremities developed and she transferred to our hospital. On admission, skin scleroderma, skin pigmentation, systolic murmur, fine crackles and edema of extremities were present. Urinalysis showed both proteinuria and microhematuria and chest X ray showed cardiomegaly and lung congestion. Echocardiography demonstrated massive pericardial effusion and chest computed tomography scan showed mild interstitial change of the bilateral lower lung fields. Taking positive anti-Scl-70 antibody into consideration, she was diagnosed with scleroderma renal crisis and congestive heart failure with pericardial effusion. Both ACE inhibitor and hemodialysis were started and blood pressure before one session of hemodialysis decreased to 138/68 mmHg. Since congestion and edema of extremities improved, she was discharged from hospital on the 27th hospital day. The frequency of scleroderma renal crisis is lower in Japan than in western countries. We report a case of severe scleroderma renal crisis with pericardial effusion that progressed to end stage renal disease.
