[Lung disease in adult common variable immunodeficiency].

Rev Mal Respir. 2015 Jun 9;

Authors: Hadjadj J, Malphettes M, Fieschi C, Oksenhendler E, Tazi A, Bergeron A

Abstract
INTRODUCTION: Common variable immunodeficiency (CVID) is characterized by a defect in antibody production and may be complicated by infectious or non-infectious respiratory disease.
BACKGROUND: In addition to recurrent infectious complications, mainly due to encapsulated bacteria, CVID may be complicated by diffuse infiltrative, non-infectious lung disease. The latter may be related to granulomatosis, lymphoid interstitial pneumonia, follicular bronchiolitis, follicular nodular hyperplasia, organizing pneumonia or lymphoma. Different lymphoid histological lesions can co-exist and form a new entity called GLILD (granulomatous lymphocytic interstitial lung disease), which is associated with a poor prognosis. Replacement of immunoglobulins significantly decreases the frequency and severity of infections but has no impact on the non-infectious complications.
OUTLOOK: Studies are needed to determine the modalities of follow-up and better understand the long-term progress of GLILD. These studies should improve the management of GLILD in the context of immunosuppressive treatments, which increase the risk of infection in CVID.
CONCLUSION: The identification of GLILD, which reflects a variable histological spectrum, rather than a well-defined entity, necessitates revising the approach to diffuse infiltrative lung diseases in CVID.

PMID: 26071128 [PubMed - as supplied by publisher]