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

AAJM. van de Ven, TM. Alfaro, A. Robinson, U. Baumann, A. Bergeron, SO. Burns, AM. Condliffe, B. Fevang, AR. Gennery, F. Haerynck, J. Jacob, S. Jolles, M. Malphettes, V. Meignin, T. Milota, J. van Montfrans, A. Prasse, I. Quinti, E. Renzoni, D....

. 2020 ; 11 (-) : 606333. [pub] 20201126

Jazyk angličtina Země Švýcarsko

Typ dokumentu časopisecké články, multicentrická studie, práce podpořená grantem

Perzistentní odkaz   https://www.medvik.cz/link/bmc21019794

Background: Granulomatous-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. Aims: The 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. Methods: The 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. Results: One 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. Conclusions: These 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.

Center for Chronic Immunodeficiency Medical Center University of Freiburg Faculty of Medicine University of Freiburg Freiburg Germany

Centre for Medical Image Computing University College London London United Kingdom

Centre for Rare Disorders and Section of Clinical Immunology and Infectious Diseases Oslo University Hospital Oslo Norway

Clinic for Respiratory Medicine and Pulmonary Cell Research University Hospital Basel Basel Switzerland

Department of Clinical Immunology Hôpital Saint Louis Assistance Publique Hôpitaux de Paris Université Paris Diderot Paris France

Department of Immunology 2nd Faculty of Medicine Charles University and Motol University Hospital Prague Czech Republic

Department of Infection Immunity and Cardiovascular Diseases University of Sheffield Medical School Sheffield United Kingdom

Department of Molecular Medicine Sapienza University of Rome Rome Italy

Department of Paediatric Pulmonology Allergy and Neonatology Hannover Medical School Hannover Germany

Department of Pathology Hôpital Saint Louis Assistance Publique Hôpitaux de Paris Paris France

Department of Pediatric Immunology and Infectious Diseases University Medical Center Utrecht Utrecht Netherlands

Department of Pediatric Pulmonology and Immunology Centre for Primary Immune deficiency Ghent PID research lab Ghent University Hospital Belgium

Department of Pulmonology Hannover Medical School and DZL BREATH and Fraunhofer Institute for Toxicology and Experimental Medicine Hannover Germany

Department of Rheumatology and Clinical Immunology Medical Center University of Freiburg Faculty of Medicine University of Freiburg Freiburg Germany

Departments of Internal Medicine and Allergology Rheumatology and Clinical Immunology University Medical Center Groningen Netherlands

Immunodeficiency Centre for Wales University Hospital of Wales Cardiff United Kingdom

Institute of Immunity and Transplantation University College London Dept of Immunology Royal Free London NHS Foundation Trust London United Kingdom

Interstitial Lung Disease Unit Royal Brompton Hospital London United Kingdom

Pneumology Unit Centro Hospital e Universitário de Coimbra Coimbra Portugal and Centre of Pneumology Faculty of Medicine University of Coimbra Coimbra Portugal

Translational and Clinical Research Institute Newcastle University and Great North Children's Hospital Newcastle upon Tyne United Kingdom

UCL Respiratory University College London London United Kingdom

Université de Paris Assistance Publique Hôpitaux de Paris Hôpital Saint Louis Paris France

Citace poskytuje Crossref.org

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