title: Hyper-IgM syndrome associated with rheumatoid arthritis: report of RA in a patient with primary impaired CD40 pathway.
abstract: We studied a patient who had a typical seronegative rheumatoid arthritis (RA) and an immunodeficiency with hyper-IgM (HIM syndrome). CD40L was normally expressed by activated T cells, but CD40-mediated signal transduction was defective in B cells, preventing heavy chain switching (CD40L+ type of the HIM syndrome). These data suggest that a typical RA can develop in at least some patients with dysfunction of the CD40 pathway, i.e. in the absence of a normal co-operation between T and B cells. Accordingly, the blockade of CD40L-CD40 interactions, which has been proposed as a treatment of RA, might not be adapted to all patients.
authors.affiliation: Department of Rheumatology, Hôpital de Hautepierre, Strasbourg, France.
authors.forename: J
authors.initials: J
authors.lastname: Sibilia
authors.forename: A
authors.initials: A
authors.lastname: Durandy
authors.forename: T
authors.initials: T
authors.lastname: Schaeverbeke
authors.forename: J P
authors.initials: JP
authors.lastname: Fermand
mesh_terms: D000328:Adult; D001172:Arthritis, Rheumatoid / Q000150:complications* / Q000276:immunology; D019013:CD40 Antigens / Q000276:immunology*; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications*; D007075:Immunoglobulin M; D008297:Male
chemical_list: D019013:CD40 Antigens; D007075:Immunoglobulin M