Inherited Complement Deficiencies and Systemic Lupus Erythematosus: an Immunogenetic Puzzle
Authors: PETER F. KOHLER
Journal: Annals of Internal Medicine (1975)

Editorial Note1 March 1975Inherited Complement Deficiencies and Systemic Lupus Erythematosus: an Immunogenetic PuzzlePETER F. KOHLER, M.D.PETER F. KOHLER, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-82-3-420 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptComplement is the collective designation for a complex system of sequentially interacting serum proteins that determines in large part the consequences of antigen-antibody interactions. IgG or IgM combined with antigen starts activation of the 11 proteins or 9 components that make up the classical complement reaction pathway: trimolecular C1(q,r,s), C4, C2, C3, C5, C6, C7, C8, and C9. Inherited deficiencies, genetically autosomal recessive, have been described for all these proteins except C8 and C9. In addition, genetic deficiencies of proteins that control enzymes derived from activated components have also been documented: decreased C1 inhibitor resulting in hereditary angioedema, and decreased...References1. PICKERINGMICHAELHERDMAN RAR: The complement system in chronic glomerulonephritis: three newly associated aberrations. J Pediatr 78:30-43, 1971 CrossrefMedlineGoogle Scholar2. HAUPTMANNGROSSHANSHEID GEE: Lupus érythémateux aigu avec defecit complet de la fraction C4 du complément. Nouv Presse Med 3:881-882, 1974 MedlineGoogle Scholar3. KOHLERPERCYCAMPION PJW: Hereditary angioedema and "familial" lupus erythematosus in identical twin boys. Am J Med 56:406-411, 1974 CrossrefMedlineGoogle Scholar4. OSTERLANDEPINOZAPARKER CLL: Inherited C2 deficiency and systemic lupus erythematosus: studies on a family. Ann Intern Med 82:323-328, 1975 LinkGoogle Scholar5. DÉMANTCAPKOVÁHINZOVÁ PJE: The role of the histocompatibility-2-linked Ss-S1p region in the control of mouse complement. Proc Natl Acad Sci USA 70:863-864, 1973 CrossrefMedlineGoogle Scholar6. FUKUNKELBRUSMAN SHH: Evidence for linkage between HL-A histocompatibility genes and those involved in the synthesis of the second component of complement. J Exp Med 140:1108-1111, 1974 CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: Division of Immunology Department of Medicine University of Colorado Medical Center Denver, Colorado PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited ByA brief history of twinsThe Anti-Ro Antibody SystemOral manifestations of lupus erythematosusInherited disorders of complementDéficit héréditaire en inhibiteur de la C1 estérase Lupus et glomérulonéphriteRelationship of clinical findings in systemic lupus erythematosus to seroreactivityANA-negative SLELupus erythematosusDeficiency of c7 with systemic lupus erythematosus. solubilization of immune complexes in complement-deficient seraLack of Association between the HLA-A10 (A25), B18 and C2° Haplotype and Anaphylactoid Purpura (AP)Complement in the Pathogenesis of Rheumatic DiseaseThe effect of normalization of serum complement and anti-DNA antibody on the course of lupus nephritisHomozygous C2 deficiency with fulminant lupus erythematosus. severe nephritis via the alternative complement pathwayThe in Vivo Metabolism of the Third Component of Complement in Systemic Lupus ErythematosusInherited deficiency of the second component of complement (C2) with membranoproliferative glomerulonephritisRheumatology: An Annotated Bibliography of Recent Literature References to Journal Articles and Other PapersRHEUMATOLOGY COMMITTEE, MEDICAL-KNOWLEDGE SELF-ASSESSMENT PROGRAM IV, AMERICAN COLLEGE OF PHYSICIANSHereditary C2 deficiency. Association with skin lesions resembling the discoid lesion of systemic lupus erythematosusDeficiency of the Second Component of Complement (C2) with Chronic VasculitisPETER FRIEND, M.D., JOHN E. REPINE, M.D., YOUNGKI KIM, M.D., C. C. CLAWSON, M.D., ALFRED F. MICHAEL, M.D. 1 March 1975Volume 82, Issue 3Page: 420-421KeywordsAntigensComplex systemsEnzymesGeneticsHereditary angioedemaLupus erythematosusSerum proteins Issue Published: 1 March 1975 PDF DownloadLoading ...