Elsevier

Respiratory Medicine

Volume 95, Issue 3, March 2001, Pages 191-195
Respiratory Medicine

Regular Article
Respiratory disorders in common variable immunodeficiency

https://doi.org/10.1053/rmed.2000.1020Get rights and content
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Abstract

Common variable immunodeficiency (CVID) is a heterogeneous immunodeficiency syndrome characterized by hypogammaglobulinemia, recurrent bacterial infections, and various immunologic abnormalities. The clinical presentation is generally that of recurrent pyogenic sinopulmonary infections.

Our objectives were to study the prevalence of lung involvement and the response to intravenous immunoglobulin replacement therapy in 19 patients with CVID. Nineteen patients (12 men) with a mean age (SD) of 33¶1 (17¶1) years had a previous diagnosis of CVID and were treated with intravenous immunoglobulin replacement. All patients underwent complete pulmonary function tests and high-resolution computed tomography (HRCT) examination. Bronchiectasis was diagnosed in 11 (58%) patients and eight (42%) were multi-lobar bronchiectasis. Chronic airflow limitation (CAL) was present in 10 (53%) patients and a restrictive pattern was seen in one case. Eleven patients (58%) presented a decrease in single-breath carbon monoxide diffusing capacity of the lung (DLco). Before intravenous immunoglobulin replacement therapy (INIRT), 84% of patients had suffered from at least one episode of pneumonia. Episodes of lower respiratory tract infection decreased significantly from 0¶28 per patient and year before replacement therapy to 0¶16 per patient and year after treatment. The mean duration of replacement therapy was 7¶5 years.

In conclusion lung involvement was frequent in patients with CVID. Long-term administration of intravenous gammaglobulin resulted in a substantial reduction of pneumonic episodes.

Keywords

common variable immunodeficiency
bronchiectasis
chronic airflow limitation
pneumonia
immunoglobulin replacement therapy.

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Correspondence should be addressed to: Dr Miguel A. Martı́nez Garcı́a, Department of Pneumology, Hospital Universitario La Fe, Avda. Campanar 21, E-46009 Valencia, Spain. Fax: +34-96-3868789; E-mail: [email protected]