Assessment of Treatment Response in Granulomatous Lymphocytic Interstitial Lung Disease (GLILD)

Gkrepi, Georgia; Lowe, David M.; Burns, Siobhan O.; Seneviratne, Suranjith L.; Hurst, John R.

doi:10.1183/13993003.congress-2019.pa1408

Cited by 2 publications

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“…S avard- H eppel and B oursiquot [ 55 ] analysed the respiratory profile of common variable immunodeficiency (CVID) patients and, among them, granulomatous-lymphocytic ILD (GLILD) patients. In a series of nine patients already on immunoglobulin replacement, add-on therapy with prednisolone improved lung function with heterogeneous responses [ 56 ]. Compared to CVID patients without ILD, GLILD was associated with lower baseline IgA levels, and a nonsmoker status [ 57 ].…”

Section: Sarcoidosis and Other Granulomatous Ilds/dpldsmentioning

confidence: 99%

ERS International Congress, Madrid, 2019: highlights from the Interstitial Lung Diseases Assembly

et al. 2020

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This article discusses a selection of the scientific presentations in the field of interstitial lung diseases (ILDs) that took place at the 2019 European Respiratory Society International Congress in Madrid, Spain. There were sessions from all four groups within Assembly 12: group 12.01 “Idiopathic interstitial pneumonias”, group 12.02 “ILDs/diffuse parenchymal lung diseases (DPLDs) of known origin”, group 12.03 “Sarcoidosis and other granulomatous ILDs/DPLDs” and group 12.04 “Rare ILDs/DPLDs”. The presented studies brought cutting-edge developments on several aspects of these conditions, including pathogenesis, diagnosis and treatment. As many of the ILDs are individually rare, the sharing of experiences and new data that occur during the Congress are very important for physicians interested in ILDs and ILD patients alike.

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Section: Sarcoidosis and Other Granulomatous Ilds/dpldsmentioning

confidence: 99%

ERS International Congress, Madrid, 2019: highlights from the Interstitial Lung Diseases Assembly

et al. 2020

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“…As with many inflammatory conditions, corticosteroids are often the first choice for remission induction in GLILD. Corticosteroids often result in an improvement in GLILD, however following prednisolone therapy of 1-4 months a widely heterogeneous response was observed, as many patients do not exhibit any improvements in PFTs or had disease flares upon tapering of corticosteroid medication (16). Collectively, these findings define the need for re-evaluation of corticosteroid monotherapy as firstline treatment.…”

Section: Introductionmentioning

confidence: 99%

Managing Granulomatous–Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency Disorders: e-GLILDnet International Clinicians Survey

et al. 2020

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BackgroundGranulomatous–lymphocytic interstitial lung disease (GLILD) is a rare, potentially severe pulmonary complication of common variable immunodeficiency disorders (CVID). Informative clinical trials and consensus on management are lacking.AimsThe European GLILD network (e-GLILDnet) aims to describe how GLILD is currently managed in clinical practice and to determine the main uncertainties and unmet needs regarding diagnosis, treatment and follow-up.MethodsThe e-GLILDnet collaborators developed and conducted an online survey facilitated by the European Society for Immunodeficiencies (ESID) and the European Respiratory Society (ERS) between February–April 2020. Results were analyzed using SPSS.ResultsOne hundred and sixty-one responses from adult and pediatric pulmonologists and immunologists from 47 countries were analyzed. Respondents treated a median of 27 (interquartile range, IQR 82–maximum 500) CVID patients, of which a median of 5 (IQR 8–max 200) had GLILD. Most respondents experienced difficulties in establishing the diagnosis of GLILD and only 31 (19%) had access to a standardized protocol. There was little uniformity in diagnostic or therapeutic interventions. Fewer than 40% of respondents saw a definite need for biopsy in all cases or performed bronchoalveolar lavage for diagnostics. Sixty-six percent used glucocorticosteroids for remission-induction and 47% for maintenance therapy; azathioprine, rituximab and mycophenolate mofetil were the most frequently prescribed steroid-sparing agents. Pulmonary function tests were the preferred modality for monitoring patients during follow-up.ConclusionsThese data demonstrate an urgent need for clinical studies to provide more evidence for an international consensus regarding management of GLILD. These studies will need to address optimal procedures for definite diagnosis and a better understanding of the pathogenesis of GLILD in order to provide individualized treatment options. Non-availability of well-established standardized protocols risks endangering patients.

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Assessment of Treatment Response in Granulomatous Lymphocytic Interstitial Lung Disease (GLILD)

Cited by 2 publications

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ERS International Congress, Madrid, 2019: highlights from the Interstitial Lung Diseases Assembly

ERS International Congress, Madrid, 2019: highlights from the Interstitial Lung Diseases Assembly

Managing Granulomatous–Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency Disorders: e-GLILDnet International Clinicians Survey

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