Infections in 252 Patients with Common Variable Immunodeficiency

Oksenhendler, Éric; Gérard, Laurence; Fieschi, Claire; Malphettes, Marion; Mouillot, Gaël; Jaussaud, R.; Viallard, Jean‐François; Gardembas, Martine; Galicier, Lionel; Schleinitz, N.; Suarez, Félipe; Soulas‐Sprauel, Pauline; Hachulla, É.; Jaccard, Arnaud; Gardeur, Anaëlle; Théodorou, Ioannis; Rabian, Claire; Debré, Patrice

doi:10.1086/587669

Cited by 412 publications

(370 citation statements)

References 23 publications

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“…In patients with primary or secondary immunodeficiency, replacement therapy with IgG effectively prevents severe systemic infection. However, despite appropriate IgG supplementation, frequently occurring chronic infection/inflammation of the respiratory and gastrointestinal mucosae has been linked to low serum IgA levels (42)(43)(44). The inherent structural features of SIgA would make it a logical agent to fill this therapeutic gap in combination with standard therapy (e.g.…”

Section: Discussionmentioning

confidence: 99%

Human Plasma-derived Polymeric IgA and IgM Antibodies Associate with Secretory Component to Yield Biologically Active Secretory-like Antibodies

Longet

Miled

Lötscher

et al. 2013

Journal of Biological Chemistry

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Background: Production of SIgA or SIgM for therapeutic application remains an unsolved issue. Results: Human plasma-derived polyclonal, polymeric IgA and IgM associate with recombinant or colostrum-derived human secretory component to form digestion-resistant, functionally active SIgA-and SIgM-like molecules. Conclusion: SIgA and SIgM can be rebuilt ex vivo from plasma-derived IgA/IgM. Significance: This would enable development of SIgA/SIgM-based mucosal therapeutics.

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Section: Discussionmentioning

confidence: 99%

Human Plasma-derived Polymeric IgA and IgM Antibodies Associate with Secretory Component to Yield Biologically Active Secretory-like Antibodies

Longet

Miled

Lötscher

et al. 2013

Journal of Biological Chemistry

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“…Some cases of transient or persistent diarrhea or malabsorption are due to bacterial, parasitic or viral infection (5). Gastrointestinal infections are more frequent in patients with absent IgA given their role in mucosal defense (19). However, inflammatory diseases are frequently observed.…”

Section: Cvid and Inflammationmentioning

confidence: 99%

Common variable immunodeficiency: Crossroads between infections, inflammation and autoimmunity

Baldovino

Montin

Martino

et al. 2013

Autoimmunity Reviews

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“…However, even some patients diagnosed more recently who had appropriate IgG concentrations developed this complication, as reported in the literature, suggesting that other factors must be involved. 3,[24][25][26][27][28] In this context, our study investigated possible factors related to the development of pulmonary complications in this cohort.…”

Section: Discussionmentioning

confidence: 99%

Primary hypogammaglobulinemia: The impact of early diagnosis in lung complications

Dorna

Santos

Castro

et al. 2016

Rev. Assoc. Med. Bras.

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Objective: To describe clinical features, tomographic findings and pulmonary function in pediatric patients with primary hypogammaglobulinemia (PH). Method: A retrospective cohort study of children with PH who received intravenous immunoglobulin (IVIG) and prophylactic antibiotics between 2005 and 2010. Epidemiological and clinical features, computed tomography (CT) findings, and spirometric data were compared, assuming a 5% significance level. Results:We evaluated 30 patients with PH. After the start of IVIG replacement, there was a decline in the frequency of pneumonia (p<0.001). The 11 patients with bronchiectasis in their first CT scan were older at diagnosis (p=0.001) and had greater diagnostic delay (p=0.001) compared to patients without bronchiectasis. At the end of the study, 18 patients had bronchiectasis and 27 also had other lung disorders, alone or in combination. The Bhalla score was applied to the last CT scan of 16 patients, with a median score of 11 (range 7-21), with a positive correlation between the score and the number of pneumonias after the start of treatment (r=0.561; p=0.024). The score was also correlated with forced expiratory volume in one second (FEV1) and forced vital capacity (FVC) values in 13/16 patients, with negative correlation to FEV1 previously to bronchodilator (r=-0.778; p=0.002) and after bronchodilator p=0.002). Conclusion: Pulmonary complications were common in this cohort, despite the decrease in the frequency of pneumonia with treatment. Early investigation of patients with recurrent infections for primary immunodeficiencies can reduce the frequency of these complications. The monitoring of changes in spirometry may indicate the need to carry out radiological investigation.

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Infections in 252 Patients with Common Variable Immunodeficiency

Abstract: Although reduced within the past decade, the delay of diagnosis of common variable immunodeficiency remains unacceptable. Recurrence of upper respiratory tract infection or pneumonia should lead to systematic evaluation of serum immunoglobulin.

Cited by 412 publications

References 23 publications

Human Plasma-derived Polymeric IgA and IgM Antibodies Associate with Secretory Component to Yield Biologically Active Secretory-like Antibodies

Human Plasma-derived Polymeric IgA and IgM Antibodies Associate with Secretory Component to Yield Biologically Active Secretory-like Antibodies

Common variable immunodeficiency: Crossroads between infections, inflammation and autoimmunity

Primary hypogammaglobulinemia: The impact of early diagnosis in lung complications

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