Long-term follow-up and outcome of a large cohort of patients with common variable immunodeficiency

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...

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Bibliographic Details
Published in:Journal of Clinical Immunology 2007-05, Vol.27 (3), p.308-316
Main Authors: Quinti, Isabella, Soresina, Annarosa, Spadaro, Giuseppe, Martino, Silvana, Donnanno, Simona, Agostini, Carlo, Claudio, Pignata, Franco, Dammacco, Maria Pesce, Anna, Borghese, Federica, Guerra, Andrea, Rondelli, Roberto, Plebani, Alessandro
Format: Article
Language:English
Subjects:
Adolescent
Adult
Age
Age Distribution
Aged
Autoimmune Diseases - immunology
Autoimmune Diseases - pathology
Child
Child, Preschool
Chronic illnesses
Cohort Studies
Common variable immunodeficiency
Common Variable Immunodeficiency - epidemiology
Common Variable Immunodeficiency - immunology
Common Variable Immunodeficiency - pathology
Common Variable Immunodeficiency - therapy
Diagnosis
Diarrhea
Female
Follow-Up Studies
Granulomatosis
Humans
Immune system
Immunoglobulins
Immunoglobulins - immunology
Immunoglobulins - therapeutic use
Immunotherapy
Intravenous administration
Lung diseases
Male
Middle Aged
Morbidity
Neoplasms - complications
Neoplasms - diagnosis
Otitis
Patients
Pediatrics
Respiratory tract diseases
Sinusitis
Survival Rate
Time Factors
Treatment Outcome
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Summary: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.
ISSN:0271-9142
1573-2592
1365-2567
DOI:10.1007/s10875-007-9075-1