C3b inactivator deficiency with immune complex manifestations.
We report a complete C3b inactivator deficiency in a 28-year-old patient referred for bronchiectasis and recurrent pneumonia. In addition to these recurrent infections, previously described with this deficiency, he also had several immune complex manifestations (rheumatoid factor, circulating immune complexes and one episode of serum sickness). The consequences of C3b inactivator deficiency on complement activation, chemotaxis and opsonization, clearance of immune complexes, and on red blood cell sensitization are discussed. The study of the parents showed an autosomal dominant transmission.
D000328:Adult; D000936:Antigen-Antibody Complex / Q000032:analysis*; D001987:Bronchiectasis / Q000276:immunology*; D002634:Chemotaxis, Leukocyte; D003180:Complement C3b Inactivator Proteins / Q000172:deficiency* / Q000235:genetics; D003165:Complement System Proteins / Q000032:analysis; D003298:Coombs Test; D006801:Humans; D007123:Immunoelectrophoresis, Two-Dimensional; D008297:Male; D009504:Neutrophils / Q000276:immunology; D010587:Phagocytosis; D011014:Pneumonia / Q000276:immunology*; D012008:Recurrence
D000936:Antigen-Antibody Complex; D003180:Complement C3b Inactivator Proteins; D003165:Complement System Proteins