Our patient had recurrent upper respiratory infections and a number of asthmatic attacks despite control of environmental measures and appropriate medications.
Specific antibody deficiency with normal immunoglobulin levels is a primary immunodeficiency of unknown origin (1). It is characterized by normal concentrations of IgG, IgA, IgM, and IgG subclasses and abnormal specific IgG antibody responses which is associated with variable clinical spectrum of recurrent and/or severe respiratory tract infections (2, 3). Inability to produce specific antibodies may occur in other primary immunodeficiency disease, including X linked agammaglobulinaemia, Wiskott-Aldrich syndrome and ataxia telangiectasia (table 1). Treatment consists of daily antibiotic prophylaxis, immunoglobulin replacement and some patients may benefit from additional immunization with conjugated pneumococcal vaccines.
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Causes of selective antibody unresponsiveness |
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Primary Immunodeficiency |
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Specific antibody deficiency |
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X linked agammaglobulinaemia (btk mutation) |
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IgG subgroup deficiency |
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Common variable immunodeficiency |
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Wiskott-Aldrich syndrome |
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Ataxia telangiectasia |
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Secondary Immunodeficiency |
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Cytotoxic/myeloablative therapy |
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HIV infection |
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Chronic lymphotic leukemia |
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Multiple myeloma |
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After bone marrow transplantation |
The patient had a chronic sinusitis that was being followed-up since the age of 5 without any other severe infections. On follow up, bronchiectasis was detected and was evaluated accordingly (Table II). Kainulainen et al (4) reported that silent progression of pulmonary changes may occur in patients with primary immunodeficiency. Recently, a study was conducted on adults with idiopathic bronchiectasis and found a fraction of patients associated with selective anti-polysaccharide response deficiency (5), while Schwartz et al reported remarkable clinical benefits of IVIG on severe asthmatic patients who were found to have specific antibody deficiency (6). Other primary immunodeficiencies were not considered due to the normal findings of IgG/IgA and B-lymphocytes (CVID/ Bruton).
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CONDITIONS ASSOCIATED WITH BRONCHIECTASIS |
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Congential Conditions |
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Primary ciliary dyskinesia |
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Álpha 1- antitrypsin deficiency |
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Cystic fibrosis |
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Cartilage deficiency (Williams-Campbell syndrome) |
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Tracheobronchomegaly (Mounier-Kuhn syndrome) |
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Marfan’s syndrome |
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Immunodeficiency |
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Primary / Secondary hypogammaglobulinemia |
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Rheumatic Conditions |
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Rheumatoid arthritis |
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Systemic lupus erythematosus |
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Sjörgen’s syndrome |
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Relapsing polychondritis |
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Postinfections Conditions |
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Bacteria (Pseudomonas, haemophilus) |
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Mycobacterium tuberculosis |
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Aspergillus species |
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Virus (H?V, adenovirus, measles virus, influenzavirus) |
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Others |
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Inflammatory bowels disease |
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Young’s syndrome (secondary ciliary dyskinesia) |
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Yellow nail syndrome (yellow nails and lymphedema) |
Table 2
In conclusion, during the follow-up of children with asthma, when the symptoms persist despite a regular inhaler corticosteroid therapy, depending on characteristics of individual patients, quantitative and qualitative immunologic parameters as well as other confounding factors such as Cystic fibrosis, Ciliary dyskinesia, Gastro-eosophageal reflux etc. should be evaluated before proceeding to a stepping-up mode of therapy.
REFERENCES
(1) Ambrosino DM, Siber GR, Chilmonczyk BA, et al. An immunodeficiency characterized by impaired antibody responses to polysaccharides. N Engl J Med. 1987;316:790 –793.
(2) Sorensen RU, Moore C. Antibody deficiency syndromes. Pediatr Clin North Am. 2000;47:1225–1252.
(3) Rijkers GT, Sanders LA, Zegers BJ. Anti-capsular polysaccharide antibody deficiency states. Immunodeficiency. 1993;5(1):1-21.
(4) Kainulainen L, Varpula M, Liippo K, Svedstrom E, Nikoskelainen J, Ruuskanen O. Pulmonary abnormalities in patients with primary hypogammaglobulinemia. J Allergy Clin Immunol. 1999 Nov; 104(5):1031-6.
(5) Van Kessel DA, van Velzen-Blad H, van den Bosch JM, Rijkers GT. Impaired pneumococcal antibody response in bronchiectasis of unknown aetiology. Eur Respir J. 2005 Mar; 25(3):482-9.
(6) Schwartz HJ, Hostoffer RW, McFadden ER Jr, Berger M. The response to intravenous immunoglobulin replacement therapy in patients with asthma with specific antibody deficiency. Allergy Asthma Proc. 2006 Jan-Feb;27(1):53-8.
Key Words: Antibody response, Asthma, bronchiectasis, intravenouse immunoglobulins
Summary
Hereby we describe a case of asthma complicated by specific antibody deficiency progressing to unpleasant consequences by developing bronchiectasis. Consensus is there for recommendations and the recommendations should be tailored appropriately according to the priorities of individual patients.
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