TY - JOUR T1 - Reversible pulmonary hypertension associated with multivisceral Whipple's disease JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.03132-2020 SP - 2003132 AU - Alice Camboulive AU - Etienne-Marie Jutant AU - Laurent Savale AU - Xavier Jaïs AU - Olivier Sitbon AU - Charlotte Mussini AU - Jérémie Bénichou AU - Jean-Christophe Lagier AU - Marc Humbert AU - David Montani Y1 - 2020/01/01 UR - http://erj.ersjournals.com/content/early/2020/10/08/13993003.03132-2020.abstract N2 - Whipple's disease (WD) is a rare infectious disease developed through fecal-oral transmission and caused by Tropheryma whipplei, a ubiquitous gram bacillus [1]. The pathogenesis remains unclear, and several host factors seem to be implicated, including male sex, comorbidities and genetic susceptibility [1]. WD is a multivisceral disorder with frequent gastro-intestinal, joint and neurological involvement, as well as pulmonary, cardiovascular, mucocutaneous, and ophthalmologic lesions [2]. The diagnosis is made via small bowel biopsy with Periodic Acid Schiff staining (PAS) positivity and by polymerase chain reaction (PCR) on different biological samples [1]. Classical treatment requires prolonged antibiotic therapy and allows rapid improvement. The association of doxycycline and hydroxychloroquine over 1 year followed by lifetime treatment with doxycycline has been shown to lead to good clinical responses and fewer relapses [3]. Others have recommended initial use of intravenous antibiotics, followed by trimethoprim-sulfamethoxazole for 1 year [4]. Among lung complications of WD, pulmonary hypertension (PH) is very rare and remains poorly understood [5–8]. Here, we report the first well-documented case of a man with concomitant diagnosis of severe precapillary PH and multivisceral WD who had total reversibility in hemodynamics and clinical state after treatment of WD and PH.FootnotesThis manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.Conflict of interest: Dr. Yan reports grants from National Institutes of Health, during the conduct of the study;.Conflict of interest: Dr. Camboulive has nothing to disclose.Conflict of interest: Dr. Jutant has nothing to disclose.Conflict of interest: Dr. Savale has nothing to disclose.Conflict of interest: Dr. Jais has nothing to disclose.Conflict of interest: Dr. SITBON reports grants, personal fees and non-financial support from Actelion, personal fees from Acceleron, grants, personal fees and non-financial support from Bayer, grants, personal fees and non-financial support from MSD, grants from GlaxoSmithKline, personal fees from Ferrer, personal fees from Gossamer Bio, outside the submitted work.Conflict of interest: Dr. Mussini has nothing to disclose.Conflict of interest: Dr. Bénichou has nothing to disclose.Conflict of interest: Dr. Lagier has nothing to disclose.Conflict of interest: Dr. Humbert reports personal fees from Acceleron, grants and personal fees from Actelion, grants and personal fees from Bayer Heathcare, personal fees from GSK, personal fees from Merck, personal fees from Novartis, personal fees from Astrazeneca, personal fees from Sanofi, outside the submitted work.Conflict of interest: Dr. Montani has nothing to disclose. ER -