PULMONARY PATHOLOGIES AND MANAGEMENT STRATEGIES IN PRIMARY IMMUNODEFICIENCIES

pulmonary_pathologies_kapak

PULMONARY PROPHYLACTIC TREATMENTIN PATIENTS WITH PRIMARY IMMUNODEFICIENCY

Emrah Harman1
Şevket Arslan2

1Konya Necmettin Erbakan University, Faculty of Medicine, Department of Immunology and Allergic Diseases, Konya, Türkiye
2Konya Necmettin Erbakan University, Faculty of Medicine, Department of Immunology and Allergic Diseases, Konya, Türkiye

Harman E, Arslan Ş. Pulmonary Prophylactic Treatment in Patients with Primary Immunodeficiency. In: Arslan Ş editor. Pulmonary Pathologies and Management Strategies in Primary Immunodeficiencies. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.117-127.

ABSTRACT

In patients with primary immunodeficiency (PID), despite adequate immunoglobulin replacement, various respiratory complications may develop, often due to underlying infections and inflammation. These complications include acute infections, infectious sequelae such as bronchiectasis, non-infectious immune-mediated manifestations such as granulomatous-lymphocytic interstitial lung disease, and malignancies such as lymphoma. Respiratory tract complications in PID are of considerable importance due to their negative impact on mortality and morbidity. Therefore, regular assessment of the structural and functional status of the lungs and upper respiratory tract is essential to minimize loss of function as well as treatment.This text will provide information on the key pulmonary findings in primary immunodeficiencies, imaging findings, other diagnostic methods, and treatment approaches.

Keywords: Primary immunodeficiency (PID); Pulmonary complications; Pulmonary protection

Referanslar

  1. Resnick ES, Moshier EL, Godbold JH, Cunningham-Rundles C. Morbidity and mortality in common variable immune deficiency over 4 decades. Blood. 2012;119(7):1650-1657. [Crossref]  [PubMed]  [PMC]
  2. Martinez Garcia MA, et al. Respiratory disorders in common variable immunodeficiency. Respir Med. 2001;95(3):191-195. [Crossref]  [PubMed]
  3. Freeman AF, Holland SM. Antimicrobial prophylaxis for primary immunodeficiencies. Curr Opin Allergy Clin Immunol. 2009;9(6):525-530. [Crossref]  [PubMed]
  4. Mason RJ, Broaddus VC, Martin TR. In: Murray and Nadel's Textbook of Respiratory Medicine. 2010.
  5. Reynolds JH, McDonald G, Alton H, Gordon SB. Pneumonia in the immunocompetent patient. Br J Radiol. 2010;83(996):998-1009. [Crossref]  [PubMed]  [PMC]
  6. Debnath SK, Debnath M, Srivastava R. Opportunistic etiological agents causing lung infections: emerging need to transform lung-targeted delivery. Heliyon. 2022;8(12):e12620. [Crossref]  [PubMed]  [PMC]
  7. Lee WI, et al. Clinical features and lymphocyte immunophenotyping analysis in primary immunodeficiency patients with non-transplant lymphoproliferative disorders. Clin Immunol. 2024;265:110269. [Crossref]  [PubMed]
  8. Stafler P, Carr SB. Non-cystic fibrosis bronchiectasis: its diagnosis and management. Arch Dis Child Educ Pract Ed. 2010;95(3):73-82. [Crossref]  [PubMed]
  9. Deerojanawong J, Chang AB, Eng PA, Robertson CF, Kemp AS. Pulmonary diseases in children with severe combined immune deficiency and DiGeorge syndrome. Pediatr Pulmonol. 1997;24(5):324-330. [Crossref]
  10. Pipavath SJ, Lynch DA, Cool C, Brown KK, Newell JD. Radiologic and pathologic features of bronchiolitis. AJR Am J Roentgenol. 2005;185(2):354-363. [Crossref]  [PubMed]
  11. Vidarsson G, Dekkers G, Rispens T. IgG subclasses and allotypes: from structure to effector functions. Front Immunol. 2014;5:520. [Crossref]  [PubMed]  [PMC]
  12. Gathmann B, et al. Clinical picture and treatment of 2212 patients with common variable immunodeficiency. J Allergy Clin Immunol. 2014;134(1):116-126. [Crossref]  [PubMed]
  13. Moctezuma SI, Panizo CM, Landecho MF. Common variable immunodeficiency-associated granulomatous and lymphocytic interstitial lung disease successfully treated with a combination regimen of rituximab and azathioprine. Med Clin (Barc). 2017;149(7):312-313. [Crossref]  [PubMed]
  14. Mukhopadhyay S, Gal AA. Granulomatous lung disease: an approach to the differential diagnosis. Arch Pathol Lab Med. 2010;134(5):667-690. [Crossref]  [PubMed]
  15. Lopez-Herrera G, et al. Deleterious mutations in LRBA are associated with a syndrome of immune deficiency and autoimmunity. Am J Hum Genet. 2012;90(6):986-1001. [Crossref]  [PubMed]  [PMC]
  16. Marciano BE, et al. Pulmonary manifestations of GATA2 deficiency. Chest. 2021;160(4):1350-1359. [Crossref]  [PubMed]  [PMC]
  17. Suzuki T, Trapnell BC. Pulmonary alveolar proteinosis syndrome. Clin Chest Med. 2016;37(3):431-440. [Crossref]  [PubMed]  [PMC]
  18. Rich AL, Le Jeune IR, McDermott L, Kinnear WJ. Serial lung function tests in primary immune deficiency. Clin Exp Immunol. 2008;151(1):110-113. [Crossref]  [PubMed]  [PMC]
  19. Maffucci P, et al. Genetic diagnosis using whole exome sequencing in common variable immunodeficiency. Front Immunol. 2016;7:220. [Crossref]  [PubMed]  [PMC]
  20. van de Ven AA, et al. Airway and interstitial lung disease are distinct entities in paediatric common variable immunodeficiency. Clin Exp Immunol. 2011;165(2):235-242. [Crossref]  [PubMed]  [PMC]
  21. Verbsky JW, et al. Rituximab and antimetabolite treatment of granulomatous and lymphocytic interstitial lung disease in common variable immunodeficiency. J Allergy Clin Immunol. 2021;147(2):704-712.e17. [Crossref]  [PubMed]
  22. Quinti I, et al. Long-term follow-up and outcome of a large cohort of patients with common variable immunodeficiency. J Clin Immunol. 2007;27(3):308-316. [Crossref]  [PubMed]
  23. Gregersen S, et al. Lung disease, T-cells and inflammation in common variable immunodeficiency disorders. Scand J Clin Lab Invest. 2013;73(6):514-522. [Crossref]  [PubMed]
  24. Sigurdsson MI, Isaksson HJ, Gudmundsson G, Gudbjartsson T. Diagnostic surgical lung biopsies for suspected interstitial lung diseases: a retrospective study. Ann Thorac Surg. 2009;88(1):227-232. [Crossref]  [PubMed]
  25. Engum SA. Minimal access thoracic surgery in the pediatric population. Semin Pediatr Surg. 2007;16(1):14-26. [Crossref]  [PubMed]
  26. Busse PJ, Razvi S, Cunningham-Rundles C. Efficacy of intravenous immunoglobulin in the prevention of pneumonia in patients with common variable immunodeficiency. J Allergy Clin Immunol. 2002;109(6):1001-1004. [Crossref]  [PubMed]
  27. de Gracia J, et al. Immunoglobulin therapy to control lung damage in patients with common variable immunodeficiency. Int Immunopharmacol. 2004;4(6):745-753. [Crossref]  [PubMed]
  28. Baumann U, Göcke K, Gewecke B, Freihorst J, von Specht BU. Assessment of pulmonary antibodies with induced sputum and bronchoalveolar lavage induced by nasal vaccination against Pseudomonas aeruginosa: a clinical phase I/II study. Respir Res. 2007;8(1):57. [Crossref]  [PubMed]  [PMC]
  29. Twigg HL 3rd. Humoral immune defense (antibodies): recent advances. Proc Am Thorac Soc. 2005;2(5):417-421. [Crossref]  [PubMed]
  30. Seidel MG, et al. The European Society for Immunodeficiencies (ESID) Registry Working Definitions for the Clinical Diagnosis of Inborn Errors of Immunity. J Allergy Clin Immunol Pract. 2019;7(6):1763-1770. [Crossref]  [PubMed]
  31. Quinti I, et al. Effectiveness of immunoglobulin replacement therapy on clinical outcome in patients with primary antibody deficiencies: results from a multicenter prospective cohort study. J Clin Immunol. 2011;31(3):315-322. [Crossref]  [PubMed]
  32. Hodkinson JP. Considerations for dosing immunoglobulin in obese patients. Clin Exp Immunol. 2017;188(3):353-362. [Crossref]  [PubMed]  [PMC]
  33. Plebani A, et al. Clinical, immunological, and molecular analysis in a large cohort of patients with X-linked agammaglobulinemia: an Italian multicenter study. Clin Immunol. 2002;104(3):221-230. [Crossref]  [PubMed]
  34. Rojavin MA, Hubsch A, Lawo JP. Quantitative evidence of wear-off effect at the end of the intravenous IgG (IVIG) dosing cycle in primary immunodeficiency. J Clin Immunol. 2016;36(3):210-219. [Crossref]  [PubMed]  [PMC]
  35. Segal BH, et al. Posaconazole as salvage therapy in patients with chronic granulomatous disease and invasive filamentous fungal infection. Clin Infect Dis. 2005;40(11):16841688. [Crossref]  [PubMed]
  36. Yong PL, et al. Use of intravenous immunoglobulin and adjunctive therapies in the treatment of primary immunodeficiencies: a working group report of and study by the Primary Immunodeficiency Committee of the American Academy of Allergy Asthma and Immunology. Clin Immunol. 2010;135(2):255-263. [Crossref]  [PubMed]
  37. Milito C, et al. Double-blind, placebo-controlled, randomized trial on low-dose azithromycin prophylaxis in patients with primary antibody deficiencies. J Allergy Clin Immunol. 2019;144(2):584-593.e7. [Crossref]  [PubMed]
  38. Stern A, Green H, Paul M, Vidal L, Leibovici L. Prophylaxis for Pneumocystis pneumonia (PCP) in non-HIV immunocompromised patients. Cochrane Database Syst Rev. 2014;2014(10):Cd005590. [Crossref]  [PubMed]  [PMC]
  39. Sobh A, Bonilla FA. Vaccination in primary immunodeficiency disorders. J Allergy Clin Immunol Pract. 2016;4(6):10661075. [Crossref]  [PubMed]
  40. Suresh S, et al. Physician vaccination practices in mild to moderate inborn errors of immunity and retrospective review of vaccine completeness in IEI: results from the Canadian Immunization Research Network. Allergy Asthma Clin Immunol. 2022;18(1):32. [Crossref]  [PubMed]  [PMC]
  41. Bonilla FA, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. Ann Allergy Asthma Immunol. 2005;94(5 Suppl 1):S1-S63. [Crossref]  [PubMed]
  42. Ansari R, et al. Primary and acquired immunodeficiencies associated with severe varicella-zoster virus infections. Clin Infect Dis. 2021;73(9):e2705-e2712. [Crossref]  [PubMed]  [PMC]