[Granulomatous disease in common variable immunodeficiency].
OBJECTIVE
Common variable immunodeficiency (CVID), defined by defective production of immunoglobulins, is the most common primary immunodeficiency in adulthood requiring a medical follow-up. Repeated bacterial infections and/or autoimmune manifestations and/or benign lymphoproliferation (including follicular hyperplasia and/or granulomatous disease) are the hallmark of the disease. This review aims at describing recent advances in the understanding and treatment of granulomatous disease in CVID.


BACKGROUND
Clinical features of granulomatous disease in CVID can mimic sarcoidosis, remarkable by the low levels of circulating immunoglobulins. Granulomas may be found in several organs in a single patient, and the main features are pulmonary, lymphoid, cutaneous, hepatic or splenic. The features of CVID is remarkable by the high frequency of autoimmune diseases complicating the immunodeficiency. Some immunological abnormalities have been described in such patients, including lymphopenia, decreased T-cells proliferations to mitogens and antigens. Rare polymorphisms in the gene encoding TNFalpha (Tumor Necrosis Factor) have been identified in CVID patients with granulomatous disease.


CONCLUSIONS
The evolution of the disease is severe, particularly when the lung is involved. Treatment consists in immunoglobulins substitution, immunosuppressive agents (corticosteroids, cyclophosphamide) and anti-TNFalpha antibodies. These treatments are difficult to manage in such immunocompromised patients.
Département d'immunologie clinique, hôpital Saint-Louis, 1 avenue Claude-Vellefaux, Paris, France.
M
M
Malphettes
E
E
Oksenhendler
L
L
Galicier
C
C
Fieschi
D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D017074:Common Variable Immunodeficiency / Q000150:complications* / Q000188:drug therapy; D004359:Drug Therapy, Combination; D006099:Granuloma / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology* / Q000503:physiopathology; D006801:Humans; D007136:Immunoglobulins / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016896:Treatment Outcome
D000911:Antibodies, Monoclonal; D007136:Immunoglobulins; D007155:Immunologic Factors; D007166:Immunosuppressive Agents