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Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders

Yazdani R1,2,3, Abolhassani H2,4, Asgardoon MH2, Shaghaghi M2,5, Modaresi M6, Azizi G7, Aghamohammadi A2

1Department of Immunology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran
2Research Center for Immunodeficiencies, Children’s Medical Center, Tehran University of Medical Sciences,
Tehran, Iran
3Molecular Immunology Interest Group (MIIG), Universal Scientific Education and Research Network (USERN),
Isfahan, Iran
4Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institutet at Karolinska
University Hospital Huddinge, Stockholm, Sweden
5Network of Immunology in Infections, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education
and Research Network (USERN), Tehran, Iran
6Department of Pediatric Pulmonary and Sleep Medicine, Children Medical Center, Teharn University of Medical
Sciences, Tehran, Iran
7Imam Hassan Mojtaba Hospital, Alborz University of Medical Sciences, Karaj, Iran

J Investig Allergol Clin Immunol 2017; Vol 27(4) : 213-224
doi: 10.18176/jiaci.0166

Primary immunodeficiency disorders (PIDs) are caused by 1 or more defects of the immune system. Patients are more likely to experience recurrent and/or severe infections and tend to develop a wide range of complications. Respiratory diseases are the main and initial manifestation in most cases and the most common complication. Pulmonary complications cause significant morbidity and mortality in patients with PIDs. Early diagnosis and appropriate treatment can prevent or at least slow the development of respiratory complications. Since the spectrum of pulmonary complications in PIDs is broad, we divided pulmonary complications into upper respiratory complications (eg, sinusitis, otitis media, and laryngeal angioedema) and lower respiratory complications (eg, pneumonia, bronchitis, bronchiectasis, interstitial lung diseases, organizing pneumonia, pulmonary adenopathies and malignancies, hyperreactive airway diseases, pulmonary dysgenesis, and adverse reactions to treatment). This review covers the main respiratory manifestations in patients with PIDs.

Key words: Primary immunodeficiency disorders, Pulmonary complications, Upper respiratory tract, Lower respiratory tract