Bronchoalveolar lavage cytology resembles sarcoidosis in a subgroup of granulomatous CVID

Naccache, Jean‐Marc; Bouvry, Diane; Valeyre, Dominique

doi:10.1183/09031936.00170013

Cited by 10 publications

(8 citation statements)

References 4 publications

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“…Broncho-alveolar lavage is routinely used in GLILD workup for microbiological differential diagnosis. However, BALF findings might also provide data on the different pathogenetic mechanisms and patients' prognosis (18,33). Thus, we may hypothesize that a more widespread use of BALF analysis and uniformed lymphocyte phenotyping might help to dissect the ongoing lung inflammatory processes (e.g., presence of a CD4+ alveolitis, B cell increase and activation, mediators potentially acting as activity biomarkers) and to potentially define tailored treatments that, at present, are provided only by histologic evaluation.…”

Section: Discussionmentioning

confidence: 99%

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Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) in Common Variable Immunodeficiency (CVID): A Multicenter Retrospective Study of Patients From Italian PID Referral Centers

et al. 2021

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Background: Granulomatous and Lymphocytic Interstitial Lung Diseases (GLILD) is a severe non-infectious complication of Common Variable Immunodeficiency (CVID), often associated with extrapulmonary involvement. Due to a poorly understood pathogenesis, GLILD diagnosis and management criteria still lack consensus. Accordingly, it is a relevant cause of long-term loss of respiratory function and is closely associated with a markedly reduced survival. The aim of this study was to describe clinical, immunological, laboratory and functional features of GLILD, whose combination in a predictive model might allow a timely diagnosis.Methods: In a multicenter retrospective cross-sectional study we enrolled 73 CVID patients with radiologic features of interstitial lung disease (ILD) associated to CVID (CVID-ILD) and 125 CVID patients without ILD (controls). Of the 73 CVID-ILD patients, 47 received a definite GLILD diagnosis while 26 received a clinical-radiologic diagnosis of CVID related ILD defined as uILD.Results: In GLILD group we found a higher prevalence of splenomegaly (84.8 vs. 39.2%), autoimmune cytopenia (59.6 vs. 6.4%) and bronchiectasis (72.3 vs. 28%), and lower IgA and IgG serum levels at CVID diagnosis. GLILD patients presented lower percentage of switched-memory B cells and marginal zone B cells, and a marked increase in the percentage of circulating CD21lo B cells (14.2 vs. 2.9%). GLILD patients also showed lower total lung capacity (TLC 87.5 vs. 5.0%) and gas transfer (DLCO 61.5 vs. 5.0%) percent of predicted. By univariate logistic regression analysis, we found IgG and IgA levels at CVID diagnosis, presence of splenomegaly and autoimmune cytopenia, CD21lo B cells percentage, TLC and DCLO percent of predicted to be associated to GLILD. The joint analysis of four variables (CD21lo B cells percentage, autoimmune cytopenia, splenomegaly and DLCO percent of predicted), together in a multiple logistic regression model, yielded an area under the ROC curve (AUC) of 0.98 (95% CI: 0.95-1.0). The AUC was only slightly modified when pooling together GLILD and uILD patients (0.92, 95% CI: 0.87-0.97).Conclusions: we propose the combination of two clinical parameters (splenomegaly and autoimmune cytopenia), one lung function index (DLCO%) and one immunologic variable (CD21lo%) as a promising tool for early identification of CVID patients with interstitial lung disease, limiting the use of aggressive diagnostic procedures.

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Section: Discussionmentioning

confidence: 99%

“…In addition, the introduction of MRI may represent a reliable radiation-free technique for diagnosis and follow-up of GLILD patients (16,17), associated with evaluation of the bronchoalveolar lavage (BAL) in terms of GLILD related markers such as inflammatory cytokines and lymphocyte subsets (18).…”

Section: Introductionmentioning

confidence: 99%

Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) in Common Variable Immunodeficiency (CVID): A Multicenter Retrospective Study of Patients From Italian PID Referral Centers

et al. 2021

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“…CD21low B-cells have also been claimed as the dominant cells in the BALF of patients diagnosed with GLILD [96]. These findings may suggest possibly distinct pathogenic mechanisms of GLILD in different patients, which might derive from diverse triggers and be correlated to heterogeneous clinical and prognostic phenotypes [113,114]. Further work is needed, however, to elucidate the contribution of these lymphocyte sub-populations to the development of ILD and to understand whether flow-cytometric analysis of BALF cells may have any diagnostic or prognostic value, in the absence of histological examination, as it is for sarcoidosis [28,115].…”

Section: Glildmentioning

confidence: 91%

“…Bronchoalveolar lavage fluid (BALF) cytology in adult CVID patients with a diagnosis of GLILD has been described as lymphocyte enriched (>20%). Both an increased and a normal CD4/CD8 ratio of BALF lymphocytes have been reported [105,113,114]. CD21low B-cells have also been claimed as the dominant cells in the BALF of patients diagnosed with GLILD [96].…”

Section: Glildmentioning

confidence: 99%

The broad spectrum of lung diseases in primary antibody deficiencies

et al. 2018

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Human primary immunodeficiency diseases (PIDs) represent a heterogeneous group of more than 350 disorders. They are rare diseases, but their global incidence is more relevant than generally thought. The underlying defect may involve different branches of the innate and/or adaptive immune response. Thus, the clinical picture may range from severe phenotypes characterised by a broad spectrum of infections to milder infectious phenotypes due to more selective (and frequent) immune defects. Moreover, infections may not be the main clinical features in some PIDs that might present with autoimmunity, auto-inflammation and/or cancer. Primary antibody deficiencies (PADs) represent a small percentage of the known PIDs but they are the most frequently diagnosed, particularly in adulthood. Common variable immunodeficiency (CVID) is the most prevalent symptomatic PAD.PAD patients share a significant susceptibility to respiratory diseases that represent a relevant cause of morbidity and mortality. Pulmonary complications include acute and chronic infection-related diseases, such as pneumonia and bronchiectasis. They also include immune-mediated interstitial lung diseases, such as granulomatous-lymphocytic interstitial lung disease (GLILD) and cancer. Herein we will discuss the main pulmonary manifestations of PADs, the associated functional and imaging findings, and the relevant role of pulmonologists and chest radiologists in diagnosis and surveillance.

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“…It has been suggested that GLILD may be associated with a BAL lymphocytosis (>20%) in the majority of patients [52,53] , but whether further analysis of cells retrieved at BAL is able to avoid surgical biopsy has yet to be properly examined. The CD4/CD8 preponderance has varied between studies, and a higher ratio has been associated with a more favourable prognosis in terms of lung function decline in one study [53] , but not in another [54] . It is recognised that abnormalities present in blood lymphocyte populations in CVID may also be present in BAL [55] , but in general there is a paucity of data on lung lymphocyte populations in CVID One study [53] included an assessment with trans-bronchial biopsy but this was not adequate to make a firm diagnosis in over 2/3 of patients.…”

Section: Figure 5: Histology Of Glild Demonstrating a Polymorphous Inmentioning

confidence: 99%

Lung disease in primary antibody deficiency

Verma

Grimbacher

Hurst

2015

The Lancet Respiratory Medicine

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This Review summarises current knowledge on the pulmonary manifestations of primary antibody deficiency (PAD) syndromes in adults. We describe the major PAD syndromes, with a particular focus on common variable immunodeficiency (CVID). Respiratory infection is a common presenting feature of PAD syndromes. Respiratory complications are frequent and responsible for much of the morbidity and mortality associated with these syndromes. Respiratory complications include acute infections, the sequelae of infection (eg, bronchiectasis), non-infectious immune-mediated manifestations (notably the development of granulomatous-lymphocytic interstitial lung disease in CVID), and an increased risk of lymphoma. Although minor abnormalities are detectable in the lungs of most patients with CVID by CT scanning, not all patients develop lung complications. Mechanisms associated with the maintenance of lung health versus lung disease, and the development of bronchiectasis versus immune-mediated complications, are now being dissected. We review the investigation, treatment, and management strategies for PAD syndromes, and include key research questions relating to both infectious and non-infectious complications of PAD in the lung.

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Bronchoalveolar lavage cytology resembles sarcoidosis in a subgroup of granulomatous CVID

Cited by 10 publications

References 4 publications

Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) in Common Variable Immunodeficiency (CVID): A Multicenter Retrospective Study of Patients From Italian PID Referral Centers

Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) in Common Variable Immunodeficiency (CVID): A Multicenter Retrospective Study of Patients From Italian PID Referral Centers

The broad spectrum of lung diseases in primary antibody deficiencies

Lung disease in primary antibody deficiency

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