Abstract
A 54 year-old woman was admitted because of acute progression of progressive systemic sclerosis (PSS).
Significant scleroderma on the face and extremities, limitation of range of movement and systolic murmur on the 3rd intercostal space at the left sternal border were observed. Cardiomegaly, bilateral pleuro-pericardial effusion were recognized by echocardiogram.
Under the diagnosis of PSS with pleulopericardial effusion, the prednisolone therapy (60 mg/day) was started. Soon later, thrombocytopenia, hemolytic anemia and marked red cell fragmentation appeared in her peripheral blood. Under the diagnosis of microangiopathic hemolitic anemia (MHA), the patient received prostaglandin E1 (PGE1), gabexate mesilate (FOY) and ticlopidine, but she died of a progressive acute renal failure.
The autopsy findings revealed thickened walls and fibrin thrombi in the arteries of the kidneys and the lungs.
These results may suggest that prednisolone played an important role in the occurence of hypercoagulable state in PSS.
