Mannan-binding lectin - associated serine protease 2 deficiency

Patients have frequent infections (pneumococcal pneumonia) and chronic inflammatory disease, including pulmonary fibrosis. Hypocomplementemia contributes also to an impaired immune defense.

Therapeutic options

  • Fresh frozen plasma is used for emergent replacement of complement components. Prophylactic antibiotics for the infections. Acute infections are treated with appropriate antibiotics and prevented also with vaccination (meningococcal, pneumococcal, and haemophilus).
  • Complement deficiency, eMedicine
  • Complement deficiency, eMedicine