National Consensus on Diagnosis and Management Guidelines for Primary Immunodeficiency

Document Type : Review

Authors

1 Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute at Karolinska University Hospital Huddinge, Stockholm, Sweden

2 Non-communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran

3 Pediatric Infections Research Center, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran

4 Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran

5 Department of Allergy and Clinical Immunology, Child Growth and Development Research Center, Research Institute for Primordial Prevention of Non-Communicable Disease, Isfahan University of Medical Sciences, Isfahan, Iran

6 Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran, and the University of Medical Science, Tehran, Iran

7 Non-Communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran

8 Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center, Tehran, University of Medical Sciences, Tehran, Iran

9 Hematology, Oncology and Stem Cell Transplantation Research Centre, Tehran University of Medical Sciences, Tehran, Iran

10 Department of Allergy and Clinical Immunology, Shahid Sadoughi University of Medical Sciences, Yazd, Iran

11 Department of Allergy and Clinical Immunology, Rasool e Akram Hospital, Iran University of Medical Sciences, Tehran, Iran

12 Department of Allergy and Clinical Immunology, Rasool-e-Akram Hospital, Iran University of Medical Sciences, Tehran, Iran.

13 Department of Pediatrics, Bo-Ali children's Hospital of Ardabil University of Medical Sciences, Ardabil, Iran

14 Acquired Immunodeficiency Research Center, lsfahan University of Medical Sciences, Isfahan, lran

15 Allergy and Clinical Immunology Department, Bushehr University of Medical Science, School of Medicine, Bushehr, Iran

16 Allergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran

17 Allergy Research Center, Mashhad University of Medical Sciences, Mashhad, Iran

18 Noncommunicable Pediatric Diseases Research Center, Amirkola Hospital, Babol University of Medical Sciences, Babol, Iran

19 Department of Pediatrics, Mazandaran University of Medical Sciences, Sari, Iran

20 Department of Immunology, Bahrami Hospital, Tehran University of Medical Sciences, Tehran, Iran

21 Department of Immunology and Allergy, Imam Khomeini Hospital, Tehran University of Medical Sciences, Tehran, Iran

22 Immunology and Allergy Department, Mofid Children's Hospital, Shahid Beheshti University of Medical Science, Tehran, Iran

23 Department of Pediatrics, School of Medicine, Arak University of Medical Sciences, Arak, Iran

24 Department of Pediatrics, Hamadan University of Medical Sciences, Hamadan, Iran

25 Department of Pediatrics, 17th Shahrivar Children's Hospital, Guilan University of Medical Sciences, Rasht, Iran

26 Department of Immunology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran

27 Cellular & Molecular Research Center, Kurdistan University of Medical Sciences, Sanandaj, Iran

28 Department of Pediatrics, Kermanshah University of Medical Sciences, Kermanshah, Iran

29 Department of Pediatrics, Qom University of Medical Sciences, Qom, Iran

30 Department of Pediatrics, Shahid Sadoughi University of Medical Sciences, Yazd, Iran

31 Department of Immunology and Allergy, Tabriz University of Medical Sciences, Tabriz, Iran

32 Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD)

33 Department of Immunology and Allergy, Ahvaz University of Medical Sciences, Ahvaz, Iran

34 Mousavi Hospital, Zanjan University of Meical Sciences, Zanjan, Iran

35 Department of Immunology and Allergy, Hormozgan University of Medical Sciences, Bandar Abbas, Iran

36 Department of Immunology and Allergy, Semnan University of Medical Sciences, Semnan, Iran

37 Department of Immunology and Allergy, Kashan University of Medical Sciences, Kashan, Iran

38 Department of Immunology and Allergy, Qazvin University of Medical Sciences, Qazvin, Iran

39 Department of Immunology and Allergy, Ilam University of Medical Sciences, Ilam, Iran

40 Department of Immunology and Allergy, Kerman University of Medical Sciences, Kerman, Iran

41 Department of Immunology and Allergy, Shahrekord University of Medical Sciences, Shahrekord , Iran

42 Department of Pediatrics, Lorestan University of Medical Sciences, Khorramabad, Iran

43 Department of Internal Medicine Shahid Sadoughi University of Medical Sciences, Yazd, Iran

44 Pediatric Infectious Diseases Research Center, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Science, Tehran, Iran

45 Iranian Primary Immunodeficiencies Network (IPIN), Tehran University of Medical Science, Tehran, Iran

Abstract

Primary immunodeficiency (PID) is a group of more than 400 distinct genetic disorders affecting both children and adults. As signs and symptoms of PID are usually heterogeneous and unspecific, based on the available human resources and laboratory facilities, diagnosis and follow-up of these patients can be challenging. In line with this notion, management and treatment of PID are practiced divergently in different PID centers. In order to reach a distinct national protocol and given the little evidence on appropriate or universal guidelines to improve the current status of management of the disease, the Iranian PID network designed a consensus suitable for regional resources. This review summarizes this PID guideline based on the importance of different clinical complications and the level of medical authority visiting those at the first line. Further, for each complication, appropriate interventions are mentioned to improve the approach.

Keywords


1. Picard C, Bobby Gaspar H, Al-Herz W, Bousfiha A, Casanova JL, Chatila T, et al. International Union of Immunological Societies: 2017 Primary Immunodeficiency Diseases Committee Report on Inborn Errors of Immunity. Journal of clinical immunology. 2018 Jan;38(1):96-128. PubMed PMID: 29226302. Pubmed Central PMCID: 5742601. 2. Aghamohammadi A, Moin M, Rezaei N. History of primary immunodeficiency diseases in iran. Iranian journal of pediatrics. 2010 Mar;20(1):16-34. PubMed PMID: 23056678. Pubmed Central PMCID: 3446008. 3. Abolhassani H, Rezaei N, Aghamohammadi A. Recent Advances and Current Status of Primary Immunodeficiency Disease in Iran. Immunology and Genetics Journal. 2018;1(1):1-33. 4. Richardson AM, Moyer AM, Hasadsri L, Abraham RS. Diagnostic Tools for Inborn Errors of Human Immunity (Primary Immunodeficiencies and Immune Dysregulatory Diseases). Current allergy and asthma reports. 2018 Feb 22;18(3):19. PubMed PMID: 29470720. 5. Aghamohammadi A, Moin M, Farhoudi A, Rezaei N, Pourpak Z, Movahedi M, et al. Efficacy of intravenous immunoglobulin on the prevention of pneumonia in patients with agammaglobulinemia. FEMS immunology and medical microbiology. 2004 Mar 8;40(2):113- 8. PubMed PMID: 14987729. 6. Rezaei N, Abolhassani H, Aghamohammadi A, Ochs HD. Indications and safety of intravenous and subcutaneous immunoglobulin therapy. Expert review of clinical immunology. 2011 May;7(3):301-16. PubMed PMID: 21595597. 7. Mozaffari H, Pourpak Z, Pourseyed S, Moin M, Farhoodi A, Aghamohammadi A, et al.Health-related quality of life in primary immune deficient patients. Iranian journal of allergy, asthma, and immunology. 2006 Mar;5(1):23-7. PubMed PMID: 17242500. 8. Aghamohammadi A, Pouladi N, Parvaneh N, Yeganeh M, Movahedi M, Gharagolou M, et al. Mortality and morbidity in common variable immunodeficiency. Journal of tropical pediatrics. 2007 Feb;53(1):32-8. PubMed PMID: 17166933. 9. Aghamohammadi A, Farhoudi A, Moin M, Pourpak Z, Rezaei N, Nikzad M, et al. Adverse effects of intravenous immunoglobulin therapy in patients with antibody deficiency. Iranian journal of allergy, asthma, and immunology. 2003 Sep;2(3):121-6. PubMed PMID: 17301367. 10. Dashti-Khavidaki S, Aghamohammadi A, Farshadi F, Movahedi M, Parvaneh N, Pouladi N, et al. Adverse reactions of prophylactic intravenous immunoglobulin; a 13-year experience with 3004 infusions in Iranian patients with primary immunodeficiency diseases. Journal of investigational allergology & clinical immunology. 2009;19(2):139-45. PubMed PMID: 19476018. 11. Aghamohammadi A, Farhoudi A, Nikzad M, Moin M, Pourpak Z, Rezaei N, et al. Adverse reactions of prophylactic intravenous immunoglobulin infusions in Iranian patients with primary immunodeficiency. Annals of allergy, asthma & immunology: official publication of the American College of Allergy, Asthma, & Immunology. 2004 Jan;92(1):60-4. PubMed PMID: 14756466. 12. Abolhassani H, Sadaghiani MS, Aghamohammadi A, Ochs HD, Rezaei N. Home-based subcutaneous immunoglobulin versus hospital-based intravenous immunoglobulin in treatment of primary antibody deficiencies: systematic review and meta analysis. Journal of clinical immunology. 2012 Dec;32(6):1180-92. PubMed PMID: 22730009. 13. Ghavamzadeh A, Alimoghaddam K, Ghaffari F, Derakhshandeh R, Jalali A, Jahani M. Twenty years of experience on stem cell transplantation in iran. Iranian Red Crescent medical journal. 2013 Feb;15(2):93-100. PubMed PMID: 23682320. Pubmed Central PMCID: 3652510. 14. Hamidieh A, Pourpak Z, Jalili M, Fazlollahi M, Behfar M, Movahedi M, et al. Haematopoietic stem cell transplantation in primary immunodeficiency diseases: the Iranian experience. 2012. 15. Nourijelyani K, Aghamohammadi A, Salehi Sadaghiani M, Behniafard N, Abolhassani H, Pourjabar S, et al. Physicians awareness on primary immunodeficiency disorders in Iran. Iranian journal of allergy, asthma, and immunology. 2012 Mar;11(1):57- 64. PubMed PMID: 22427477. 16. Isaian A, Moin M, Pourpak Z, Rezaei N, Aghamohammadi A, Movahedi M, et al. DNA banking of primary immunodeficiency disorders in iran. Iranian journal of allergy, asthma, and immunology. 2006 Dec;5(4):201- 2. PubMed PMID: 17237575.