title: Pulmonary Manifestations of Primary Immunodeficiency Disorders.
abstract: Pulmonary disease, ranging from infectious pneumonia, lung abscess, and empyema to structural lung diseases to malignancy, significantly increase morbidity and mortality in primary immune deficiency. Treatment with supplemental immunoglobulin (intravenous or subcutaneous) and antimicrobials is beneficial in reducing infections but are largely ineffective in preventing noninfectious complications, including interstitial lung disease, malignancy, and autoimmune disease. A low threshold for suspecting pulmonary complications is necessary for the early diagnosis of pulmonary involvement in primary immunodeficiency disorders, before irreversible damage is done, to improve patient outcomes.
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Oregon Health and Science University, 3181 Southwest Sam Jackson Park Road, UHN-67, Portland, OR 97239, USA. Electronic address: nonas@ohsu.edu.
authors.forename: Stephanie
authors.initials: S
authors.lastname: Nonas
mesh_terms: D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications* / Q000175:diagnosis*; D008171:Lung Diseases / Q000175:diagnosis* / Q000209:etiology*
keywords: Bronchiectasis; Common variable immune deficiency; Interstitial lung disease; Primary immunodeficiency disease; Pulmonary complication