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Patients have higher susceptibility to bacterial infections; especially to meningococcal infections. More than half of patients with properdin deficiency have had meningococcal disease. Patients with properdin deficiency have an intact classical pathway and normal levels of serum C3 and other components of the alternative pathway are present in normal levels. SLE (systemic lupus erythematous) and discoid lupus are possible.
Diagnosis
Diagnostic recommendations
Additional Information
Diagnostic laboratories
Clinical:
Genetic:
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