title: Good syndrome: an adult-onset immunodeficiency remarkable for its high incidence of invasive infections and autoimmune complications.
abstract: BACKGROUND
  Good syndrome (GS) is a rare condition in which thymoma is associated with hypogammaglobulinemia. It is characterized by increased susceptibility to bacterial, viral, and fungal infections, as well as autoimmunity. Most patients have no circulating B cells.
  
  
  METHODS
  The French DEFicit Immunitaire de l'adulte cohort provides detailed clinical and immunological descriptions of 690 adults with primary hypogammaglobulinemia. Comparisons between patients with GS, those with common variable immunodeficiency (CVID), and those with B(-) CVID (circulating B cells <1%) were performed.
  
  
  RESULTS
  Twenty-one patients had GS and 440 had CVID, including 39 B(-) CVID, with a median age at diagnosis of 60, 35, and 34 years, respectively. Invasive bacterial infections were observed in 90.5% of GS, 54% of CVID, and 72% of B(-) CVID patients. Eight patients with GS had opportunistic infections, despite normal peripheral CD4(+) T-cell numbers. Autoimmune complications were demonstrated in 76% of GS, 29% of CVID, and 26% of B(-) CVID patients. The spectrum of autoimmunity in GS was uncommon, consisting of oral lichen planus, graft-vs-host disease-like colitis, and pure red cell aplasia, different from the pattern observed in CVID patients. GS patients did not display lymphoid hyperplasia nor lymphoma, unlike those with CVID or B(-) CVID.
  
  
  CONCLUSIONS
  GS differs notably from CVID and B(-) CVID: very late onset, no familial cases, and absence of lymphoid hyperplasia. The key observation is the very high frequency of invasive bacterial infections in GS, an issue that physicians should be aware of.
authors.affiliation: Département d'immunologie, Assistance Publique Hôpitaux de Paris (AP-HP) INSERM U1126, Centre Hayem, Hôpital Saint-Louis.
authors.forename: Marion
authors.initials: M
authors.lastname: Malphettes
authors.affiliation: Département d'immunologie, Assistance Publique Hôpitaux de Paris (AP-HP) EA3518, Université Paris Diderot, Paris.
authors.forename: Laurence
authors.initials: L
authors.lastname: Gérard
authors.affiliation: Département d'immunologie, Assistance Publique Hôpitaux de Paris (AP-HP) EA3518, Université Paris Diderot, Paris.
authors.forename: Lionel
authors.initials: L
authors.lastname: Galicier
authors.affiliation: Département d'immunologie, Assistance Publique Hôpitaux de Paris (AP-HP) INSERM U1126, Centre Hayem, Hôpital Saint-Louis.
authors.forename: David
authors.initials: D
authors.lastname: Boutboul
authors.affiliation: Département d'immunologie, Assistance Publique Hôpitaux de Paris (AP-HP) INSERM U1126, Centre Hayem, Hôpital Saint-Louis.
authors.forename: Bouchra
authors.initials: B
authors.lastname: Asli
authors.affiliation: Département d'immunologie, Assistance Publique Hôpitaux de Paris (AP-HP) INSERM U1126, Centre Hayem, Hôpital Saint-Louis.
authors.forename: Raphael
authors.initials: R
authors.lastname: Szalat
authors.affiliation: Médecine Interne, CHU Hôpital Sud, Rennes.
authors.forename: Antoinette
authors.initials: A
authors.lastname: Perlat
authors.affiliation: Médecine Interne, CHU de Nantes.
authors.forename: Agathe
authors.initials: A
authors.lastname: Masseau
authors.affiliation: Service de Médecine Interne, Hôpital de la Conception, Marseille.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Schleinitz
authors.affiliation: Service de Médecine Interne, CHU Clermont-Ferrand.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Le Guenno
authors.affiliation: Médecine Interne et Maladies Infectieuses, Hôpital Haut-Lévêque, Pessac.
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Viallard
authors.affiliation: Médecine Interne et Immunologie Clinique, CHU Bocage, Dijon.
authors.forename: Bernard
authors.initials: B
authors.lastname: Bonnotte
authors.affiliation: Service de Médecine Interne, CHU Purpan, Toulouse.
authors.forename: Marie-Françoise
authors.initials: MF
authors.lastname: Thiercelin-Legrand
authors.affiliation: Service d'hématologie Clinique, Perpignan.
authors.forename: Laurence
authors.initials: L
authors.lastname: Sanhes
authors.affiliation: Service de Pneumologie A, Hôpital Bichat.
authors.forename: Raphael
authors.initials: R
authors.lastname: Borie
authors.affiliation: Service de Médecine Interne, Hôpital Tenon, AP-HP, Paris, France.
authors.forename: Sophie
authors.initials: S
authors.lastname: Georgin-Lavialle
authors.affiliation: Département d'immunologie, Assistance Publique Hôpitaux de Paris (AP-HP) INSERM U1126, Centre Hayem, Hôpital Saint-Louis.
authors.forename: Claire
authors.initials: C
authors.lastname: Fieschi
authors.affiliation: Département d'immunologie, Assistance Publique Hôpitaux de Paris (AP-HP) EA3518, Université Paris Diderot, Paris.
authors.forename: Eric
authors.initials: E
authors.lastname: Oksenhendler
mesh_terms: D000328:Adult; D000361:Agammaglobulinemia / Q000150:complications; D000367:Age Factors; D000368:Aged; D015551:Autoimmunity; D001402:B-Lymphocytes / Q000276:immunology; D001424:Bacterial Infections / Q000175:diagnosis / Q000209:etiology* / Q000276:immunology* / Q000382:microbiology; D003092:Colitis / Q000175:diagnosis / Q000209:etiology; D017074:Common Variable Immunodeficiency / Q000150:complications; D005260:Female; D006086:Graft vs Host Disease / Q000175:diagnosis; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications* / Q000276:immunology / Q000382:microbiology*; D015994:Incidence; D017676:Lichen Planus, Oral / Q000175:diagnosis / Q000209:etiology; D008297:Male; D008875:Middle Aged; D009894:Opportunistic Infections / Q000175:diagnosis / Q000209:etiology*; D012010:Red-Cell Aplasia, Pure / Q000175:diagnosis / Q000209:etiology; D013601:T-Lymphocytes / Q000276:immunology; D013945:Thymoma / Q000276:immunology
keywords: CVID; Good syndrome; autoimmunity; infection; thymoma