title: [Common variable immunodeficiency with autoimmune manifestations: study of nine cases; interest of a peripheral B-cell compartment analysis in seven patients].
abstract: OBJECTIVE
  Autoimmune manifestations (AIM) are associated to common variable immunodeficiency (CVI) in about 20 to 25% of the cases. This study presents the clinical, biological characteristics and the evolution of nine patients developing CVI and AIM. A peripheral B-cell compartment analysis has been performed in seven cases.
  
  
  METHODS
  This multicenter retrospective study analyses nine patients, six men and three women, within a population of 32 CVI.
  
  
  RESULTS
  The mean age was 27 years at the time of diagnosis of AIM and 30 years at the time of diagnosis of CVI. The diagnosis of AIM preceded the diagnosis of CVI in five cases. Thirteen AIM of different types were observed: autoimmune hemolytic anemia (AHA, 3), immune thrombocytopenic purpura (ITP, 2), Evan's syndrome (2), primary biliary cirrhosis (1), rheumatoid arthritis (1), alopecia totalis (1), myasthenia gravis (1). The peripheral B-cell compartment was investigated in seven patients: five patients with autoimmune cytopenia presented with a diminution of memory B cells (CD27+IgD-) and immature B cells (CD21-) levels; the patient with primary biliary cirrhosis and myasthenia gravis had only a diminution of memory B cells level; the last patient with ITP presented with a normal level of memory B cells. Five among the seven patients with autoimmune cytopenia required a specific treatment using corticosteroids, high dosages of intravenous immunoglobulin, then splenectomy after failure of the medical management, with severe infectious complications in one case.
  
  
  CONCLUSIONS
  The association of AIM and CVI is not fortuitous. The most common AIM is autoimmune cytopenia. The peripheral B-cell compartment analyses show that a majority of patients have a defect in memory B-cells. Treatment regimens are not standardized and splenectomy increases the risk of infectious complications.
authors.affiliation: Service de médecine interne, hôpital d'instruction des armées Desgenettes, 108, boulevard Pinel, 69003 Lyon, France. m.pavic@chello.fr
authors.forename: M
authors.initials: M
authors.lastname: Pavic
authors.forename: P
authors.initials: P
authors.lastname: Sève
authors.forename: C
authors.initials: C
authors.lastname: Malcus
authors.forename: F
authors.initials: F
authors.lastname: Sarrot-Reynault
authors.forename: D
authors.initials: D
authors.lastname: Peyramond
authors.forename: P
authors.initials: P
authors.lastname: Debourdeau
authors.forename: D
authors.initials: D
authors.lastname: Andriamanantena
authors.forename: D
authors.initials: D
authors.lastname: Bouhour
authors.forename: N
authors.initials: N
authors.lastname: Philippe
authors.forename: H
authors.initials: H
authors.lastname: Rousset
authors.forename: C
authors.initials: C
authors.lastname: Broussolle
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000367:Age Factors; D000505:Alopecia / Q000150:complications / Q000276:immunology; D000743:Anemia, Hemolytic / Q000150:complications / Q000276:immunology; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications / Q000276:immunology; D001172:Arthritis, Rheumatoid / Q000150:complications / Q000276:immunology; D001327:Autoimmune Diseases / Q000150:complications* / Q000209:etiology / Q000276:immunology; D001402:B-Lymphocytes / Q000276:immunology; D017074:Common Variable Immunodeficiency / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D005260:Female; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications / Q000276:immunology; D007075:Immunoglobulin M; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage / Q000627:therapeutic use; D016130:Immunophenotyping; D007223:Infant; D008105:Liver Cirrhosis, Biliary / Q000150:complications / Q000276:immunology; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D015337:Multicenter Studies as Topic; D009157:Myasthenia Gravis / Q000150:complications / Q000276:immunology; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000150:complications / Q000276:immunology; D012189:Retrospective Studies; D013156:Splenectomy; D013577:Syndrome; D013921:Thrombocytopenia / Q000150:complications / Q000276:immunology
chemical_list: D000305:Adrenal Cortex Hormones; D007075:Immunoglobulin M; D016756:Immunoglobulins, Intravenous