Granulomatous–lymphocytic interstitial lung disease: an international research prioritisation

Hurst, John R.; Abbas, Syedda; Bintalib, Heba M.; Alfaro, Tiago M.; Baumann, Ulrich; Burns, Siobhan O.; Condliffe, Alison M.; Davidsen, Jesper Rømhild; Fevang, Børre; Gennery, Andrew R.; Haerynck, Filomeen; Jacob, Joseph; Jolles, Stephen; Lamers, Olivia A. C.; Bergeron, Anne; Malphettes, Marion; Meignin, Véronique; Milito, Cinzia; Milota, Tomáš; Pergent, Martine; Prasse, Antje; Quinti, Isabella; Renzoni, Elisabetta; Šedivá, Anna; Stolz, Daiana; Smits, Bas M.; Strauss, Friedolin; Ven, Annick A. J. M. van de; Montfrans, Joris van; Warnatz, Klaus

doi:10.1183/23120541.00467-2021

Cited by 6 publications

(3 citation statements)

References 6 publications

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“…The superiority of first-line treatment over watchful waiting was one of the top-ranked questions in a recent research prioritisation exercise in GLILD [ 14 ]. We are the first complete, single-centre cohort reporting the change in lung function over time for GLILD patients who were not receiving immunosuppression treatment.…”

Section: Discussionmentioning

confidence: 99%

Corticosteroid-induced remission and mycophenolate maintenance therapy in granulomatous lymphocytic interstitial lung disease: long-term, longitudinal change in lung function in a single-centre cohort

et al. 2022

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AimThe aim of the study was to evaluate the response in lung function to different treatment regimens for common variable immunodeficiency patients with granulomatous lymphocytic interstitial lung disease (GLILD).MethodA longitudinal retrospective cohort study was carried out. Patients were divided into three groups. To assess the response to different treatments, we compared baseline lung function with post-treatment tests.Results14 patients with GLILD were included, seven of whom were treated with acute corticosteroids for a mean duration of 132±65 days. Spirometry results were unchanged, but there was a significant improvement in diffusing capacity of the lung for carbon monoxide (DLCO)% and transfer coefficient of the lung for carbon monoxide (KCO)% (median change inDLCO%=7%, p=0.04, andKCO%=13%, p=0.02). Relapse occurred in three out of seven patients. Five patients were treated with long-term mycophenolate mofetil (MMF) with/without corticosteroids for a mean duration of 1277±917 days. No changes were found in spirometry; however, there was a significant increase inDLCO% andKCO% (median change in each ofDLCO% andKCO%=10%, p=0.04). Four patients on steroids with MMF successfully weaned the prednisone dose over 12 months. Four patients never received immunosuppression therapy. A significant decline was found in their lung function assessed over 7.5 years. The median reduction in the forced vital capacity (FVC)%, forced expiratory volume in 1 s (FEV1)% andDLCO% was 15%, 7% and 15%, equivalent to 2%, 1% and 2% per year, respectively.ConclusionCorticosteroids improve gas transfer in GLILD, but patients often relapse. The use of MMF was associated with long-term effectiveness in GLILD and permits weaning of corticosteroids. A delay in initiating and continuing maintenance treatment could lead to disease progression.

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Section: Discussionmentioning

confidence: 99%

Corticosteroid-induced remission and mycophenolate maintenance therapy in granulomatous lymphocytic interstitial lung disease: long-term, longitudinal change in lung function in a single-centre cohort

et al. 2022

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“…CVID-ILDs share clinical and histological characteristics with other conditions, and there is currently no single consensus on the diagnostic criteria for CVID-ILD. The understanding of pathogenesis is limited, and significant gaps in knowledge about diagnosis and management remain ( 5 , 7 ). No evidence-based guideline for diagnosis or treatment is currently available, and management generally relies on clinicians’ expert opinions ( 7 , 8 ).…”

Section: Introductionmentioning

confidence: 99%

“…The understanding of pathogenesis is limited, and significant gaps in knowledge about diagnosis and management remain ( 5 , 7 ). No evidence-based guideline for diagnosis or treatment is currently available, and management generally relies on clinicians’ expert opinions ( 7 , 8 ).…”

Section: Introductionmentioning

confidence: 99%

Diagnostic testing for interstitial lung disease in common variable immunodeficiency: a systematic review

et al. 2023

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IntroductionCommon variable immunodeficiency related interstitial lung disease (CVID-ILD, also referred to as GLILD) is generally considered a manifestation of systemic immune dysregulation occurring in up to 20% of people with CVID. There is a lack of evidence-based guidelines for the diagnosis and management of CVID-ILD.AimTo systematically review use of diagnostic tests for assessing patients with CVID for possible ILD, and to evaluate their utility and risks.MethodsEMBASE, MEDLINE, PubMed and Cochrane databases were searched. Papers reporting information on the diagnosis of ILD in patients with CVID were included.Results58 studies were included. Radiology was the investigation modality most commonly used. HRCT was the most reported test, as abnormal radiology often first raised suspicion of CVID-ILD. Lung biopsy was used in 42 (72%) of studies, and surgical lung biopsy had more conclusive results compared to trans-bronchial biopsy (TBB). Analysis of broncho-alveolar lavage was reported in 24 (41%) studies, primarily to exclude infection. Pulmonary function tests, most commonly gas transfer, were widely used. However, results varied from normal to severely impaired, typically with a restrictive pattern and reduced gas transfer.ConclusionConsensus diagnostic criteria are urgently required to support accurate assessment and monitoring in CVID-ILD. ESID and the ERS e-GLILDnet CRC have initiated a diagnostic and management guideline through international collaboration.Systematic review registrationhttps://www.crd.york.ac.uk/prospero/, identifier CRD42022276337.

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Common and Uncommon CT Findings in CVID-Related GL-ILD: Correlations with Clinical Parameters, Therapeutic Decisions and Potential Implications in the Differential Diagnosis

Scarpa,

Cinetto,

Milito

et al. 2023

J Clin Immunol

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Purpose To investigate computed tomography (CT) findings of Granulomatous Lymphocytic Interstitial Lung Disease (GL-ILD) in Common Variable Immunodeficiency (CVID), also in comparison with non-GL-ILD abnormalities, correlating GL-ILD features with functional/immunological parameters and looking for GL-ILD therapy predictive elements. Methods CT features of 38 GL-ILD and 38 matched non-GL-ILD subjects were retrospectively described. Correlations of GL-ILD features with functional/immunological features were assessed. A logistic regression was performed to find a predictive model of GL-ILD therapeutic decisions. Results Most common GL-ILD CT findings were bronchiectasis, non-perilymphatic nodules, consolidations, Ground Glass Opacities (GGO), bands and enlarged lymphnodes. GL-ILD was usually predominant in lower fields. Multiple small nodules (≤10 mm), consolidations, reticulations and fibrotic ILD are more indicative of GL-ILD. Bronchiectasis, GGO, Reticulations and fibrotic ILD correlated with decreased lung performance. Bronchiectasis, GGO and fibrotic ILD were associated with low IgA levels, whereas high CD4+ T cells percentage was related to GGO. Twenty out of 38 patients underwent GL-ILD therapy. A model combining Marginal Zone (MZ) B cells percentage, IgA levels, lower field consolidations and lymphnodes enlargement showed a good discriminatory capacity with regards to GL-ILD treatment. Conclusions GL-ILD is a lower field predominant disease, commonly characterized by bronchiectasis, non-perilymphatic small nodules, consolidations, GGO and bands. Multiple small nodules, consolidations, reticulations and fibrotic ILD may suggest the presence of GL-ILD in CVID. MZ B cells percentage, IgA levels at diagnosis, lower field consolidations and mediastinal lymphnodes enlargement may predict the need of a specific GL-ILD therapy.

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Granulomatous–lymphocytic interstitial lung disease: an international research prioritisation

Cited by 6 publications

References 6 publications

Corticosteroid-induced remission and mycophenolate maintenance therapy in granulomatous lymphocytic interstitial lung disease: long-term, longitudinal change in lung function in a single-centre cohort

Corticosteroid-induced remission and mycophenolate maintenance therapy in granulomatous lymphocytic interstitial lung disease: long-term, longitudinal change in lung function in a single-centre cohort

Diagnostic testing for interstitial lung disease in common variable immunodeficiency: a systematic review

Common and Uncommon CT Findings in CVID-Related GL-ILD: Correlations with Clinical Parameters, Therapeutic Decisions and Potential Implications in the Differential Diagnosis

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