C3B INACTIVATOR DEFICIENCY WITH IMMUNE-COMPLEX MANIFESTATIONS

  • 1 January 1982
    • journal article
    • research article
    • Vol. 47 (1), 197-205
Abstract
A complete complement component C3b inactivator deficiency was reported in a 28 yr 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, circuating immune complexes and 1 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.