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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....
Jazyk angličtina Země Švýcarsko
Typ dokumentu časopisecké články, multicentrická studie, práce podpořená grantem
NLK
Directory of Open Access Journals
od 2010
Free Medical Journals
od 2010
PubMed Central
od 2010
Europe PubMed Central
od 2010
Open Access Digital Library
od 2010-01-01
Open Access Digital Library
od 2010-01-01
ROAD: Directory of Open Access Scholarly Resources
od 2010
- MeSH
- alergologie a imunologie trendy MeSH
- běžná variabilní imunodeficience diagnóza farmakoterapie imunologie MeSH
- biologické přípravky terapeutické užití MeSH
- disparity zdravotní péče trendy MeSH
- granulom dýchacího systému diagnóza farmakoterapie imunologie MeSH
- imunosupresiva škodlivé účinky terapeutické užití MeSH
- internet MeSH
- intersticiální plicní nemoci diagnóza farmakoterapie imunologie MeSH
- lékařská praxe - způsoby provádění trendy MeSH
- lidé MeSH
- pediatři trendy MeSH
- pediatrie trendy MeSH
- plicní lékaři trendy MeSH
- pneumologie trendy MeSH
- prognóza MeSH
- průzkumy zdravotní péče MeSH
- steroidy terapeutické užití MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
- multicentrická studie MeSH
- práce podpořená grantem MeSH
- Geografické názvy
- Evropa MeSH
- Spojené státy americké MeSH
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.
Centre for Medical Image Computing University College London London United Kingdom
Department of Molecular Medicine Sapienza University of Rome Rome Italy
Department of Pathology Hôpital Saint Louis Assistance Publique Hôpitaux de Paris Paris France
Immunodeficiency Centre for Wales University Hospital of Wales Cardiff United Kingdom
Interstitial Lung Disease Unit Royal Brompton Hospital London 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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- $a van de Ven, Annick A J M $u Departments of Internal Medicine and Allergology, Rheumatology and Clinical Immunology, University Medical Center Groningen, Netherlands
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- $a 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.
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