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Connective tissue diseases, including systemic sclerosis and idiopathic inflammatory myopathies (IIMs), are a very rare cause of thrombotic microangiopathies (TMAs). Whether dysregulation of the complement pathways underlies these secondary forms of TMA and may be targeted by complement blocking agents remains elusive.

Role of C5 inhibition in Idiopathic Inflammatory Myopathies and Scleroderma Renal Crisis–Induced Thrombotic Microangiopathies