Scleroderma renal crisis following pericardial effusion in a Japanese female

Scleroderma renal crisis (SRC) occurs in 4-5% of Japanese patients with systemic sclerosis (SSc) and is one of the most severe complications, along with interstitial pneumonia and pulmonary hypertension, which lead to a poor outcome. The factors predicting SRC include diffuse progressive skin thickening, a duration of SSc of 4 years or less, having autoantibodies against RNA polymerase III, systemic corticosteroid (CS) administration and recent cardiac events, including pericarditis. We herein report a female case of SRC preceded by pericardial effusion during the treatment with CS for rapidly progressive skin thickening. She had none of the known autoantibodies and was successfully treated with an angiotensin II receptor blocker.