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Current Allergy and Asthma Reports
Published in: Current Allergy and Asthma Reports 6/2014

01-06-2014 | BASIC AND APPLIED SCIENCE (M FRIERI AND PJ BRYCE, SECTION EDITORS)

Good’s Syndrome, CVID, and Selective Antibody Deficiency in Patients with Chronic Rhinosinusitis

Author: Marianne Frieri

Published in: Current Allergy and Asthma Reports | Issue 6/2014

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Abstract

Good’s syndrome and common variable immune deficiency (CVID) are associated with chronic rhinosinusitis. Good’s syndrome is characterized by hypogammaglobulinemia, B-cell depletion, variable defects in cellular immunity and thymoma. Immunodeficiency and recurrent infections can initially present after thymectomy. The pathogenesis can involve cytokines from bone marrow along with genetic defects. Intravenous gamma globulin (IVIG) restores defective signaling and can reestablish immune homeostasis. IVIG at regular intervals is the most effective way to improve the clinical symptoms and reduce patient mortality. Increased awareness of the clinical and immunological profile of this syndrome may increase its early recognition. CVID patients have hypogammaglobulinemia, respond to IVIG and have a dysregulated antimicrobial peptide response to pathogenic bacteria in the upper respiratory tract. This article reviewed selected literature on Good’s syndrome, described an unusual case of Good’s syndrome, CVID including SAD related to chronic rhinosinusitis.
Literature
1.
Good RA. Agammaglobulinemia. A provocative experiment of nature. Bull Univ Minn. 1954;26:1–19.
2.
Primary immunodeficiency diseases. Report of an IUI. Scientific Committee. International Union of Immunological Societies. Clin Exp Immunol 1999;118 (suppl 1) 1–28
3.
Tarr PE, Sneller MC, Mechanic IJ, et al. Infectious in patients with immunodeficiency with thymoma (Good syndrome). Report of 5 cases and review of the literature. Medicine. 2001;80:123–33.PubMedCrossRef
4.
5.
Dzhindzhikhashvili M, Absy-Jaghab M, Frieri M. Lymphadenopathy, productive cough, eosinophilia, and a new-onset acquired immunodeficiency syndrome. Allergy Asthma Proc. 2001;32(2):178–83.CrossRef
6.
Bouboulis DA, Rubinstein A, Shliozberg J, Madden J, Frieri M. Cerebral toxoplasmosis in childhood and adult HIV infection treated with 1–4 hydroxynaphthoquinone and rapid desensitization with pyrimethamine. Ann Allergy Asthma Immunol. 1995;74(6):491–4.PubMed
7.
Mansur A, Therattil J, Young RM, Frieri M. An atypical case of hypogammaglobulinemia. Ann Allergy Asthma Immunol. 2000;84(6):583–6.PubMedCrossRef
8.
Ternavasio-de la Vega HG, Velasco-Tirado V, Pozo-Rosado L et al. Persistence of immunological alterations after thymectomy in Good’s syndrome: a clue to its pathogenesis. Cytometry 2011; Part B.80B: 339–342.
9.
Oritani K, Hirota S, Nakagawa T, et al. T lymphocytes constitutively produce an interferon like cytokine limiting characterized as a heat- and acid-stable and heparin-binding glycoprotein. Blood. 2003;101:178–85.PubMedCrossRef
10.
Oritani K, Medina KL, Tomiyama Y, et al. Limitin: An interferon-like cytokine that preferentially influences B-lymphocyte precursors. Nat Med. 2000;6:659–66.PubMedCrossRef
11.
Cook MC, Tangye SG. Primary immune deficiencies affecting lymphocyte differentiation: lessons from the spectrum of resulting infections. Int Immunol. 2009;21(9):1003–11.PubMedCrossRef
12.
Degos L, Faille A, Housset M, et al. Syndrome of neutrophil agranulocytosis hypogammaglobulinemia, and thymoma. Blood. 1982;60:968–72.PubMed
13.
14.
15.
Lanio N, Sarmiento E, Gallego A, et al. Immunophenotypic profile of T cells in common variable immunodeficiency: is there an association with different clinical findings? Allergol Immunopathol (Madr). 2009;37:14–20.CrossRef
16.
17.••
Orange JS, Glessner JT, Resnick E, et al. Genome-wide association identifies diverse causes of common variable immunodeficiency. J Allergy Clin Immunol. 2011;127(6):1360–7. This excellent article addressed the underlying immunopathogenesis of CVID and comorbidities, and conducted the first genome-wide association and gene copy number variation (CNV) study in patients with CVID. It provided new mechanistic insights into immunopathogenesis based on these unique genetic variations and might allow for improved diagnosis of CVID based on accurate prediction of the CVID clinical phenotypes by using the Support Vector Machine model.PubMedCentralPubMedCrossRef
18.••
van de Ven AA, Compeer EB, van Montfrans JM, et al. B-cell defects in common variable immunodeficiency: BCR signaling, protein clustering and hardwired gene mutations. Crit Rev Immunol. 2011;31:85–98. This excellent article evaluated CD20 dissociation from the BCR during early B-cell activation that might contribute to the development of CVID disorders. The authors stated that CD20 dissociation from the BCR signalosome is pivotal to BCR-mediated calcium mobilization in the cytosol. And defects in CD20/BCR signalosome conformation might predispose to the spectrum of CVID disorders.PubMedCrossRef
19.
Park JH, Resnick ES, Cunningham-Rundles C. Perspectives on common variable immune deficiency. Ann N Y Acad Sci. 2012;1246:41–9.CrossRef
20.
Martinez-Gallo M, Radigan L, Belen Almejun M, et al. TACI mutations and impaired B-cell function in subjects with CVID and healthy heterozygotes. J Allergy Clin Immunol. 2013;131:468–76.PubMedCentralPubMedCrossRef
21.
Cederlund A, Oliver M, Rekha RS, et al. Impaired release of antimicrobial peptides into nasal fluid of hyper-IgE and CVID patients. Plos One. 2011;6(12):e29316.PubMedCentralPubMedCrossRef
22.
Kelesidis T, Yang O. Good's syndrome remains a mystery after 55 years: a systematic review of the scientific evidence. Clin Immunol. 2010;135(3):347–63.PubMedCrossRef
23.
Narayan A, Frieri M. CVID in SLE with hypogammaglobulinemia. Clinical Immunology Society (CIS), May 13, 2005.
24.
Frieri M. Mechanisms of disease for the clinician: systemic lupus erythematosus. Ann Allergy Asthma Immunol. 2013;110:228–32.PubMedCrossRef
25.
Ibrahim E, Horne N, Frieri M. CVID as an abdominal mass. American College of Physicians (ACP) poster session, 2013.
26.
Frieri M. Good’s syndrome. Acquired immunodeficiency with chronic sinusitis and thymoma. North American Phinology and Allergy Conference (NARAC), Puerto Rico, Abs. Jan, 2013.
27.
Martinez FJ, Simon RH. Clinical implications of macrolide therapy in chronic sinopulmonary diseases. Curr Pharm Des. 2004;10:3095–110.PubMedCrossRef
28.
Narayan AR, Huang YC, Wang XP, Goodwin L, Guida L, Frieri MF. Pseudomonas lipopolysaccharide and A. fumigatus stimulated TNFα, RANTES and nitric oxide from peripheral blood mononuclear cells from cystic fibrosis patients with bronchial epithelial cells. Modulation by rhDNAase. J Allergy Clin Immunol. 2005;115:S221a.CrossRef
29.
Singhal D, Baker L, Wormald PJ, et al. Aspergillus fumigatus biofilm on primary human sinonasal epithelial culture. Am J Rhinol Allergy. 2011;25(4):219–25.PubMedCrossRef
30.
Tsou YA, Chen CM, Lin TC et al. Decreased SPLUNC1 expression is associated with Pseudomonas infection in surgically treated chronic rhinosinusitis patients who may require repeated sinus surgery Laryngoscope. 2013.
31.
Brandt D, Gershwin ME. Common variable immunodeficiency and autoimmunity. Autoimmun Rev. 2006;7:465–70.CrossRef
32.
Frieri M. Complement-related diseases. Allergy Asthma Proc. 2002;23:319–24.PubMed
33.
Frieri M. Complement systems and allergic diseases. Chapter 23. In: Allergy & Asthma. Practical Diagnosis and Management. Ed: Massoud Mahmoudi. McGraw Hill, 2008;23:206–214.
34.
LaRosa DF, Patel CS. Clinical characteristics of adult patients with isolated low IgG and abnormal response to Pneumovax. J Allergy Clin Immunol. 2012;129(Issue 2, Supplement):AB83.
35.
Wilson SP, Ballas ZK. Progression of specific antibody deficiency (SAD) to common variable immunodeficiency (CVID). February. J Allergy Clin Immunol. 2010;125(Issue 2, Supplement 1):AB73.
36.
Frieri M. Chronic rhinosinusitis with an uncommon immune deficiency. North American Rhinology & Allergy Conference. Amer J Rhinology. abs. 2011.
37.••
Madeo J, Frieri M. Bacterial biofilms and chronic rhinosinusitis. Allergy Asthma Proc. 2013;34:335–41. This literature review focused on factors involved in biofilm formation and highlights the current research in this field. It discussed Staphylococcus aureus biofilms and exotoxins that act as superantigens and have been implicated in playing an important pathological role in the incidence, maintenance and ongoing burden of chronic rhinosinusitis. A better understanding of the interplay among bacterial factors, host factors and the environment will facilitate better management of this disease.PubMedCrossRef
38.
Paris K, Sorensen RU. Assessment and clinical interpretation of polysaccharide antibody responses. Ann Allergy Asthma Immunol. 2007;99(5):462–4.PubMedCrossRef
39.
Carr TF, Koterba AP, Chandra R, et al. Characterization of specific antibody deficiency in adults with medically refractory chronic rhinosinusitis. Am J Rhinol Allergy. 2011;25(4):241–4.PubMedCentralPubMedCrossRef
40.
Rizzi M, Neumann C, Feidling AK, et al. Outcome of allogeneic stem cell transplantation in adults with common variable immunodeficiency. Short Communication. J Allergy Clin Immunol. 2011;128(6):1371–4.PubMedCrossRef
41.
Sherkat R, Shoaei P, Parvaneh N, et al. Selective antibody deficiency and its relation to the IgG2 and IgG3 subclass titers in recurrent respiratory infections. Iran J Immunol. 2013;10(1):55–60.PubMed
42.
Gelfand EW, Ochs HD, Shearer WT. Controversies in IgG replacement therapy in patients with antibody deficiency diseases. J Allergy Clin Immunol. 2013;131:1001–5.PubMedCrossRef
43.
Frieri M. Chronic sinusitis and steroid unresponsive cough-induced asthma North American Rhinology and Allergy Conference, Puerto Rico, Abs. 2012.
44.
Frisella PD, Silverberg J, Joks R, Frieri M. Transforming growth factor-beta. Role in the upper airway and rhinosinusitis: D. Pteronyssinusis induced apoptosis with pulmonary alveolar cells. Am J Rhinol. 2011;25:231–5.CrossRef
45.
Lin DC, Chandra RK, Tan BK, et al. Association between severity of asthma and degree of chronic rhinosinusitis. Am J Rhinol Allergy. 2011;25(4):205–8.PubMedCentralPubMedCrossRef
46.•
Ocampo CJ, Peters AT. Antibody deficiency in chronic rhinosinusitis: epidemiology and burden of illness. Am J Rhinol Allergy. 2013;27(1):34–8. This paper reviewed four primary immunodeficiencies reported in individuals with chronic rhinosinusitis, common variable immune deficiency, selective IgA deficiency, IgG subclass deficiency and specific antibody deficiency. It also reviewed treatment options for individuals with both chronic rhinosinusitis and a concomitant immune defect.PubMedCentralPubMedCrossRef
Metadata
Title
Good’s Syndrome, CVID, and Selective Antibody Deficiency in Patients with Chronic Rhinosinusitis
Author
Marianne Frieri
Publication date
01-06-2014
Publisher
Springer US
Published in
Current Allergy and Asthma Reports / Issue 6/2014
Print ISSN: 1529-7322
Electronic ISSN: 1534-6315
DOI
https://doi.org/10.1007/s11882-014-0438-4

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