Clinical features, long-term follow-up and outcome of a large cohort of patients with Chronic Granulomatous Disease: An Italian multicenter study

Baldassarre Martire, Roberto Rondelli, Annarosa Soresina, Claudio Pignata, Teresa Broccoletti, Andrea Finocchi, Paolo Rossi, Marco Gattorno, Marco Rabusin, Chiara Azzari, Rosa M. Dellepiane, Maria C. Pietrogrande, Antonino Trizzino, Paolo Di Bartolomeo, Silvana Martino, Luigi Carpino, Fausto Cossu, Franco Locatelli, Rita Maccario, Paolo PieraniMaria C. Putti, Achille Stabile, Luigi D. Notarangelo, Alberto G. Ugazio, Alessandro Plebani, Domenico De Mattia

Research output: Contribution to journalArticle

Abstract

A retrospective clinical and immunological survey was conducted in 60 patients with Chronic Granulomatous Disease. A prospective controlled non-randomized study of the efficacy of long-term IFNγ treatment was carried out. The mean age at the time of diagnosis was 4.4 years; mean duration of follow-up was 10.4 years. Lung and skin infections were the most frequent manifestations both prior to diagnosis and during follow-up. Aspergillus species was the first cause of infection and of death in our cohort. The mortality rate was 13%. Long term prophylaxis with IFNγ did not significantly change the rate of total infection per patient-year compared to controls (p = 0.07). Our data provide clear evidence that protocols of continuing intensive surveillance and monitoring of compliance with anti-infective regimens may significantly improve the quality of life and long-term survival in patients with CGD. No evidence justifying long-term prophylaxis with IFNγ was obtained.

Original languageEnglish
Pages (from-to)155-164
Number of pages10
JournalClinical Immunology
Volume126
Issue number2
DOIs
Publication statusPublished - Feb 2008

Keywords

  • Chronic Granulomatous Disease (CGD)
  • Cotrimoxazole (CTX)
  • Follow up
  • Infections
  • Interferon gamma (IFNγ)
  • Itraconazole (ITRA)

ASJC Scopus subject areas

  • Immunology
  • Immunology and Allergy

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    Martire, B., Rondelli, R., Soresina, A., Pignata, C., Broccoletti, T., Finocchi, A., Rossi, P., Gattorno, M., Rabusin, M., Azzari, C., Dellepiane, R. M., Pietrogrande, M. C., Trizzino, A., Di Bartolomeo, P., Martino, S., Carpino, L., Cossu, F., Locatelli, F., Maccario, R., ... De Mattia, D. (2008). Clinical features, long-term follow-up and outcome of a large cohort of patients with Chronic Granulomatous Disease: An Italian multicenter study. Clinical Immunology, 126(2), 155-164. https://doi.org/10.1016/j.clim.2007.09.008