Published in:
01-01-2013 | Original Research
Granulomatous Disease in CVID: Retrospective Analysis of Clinical Characteristics and Treatment Efficacy in a Cohort of 59 Patients
Authors:
Jean-Nicolas Boursiquot, Laurence Gérard, Marion Malphettes, Claire Fieschi, Lionel Galicier, David Boutboul, Raphael Borie, Jean-François Viallard, Pauline Soulas-Sprauel, Alice Berezne, Arnaud Jaccard, Eric Hachulla, Julien Haroche, Nicolas Schleinitz, Laurent Têtu, Eric Oksenhendler, the DEFI study group
Published in:
Journal of Clinical Immunology
|
Issue 1/2013
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Abstract
Background
Granulomatous disease (GD) will develop in a subset of patients with common variable immunodeficiency (CVID). Little is known about the efficacy of therapeutic agents used for treating this disorder.
Objective
To evaluate the efficacy of immunosuppressive drugs with the help of a set of clinical, biological and radiological criteria.
Method
Clinical and laboratory features of CVID patients were collected from the French DEFI cohort, a prospective study on adults with hypogammaglobulinemia. The medical charts of 55 patients (93 %) of the GD cohort were reviewed.
Results
Among 436 subjects with CVID, 59 patients (13.5 %) were diagnosed with GD. Of the 55 patients in whom medical charts were available, 32 patients received treatment for the granulomatous disease. Corticosteroids were the most frequently used drug. Complete response to treatment was infrequent. It was achieved with corticosteroids, cyclophosphamide, hydroxychloroquine, rituximab and methotrexate. Azathioprine, cyclosporine, mycophenolate mofetil, sirolimus, infliximab and thalidomide led to partial or absence of response. Complete and partial responses were observed in lymph nodes, lungs, liver, skin, bone marrow and central nervous system. Absent of response for gastrointestinal tract granulomas was noted in all cases of treatment attempt.
Conclusion
CVID patients with GD exhibit a particular biological phenotype. Treatment should be considered in any symptomatic patient or if there is evidence of organ dysfunction. Corticosteroids are the drug of choice in most instances but response to treatment is often unsatisfactory.