Lung disease in primary antibody deficiency

Verma, Nisha; Grimbacher, Bodo; Hurst, John R.

doi:10.1016/s2213-2600(15)00202-7

Cited by 88 publications

(76 citation statements)

References 68 publications

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“…Additionally, patients with primary immunodeficiency disorder, particularly those suffering from predominantly antibody deficiencies, should be regularly screened for bronchiectasis using high-resolution computed tomography. 34 Asthma affected only 11% of our cohort, whereas its prevalence in our population was found to exceed 15%. 35 This could be due to underreporting of symptoms or underdiagnosis.…”

Section: Discussioncontrasting

confidence: 53%

Sinopulmonary Complications in Subjects With Primary Immunodeficiency

Owayed

Al‐Herz

2016

Respir Care

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BACKGROUND: The aim of this work was to describe the frequency and spectrum of sinopulmonary complications among subjects with primary immunodeficiency disorders. METHODS: The subjects included all patients with primary immunodeficiency who were registered prospectively between January 2004 and December 2013 in the Kuwait National Primary Immunodeficiency Disorders Registry. RESULTS: A total of 202 subjects were registered during the study period. Subjects with combined immunodeficiencies were the most prevalent (65 subjects, 32.1%), followed by well-defined syndromes with immunodeficiency (45 subjects, 22.2%) and predominantly antibody deficiencies (35 subjects, 17.3%). A total of 295 sinopulmonary manifestations were observed in 127 subjects (63%); 157 manifestations (53.2%) were observed among the presenting symptoms, and 138 manifestations (46.8%) occurred after establishment of the primary immunodeficiency disorder diagnosis. Sinopulmonary manifestations were more common in subjects with predominantly antibody deficiencies (2.3 manifestations/subject), followed by subjects with combined immunodeficiencies (1.75 manifestations/subject). Pneumonia was the most common manifestation (108 episodes affecting 80 subjects), followed by otitis media (81 episodes affecting 59 subjects), bronchiectasis in 28 subjects (13.8%), and asthma in 22 subjects (11%). Microbial organisms were isolated during 46 episodes of pneumonia (42.5%) (cytomegalovirus and Pneumocystis jirovecii were the most common). There were 57 deaths (28%) during the study period. Twenty-four deaths (42%) were due to pulmonary complications as follows: pneumonia (16 subjects, 8%), pulmonary hemorrhage (6 subjects, 3%), and aspiration pneumonia (2 subjects, 1%). CONCLUSIONS: Sinopulmonary complications are common in subjects with primary immunodeficiency. They can be serious and continue to occur even after proper treatment is initiated. The pulmonologist should play an important role in the management of subjects with primary immunodeficiency disorder.

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Section: Discussioncontrasting

confidence: 53%

Sinopulmonary Complications in Subjects With Primary Immunodeficiency

Owayed

Al‐Herz

2016

Respir Care

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“…16 It is one of the major long-term clinical complications in patients with SAD and CVID. 17 Bronchiectasis was present in 50% of the patients with SAD and CVID but none of our patients with AT developed bronchiectasis. Recurrent lower respiratory tract infections are common in AT and can be associated with the development of bronchiectasis.…”

Section: Volume 59 • Number 5 Infection Profiles Of Turkish Patients mentioning

confidence: 62%

Defective pneumococcal antibody response in patients with recurrent respiratory tract infections

Erman¹,

Demirtaş

Bildik

et al. 2017

Turk J Pediatr

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Streptococcus pneumoniae is a common pathogen responsible for pulmonary infections and the leading cause of mortality and morbidity in patients with particularly B cell immunodeficiencies. Antibody production is the principal protective immune response against S. pneumoniae and measurement of the production of antipolysaccharide antibodies is important in the evaluation of B cell deficiencies. We quantified serotype-specific immunoglobulin G antibodies against seven common pneumococcal serotypes before and three weeks after unconjugated vaccine in 416 patients with recurrent respiratory tract infections; fifty-five (13%) of whom showed impaired antibody response. We could evaluate 41 of these 55 patients for their particular clinical features. Specific antibody deficiency, was diagnosed in 10 of these patients, common variable immunodeficiency in 18, ataxia telangiectasia in 10 and other antibody deficiencies in 7 (transient hypogammaglobulinemia in 4, IgG subclass deficiency in 1, partial and selective IgA deficiency in 1) patients. Evaluation of the antibody response to polysaccharide antigens should be considered early on in patients with recurrent respiratory infections and required particularly for the diagnosis of specific antibody deficiency and the decision of the appropriate treatment approaches.

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“…Immune thrombocytopenia or hemolytic anemia, splenomegaly, polyarthritis, a decrease in switched memory B cells, high blood CD4/CD8 T cell ratio, and high serum IgM may help identify patients with GLILD [21,32,34,45]. PFTs and thoracic CT at baseline plus every year for PFTs and every 5 years for CT are recommended in CVID-ILD [7,46]. 18F FDG-PET/CT shows an increased uptake in lungs or extrathoracic organs [47].…”

Section: Lung Manifestations In Common Variable Immunodeficiencymentioning

confidence: 99%

“…Immunosuppressive agents expose to infectious and malignant diseases. Monitoring corticosteroid-induced CD4 lymphopenia and pneumocystis prophylaxis are recommended [46]. Despite weak evidence of efficacy, corticosteroids may be used as first-line therapy [38].…”

Section: Lung Manifestations In Common Variable Immunodeficiencymentioning

confidence: 99%

The Lung in Dysregulated States of Humoral Immunity

Uzunhan¹,

Jeny²,

Kambouchner³

et al. 2017

Respiration

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In common variable immunodeficiency, lung manifestations are related to different mechanisms: recurrent pneumonias due to encapsulated bacteria responsible for diffuse bronchiectasis, diffuse infiltrative pneumonia with various patterns, and lymphomas, mostly B cell extranodal non-Hodgkin type. The diagnosis relies on significant serum Ig deficiency and the exclusion of any primary or secondary cause. Histopathology may be needed. Immunoglobulin (IgG) replacement is crucial to prevent infections and bronchiectasis. IgG4-related respiratory disease, often associated with extrapulmonary localizations, presents with solitary nodules or masses, diffuse interstitial lung diseases, bronchiolitis, lymphadenopathy, and pleural or pericardial involvement. Diagnosis relies on international criteria including serum IgG4 dosage and significantly increased IgG4/IgG plasma cells ratio in pathologically suggestive biopsy. Respiratory amyloidosis presents with tracheobronchial, nodular, and cystic or diffuse interstitial lung infiltration. Usually of AL (amyloid light chain) subtype, it may be localized or systemic, primary or secondary to a lymphoproliferative process. Very rare other diseases due to nonamyloid IgG deposits are described. Among the various lung manifestations of dysregulated states of humoral immunity, this article covers only those associated with the common variable immunodeficiency, IgG4-related disease, amyloidosis, and pulmonary light-chain deposition disease. Autoimmune connective-vascular tissue diseases or lymphoproliferative disorders are addressed in other chapters of this issue.

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Lung disease in primary antibody deficiency

Cited by 88 publications

References 68 publications

Sinopulmonary Complications in Subjects With Primary Immunodeficiency

Sinopulmonary Complications in Subjects With Primary Immunodeficiency

Defective pneumococcal antibody response in patients with recurrent respiratory tract infections

The Lung in Dysregulated States of Humoral Immunity

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