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Chest Complications in Patients with Primary Antibody Deficiency Syndromes (PADS)

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Book cover Humoral Primary Immunodeficiencies

Part of the book series: Rare Diseases of the Immune System ((RDIS))

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Abstract

Symptomatic primary antibody deficiency syndromes (PADS) range from severe forms, such as common variable immunodeficiency disorders and X-linked agammaglobulinemia, to partial antibody deficiencies. All patients with failure of antibody production suffer from chest infections with common organisms, which should prompt diagnosis. Patients with proven antibody failure require replacement immunoglobulin therapy.

Chest infections are treated with appropriate antibiotics early and for longer in antibody failure than in immunocompetent individuals to prevent structural lung damage, since bronchiectasis is a common feature in patients with severe PADS. Bronchiectasis necessitates breakthrough infection documentation as well as CT monitoring for progression, which is not inevitable in patients receiving adequate doses of replacement immunoglobulin.

Interstitial lung disease is a particular feature in about 15% or so of sporadic CVID patients, though this may represent several different conditions depending on aetiology, and prognosis is uncertain. Biopsy is essential to distinguish lymphoid malignancy and to determine appropriate therapeutic interventions. Until there is a standard protocol for reporting CT findings, and an internationally agreed histological classification for biopsies that can predict prognosis, this complication remains difficult to investigate and treat successfully.

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References

  1. Bleesing J, Souto-Carneiro MM, Savage WJ, Brown MR, Martinez C, Yavuz S, Brenner S, Siegel RM, Horwitz ME, Lipsky PE, Malech HL, Fleisher TA. Patients with chronic granulomatous disease have a reduced peripheral blood memory B cell compartment. J Immunol. 2006;176:7096–8103.

    Article  CAS  Google Scholar 

  2. Bacchelli C, Matharu K, Zarember KA, Marciano BE, Kuhns DB, Spalding C, Garofalo M, Dimaggio T, Estwick T, Huang CY, Fink D, Priel DL, Fleisher TA, Holland SM, Malech HL, Gallin JI. Cell activating factor (BAFF) is elevated in chronic granulomatous disease. Clin Immunol. 2013;148:258–64.

    Article  Google Scholar 

  3. Kienzler A, van Schouwenburg PA, Taylor J, Marwah I, SharmA RU, Noakes C, Thomson K, Sadler R, Segal S, Ferry B, Taylor JC, Blair E, Chapel H, Patel SY. Hypomorphic function and somatic reversion of DOCK8 cause combined immunodeficiency without hyper-IgE. Clin Immunol. 2016;163:17–21.

    Article  CAS  Google Scholar 

  4. Al-Herz W, Bousfiha A, Casanova JL, Chapel H, Conley ME, Cunningham-Rundles C, Etzioni A, Fischer A, Franco JL, Geha RS, Hammarström L, Nonoyama S, Notarangelo LG, Ochs HD, Puck JM, Roifman CM, Seger R, Tang MLK. Primary immunodeficiency diseases: an update on the classification from the International Union of Immunological Societies Expert Committee for Primary Immunodeficiency. Front Immunol. 2011;2:1–26.

    Article  Google Scholar 

  5. Driessen G, van der Burg M. Primary antibody deficiencies. Eur J Pediatr. 2011;170:693–702.

    Article  Google Scholar 

  6. Lopez-Granados E, Stacey M, Kienzler AK, Sierro S, Willberg CB, Fox CP, Rigaud S, Long HM, Hislop AD, Rickinson AB, Patel S, Latour S, Klenerman P, Chapel H. A mutation in X-linked inhibitor of apoptosis (G466X) leads to memory inflation of Epstein-Barr virus-specific T cells. Clin Exp Immunol. 2014;178:470–82.

    Article  CAS  Google Scholar 

  7. Resnick ES, Moshier EL, Godbold JH, Cunningham-Rundles C. Morbidity and mortality in common variable immune deficiency over 4 decades. Blood. 2012;119(7):1650–7.

    Article  CAS  Google Scholar 

  8. Chapel H, Lucas M, Lee M, Bjorkander J, Webster D, Grimbacher B, Fieschi C, Thon V, Abedi MR, Hammarstrom L. Common variable immunodeficiency disorders: division into distinct clinical phenotypes. Blood. 2008;112:277–86.

    Article  CAS  Google Scholar 

  9. Plebani A, Soresina A, Rondelli R, Amato G, Azzari C, Cardinale F, Cazzola G, Consolini R, De Mattia D, Dell'Erba G, Duse M, Fiorini M, Martino S, Martire B, Masi M, Monafo V, Moschese V, Notarangelo LD, Orlandi P, Panei P, Pession A, Pietrogrande MC, Pignata C, Quinti I, Ragno V, Rossi P, Sciotto A, Stabile A, Italian Pediatric Group for XLA-AIEOP. Clinical, Immunological and molecular analysis in a large cohort of patients with X-linked agammaglobulinaemia: an Italian multicenter study. Clin Immunol. 2002;104:221–30.

    Article  CAS  Google Scholar 

  10. Quinti I, Soresina A, Sparado G, Martino S, Donnanno S, Agostini C, Pignata P, Dammacco D, Pesce AM, Borgese F, Guerra A, Rondelli R, Plebani A. Long-term follow-up and outcome of a large cohort of patients with common variable immunodeficiency. J Clin Immunol. 2007;27:308–16.

    Article  Google Scholar 

  11. 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;125:1354–60.

    Article  CAS  Google Scholar 

  12. Winkelstein J, Marino MC, Lederman HM, Jones SM, Sullivan K, Burks AW, et al. X-linked agammaglobulinemia: report on a United States registry of 201 patients. Medicine (Baltimore). 2006;85:193–202.

    Article  Google Scholar 

  13. Orange J, Belohradsky BH, Berger M, Borte M, Hagan J, Jolles S, Wasserman RL, Baggish JS, Saunders R, Grimbacher B. Evaluation of correlation between dose and clinical outcomes in subcutaneous immunoglobulin replacement therapy. Clin Exp Immunol. 2012;169:172–81.

    Article  CAS  Google Scholar 

  14. Harville T. Can we effectively use radiographic imaging and clinical parameters for making an earlier diagnosis of granulomatous interstitial lung disease in patients with common variable immunodeficiency? Ann Allergy Asthma Immunol. 2017;118:529–30.

    Article  Google Scholar 

  15. Orange J, Grossman WJ, Navickis RJ, Wilkes MM. Impact of trough IgG on pneumonia incidence in primary immunodeficiency: a meta-analysis of clinical studies. Clin Immunol. 2010;137:21–30.

    Article  CAS  Google Scholar 

  16. Milito C, Pulvirenti F, Pesce AM, Digiulio MA, Pandolfi F, Visentini M, Quinti I. Adequate patient’s outcome achieved with short immunoglobulin replacement intervals in severe antibody deficiencies. J Clin Immunol. 2014;34:813–9.

    Article  CAS  Google Scholar 

  17. Popa V, Colby TV, Reich SV. Pulmonary interstitial disease in Ig deficiency. Chest. 2002;122:1594–603.

    Article  Google Scholar 

  18. Quinti I, Soresina A, Guerra A, Rondelli R, Spadaro G, Agostini C, Milito C, Trombetta AC, Visentini V, Martini H, Plebani A, Fiorilli M, IPINet Investigators. 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;31:315–22.

    Article  CAS  Google Scholar 

  19. Ni Y, Shi G, Yu Y, Hao J, Chen T, Song H. Clinical characteristics of patients with chronic obstructive pulmonary disease with comorbid bronchiectasis: a systemic review and meta-analysis. Int J COPD. 2015;10:1465–75.

    Article  CAS  Google Scholar 

  20. Stead A, Douglas JG, Broadfoot CJ, Kaminski ER, Herriot R. Humoral immunity and bronchiectasis. Clin Exp Immunol. 2002;130:325–30.

    Article  CAS  Google Scholar 

  21. Schütz K, Alecsandru D, Babar J, Banerjee AK, Bondioni P, Bruining A, Chapel H, Condliffe AM, Dettmer S, Driessen G, Exley A, Farber CM, Fraioli F, Galal N, Gathmann B, Grimbacher B, Gonzalez-Granado LI, Hackett S, Haddock J, van Hagen PM, Hartmann Hurst JL, Lougaris V, Lucas M, Meyts I, Milito C, Mitrevski M, Patel S, Plebani A, Postranecka V, Quinti I, Screaton N, Serra G, Sirignano C, Soresina A, Spadaro G, Stirling R, Thon V, de Vries E, Warnatz K, Baumann U. Imaging of bronchial pathology in primary antibody deficiencies: data from the European Chest CT Group. in submission.

    Google Scholar 

  22. Litzman JFT, Grimbacher B, Gathmann B, Salzer U, Pavlík T, Vlcek J, Postránecká V, Trávnícková Z, Thon V. Mannose-binding lectin gene polymorphic variants predispose to the development of bronchopulmonary complications but have no influence on other clinical and laboratory symptoms or signs of common variable immunodeficiency. Clin Exp Immunol. 2008;153:324–30.

    Article  CAS  Google Scholar 

  23. Costa-Carvalho B, Wandalsen GF, Pulici G, Sanchez Aranda C, Solé D. Pulmonary complications in patients with antibody deficiency. Allergol Immunopathol (Madr). 2011;39:128–32.

    Article  Google Scholar 

  24. Touw CM, van de Ven AA, de Jong PA, Terheggen-Lagro S, Beek E, Sanders EA, van Montfrans JM. Detection of pulmonary complications in common variable immunodeficiency. Pediatr Allergy Immunol. 2010;21:793–805.

    Article  Google Scholar 

  25. Hampson F, Chandra A, Screaton NJ, Condliffe A, Kumararatne DS, Exley AR, et al. Respiratory disease in common variable immunodeficiency and other primary immunodeficiency disorders. Clin Radiol. 2012;67:587–95.

    Article  CAS  Google Scholar 

  26. Cereser L, Girometti R, d’Angelo P, De Carli M, De Pellegrin A, Zuiani C. Humoral primary immunodeficiency diseases: clinical overview and chest high-resolution computed tomography (HRCT) features in the adult population. Clinical Radiology. 2017;72:534–42.

    Article  CAS  Google Scholar 

  27. Arslan S, Poyraz N, Ucar R, Yesildag M, Yesildag A, Caliskaner AZ. Magnetic resonance imaging may be a valuable radiation-free technique for lung pathologies in patients with primary immunodeficiency. J Clin Immunol. 2016;36:66–72.

    Article  CAS  Google Scholar 

  28. Milito C, Pulvirenti F, Serra G, Valente M, Pesce AM, Granata G, Catalano C, Fraioli F, Quinti I. Magnetic resonance imaging may be a valuable radiation-free technique for lung pathologies in patients with primary immunodeficiency. J Clin Immunol. 2015;35:491–500.

    Article  CAS  Google Scholar 

  29. Chapel H, Prevot J, Gaspar HB, Español T, Bonilla FS, Solis L, Drabwell J, Editorial Board for Working Party on Principles of Care at IPOPI. Primary immunodeficiencies – principles of care. Front Immunol. 2014.; ISSN: 1664-3224;5:627. https://doi.org/10.3389/fimmu.2014.00627.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  30. Freiberger T, Grodecká L, Ravcuková B, Kurecová B, Postránecká V, Vlcek J, Jarkovský J, Thon V, Litzman J. Association of FcRn expression with lung abnormalities and IVIG catabolism in patients with common variable immunodeficiency. Clin Immunol. 2010;136:419–25.

    Article  CAS  Google Scholar 

  31. Patel S, Anzilotti C, Lucas M, Jain R, Moore N, Chapel H. Outcome of interstitial lung disease in patients with common variable immunodeficiency disorders – common pathology or more than one disease? in press.

    Google Scholar 

  32. Kokosi M, Nicholson AG, Hansell DM, Wells AU. Rare idiopathic interstitial pneumonias: LIP and PPFE and rare histologic patterns of interstitial pneumonias: AFOP and BPIP. Respirology. 2016;21:600–14.

    Article  Google Scholar 

  33. Hurst J, Verma N, Lowe D, Baxendale HE, Jolles J, Kelleher P, Longhurst HJ, Y Patel S, Renzoni EA, Sander CR, Avery GA, Babar JL, Buckland MS, Burns S, Egner W, Gompels MM, Gordins P, Haddock JA, Hart Hayman GR, Herriot R, Hoyles RK, Huissoon AP, Jacob J, Nicholson AG, Rassl DM, Sargur RB, Savic S, Seneviratne SL, Sheaff M, Vaitla PM, Walters GI, Whitehouse GL, Wright PA, Condliffe AM, BLF/UKPIN Consensus Statement on the Definition. Diagnosis and Management of Granulomatous-Lymphocytic Interstitial Lung Disease (GLILD) in Common Variable Immunodeficiency Disorders (CVID). J Allergy Clin Immunol Pract. 2017;5:938–45.

    Article  Google Scholar 

  34. Kokosi M, Nicholson AG, Hansell DM, Wells AU. Respiratory. Respirology. 2016;21:600–14.

    Article  Google Scholar 

  35. Maglione P, Ko HM, Beasley MB, Strauchen JA, Cunningham-Rundles C. Tertiary lymphoid neogenesis is a component of pulmonary lymphoid hyperplasia in patients with common variable immunodeficiency. J Allergy Clin Immunol. 2014;133:535–42.

    Article  Google Scholar 

  36. Guinee DJ. Update on nonneoplastic pulmonary lymphoproliferative disorders and related entities. Arch Pathol Lab Med. 2010;134:691–701.

    PubMed  Google Scholar 

  37. Rao N, Mackinnon AC, Routes JM. Granulomatous and lymphocytic interstitial lung disease: a spectrum of pulmonary histopathologic lesions in common variable immunodeficiency—histologic and immunohistochemical analyses of 16 cases. Hum Pathol. 2015;46:1306–14.

    Article  Google Scholar 

  38. Chase N, Verbsky JW, Hintermeyer MK, Waukau JK, Tomita-Mitchell A, Casper JT, Singh S, Shahir KS, Tisol WB, Nugent ML, Rao RN, Mackinnon AC, Goodman LR, Simpson PM, Routes JM. Use of combination chemotherapy for treatment of granulomatous and lymphocytic interstitial lung disease (GLILD) in patients with common variable immunodeficiency (CVID). J Clin Immunol. 2013;33:30–9.

    Article  CAS  Google Scholar 

  39. Chapel H, Lucas M, Patel S, Lee M, Cunningham-Rundles C, Resnick E, Gerard L, Oksenhendler E. Confirmation and improvement of criteria for clinical phenotyping in common variable immunodeficiency disorders in replicate cohorts. JACI. 2012;130:1197–8.

    Google Scholar 

  40. Hartono S, Motosue MS, Khan S, Rodriguez V, Iyer VN, Divekar R, Joshi AJ. Predictors of granulomatous lymphocytic interstitial lung disease in common variable immunodeficiency. Ann Allergy Asthma Immunol. 2017;118:614–20.

    Article  Google Scholar 

  41. Zdziarski P, Gamian A, Dworacki G. A case report of lymphoid interstitial pneumonia in common variable immunodeficiency. Oligoclonal expansion of effector lymphocytes with preferential cytomegalovirus-specific immune response and lymphoproliferative disease promotion. Medicine (Baltimore). 2017;96:23.

    Article  Google Scholar 

  42. Hasegawa M, Sakai F, Okabayashi A, Sato A, Yokohori N, Katsura H, Asano C, Kamata T, Koh E, Sekine Y, Hiroshima K, Ogura T, Takemura T. Intravenous immunoglobulin monotherapy for granulomatous lymphocytic interstitial lung disease in common variable immunodeficiency. Intern Med. 2017;56(21):2899–902. https://doi.org/10.2169/internalmedicine.7757-16.

    Article  PubMed  PubMed Central  Google Scholar 

  43. Bucciol G, Petrone A, Putti MC. Efficacy of mycophenolate on lung disease and autoimmunity in children with immunodeficiency. Pediatr Pulmonol. 2017;52:E73–E6.

    Article  Google Scholar 

  44. Mannina A, Chung, JH., Swigris, JJ., Solomon, JJ., Huie, TJ., Yunt, ZX., Truong, TQ., Brown, K., Achcar, RD., Olson, AL., Cox, CW., Kligerman, SJ>, Curran-Everett, D., Fernandez-Perez, ER. Clinical predictors of a diagnosis of common variable immunodeficiency-related granulomatous-lymphocytic interstitial lung disease. Ann Am Thorac Soc 2016;13:1042–1049.

    Article  Google Scholar 

  45. Schussler E, Beasley MB, Maglione PJ. Lung disease in primary antibody deficiencies. J Allergy Clin Immunol Pract. 2016;4:1039–52.

    Article  Google Scholar 

  46. Gompels MM, Hodges E, Lock RJ, Angus B, White H, Larkin A, Chapel HM, Spickett GP, Misbah SA, Smith JL, Associated Study Group. Lymphoproliferative disease in antibody deficiency: a multi-centre study. Clin Exp Immunol. 2003;134:314–20.

    Article  CAS  Google Scholar 

  47. Dhalla F, da Silva SP, Lucas M, Travis S, Chapel H. Review of gastric cancer risk factors in patients with common variable immunodeficiency disorders, resulting in a proposal for a surveillance programme. Clin Exp Immunol. 2011;165(1):1–7.

    Article  CAS  Google Scholar 

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Authors and Affiliations

  1. Primary Immunodeficiency Unit, Experimental Medicine, Nuffield Department of Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK

    Helen M. Chapel MA. MD. FRCP. FRCPath, Consuelo Anzilotti MD. PhD & Smita Y. Patel MBBS. BSc. MRCP. FRCPath. PhD

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  1. Helen M. Chapel MA. MD. FRCP. FRCPath
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  2. Consuelo Anzilotti MD. PhD
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  3. Smita Y. Patel MBBS. BSc. MRCP. FRCPath. PhD
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Correspondence to Helen M. Chapel MA. MD. FRCP. FRCPath .

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Editors and Affiliations

  1. Careggi Hospital, Department of Clinical and Experimental Medicine, University of Florence, Firenze, Italy

    Mario Milco D'Elios

  2. University Medical Centre Freiburg, Department of Rheumatology and Clinical Immunology and Center for Chronic Immunodeficiency, Freiburg, Germany

    Marta Rizzi

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Chapel, H.M., Anzilotti, C., Patel, S.Y. (2019). Chest Complications in Patients with Primary Antibody Deficiency Syndromes (PADS). In: D'Elios, M., Rizzi, M. (eds) Humoral Primary Immunodeficiencies. Rare Diseases of the Immune System. Springer, Cham. https://doi.org/10.1007/978-3-319-91785-6_25

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  • DOI: https://doi.org/10.1007/978-3-319-91785-6_25

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