C4A deficiency

Most C4 deficient patients have discoid or systemic lupus erythematous, with or without associated glomerulonephritis. The disease onset is earlier than for systemic lupus erythematous (SLE) in complement-sufficient patients, and cutaneous features, as Raynaud's phenomenon and vasculitic ulcers are common. Bacterial infections are a common cause of morbidity and mortality. Systemic lupus erythematous (SLE) patients with C4A deficiency have a lower prevalence of anticardiolipin, anti-Ro, anti-dsDNA, and anti-Sm antibodies, less neurologic disease and renal disease, and more photosensitivity than other systemic lupus erythematous (SLE) patients.

Therapeutic options