Inherited complement deficiency states: implications for immunity and immunological disease.
The study of complement deficiency states and their influence on immune function has generated new insights and still provides a challenge to continued investigation. The association of classical pathway deficiencies (C1, C4, C2 or C3) with immunological diseases such as SLE and glomerulonephritis has contributed to current knowledge concerning complement-dependent immune complex handling and elimination. Susceptibility to systemic infection with encapsulated bacteria is encountered in most forms of inherited complement deficiency. Recurrent neisserial infection is the only clinical manifestation clearly associated with defects of the membranolytic sequence C5-C9, while deficiency of properdin, a component of the alternative activation pathway, appears to predispose to nonrecurrent meningococcal disease. Inherited complement deficiency is rare, but the perspective is widened by the more common occurence of acquired defects in immunological diseases, and the apparent requirement for efficient complement recruitment in host defense. Another aspect is the possibility that complement deficiency might alleviate or prevent inflammatory symptoms. Notably, complement deficiency has not been reported in classical rheumatoid arthritis. Considerations of this kind would be refuted or modified by findings of complement deficiency in single patients.
Sjöholm
A G
AG
Department of Medical Microbiology, Lund University, Sweden.
D000818:Animals; D001424:Bacterial Infections / Q000276:immunology; D003141:Communicable Diseases / Q000235:genetics; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency / Q000235:genetics; D003180:Complement C3b Inactivator Proteins / Q000172:deficiency / Q000235:genetics; D011416:Complement Factor D / Q000172:deficiency; D017242:Complement Factor H; D003170:Complement Pathway, Alternative; D003165:Complement System Proteins / Q000172:deficiency* / Q000235:genetics / Q000502:physiology; D006801:Humans; D007249:Inflammation / Q000276:immunology; D011414:Properdin / Q000172:deficiency / Q000235:genetics
C493495:CFH protein, human; D003174:Complement C1 Inactivator Proteins; D003180:Complement C3b Inactivator Proteins; D011414:Properdin; D017242:Complement Factor H; D003165:Complement System Proteins; C493488:CFD protein, human; D011416:Complement Factor D