National Consensus Guideline on Diagnosis and Management of Predominantly Antibody Deficiencies
-
Reza Yazdani
,
Samaneh Abdolahzade
,
Parisa Ashournia
,
Aida Askarisarvestani
,
Sima Bahrami
,
Maryam Behfar
,
Zahra Daneshmandi
,
Mohammad Hassan Bemanian
,
Taher Cheraghi
,
Kian Darabi
,
Sepideh Darougar
,
Samaneh Delavari
,
Sarehsadat Ebrahimi
,
Shabnam Eskandarzadeh
,
Golnaz Eslamian
,
Narges Eslami
,
Shahrzad Fallah
,
Morteza Fallahpour
,
Saba Fekrvand
,
Ali Haghbin
,
Amir Ali Hamidieh
,
Zohre Hassanpoor
,
Zahra Hamidi Esfahani
,
Tolue Mahdavi
,
Mehrnaz Mesdaghi
,
Homa Hatefi Minaei
,
Majid Marjani
,
Shahla Mirazizi
,
Mohammadreza Modaresi
,
Mahshid Movahedi
,
Mohammamd Nabavi
,
Mojtaba Ranjbar
,
Anahita Razaghian
,
Mohammad Saberi
,
Mahnaz Sadeghi-Shabestari
,
Mitra Sahragard
,
Fereshte Salami
,
Sahar Seraj
,
Seyedehshabnam Seyedsalehi
,
Alireza Shafiei
,
Roya Sherkat
,
Reza Shiari
,
Sima Shokri
,
Saman Tavakoli
,
Ahmad Vosughimotlagh
,
Niloufar Yazdanpanah
,
Vahid Ziaee
,
Nima Rezaei
Abstract
At present, a national consensus or guideline for diagnosing and managing patients suspected of having predominantly antibody deficiencies (PADs) is lacking. This consensus is written based on a combination of scientific literature and comments from the expert panel of Iranian immunologists. A group of clinical immunologists reviewed the current consensus, presented their comments at a meeting titled 'First Meeting on the Diagnosis of Inborn Errors of Immunity (IEI) by IEI Experts,' and agreed on this consensus. This consensus guideline provides recommendations on the diagnosis, antimicrobial prophylaxis, management of clinical manifestations, and immunoglobulin replacement therapy (IgRT) for patients with PAD.
1. Vilela M. Human Inborn Errors of Immunity (HIEI): predominantly antibody deficiencies (PADs): if you suspect it, you can detect it. J Pediatr (Rio J). 2021;97 Suppl 1(Suppl 1):S67-s74.
2. Abolhassani H, Azizi G, Sharifi L, Yazdani R, Mohsenzadegan M, Delavari S, et al. Global systematic review of primary immunodeficiency registries. Expert Rev Clin Immunol. 2020;16(7):717-32.
3. Tangye SG, Al-Herz W, Bousfiha A, Cunningham-Rundles C, Franco JL, Holland SM, et al. Human Inborn Errors of Immunity: 2022 Update on the Classification from the International Union of Immunological Societies Expert Committee. J Clin Immunol. 2022;42(7):1473-507.
4. Seidel MG, Kindle G, Gathmann B, Quinti I, Buckland M, van Montfrans J, 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):1763-70.
5. Conley ME, Notarangelo LD, Etzioni A. Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies). Clin Immunol. 1999;93(3):190-7.
6. Bousfiha A, Moundir A, Tangye SG, Picard C, Jeddane L, Al-Herz W, et al. The 2022 Update of IUIS Phenotypical Classification for Human Inborn Errors of Immunity. J Clin Immunol. 2022;42(7):1508-20.
7. Yazdani R, Habibi S, Sharifi L, Azizi G, Abolhassani H, Olbrich P, et al. Common Variable Immunodeficiency: Epidemiology, Pathogenesis, Clinical Manifestations, Diagnosis, Classification, and Management. J Investig Allergol Clin Immunol. 2020;30(1):14-34.
8. Chapel H, Lucas M, Lee M, Bjorkander J, Webster D, Grimbacher B, et al. Common variable immunodeficiency disorders: division into distinct clinical phenotypes. Blood. 2008;112(2):277-86.
9. Ameratunga R, Brewerton M, Slade C, Jordan A, Gillis D, Steele R, et al. Comparison of diagnostic criteria for common variable immunodeficiency disorder. Front Immunol. 2014;5:415.
10. Yazdani R, Aghamohammadi A, Rezaei N. Application of Flow Cytometry in Predominantly Antibody Deficiencies. Endocr Metab Immune Disord Drug Targets. 2021;21(4):647-63.
11. Yazdani R, Fekrvand S, Shahkarami S, Azizi G, Moazzami B, Abolhassani H, et al. The hyper IgM syndromes: Epidemiology, pathogenesis, clinical manifestations, diagnosis and management. Clin Immunol. 2019;198:19-30.
12. Kutukculer N, Moratto D, Aydinok Y, Lougaris V, Aksoylar S, Plebani A, et al. Disseminated cryptosporidium infection in an infant with hyper-IgM syndrome caused by CD40 deficiency. J Pediatr. 2003;142(2):194-6.
13. Kim D, Shin JA, Han SB, Chung NG, Jeong DC. Pneumocystis jirovecii pneumonia as an initial manifestation of hyper-IgM syndrome in an infant: A case report. Medicine (Baltimore). 2019;98(7):e14559.
14. Bonilla FA, Khan DA, Ballas ZK, Chinen J, Frank MM, Hsu JT, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. J Allergy Clin Immunol. 2015;136(5):1186-205.e1-78.
15. Sadighi Akha AA, Tschumper RC, Mills JR, Isham CR, Witty EE, Viswanatha DS, et al. A rare case of selective Igκ chain deficiency: Biologic and clinical implications. J Allergy Clin Immunol. 2020;146(5):1208-10.e6.
16. Perez EE, Ballow M. Diagnosis and management of Specific Antibody Deficiency. Immunol Allergy Clin North Am. 2020;40(3):499-510.
17. Keles S, Artac H, Kara R, Gokturk B, Ozen A, Reisli I. Transient hypogammaglobulinemia and unclassified hypogammaglobulinemia: 'similarities and differences'. Pediatr Allergy Immunol. 2010;21(5):843-51.
18. Bonagura VR. Using intravenous immunoglobulin (IVIG) to treat patients with primary immune deficiency disease. J Clin Immunol. 2013;33 Suppl 2:S90-4.
19. Bonagura VR, Marchlewski R, Cox A, Rosenthal DW. Biologic IgG level in primary immunodeficiency disease: the IgG level that protects against recurrent infection. J Allergy Clin Immunol. 2008;122(1):210-2.
20. Quinti I, Soresina A, Spadaro G, Martino S, Donnanno S, Agostini C, et al. Long-term follow-up and outcome of a large cohort of patients with common variable immunodeficiency. J Clin Immunol. 2007;27(3):308-16.
21. Lindberg K, Gustafson R, Samuelson A, Rynnel-Dagöö B. Impact of IgG replacement therapy and antibiotic treatment on the colonization of non-encapsulated Haemophilus influenzae in the nasopharynx in patients with hypogammaglobulinaemia. Scand J Infect Dis. 2001;33(12):904-8.
22. Bayrakci B, Ersoy F, Sanal O, Kiliç S, Metin A, Tezcan I. The efficacy of immunoglobulin replacement therapy in the long-term follow-up of the B-cell deficiencies (XLA, HIM, CVID). Turk J Pediatr. 2005;47(3):239-46.
23. Lucas M, Lee M, Lortan J, Lopez-Granados E, Misbah S, Chapel H. Infection outcomes in patients with common variable immunodeficiency disorders: relationship to immunoglobulin therapy over 22 years. J Allergy Clin Immunol. 2010;125(6):1354-60.e4.
24. Hoernes M, Seger R, Reichenbach J. Modern management of primary B-cell immunodeficiencies. Pediatr Allergy Immunol. 2011;22(8):758-69.
25. Kuruvilla M, de la Morena MT. Antibiotic prophylaxis in primary immune deficiency disorders. J Allergy Clin Immunol Pract. 2013;1(6):573-82.
26. Yel L. Selective IgA deficiency. J Clin Immunol. 2010;30(1):10-6.
27. López-Boado YS, Rubin BK. Macrolides as immunomodulatory medications for the therapy of chronic lung diseases. Curr Opin Pharmacol. 2008;8(3):286-91.
28. Luisi F, Gandolfi TD, Daudt AD, Sanvitto JP, Pitrez PM, Pinto LA. Anti-inflammatory effects of macrolides in childhood lung diseases. J Bras Pneumol. 2012;38(6):786-96.
29. Langelot M, Cellerin L, Germaud P. [Anti-inflammatory effects of macrolides: applications in lung disease]. Rev Pneumol Clin. 2006;62(4):215-22.
30. Sewell WA, Buckland M, Jolles SR. Therapeutic strategies in common variable immunodeficiency. Drugs. 2003;63(13):1359-71.
31. Lederman HM, Winkelstein JA. X-linked agammaglobulinemia: an analysis of 96 patients. Medicine (Baltimore). 1985;64(3):145-56.
32. Yazdani R, Abolhassani H, Asgardoon MH, Shaghaghi M, Modaresi M, Azizi G, et al. Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders. J Investig Allergol Clin Immunol. 2017;27(4):213-24.
33. Wood P. Primary antibody deficiencies: recognition, clinical diagnosis and referral of patients. Clin Med (Lond). 2009;9(6):595-9.
34. Bagheri Y, Vosughi A, Azizi G, Yazdani R, Kiaee F, Hafezi N, et al. Comparison of clinical and immunological features and mortality in common variable immunodeficiency and agammaglobulinemia patients. Immunol Lett. 2019;210:55-62.
35. Rezaei N, Abolhassani H, Aghamohammadi A, Ochs HD. Indications and safety of intravenous and subcutaneous immunoglobulin therapy. Expert Rev Clin Immunol. 2011;7(3):301-16.
36. Abolhassani H, Sagvand BT, Shokuhfar T, Mirminachi B, Rezaei N, Aghamohammadi A. A review on guidelines for management and treatment of common variable immunodeficiency. Expert Rev Clin Immunol. 2013;9(6):561-74; quiz 75.
37. Cunningham-Rundles C. How I treat common variable immune deficiency. Blood. 2010;116(1):7-15.
38. Bethune C, Egner W, Garcez T, Huissoon A, Jolles S, Karim Y, et al. British Society for Immunology/United Kingdom Primary Immunodeficiency Network consensus statement on managing non-infectious complications of common variable immunodeficiency disorders. Clin Exp Immunol. 2019;196(3):328-35.
39. Fried AJ, Bonilla FA. Pathogenesis, diagnosis, and management of primary antibody deficiencies and infections. Clin Microbiol Rev. 2009;22(3):396-414.
40. Yalçin E, Kiper N, Ozçelik U, Doğru D, Firat P, Sahin A, et al. Effects of claritromycin on inflammatory parameters and clinical conditions in children with bronchiectasis. J Clin Pharm Ther. 2006;31(1):49-55.
41. Costagliola G, Cappelli S, Consolini R. Autoimmunity in Primary Immunodeficiency Disorders: An Updated Review on Pathogenic and Clinical Implications. J Clin Med. 2021;10(20).
42. Quartier P, Bustamante J, Sanal O, Plebani A, Debré M, Deville A, et al. Clinical, immunologic and genetic analysis of 29 patients with autosomal recessive hyper-IgM syndrome due to Activation-Induced Cytidine Deaminase deficiency. Clin Immunol. 2004;110(1):22-9.
43. Yazdani R, Latif A, Tabassomi F, Abolhassani H, Azizi G, Rezaei N, et al. Clinical phenotype classification for selective immunoglobulin A deficiency. Expert Rev Clin Immunol. 2015;11(11):1245-54.
44. Gobert D, Bussel JB, Cunningham-Rundles C, Galicier L, Dechartres A, Berezne A, et al. Efficacy and safety of rituximab in common variable immunodeficiency-associated immune cytopenias: a retrospective multicentre study on 33 patients. Br J Haematol. 2011;155(4):498-508.
45. Amaya-Uribe L, Rojas M, Azizi G, Anaya JM, Gershwin ME. Primary immunodeficiency and autoimmunity: A comprehensive review. J Autoimmun. 2019;99:52-72.
46. Andersen JB, Midttun K, Feragen KJB. Measuring quality of life of primary antibody deficiency patients using a disease-specific health-related quality of life questionnaire for common variable immunodeficiency (CVID_QoL). J Patient Rep Outcomes. 2019;3(1):15.
47. Agarwal S, Mayer L. Diagnosis and treatment of gastrointestinal disorders in patients with primary immunodeficiency. Clin Gastroenterol Hepatol. 2013;11(9):1050-63.
48. Both T, Dalm V, Richardson SA, van Schie N, van den Broek LM, de Vries AC, et al. Inflammatory bowel disease in primary immunodeficiency disorders is a heterogeneous clinical entity requiring an individualized treatment strategy: A systematic review. Autoimmun Rev. 2021;20(8):102872.
49. Chua I, Standish R, Lear S, Harbord M, Eren E, Raeiszadeh M, et al. Anti-tumour necrosis factor-alpha therapy for severe enteropathy in patients with common variable immunodeficiency (CVID). Clin Exp Immunol. 2007;150(2):306-11.
50. Agarwal S, Cunningham-Rundles C. Autoimmunity in common variable immunodeficiency. Ann Allergy Asthma Immunol. 2019;123(5):454-60.
51. Daniels JA, Lederman HM, Maitra A, Montgomery EA. Gastrointestinal tract pathology in patients with common variable immunodeficiency (CVID): a clinicopathologic study and review. Am J Surg Pathol. 2007;31(12):1800-12.
52. Bouvry D, Mouthon L, Brillet PY, Kambouchner M, Ducroix JP, Cottin V, et al. Granulomatosis-associated common variable immunodeficiency disorder: a case-control study versus sarcoidosis. Eur Respir J. 2013;41(1):115-22.
53. de Gracia J, Vendrell M, Alvarez A, Pallisa E, Rodrigo MJ, de la Rosa D, et al. Immunoglobulin therapy to control lung damage in patients with common variable immunodeficiency. Int Immunopharmacol. 2004;4(6):745-53.
54. Boursiquot JN, Gérard L, Malphettes M, Fieschi C, Galicier L, Boutboul D, et al. Granulomatous disease in CVID: retrospective analysis of clinical characteristics and treatment efficacy in a cohort of 59 patients. J Clin Immunol. 2013;33(1):84-95.
55. von Spee-Mayer C, Echternach C, Agarwal P, Gutenberger S, Soetedjo V, Goldacker S, et al. Abatacept Use Is Associated with Steroid Dose Reduction and Improvement in Fatigue and CD4-Dysregulation in CVID Patients with Interstitial Lung Disease. J Allergy Clin Immunol Pract. 2021;9(2):760-70.e10.
56. Verbsky JW, Hintermeyer MK, Simpson PM, Feng M, Barbeau J, Rao N, et al. Rituximab and antimetabolite treatment of granulomatous and lymphocytic interstitial lung disease in common variable immunodeficiency. J Allergy Clin Immunol. 2021;147(2):704-12.e17.
57. Hayward AR, Levy J, Facchetti F, Notarangelo L, Ochs HD, Etzioni A, et al. Cholangiopathy and tumors of the pancreas, liver, and biliary tree in boys with X-linked immunodeficiency with hyper-IgM. J Immunol. 1997;158(2):977-83.
58. Salavoura K, Kolialexi A, Tsangaris G, Mavrou A. Development of cancer in patients with primary immunodeficiencies. Anticancer Res. 2008;28(2b):1263-9.
59. Fekrvand S, Abolhassani H, Hamidi Esfahani Z, Nameh Goshay Fard N, Amiri M, Salehi H. Cancer Trends in Inborn Errors of Immunity: A Systematic Review and Meta-Analysis. J Clin Immunol. 2024;45(34).
60. Shapiro RS. Malignancies in the setting of primary immunodeficiency: Implications for hematologists/oncologists. Am J Hematol. 2011;86(1):48-55.
61. Ochtrop ML, Goldacker S, May AM, Rizzi M, Draeger R, Hauschke D, et al. T and B lymphocyte abnormalities in bone marrow biopsies of common variable immunodeficiency. Blood. 2011;118(2):309-18.
62. Grigoriadou S, Clubbe R, Garcez T, Huissoon A, Grosse-Kreul D, Jolles S, et al. British Society for Immunology and United Kingdom Primary Immunodeficiency Network (UKPIN) consensus guideline for the management of immunoglobulin replacement therapy. Clin Exp Immunol. 2022;210(1):1-13.
63. Roifman CM, Levison H, Gelfand EW. High-dose versus low-dose intravenous immunoglobulin in hypogammaglobulinaemia and chronic lung disease. Lancet. 1987;1(8541):1075-7.
64. Eijkhout HW, van Der Meer JW, Kallenberg CG, Weening RS, van Dissel JT, Sanders LA, et al. The effect of two different dosages of intravenous immunoglobulin on the incidence of recurrent infections in patients with primary hypogammaglobulinemia. A randomized, double-blind, multicenter crossover trial. Ann Intern Med. 2001;135(3):165-74.
65. Favre O, Leimgruber A, Nicole A, Spertini F. Intravenous immunoglobulin replacement prevents severe and lower respiratory tract infections, but not upper respiratory tract and non-respiratory infections in common variable immune deficiency. Allergy. 2005;60(3):385-90.
66. Condino-Neto A, Costa-Carvalho BT, Grumach AS, King A, Bezrodnik L, Oleastro M, et al. Guidelines for the use of human immunoglobulin therapy in patients with primary immunodeficiencies in Latin America. Allergol Immunopathol (Madr). 2014;42(3):245-60.
67. Smits BM, Kleine Budde I, de Vries E, Ten Berge IJM, Bredius RGM, van Deuren M, et al. Immunoglobulin Replacement Therapy Versus Antibiotic Prophylaxis as Treatment for Incomplete Primary Antibody Deficiency. J Clin Immunol. 2021;41(2):382-92.
68. Paris K, Wall LA. The Treatment of Primary Immune Deficiencies: Lessons Learned and Future Opportunities. Clin Rev Allergy Immunol. 2023;65(1):19-30.
2. Abolhassani H, Azizi G, Sharifi L, Yazdani R, Mohsenzadegan M, Delavari S, et al. Global systematic review of primary immunodeficiency registries. Expert Rev Clin Immunol. 2020;16(7):717-32.
3. Tangye SG, Al-Herz W, Bousfiha A, Cunningham-Rundles C, Franco JL, Holland SM, et al. Human Inborn Errors of Immunity: 2022 Update on the Classification from the International Union of Immunological Societies Expert Committee. J Clin Immunol. 2022;42(7):1473-507.
4. Seidel MG, Kindle G, Gathmann B, Quinti I, Buckland M, van Montfrans J, 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):1763-70.
5. Conley ME, Notarangelo LD, Etzioni A. Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies). Clin Immunol. 1999;93(3):190-7.
6. Bousfiha A, Moundir A, Tangye SG, Picard C, Jeddane L, Al-Herz W, et al. The 2022 Update of IUIS Phenotypical Classification for Human Inborn Errors of Immunity. J Clin Immunol. 2022;42(7):1508-20.
7. Yazdani R, Habibi S, Sharifi L, Azizi G, Abolhassani H, Olbrich P, et al. Common Variable Immunodeficiency: Epidemiology, Pathogenesis, Clinical Manifestations, Diagnosis, Classification, and Management. J Investig Allergol Clin Immunol. 2020;30(1):14-34.
8. Chapel H, Lucas M, Lee M, Bjorkander J, Webster D, Grimbacher B, et al. Common variable immunodeficiency disorders: division into distinct clinical phenotypes. Blood. 2008;112(2):277-86.
9. Ameratunga R, Brewerton M, Slade C, Jordan A, Gillis D, Steele R, et al. Comparison of diagnostic criteria for common variable immunodeficiency disorder. Front Immunol. 2014;5:415.
10. Yazdani R, Aghamohammadi A, Rezaei N. Application of Flow Cytometry in Predominantly Antibody Deficiencies. Endocr Metab Immune Disord Drug Targets. 2021;21(4):647-63.
11. Yazdani R, Fekrvand S, Shahkarami S, Azizi G, Moazzami B, Abolhassani H, et al. The hyper IgM syndromes: Epidemiology, pathogenesis, clinical manifestations, diagnosis and management. Clin Immunol. 2019;198:19-30.
12. Kutukculer N, Moratto D, Aydinok Y, Lougaris V, Aksoylar S, Plebani A, et al. Disseminated cryptosporidium infection in an infant with hyper-IgM syndrome caused by CD40 deficiency. J Pediatr. 2003;142(2):194-6.
13. Kim D, Shin JA, Han SB, Chung NG, Jeong DC. Pneumocystis jirovecii pneumonia as an initial manifestation of hyper-IgM syndrome in an infant: A case report. Medicine (Baltimore). 2019;98(7):e14559.
14. Bonilla FA, Khan DA, Ballas ZK, Chinen J, Frank MM, Hsu JT, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. J Allergy Clin Immunol. 2015;136(5):1186-205.e1-78.
15. Sadighi Akha AA, Tschumper RC, Mills JR, Isham CR, Witty EE, Viswanatha DS, et al. A rare case of selective Igκ chain deficiency: Biologic and clinical implications. J Allergy Clin Immunol. 2020;146(5):1208-10.e6.
16. Perez EE, Ballow M. Diagnosis and management of Specific Antibody Deficiency. Immunol Allergy Clin North Am. 2020;40(3):499-510.
17. Keles S, Artac H, Kara R, Gokturk B, Ozen A, Reisli I. Transient hypogammaglobulinemia and unclassified hypogammaglobulinemia: 'similarities and differences'. Pediatr Allergy Immunol. 2010;21(5):843-51.
18. Bonagura VR. Using intravenous immunoglobulin (IVIG) to treat patients with primary immune deficiency disease. J Clin Immunol. 2013;33 Suppl 2:S90-4.
19. Bonagura VR, Marchlewski R, Cox A, Rosenthal DW. Biologic IgG level in primary immunodeficiency disease: the IgG level that protects against recurrent infection. J Allergy Clin Immunol. 2008;122(1):210-2.
20. Quinti I, Soresina A, Spadaro G, Martino S, Donnanno S, Agostini C, et al. Long-term follow-up and outcome of a large cohort of patients with common variable immunodeficiency. J Clin Immunol. 2007;27(3):308-16.
21. Lindberg K, Gustafson R, Samuelson A, Rynnel-Dagöö B. Impact of IgG replacement therapy and antibiotic treatment on the colonization of non-encapsulated Haemophilus influenzae in the nasopharynx in patients with hypogammaglobulinaemia. Scand J Infect Dis. 2001;33(12):904-8.
22. Bayrakci B, Ersoy F, Sanal O, Kiliç S, Metin A, Tezcan I. The efficacy of immunoglobulin replacement therapy in the long-term follow-up of the B-cell deficiencies (XLA, HIM, CVID). Turk J Pediatr. 2005;47(3):239-46.
23. Lucas M, Lee M, Lortan J, Lopez-Granados E, Misbah S, Chapel H. Infection outcomes in patients with common variable immunodeficiency disorders: relationship to immunoglobulin therapy over 22 years. J Allergy Clin Immunol. 2010;125(6):1354-60.e4.
24. Hoernes M, Seger R, Reichenbach J. Modern management of primary B-cell immunodeficiencies. Pediatr Allergy Immunol. 2011;22(8):758-69.
25. Kuruvilla M, de la Morena MT. Antibiotic prophylaxis in primary immune deficiency disorders. J Allergy Clin Immunol Pract. 2013;1(6):573-82.
26. Yel L. Selective IgA deficiency. J Clin Immunol. 2010;30(1):10-6.
27. López-Boado YS, Rubin BK. Macrolides as immunomodulatory medications for the therapy of chronic lung diseases. Curr Opin Pharmacol. 2008;8(3):286-91.
28. Luisi F, Gandolfi TD, Daudt AD, Sanvitto JP, Pitrez PM, Pinto LA. Anti-inflammatory effects of macrolides in childhood lung diseases. J Bras Pneumol. 2012;38(6):786-96.
29. Langelot M, Cellerin L, Germaud P. [Anti-inflammatory effects of macrolides: applications in lung disease]. Rev Pneumol Clin. 2006;62(4):215-22.
30. Sewell WA, Buckland M, Jolles SR. Therapeutic strategies in common variable immunodeficiency. Drugs. 2003;63(13):1359-71.
31. Lederman HM, Winkelstein JA. X-linked agammaglobulinemia: an analysis of 96 patients. Medicine (Baltimore). 1985;64(3):145-56.
32. Yazdani R, Abolhassani H, Asgardoon MH, Shaghaghi M, Modaresi M, Azizi G, et al. Infectious and Noninfectious Pulmonary Complications in Patients With Primary Immunodeficiency Disorders. J Investig Allergol Clin Immunol. 2017;27(4):213-24.
33. Wood P. Primary antibody deficiencies: recognition, clinical diagnosis and referral of patients. Clin Med (Lond). 2009;9(6):595-9.
34. Bagheri Y, Vosughi A, Azizi G, Yazdani R, Kiaee F, Hafezi N, et al. Comparison of clinical and immunological features and mortality in common variable immunodeficiency and agammaglobulinemia patients. Immunol Lett. 2019;210:55-62.
35. Rezaei N, Abolhassani H, Aghamohammadi A, Ochs HD. Indications and safety of intravenous and subcutaneous immunoglobulin therapy. Expert Rev Clin Immunol. 2011;7(3):301-16.
36. Abolhassani H, Sagvand BT, Shokuhfar T, Mirminachi B, Rezaei N, Aghamohammadi A. A review on guidelines for management and treatment of common variable immunodeficiency. Expert Rev Clin Immunol. 2013;9(6):561-74; quiz 75.
37. Cunningham-Rundles C. How I treat common variable immune deficiency. Blood. 2010;116(1):7-15.
38. Bethune C, Egner W, Garcez T, Huissoon A, Jolles S, Karim Y, et al. British Society for Immunology/United Kingdom Primary Immunodeficiency Network consensus statement on managing non-infectious complications of common variable immunodeficiency disorders. Clin Exp Immunol. 2019;196(3):328-35.
39. Fried AJ, Bonilla FA. Pathogenesis, diagnosis, and management of primary antibody deficiencies and infections. Clin Microbiol Rev. 2009;22(3):396-414.
40. Yalçin E, Kiper N, Ozçelik U, Doğru D, Firat P, Sahin A, et al. Effects of claritromycin on inflammatory parameters and clinical conditions in children with bronchiectasis. J Clin Pharm Ther. 2006;31(1):49-55.
41. Costagliola G, Cappelli S, Consolini R. Autoimmunity in Primary Immunodeficiency Disorders: An Updated Review on Pathogenic and Clinical Implications. J Clin Med. 2021;10(20).
42. Quartier P, Bustamante J, Sanal O, Plebani A, Debré M, Deville A, et al. Clinical, immunologic and genetic analysis of 29 patients with autosomal recessive hyper-IgM syndrome due to Activation-Induced Cytidine Deaminase deficiency. Clin Immunol. 2004;110(1):22-9.
43. Yazdani R, Latif A, Tabassomi F, Abolhassani H, Azizi G, Rezaei N, et al. Clinical phenotype classification for selective immunoglobulin A deficiency. Expert Rev Clin Immunol. 2015;11(11):1245-54.
44. Gobert D, Bussel JB, Cunningham-Rundles C, Galicier L, Dechartres A, Berezne A, et al. Efficacy and safety of rituximab in common variable immunodeficiency-associated immune cytopenias: a retrospective multicentre study on 33 patients. Br J Haematol. 2011;155(4):498-508.
45. Amaya-Uribe L, Rojas M, Azizi G, Anaya JM, Gershwin ME. Primary immunodeficiency and autoimmunity: A comprehensive review. J Autoimmun. 2019;99:52-72.
46. Andersen JB, Midttun K, Feragen KJB. Measuring quality of life of primary antibody deficiency patients using a disease-specific health-related quality of life questionnaire for common variable immunodeficiency (CVID_QoL). J Patient Rep Outcomes. 2019;3(1):15.
47. Agarwal S, Mayer L. Diagnosis and treatment of gastrointestinal disorders in patients with primary immunodeficiency. Clin Gastroenterol Hepatol. 2013;11(9):1050-63.
48. Both T, Dalm V, Richardson SA, van Schie N, van den Broek LM, de Vries AC, et al. Inflammatory bowel disease in primary immunodeficiency disorders is a heterogeneous clinical entity requiring an individualized treatment strategy: A systematic review. Autoimmun Rev. 2021;20(8):102872.
49. Chua I, Standish R, Lear S, Harbord M, Eren E, Raeiszadeh M, et al. Anti-tumour necrosis factor-alpha therapy for severe enteropathy in patients with common variable immunodeficiency (CVID). Clin Exp Immunol. 2007;150(2):306-11.
50. Agarwal S, Cunningham-Rundles C. Autoimmunity in common variable immunodeficiency. Ann Allergy Asthma Immunol. 2019;123(5):454-60.
51. Daniels JA, Lederman HM, Maitra A, Montgomery EA. Gastrointestinal tract pathology in patients with common variable immunodeficiency (CVID): a clinicopathologic study and review. Am J Surg Pathol. 2007;31(12):1800-12.
52. Bouvry D, Mouthon L, Brillet PY, Kambouchner M, Ducroix JP, Cottin V, et al. Granulomatosis-associated common variable immunodeficiency disorder: a case-control study versus sarcoidosis. Eur Respir J. 2013;41(1):115-22.
53. de Gracia J, Vendrell M, Alvarez A, Pallisa E, Rodrigo MJ, de la Rosa D, et al. Immunoglobulin therapy to control lung damage in patients with common variable immunodeficiency. Int Immunopharmacol. 2004;4(6):745-53.
54. Boursiquot JN, Gérard L, Malphettes M, Fieschi C, Galicier L, Boutboul D, et al. Granulomatous disease in CVID: retrospective analysis of clinical characteristics and treatment efficacy in a cohort of 59 patients. J Clin Immunol. 2013;33(1):84-95.
55. von Spee-Mayer C, Echternach C, Agarwal P, Gutenberger S, Soetedjo V, Goldacker S, et al. Abatacept Use Is Associated with Steroid Dose Reduction and Improvement in Fatigue and CD4-Dysregulation in CVID Patients with Interstitial Lung Disease. J Allergy Clin Immunol Pract. 2021;9(2):760-70.e10.
56. Verbsky JW, Hintermeyer MK, Simpson PM, Feng M, Barbeau J, Rao N, et al. Rituximab and antimetabolite treatment of granulomatous and lymphocytic interstitial lung disease in common variable immunodeficiency. J Allergy Clin Immunol. 2021;147(2):704-12.e17.
57. Hayward AR, Levy J, Facchetti F, Notarangelo L, Ochs HD, Etzioni A, et al. Cholangiopathy and tumors of the pancreas, liver, and biliary tree in boys with X-linked immunodeficiency with hyper-IgM. J Immunol. 1997;158(2):977-83.
58. Salavoura K, Kolialexi A, Tsangaris G, Mavrou A. Development of cancer in patients with primary immunodeficiencies. Anticancer Res. 2008;28(2b):1263-9.
59. Fekrvand S, Abolhassani H, Hamidi Esfahani Z, Nameh Goshay Fard N, Amiri M, Salehi H. Cancer Trends in Inborn Errors of Immunity: A Systematic Review and Meta-Analysis. J Clin Immunol. 2024;45(34).
60. Shapiro RS. Malignancies in the setting of primary immunodeficiency: Implications for hematologists/oncologists. Am J Hematol. 2011;86(1):48-55.
61. Ochtrop ML, Goldacker S, May AM, Rizzi M, Draeger R, Hauschke D, et al. T and B lymphocyte abnormalities in bone marrow biopsies of common variable immunodeficiency. Blood. 2011;118(2):309-18.
62. Grigoriadou S, Clubbe R, Garcez T, Huissoon A, Grosse-Kreul D, Jolles S, et al. British Society for Immunology and United Kingdom Primary Immunodeficiency Network (UKPIN) consensus guideline for the management of immunoglobulin replacement therapy. Clin Exp Immunol. 2022;210(1):1-13.
63. Roifman CM, Levison H, Gelfand EW. High-dose versus low-dose intravenous immunoglobulin in hypogammaglobulinaemia and chronic lung disease. Lancet. 1987;1(8541):1075-7.
64. Eijkhout HW, van Der Meer JW, Kallenberg CG, Weening RS, van Dissel JT, Sanders LA, et al. The effect of two different dosages of intravenous immunoglobulin on the incidence of recurrent infections in patients with primary hypogammaglobulinemia. A randomized, double-blind, multicenter crossover trial. Ann Intern Med. 2001;135(3):165-74.
65. Favre O, Leimgruber A, Nicole A, Spertini F. Intravenous immunoglobulin replacement prevents severe and lower respiratory tract infections, but not upper respiratory tract and non-respiratory infections in common variable immune deficiency. Allergy. 2005;60(3):385-90.
66. Condino-Neto A, Costa-Carvalho BT, Grumach AS, King A, Bezrodnik L, Oleastro M, et al. Guidelines for the use of human immunoglobulin therapy in patients with primary immunodeficiencies in Latin America. Allergol Immunopathol (Madr). 2014;42(3):245-60.
67. Smits BM, Kleine Budde I, de Vries E, Ten Berge IJM, Bredius RGM, van Deuren M, et al. Immunoglobulin Replacement Therapy Versus Antibiotic Prophylaxis as Treatment for Incomplete Primary Antibody Deficiency. J Clin Immunol. 2021;41(2):382-92.
68. Paris K, Wall LA. The Treatment of Primary Immune Deficiencies: Lessons Learned and Future Opportunities. Clin Rev Allergy Immunol. 2023;65(1):19-30.
| Files | ||
| Issue | Vol 7, No 2 (2024) | |
| Section | Review Article | |
| DOI | https://doi.org/10.18502/igj.v7i2.17850 | |
| Keywords | ||
| Diagnosis Inborn Errors of Immunity Immunoglobulin Replacement Therapy Management National Consensus Predominantly Antibody Deficiencies | ||
| Rights and permissions | |
|
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. |
How to Cite
1.
Yazdani R, Abdolahzade S, Ashournia P, Askarisarvestani A, Bahrami S, Behfar M, Daneshmandi Z, Bemanian MH, Cheraghi T, Darabi K, Darougar S, Delavari S, Ebrahimi S, Eskandarzadeh S, Eslamian G, Eslami N, Fallah S, Fallahpour M, Fekrvand S, Haghbin A, Hamidieh AA, Hassanpoor Z, Hamidi Esfahani Z, Mahdavi T, Mesdaghi M, Hatefi Minaei H, Marjani M, Mirazizi S, Modaresi M, Movahedi M, Nabavi M, Ranjbar M, Razaghian A, Saberi M, Sadeghi-Shabestari M, Sahragard M, Salami F, Seraj S, Seyedsalehi S, Shafiei A, Sherkat R, Shiari R, Shokri S, Tavakoli S, Vosughimotlagh A, Yazdanpanah N, Ziaee V, Rezaei N. National Consensus Guideline on Diagnosis and Management of Predominantly Antibody Deficiencies. Immunol Genet J. 2024;7(2):47-52.
