Bronchiectasis in common variable immunodeficiency: A systematic review and meta‐analysis

Ramzi, Nasim; Jamee, Mahnaz; Bakhtiyari, Mahmood; Rafiemanesh, Hosein; Zainaldain, Hamed; Tavakol, Marzieh; Rezaei, Amir; Kalvandi, Mustafa; Zian, Zeineb; Mohammadi, Hamed; Jadidi‐Niaragh, Farhad; Yazdani, Reza; Abolhassani, Hassan; Aghamohammadi, Asghar; Azizi, Gholamreza

doi:10.1002/ppul.24599

Cited by 36 publications

(26 citation statements)

References 33 publications

(79 reference statements)

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“…Patients in our follow-up cohort had a strikingly low prevalence of lymphopenia, which may underlie the milder course of immunodeficiency and possibly explain the absence of bronchiectasis. The finding of higher prevalence of low IgM levels in patients with subtle bronchiectasis changes is in concordance with reported correlations in patients with common variable immunodeficiency and bronchiectasis [16].…”

Section: Discussionsupporting

confidence: 91%

Pulmonary Follow-Up Imaging in Cartilage-Hair Hypoplasia: a Prospective Cohort Study

et al. 2021

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Cartilage-hair hypoplasia is a syndromic immunodeficiency with short stature, chondrodysplasia, and variable degree of immune dysfunction. Patients with cartilage-hair hypoplasia are prone to recurrent respiratory tract infections, and the prevalence of bronchiectasis ranges from 29 to 52%. Pulmonary complications contribute significantly to the mortality; therefore, regular lung imaging is essential. However, the optimal schedule for repeated lung imaging remains unestablished. We determined the rate and correlates of progression of structural lung changes in a prospectively followed cohort of 16 patients with cartilage-hair hypoplasia. We analyzed clinical, laboratory, and pulmonary functional testing data and performed lung magnetic resonance imaging at a median interval of 6.8 years since previous imaging. Imaging findings remained identical or improved due to disappearance of inflammatory changes in all evaluated patients. Patients with subtle signs of bronchiectasis on imaging tended to have low immunoglobulin M levels, as well as suffered from pneumonia during the follow-up. In conclusion, our results suggest slow if any development of bronchiectasis in selected subjects with cartilage-hair hypoplasia.

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Section: Discussionsupporting

confidence: 91%

Pulmonary Follow-Up Imaging in Cartilage-Hair Hypoplasia: a Prospective Cohort Study

et al. 2021

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“…Therefore, the generalizability of the findings for the Manual Push Cohort in this study are limited to adults (aged ≥ 18 to 65 years) and normal-weight or obese individuals (BMI ≥ 19 kg/m 2 ). Finally, while the proportion of patients with bronchiectasis in this cohort (25%) compares well with the average proportion among patients with CVID in Europe (25%, range 0-66) [29], it is lower than the recently reported global average for patients with CVID (34%; 95% confidence interval: 30-38) [30]. Overall, the proportion of patients with bronchiectasis among patients with PID depends on many factors and shows great regional variability [31].…”

Section: Discussionsupporting

confidence: 45%

Safety and Tolerability of Manual Push Administration of Subcutaneous IgPro20 at High Infusion Rates in Patients with Primary Immunodeficiency: Findings from the Manual Push Administration Cohort of the HILO Study

et al. 2020

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Purpose To evaluate the safety and tolerability of IgPro20 manual push (also known as rapid push) infusions at flow rates of 0.5–2.0 mL/min. Methods Patients with primary immunodeficiency (PID) with previous experience administering IgPro20 (Hizentra®, CSL Behring, King of Prussia, PA, USA) were enrolled in the Hizentra® Label Optimization (HILO) study (NCT03033745) and assigned to Pump-assisted Volume Cohort, Pump-assisted Flow Rate Cohort, or Manual Push Flow Rate Cohort; this report describes the latter. Patients administered IgPro20 via manual push at 0.5, 1.0, and 2.0 mL/min/site for 4 weeks each. Responder rates (percentage of patients who completed a predefined minimum number of infusions), safety outcomes, and serum immunoglobulin G (IgG) trough levels were evaluated. Results Sixteen patients were treated; 2 patients (12.5%) discontinued at the 1.0-mL/min level (unrelated to treatment). Responder rates were 100%, 100%, and 87.5% at 0.5-, 1.0-, and 2.0-mL/min flow rates, respectively. Mean weekly infusion duration decreased from 103–108 to 23–28 min at the 0.5- and 2.0-mL/min flow rates, respectively. Rates of treatment-related treatment-emergent adverse events (TEAEs) per infusion were 0.023, 0.082, and 0.025 for the 0.5-, 1.0-, and 2.0-mL/min flow rates, respectively. Most TEAEs were mild local reactions and tolerability (infusions without severe local reactions/total infusions) was 100% across flow rate levels. Serum IgG levels (mean [SD]) were similar at study start (9.36 [2.53] g/L) and end (9.58 [2.12] g/L). Conclusions Subcutaneous IgPro20 manual push infusions at flow rates up to 2.0 mL/min were well tolerated and reduced infusion time in treatment-experienced patients with PID. Trial Registration NCT03033745

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“…Bacterial sinopulmonary infections are often the presenting feature, most frequently caused by Haemophilus influenzae or Streptococcus pneumoniae (11,12). Recurrent and/ or severe lower respiratory tract infections, particularly pneumonia, lead to bronchiectasis with an overall estimated prevalence of 30-35% among CVID patients, which, when present in isolation, does not contribute to increased mortality (8,(11)(12)(13)(14). Interstitial lung disease (ILD), on the other hand, probably occurs due to immune dysregulation and/or viral infection rather than as a consequence of bacterial infection (7,15,16), and occurs alongside other diseaserelated complications, and shortens survival (7)(8)(9)16).…”

Section: Introductionmentioning

confidence: 99%

Histology of Interstitial Lung Disease in Common Variable Immune Deficiency

et al. 2020

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Interstitial lung disease (ILD) is an important non-infectious complication in several primary immune deficiencies. In common variable immune deficiency (CVID) it is associated with complex clinical phenotypes and adverse outcomes. The histology of ILD in CVID is heterogeneous and mixed patterns are frequently observed within a single biopsy, including non-necrotising granulomatous inflammation, lymphoid interstitial pneumonitis, lymphoid hyperplasia, follicular bronchiolitis, organizing pneumonia, and interstitial fibrosis; ILD has to be differentiated from lymphoma. The term granulomatous-lymphocytic interstitial lung disease (GLILD), coined to describe the histopathological findings within the lungs of patients with CVID with or without multisystem granulomata, is somewhat controversial as pulmonary granulomata are not always present on histology and the nature of infiltrating lymphocytes is variable. In this mini review we summarize the literature on the histology of CVID-related ILD and discuss some of the factors that may contribute to the inter- and intra- patient variability in the histological patterns reported. Finally, we highlight areas for future development. In particular, there is a need for standardization of histological assessments and reporting, together with a better understanding of the immunopathogenesis of CVID-related ILD to resolve the apparent heterogeneity of ILD in this setting and guide the selection of rational targeted therapies in different patients.

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Bronchiectasis in common variable immunodeficiency: A systematic review and meta‐analysis

Cited by 36 publications

References 33 publications

Pulmonary Follow-Up Imaging in Cartilage-Hair Hypoplasia: a Prospective Cohort Study

Pulmonary Follow-Up Imaging in Cartilage-Hair Hypoplasia: a Prospective Cohort Study

Safety and Tolerability of Manual Push Administration of Subcutaneous IgPro20 at High Infusion Rates in Patients with Primary Immunodeficiency: Findings from the Manual Push Administration Cohort of the HILO Study

Histology of Interstitial Lung Disease in Common Variable Immune Deficiency

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