2006 Volume 116 Issue 1 Pages 71-78
We reported a rare case of diffuse cutaneous systemic sclerosis (SSc) with catastrophic antiphospholipid syndrome (CAPS). A 70-year-old woman with SSc visited our hospital in November 7, 2002, and she also had extreme hypertension. Despite treatments with an inhibitor of angiotensin converting enzyme (ACEI) to lower her blood pressure, her renal function gradually deteriorated until finally she had to receive dialysis treatment. About two years after the first visit, she complained of general fatigue and severe cough ; pneumonia and heart failure were suspected. After a brief improvement with treatment, she suddenly suffered from circulation failure of the limbs and drastic disturbance of consciousness. Because of her positive lupus anticoagulant (LA) and of thrombosis detected in many internal organs, we diagnosed her with CAPS. We tried anticoagulant therapy and plasma exchange, but she died without improving. It is well known that a severe, rapidly progressive form characterized by clinical involvement of multiple different organ systems with histopathological evidence of small and large vessel occlusion is termed CAPS, and that patients with systemic lupus erythematosus (SLE) often suffer from CAPS. Some previous studies have indicated that more than 50% of SSc patients have LA. However, it is still not clear whether or not the patients with SSc have a high incidence of APS or CAPS, as in this case of SLE.
