Thrombotic thrombocytopenia purpura in a patient with systemic sclerosis

Thrombotic thrombocytopenic purpura (TTP) has been associated with sclerode rma renal crises (SRC) in the past. However such reports markedly diminishe d after the onset of ACE inhibitor use. Recently, reports again have surfac ed that describe scleroderma patients presenting with clinical evidence of TTP We describe a 50-year-old female with longstanding limited cutaneous sc leroderma who presented with hematochezia and thrombocytopenia along with o ther findings suggesting TTP. A colon biopsy revealed thrombi within the lu men. Her course was complicated by renal failure and hypertension that did not respond to ACE inhibitor therapy alone. She improved after a course of plasma exchange. She was discharged home only to return 2 months later with grand mal seizures and hypertension. During her course she developed adult respiratory distress syndrome. She again responded to plasma exchange and she was discharged home. She has remained stable for 2 years. This report emphasizes the importance of fully evaluating patients with lon gstanding limited cutaneous scleroderma who present with renal failure, hyp ertension, and thrombocytopenia in association with multiorgan complication s. All possible etiologies, including SRC, TTP, vasculitis, and sepsis shou ld be considered. Tissue biopsies (in this case, a colon biopsy revealed th rombi within the vessel lumen) may prove beneficial in assisting with the d iagnosis. For such patients who fail treatment with ACE inhibitors, plasma exchange may be considered.