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Pulmonary complications of primary immunodeficiencies

James Verbsky, MD, PhD
John M Routes, MD
Section Editor
Jordan S Orange, MD, PhD
Deputy Editors
Anna M Feldweg, MD
Helen Hollingsworth, MD


Pulmonary disease is common among patients with primary immunodeficiencies (PIDs) [1-4]. As therapy for these disorders improves and life expectancy of patients with PIDs increases, the prevalence of pulmonary complications is likely to increase in parallel. Consequently, knowledge of the detection and management of pulmonary disorders related to PIDs is critical for optimal management.

This topic will review the pulmonary findings that should raise suspicion of an underlying immune disorder, as well as the various pulmonary conditions that are seen in specific immunodeficiency disorders. Monitoring patients with PIDs for the development of pulmonary problems is also discussed. The evaluation of secondary immunodeficiency (eg, due to underlying diabetes, hemoglobinopathies, malignancy, human immunodeficiency virus [HIV] infection, drug-induced immunosuppression) and an overview of the medical management of patients with immunodeficiency are found separately. (See "Approach to the adult with recurrent infections", section on 'Respiratory tract infections' and "Secondary immunodeficiency due to underlying disease states, environmental exposures, and miscellaneous causes" and "Secondary immunodeficiency induced by biologic therapies" and "Primary immunodeficiency: Overview of management".)


The spectrum of pulmonary manifestations in PIDs is broad and includes acute and chronic infection, structural abnormalities (eg, bronchiectasis), malignancy, and dysregulated inflammation resulting in tissue damage (eg, granuloma, pulmonary fibrosis) [5-9]. Pulmonary disease may be the initial manifestation of a PID, and certain clinical and radiographic findings, such as specific types of pulmonary infections and structural lung abnormalities, should prompt an immunologic evaluation for an underlying PID, regardless of patient age. The laboratory tests used to evaluate the immune system are reviewed separately. (See "Laboratory evaluation of the immune system".)

Pulmonary infections — Infections that are recurrent, recalcitrant to usual therapy, or due to opportunistic or unusual pathogens are suggestive of a possible underlying PID.

Recurrent infections — In the general population, recurrent pneumonias (ie, >2/lifetime) are unusual. The association of pneumonia with recurrent sinus infections is also a clue that a PID may be present. Recurrent pneumonias in a specific lobe are suggestive of structural abnormalities, such as aberrant lung development, neoplasm, or the presence of a foreign body. Pneumonias confined to dependent lobes may indicate recurrent aspiration due to gastroesophageal reflux disease or swallowing abnormalities. In contrast, pneumonias in varying locations of the lung, particularly with interim clearing between episodes, are more indicative of underlying immune dysfunction [10-12]. The presence of unusual complications of pneumonia, such as pneumatoceles or cavitary lesions, is also concerning for immunodeficiency [13,14].

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Literature review current through: Sep 2017. | This topic last updated: Sep 11, 2017.
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