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Journal of Clinical Immunology

, Volume 27, Issue 3, pp 308–316 | Cite as

Long-Term Follow-Up and Outcome of a Large Cohort of Patients with Common Variable Immunodeficiency

  • ISABELLA QUINTIEmail author
  • ANNAROSA SORESINA
  • GIUSEPPE SPADARO
  • SILVANA MARTINO
  • SIMONA DONNANNO
  • CARLO AGOSTINI
  • PIGNATA CLAUDIO
  • DAMMACCO FRANCO
  • ANNA MARIA PESCE
  • FEDERICA BORGHESE
  • ANDREA GUERRA
  • ROBERTO RONDELLI
  • ALESSANDRO PLEBANI
Original Paper

Common Variable Immunodeficiency belongs to the group of rare diseases encompassing antibody deficiency syndromes of highly variable clinical presentation and outcome. The multicenter prospective study on a cohort of 224 patients with Common Variable Immunodeficiency provides an updated view of the spectrum of illnesses which occurred at the clinical onset and over a long period of follow-up (mean time: 11 years) and information on the effects of long-term immunoglobulin treatment. The mean age at the time of diagnosis was 26.6 years. Seventy-five patients were younger than 14 years of age. The mean age at the onset of symptoms was 16.9 years. This implicates with a mean diagnostic delay of 8.9 years. Respiratory tract infections were the most prominent clinical problem observed at diagnosis and during the follow-up. Intravenous immunoglobulin administration induced a significant reduction in the incidence of acute infections, mainly acute pneumonia and acute otitis. However, a progressive increase in the prevalence of patients with chronic diseases, mainly sinusitis and lung disease, was observed in all age groups, including the pediatric population. The morbidity of Common Variable Immunodeficiency due to all associated clinical conditions increased over time despite an adequate replacement with intravenous immunoglobulins. Our data stressed the need to develop international guidelines for the prevention and therapy of chronic lung disease, chronic sinusitis, chronic diarrhoea, and chronic granulomatosis in patients with humoral immunodeficiencies.

KEYWORDS:

common Variable Immunodeficiency intravenous immunoglobulins chronic lung disease pneumonia mortality 

Abbreviation used

CVID

Common Variable Immunodeficiency

IVIG

intravenous immunoglobulins

CLD

chronic lung disease

XLA

X-linked Agammaglobulinemia

NHL

non-Hodgkin lymphoma.

Notes

Acknowledgments

We would like to thank the Italian Primary Immunodeficiency Patient's Association (AIP) for their help and Dr. Bodo Grimbacker for critical review of the manuscript.

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Copyright information

© Springer Science+Business Media, LLC 2007

Authors and Affiliations

  • ISABELLA QUINTI
    • 1
    Email author
  • ANNAROSA SORESINA
    • 2
  • GIUSEPPE SPADARO
    • 3
  • SILVANA MARTINO
    • 4
  • SIMONA DONNANNO
    • 1
  • CARLO AGOSTINI
    • 5
  • PIGNATA CLAUDIO
    • 6
  • DAMMACCO FRANCO
    • 7
  • ANNA MARIA PESCE
    • 1
  • FEDERICA BORGHESE
    • 1
  • ANDREA GUERRA
    • 1
  • ROBERTO RONDELLI
    • 8
  • ALESSANDRO PLEBANI
    • 2
  1. 1.Department of Clinical ImmunologyUniversity of Rome “La Sapienza” RomeRomeItaly
  2. 2.Department of PaediatricsUniversity of BresciaOwensboroItaly
  3. 3.Department of Allergy and Clinical ImmunologyUniversity of Naples “Federico II,” NaplesNaplesItaly
  4. 4.Department of PaediatricsUniversity of TurinTorinoItaly
  5. 5.Department of Internal MedicineUniversity of PaduaPaduaItaly
  6. 6.Department of PediatricsUniversity of Naples “Federico II” NaplesItaly
  7. 7.Department of Biomedical SciencesUniversity of BariBariItaly
  8. 8.Department of Onco-HaematologyUniversity of BolognaBolognaItaly

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