Scleroderma Renal Crisis With Thrombotic Microangiopathy Treated With Eculizumab

We herein report the unusual case of a 52-year-old female with systemic scleroderma who was admitted to the emergency department (ED) with renal dysfunction and hypertension. Following a decline in hemoglobin (Hb) and platelet (Plt) count, the diagnosis of scleroderma renal crisis (SRC) with associated microangiopathic hemolytic anemia was made. Renal replacement therapy using hemodialysis was required. Systemic scleroderma is a chronic autoimmune multisystem vasculopathy affecting several vessel beds, including distal extremities, kidneys, and lungs. Microangiopathic hemolytic anemia occurs in almost half of patients who develop scleroderma renal crisis. This association is thought to be related to the activation of the complement system via the classical pathway. Based on that, we administered a C5 blocker (eculizumab) to our patient and reported an unprecedented positive outcome.