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Pulmonary Manifestations in a Cohort of Patients With Inborn Errors of Immunity: An 8-Year Follow-Up Study Publisher Pubmed



Movahedi M1 ; Jamee M2, 3 ; Ghaffaripour H1 ; Noori F1 ; Ghaini M4 ; Eskandarzadeh S4 ; Enayat J1 ; Eslamian G4 ; Pourdowlat G5 ; Sharifinejad N6 ; Poorabdollah M1 ; Nadji SA1 ; Fallahi M1 ; Daneshmandi Z1 Show All Authors
Authors
  1. Movahedi M1
  2. Jamee M2, 3
  3. Ghaffaripour H1
  4. Noori F1
  5. Ghaini M4
  6. Eskandarzadeh S4
  7. Enayat J1
  8. Eslamian G4
  9. Pourdowlat G5
  10. Sharifinejad N6
  11. Poorabdollah M1
  12. Nadji SA1
  13. Fallahi M1
  14. Daneshmandi Z1
  15. Heshmatnia J5
  16. Eslaminejad A5
  17. Fakharian A5
  18. Vasheghani M5
  19. Moniri A7
  20. Mirenayat MS5
  21. Tabarsi P7
  22. Marjani M7
  23. Rezaei N8, 9, 10
  24. Seppanen MRJ11
  25. Mansouri D1, 7
  26. Mahdaviani SA1
  27. Velayati AA1
Show Affiliations
Authors Affiliations
  1. 1. Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran
  2. 2. Pediatric Infections Research Center, Research Institute for Children’s Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  3. 3. Pediatric Nephrology Research Center, Research Institute for Children’s Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  4. 4. Department of Allergy and Clinical Immunology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  5. 5. Chronic Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran, Iran
  6. 6. Student Research Committee, Alborz University of Medical Sciences, Karaj, Iran
  7. 7. Clinical Tuberculosis and Epidemiology Research Centre, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran
  8. 8. Research Center for Immunodeficiencies, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  9. 9. Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran
  10. 10. Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran
  11. 11. Rare Disease and Pediatric Research Centers, Hospital for Children and Adolescents and Adult Immunodeficiency Unit, Inflammation Center, University of Helsinki and HUS Helsinki University Hospital, Helsinki, Finland

Source: Allergologia et Immunopathologia Published:2022


Abstract

Background: Inborn errors of immunity (IEIs) are a group of congenital diseases caused by genetic defects in the development and function of the immune system. The involvement of the respiratory tract is one of the most common presentations in IEIs. Methods: Overall, 117 patients with diagnosed IEIs were followed-up within 8 years at the National Research Institute of Tuberculosis and Lung Diseases (NRITLD). Demographic, clinical, and laboratory data were collected in a questionnaire. Pulmonary function test (PFT), chest X-ray (CXR), and high-resolution computed tomography (HRCT) scans were obtained where applicable. Results: Our study population consisted of 48 (41%) patients with predominantly antibody deficiencies (PADs), 39 (32%) patients with congenital defects of phagocytes, 14 (11.9%) patients with combined immunodeficiency (CID), and 16 (14%) patients with Mendelian susceptibility to mycobacterial diseases (MSMD). Recurrent pneumonia was the most common manifestation, while productive cough appeared to be the most common symptom in almost all diseases. PFT showed an obstructive pattern in patients with PAD, a restrictive pattern in patients with CID, and a mixed pattern in patients with CGD. HRCT findings were consistent with bronchiectasis in most PAD patients, whereas consolidation and mediastinal lesions were more common in the other groups. Conclusions: Pulmonary manifestations vary among different groups of IEIs. The screening for lung complications should be performed regularly to reveal respiratory pathologies in early stages and follow-up on already existing abnormalities. © 2022. Codon Publications. Published by Codon Publications.
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