title: [Granulomatosis and primary immunodeficiency in adulthood].
abstract: Primary immunodeficiency with granulomatosis in the adulthood mainly concern common variable immunodeficiency (CVID). Hypogammaglobulinemia in the adulthood is usually related to a secondary immunodeficiency. When a patient presents with the association of a hypogammaglobulinemia and a granulomatosis, an opportunistic infection must first be ruled out. For unknown reasons, about 10% of the patients affected by CVID also present with granulomatosis. Lesions usually affect the pulmonary tract or the mediastinum. Half of these patients are also affected by an autoimmune cytopenia. Treatment is not codified. Severe pulmonary complications can occur in about 50% of the patients.
authors.affiliation: Service de médecine interne et oncologie, HIA Desgenettes, Lyon, France. m.pavic@numericable.com
authors.forename: M
authors.initials: M
authors.lastname: Pavic
authors.forename: F
authors.initials: F
authors.lastname: Pasquet
authors.forename: C
authors.initials: C
authors.lastname: Fieschi
authors.forename: M
authors.initials: M
authors.lastname: Malphettes
authors.forename: P
authors.initials: P
authors.lastname: Sève
mesh_terms: D000328:Adult; D000361:Agammaglobulinemia / Q000188:drug therapy / Q000209:etiology*; D001327:Autoimmune Diseases / Q000150:complications / Q000188:drug therapy; D017074:Common Variable Immunodeficiency / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D005938:Glucocorticoids / Q000627:therapeutic use; D006099:Granuloma / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents