title: Association of FcRn expression with lung abnormalities and IVIG catabolism in patients with common variable immunodeficiency.
abstract: The neonatal Fc receptor (FcRn) acts as a key regulator of IgG homeostasis and is an important sensor of luminal infection. We analyzed the influence of FcRn expression on disease phenotype and the catabolism of therapeutically administered intravenous immunoglobulins (IVIG) in 28 patients with common variable immunodeficiency (CVID). Patients with generalized bronchiectasis and fibrosis had lower levels of FCRN mRNA compared to patients without these complications (P=0.027 and P=0.041, respectively). Moreover, FCRN mRNA levels correlated negatively with the extent of bronchiectasis and the rate of IgG decline after infusion of IVIG (P=0.027 and P=0.045, respectively). No relationship of FCRN expression with age at disease onset, age at diagnosis, diagnostic delay, IgG levels or frequency of infections before or during replacement immunoglobulin treatment, the presence of lung functional abnormalities, chronic diarrhea, granulomas, lymphadenopathy, splenomegaly or autoimmune phenomena was observed. Our results showed that FcRn might play a role in the development of lung structural abnormalities and in the catabolism of IVIG in patients with CVID.
authors.affiliation: Molecular Genetics Laboratory, Centre for Cardiovascular Surgery and Transplantation, Brno, Czech Republic. tomas.freiberger@cktch.cz
authors.forename: T
authors.initials: T
authors.lastname: Freiberger
authors.forename: L
authors.initials: L
authors.lastname: Grodecká
authors.forename: B
authors.initials: B
authors.lastname: Ravcuková
authors.forename: B
authors.initials: B
authors.lastname: Kurecová
authors.forename: V
authors.initials: V
authors.lastname: Postránecká
authors.forename: J
authors.initials: J
authors.lastname: Vlcek
authors.forename: J
authors.initials: J
authors.lastname: Jarkovský
authors.forename: V
authors.initials: V
authors.lastname: Thon
authors.forename: J
authors.initials: J
authors.lastname: Litzman
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D017074:Common Variable Immunodeficiency / Q000235:genetics / Q000276:immunology* / Q000473:pathology / Q000503:physiopathology; D005260:Female; D015870:Gene Expression; D015395:Histocompatibility Antigens Class I / Q000235:genetics*; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D016756:Immunoglobulins, Intravenous / Q000097:blood*; D008168:Lung / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D018598:Minisatellite Repeats; D010641:Phenotype; D011401:Promoter Regions, Genetic; D012333:RNA, Messenger / Q000235:genetics / Q000378:metabolism; D011961:Receptors, Fc / Q000235:genetics*; D055815:Young Adult
chemical_list: D015395:Histocompatibility Antigens Class I; D007074:Immunoglobulin G; D016756:Immunoglobulins, Intravenous; D012333:RNA, Messenger; D011961:Receptors, Fc; C100688:Fc receptor, neonatal