C4 binding protein α deficiency

Patients have angioedema, cutaneous vasculitis, and arthritis. Activation of the classical pathway increases C3 consumption with diminished inhibition of the C4b.2a enzyme. The increased production of C3a and C5a might have led to angioedema through increased vascular permeability.

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