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Journal of Clinical Immunology

, Volume 33, Issue 1, pp 84–95 | Cite as

Granulomatous Disease in CVID: Retrospective Analysis of Clinical Characteristics and Treatment Efficacy in a Cohort of 59 Patients

  • 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
Original Research

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.

Keywords

CVID granulomatous disease antibody deficiency primary immune deficiency treatment 

Abbreviations

CVID

Common variable immunodeficiency

GD

Granulomatous disease

TR

Therapeutic regimen

Notes

Disclosure

The authors declare no conflict of interest.

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Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  • Jean-Nicolas Boursiquot
    • 1
  • Laurence Gérard
    • 2
  • Marion Malphettes
    • 2
  • Claire Fieschi
    • 2
  • Lionel Galicier
    • 2
  • David Boutboul
    • 2
  • Raphael Borie
    • 3
  • Jean-François Viallard
    • 4
  • Pauline Soulas-Sprauel
    • 5
  • Alice Berezne
    • 6
  • Arnaud Jaccard
    • 7
  • Eric Hachulla
    • 8
  • Julien Haroche
    • 9
  • Nicolas Schleinitz
    • 10
  • Laurent Têtu
    • 11
  • Eric Oksenhendler
    • 2
  • the DEFI study group
  1. 1.Department of Clinical Immunology and Allergy, Centre Hospitalier Universitaire de Québec, Laval UniversityQuebec CityCanada
  2. 2.Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique- Hôpitaux de ParisParisFrance
  3. 3.Department of RespirologyHôpital TenonParisFrance
  4. 4.Department of Internal MedicineHôpital Haut LévêquePessacFrance
  5. 5.Department of Internal Medicine and INSERM UMR S737StrasbourgFrance
  6. 6.Department of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de ParisParisFrance
  7. 7.Department of HematologyHôpital DupuytrenLimogesFrance
  8. 8.Department of Internal MedicineHôpital Claude HuriezLilleFrance
  9. 9.Department of Internal Medicine, Hôpital de la Salpêtrière, Assistance Publique-Hôpitaux de ParisParisFrance
  10. 10.Department of Internal MedicineHôpital de la ConceptionMarseilleFrance
  11. 11.Service de PneumologieHôpital Rangueil-LarreyToulouseFrance

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