Primary immunodeficiency diseases in lung disease: warning signs, diagnosis and management
- PMID: 30419907
- PMCID: PMC6233514
- DOI: 10.1186/s12931-018-0923-8
Primary immunodeficiency diseases in lung disease: warning signs, diagnosis and management
Abstract
Background: Pulmonary complications are common in primary immunodeficiency diseases (PID) and contribute to morbidity and mortality in these patients. However, their varied presentation and a general lack of awareness of PID in this setting make early diagnosis and treatment difficult. The aim of this study was to define the warning signs of PID in patients with respiratory manifestations, the necessary diagnostic tests, and the therapeutic management of both children and adults.
Methods: A review of the literature was performed, and 43 PID interdisciplinary specialists were consulted.
Results: This document identifies the pulmonary and extrapulmonary manifestations that should prompt a suspicion of PID, the immunological and respiratory tests that should be included in the diagnostic process according to the level of care, recommendations regarding the use of immunoglobulin replacement therapy according to the specific immunodeficiency, and the minimum recommended immunological and pulmonary monitoring in these patients.
Conclusions: This document is the first to combine scientific evidence with the opinion of a broad panel of experts specializing in the treatment of patients with immunodeficiencies. It aims to provide a useful tool for all practitioners who are regularly involved in the management of these patients.
Keywords: “Antibodies/deficiency”[mesh]; “Immunoglobulins/administration and dosage”[mesh]; “Immunoglobulins/deficiency”[mesh]; “Immunologic Deficiency Syndromes”[mesh]; “Respiratory Tract Infections”[mesh].
Conflict of interest statement
Ethics approval and consent to participate
Not applicable.
Consent for publication
Not applicable.
Competing interests
PSP reports receiving personal fees from CSL Behring and grants from the Jeffrey Modell Foundation during the conduct of the study; and personal fees from Shire SL, and from Grifols, outside the context of the submitted work.
JG reports receiving personal fees from CLS Berhing, from the Fundación Victor Griffols, and from Shire SL, outside the context of the submitted work.
LIG reports receiving grants from Fondo de Investigación Sanitaria Carlos III and from Shire SL, and grants and personal fees from CSL Behring, during the conduct of the study.
CM reports receiving personal fees from CSL Behring, outside the context of the submitted work.
CR reports receiving personal fees from CSL Behring, during the conduct of the study.
SSR has served as speaker, consultant and advisory member for or has received research funding from CSL Behring, Grifols, Shire and Octapharma.
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