Please use this identifier to cite or link to this item: http://hdl.handle.net/10553/48608
Title: Primary immunodeficiency diseases in lung disease: warning signs, diagnosis and management
Authors: Soler-Palacín, Pere
de Gracia, Javier
González-Granado, Luis Ignacio
Martín, Carlos
Rodríguez-Gallego, Carlos 
Sánchez-Ramón, Silvia
Group, Lung Id Signal
UNESCO Clasification: 32 Ciencias médicas
3205 Medicina interna
241117 Fisiología de la respiración
Keywords: Immunoglobulins
Antibodies
Immunoglobulins
Respiratory Tract Infections
Immunologic Deficiency Syndromes
Issue Date: 2018
Journal: Respiratory Research 
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.
URI: http://hdl.handle.net/10553/48608
ISSN: 1465-993X
DOI: 10.1186/s12931-018-0923-8
Source: Respiratory research [ISSN 1465-993X] ,v. 19 (Noviembre 2018)
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