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Clin Infect Dis. 2015 Jul 15;61(2):e13-9. doi: 10.1093/cid/civ269. Epub 2015 Mar 31.

Good syndrome: an adult-onset immunodeficiency remarkable for its high incidence of invasive infections and autoimmune complications.

Author information

1
Département d'immunologie, Assistance Publique Hôpitaux de Paris (AP-HP) INSERM U1126, Centre Hayem, Hôpital Saint-Louis.
2
Département d'immunologie, Assistance Publique Hôpitaux de Paris (AP-HP) EA3518, Université Paris Diderot, Paris.
3
Médecine Interne, CHU Hôpital Sud, Rennes.
4
Médecine Interne, CHU de Nantes.
5
Service de Médecine Interne, Hôpital de la Conception, Marseille.
6
Service de Médecine Interne, CHU Clermont-Ferrand.
7
Médecine Interne et Maladies Infectieuses, Hôpital Haut-Lévêque, Pessac.
8
Médecine Interne et Immunologie Clinique, CHU Bocage, Dijon.
9
Service de Médecine Interne, CHU Purpan, Toulouse.
10
Service d'hématologie Clinique, Perpignan.
11
Service de Pneumologie A, Hôpital Bichat.
12
Service de Médecine Interne, Hôpital Tenon, AP-HP, Paris, France.

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.

KEYWORDS:

CVID; Good syndrome; autoimmunity; infection; thymoma

PMID:
25828999
DOI:
10.1093/cid/civ269
[Indexed for MEDLINE]

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