Scleroderma Renal Crisis Debute with Thrombotic Microangiopathy: A Successful Case Treated with Eculizumab

Abstract

We had the challenged to treat a 40-year-old female with Systemic Scleroderma who was showing unspecific symptoms. During her time at the hospital she rapidly develops renal dysfunction, associated with hypertension. She required renal replacement therapy initiation and we observed a decline in hemoglobin and platelets numbers. We confirm a microangiopathic hemolytic anemia and rule out other immune diseases or thrombotic thrombocytopenic purpura. Systemic Sclerosis is a chronic immune disorder of unknown origin that it is not completely understood. It is believed that environmental factors may contribute and also altered genes may be implicated in the immune system’s function. Microangiopathic hemolytic anemia occurs in 43% of patients who develop scleroderma renal crisis and an activation of the complement system through the classical pathway may be involved. Given that context we decided to treat the patients with C5 blocker Eculizumab and obtain an extraordinary positive response.