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Most C4 deficient patients have discoid or systemic lupus erythematous (SLE), with or without associated glomerulonephritis. The disease onset is earlier than for SLE in complement-sufficient patients, and cutaneous features, as Raynaud's phenomenon and vasculitic ulcers are common. Bacterial infections are also a common cause of morbidity and mortality.
Diagnosis
Diagnostic recommendations
Additional Information
Therapeutic options
- Fresh frozen plasma is used for emergent replacement of complements components. Supportive therapy is used for complement deficiencies. Prophylactic antibiotics for the infections.
- Complement deficiency, eMedicine
- Complement deficiency, eMedicine