Rheumatological features of Whipple disease

Abstract Whipple disease (WD) is a rare infectious systemic disease. Rheumatologists are at the frontline of WD diagnosis due to the early rheumatological manifestations. An early diagnosis is crucial, as usual anti-rheumatic drugs, especially TNF inhibitors, may worsen the disease course. We conduc...

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Autores principales: Alice Tison, Pauline Preuss, Clémentine Leleu, François Robin, Adrien Le Pluart, Justine Vix, Guillaume Le Mélédo, Philippe Goupille, Elisabeth Gervais, Grégoire Cormier, Jean-David Albert, Aleth Perdriger, Béatrice Bouvard, Jean-Marie Berthelot, Nathan Foulquier, Alain Saraux, the network of rheumatologists from the Grand Ouest “VICTOR HUGO”
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Publicado: Nature Portfolio 2021
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spelling oai:doaj.org-article:9b68e0d9e2bd4e8a9684d2eb215f195c2021-12-02T17:34:35ZRheumatological features of Whipple disease10.1038/s41598-021-91671-92045-2322https://doaj.org/article/9b68e0d9e2bd4e8a9684d2eb215f195c2021-06-01T00:00:00Zhttps://doi.org/10.1038/s41598-021-91671-9https://doaj.org/toc/2045-2322Abstract Whipple disease (WD) is a rare infectious systemic disease. Rheumatologists are at the frontline of WD diagnosis due to the early rheumatological manifestations. An early diagnosis is crucial, as usual anti-rheumatic drugs, especially TNF inhibitors, may worsen the disease course. We conducted a retrospective multicentre national study from January 2010 to April 2020 to better characterize the rheumatological features of WD. Classic WD (CWD) was defined by positive periodic acid-Schiff (PAS) staining of a small-bowel biopsy sample, and non-CWD (NCWD) was defined by negative PAS staining of a small-bowel biopsy sample but at least one positive Tropheryma whipplei (TW) polymerase chain reaction (PCR) for a digestive or extradigestive specimen. Sixty-eight patients were enrolled, including 11 CWD patients. Twenty patients (30%) received TNF inhibitors during the WD course, with inefficacy or symptom worsening. More digestive symptoms and systemic biological features were observed in CWD patients than in NCWD patients, but both patient groups had similar outcomes, especially concerning the response to antibiotics and relapse rate. Stool and saliva TW PCR sensitivity were both 100% for CWD and 75% for NCWD and 89% and 60% for small-bowel biopsy sample PCR, respectively. WD encountered in rheumatology units has many presentations, which might result from different pathophysiologies that are dependent on host immunity. Given the heterogeneous presentations and the presence of chronic carriage, multiple TW PCR tests on samples from specific rheumatological sites when possible should be performed, but samples from nonspecific digestive and extradigestive sites also have great value.Alice TisonPauline PreussClémentine LeleuFrançois RobinAdrien Le PluartJustine VixGuillaume Le MélédoPhilippe GoupilleElisabeth GervaisGrégoire CormierJean-David AlbertAleth PerdrigerBéatrice BouvardJean-Marie BerthelotNathan FoulquierAlain Sarauxthe network of rheumatologists from the Grand Ouest “VICTOR HUGO”Nature PortfolioarticleMedicineRScienceQENScientific Reports, Vol 11, Iss 1, Pp 1-10 (2021)
institution DOAJ
collection DOAJ
language EN
topic Medicine
R
Science
Q
spellingShingle Medicine
R
Science
Q
Alice Tison
Pauline Preuss
Clémentine Leleu
François Robin
Adrien Le Pluart
Justine Vix
Guillaume Le Mélédo
Philippe Goupille
Elisabeth Gervais
Grégoire Cormier
Jean-David Albert
Aleth Perdriger
Béatrice Bouvard
Jean-Marie Berthelot
Nathan Foulquier
Alain Saraux
the network of rheumatologists from the Grand Ouest “VICTOR HUGO”
Rheumatological features of Whipple disease
description Abstract Whipple disease (WD) is a rare infectious systemic disease. Rheumatologists are at the frontline of WD diagnosis due to the early rheumatological manifestations. An early diagnosis is crucial, as usual anti-rheumatic drugs, especially TNF inhibitors, may worsen the disease course. We conducted a retrospective multicentre national study from January 2010 to April 2020 to better characterize the rheumatological features of WD. Classic WD (CWD) was defined by positive periodic acid-Schiff (PAS) staining of a small-bowel biopsy sample, and non-CWD (NCWD) was defined by negative PAS staining of a small-bowel biopsy sample but at least one positive Tropheryma whipplei (TW) polymerase chain reaction (PCR) for a digestive or extradigestive specimen. Sixty-eight patients were enrolled, including 11 CWD patients. Twenty patients (30%) received TNF inhibitors during the WD course, with inefficacy or symptom worsening. More digestive symptoms and systemic biological features were observed in CWD patients than in NCWD patients, but both patient groups had similar outcomes, especially concerning the response to antibiotics and relapse rate. Stool and saliva TW PCR sensitivity were both 100% for CWD and 75% for NCWD and 89% and 60% for small-bowel biopsy sample PCR, respectively. WD encountered in rheumatology units has many presentations, which might result from different pathophysiologies that are dependent on host immunity. Given the heterogeneous presentations and the presence of chronic carriage, multiple TW PCR tests on samples from specific rheumatological sites when possible should be performed, but samples from nonspecific digestive and extradigestive sites also have great value.
format article
author Alice Tison
Pauline Preuss
Clémentine Leleu
François Robin
Adrien Le Pluart
Justine Vix
Guillaume Le Mélédo
Philippe Goupille
Elisabeth Gervais
Grégoire Cormier
Jean-David Albert
Aleth Perdriger
Béatrice Bouvard
Jean-Marie Berthelot
Nathan Foulquier
Alain Saraux
the network of rheumatologists from the Grand Ouest “VICTOR HUGO”
author_facet Alice Tison
Pauline Preuss
Clémentine Leleu
François Robin
Adrien Le Pluart
Justine Vix
Guillaume Le Mélédo
Philippe Goupille
Elisabeth Gervais
Grégoire Cormier
Jean-David Albert
Aleth Perdriger
Béatrice Bouvard
Jean-Marie Berthelot
Nathan Foulquier
Alain Saraux
the network of rheumatologists from the Grand Ouest “VICTOR HUGO”
author_sort Alice Tison
title Rheumatological features of Whipple disease
title_short Rheumatological features of Whipple disease
title_full Rheumatological features of Whipple disease
title_fullStr Rheumatological features of Whipple disease
title_full_unstemmed Rheumatological features of Whipple disease
title_sort rheumatological features of whipple disease
publisher Nature Portfolio
publishDate 2021
url https://doaj.org/article/9b68e0d9e2bd4e8a9684d2eb215f195c
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