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Reversible pulmonary hypertension associated with multivisceral Whipple's disease

Alice Camboulive, Etienne-Marie Jutant, Laurent Savale, Xavier Jaïs, Olivier Sitbon, Charlotte Mussini, Jérémie Bénichou, Jean-Christophe Lagier, Marc Humbert, David Montani
European Respiratory Journal 2021 57: 2003132; DOI: 10.1183/13993003.03132-2020
Alice Camboulive
1Assistance Publique - Hôpitaux de Paris (AP-HP), Dept of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
2Université Paris-Saclay, School of Medicine, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999 “Pulmonary Hypertension: Pathophysiology and Novel Therapies”, Hôpital Marie Lannelongue, Le Plessis-Robinson, France
9Contributed equally
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Etienne-Marie Jutant
1Assistance Publique - Hôpitaux de Paris (AP-HP), Dept of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
2Université Paris-Saclay, School of Medicine, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999 “Pulmonary Hypertension: Pathophysiology and Novel Therapies”, Hôpital Marie Lannelongue, Le Plessis-Robinson, France
9Contributed equally
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Laurent Savale
1Assistance Publique - Hôpitaux de Paris (AP-HP), Dept of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
2Université Paris-Saclay, School of Medicine, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999 “Pulmonary Hypertension: Pathophysiology and Novel Therapies”, Hôpital Marie Lannelongue, Le Plessis-Robinson, France
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Xavier Jaïs
1Assistance Publique - Hôpitaux de Paris (AP-HP), Dept of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
2Université Paris-Saclay, School of Medicine, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999 “Pulmonary Hypertension: Pathophysiology and Novel Therapies”, Hôpital Marie Lannelongue, Le Plessis-Robinson, France
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Olivier Sitbon
1Assistance Publique - Hôpitaux de Paris (AP-HP), Dept of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
2Université Paris-Saclay, School of Medicine, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999 “Pulmonary Hypertension: Pathophysiology and Novel Therapies”, Hôpital Marie Lannelongue, Le Plessis-Robinson, France
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Charlotte Mussini
4AP-HP, Service d'Anatomie et de Cytologie Pathologiques, Hôpital de Bicêtre, Le Kremlin Bicêtre, France
5Hôpitaux Universitaires Paris-Saclay, Le Kremlin-Bicêtre, France
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Jérémie Bénichou
5Hôpitaux Universitaires Paris-Saclay, Le Kremlin-Bicêtre, France
6AP-HP, Service d'ophtalmologie, Hôpital de Bicêtre, Le Kremlin Bicêtre, France
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Jean-Christophe Lagier
7Aix Marseille University, IRD, AP-HM, MEPHI, Marseille, France
8IHU-Méditerranée Infection, Marseille, France
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Marc Humbert
1Assistance Publique - Hôpitaux de Paris (AP-HP), Dept of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
2Université Paris-Saclay, School of Medicine, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999 “Pulmonary Hypertension: Pathophysiology and Novel Therapies”, Hôpital Marie Lannelongue, Le Plessis-Robinson, France
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David Montani
1Assistance Publique - Hôpitaux de Paris (AP-HP), Dept of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
2Université Paris-Saclay, School of Medicine, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999 “Pulmonary Hypertension: Pathophysiology and Novel Therapies”, Hôpital Marie Lannelongue, Le Plessis-Robinson, France
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  • For correspondence: david.montani@aphp.fr
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Extract

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 PCR on different biological samples [1]. Classical treatment requires prolonged antibiotic therapy and allows rapid improvement. Combination therapy with 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 haemodynamics and clinical state after treatment of WD and PH.

Abstract

A report of multivisceral Whipple's disease with severe precapillary pulmonary hypertension cured by doxycycline and hydroxychloroquine therapy associated with initial oral combination of bosentan and tadalafil. https://bit.ly/2SECU3L

Footnotes

  • Conflict of interest: A. Camboulive has nothing to disclose.

  • Conflict of interest: E-M. Jutant has nothing to disclose.

  • Conflict of interest: L. Savale has nothing to disclose.

  • Conflict of interest: X. Jais has nothing to disclose.

  • Conflict of interest: O. Sitbon reports grants, personal fees and non-financial support from Actelion, Bayer and MSD, personal fees from Acceleron, Ferrer and Gossamer Bio, grants from GlaxoSmithKline, outside the submitted work.

  • Conflict of interest: C. Mussini has nothing to disclose.

  • Conflict of interest: J. Bénichou has nothing to disclose.

  • Conflict of interest: J-C. Lagier has nothing to disclose.

  • Conflict of interest: M. Humbert reports grants and personal fees from Actelion and Bayer Heathcare, personal fees from Acceleron, GSK, Merck, Novartis, AstraZeneca and Sanofi, outside the submitted work.

  • Conflict of interest: D. Montani reports grants and personal fees from Actelion, Bayer Heathcare, MSD and GlaxoSmithKline, outside the submitted work.

  • Received August 13, 2020.
  • Accepted October 6, 2020.
  • Copyright ©ERS 2021
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Reversible pulmonary hypertension associated with multivisceral Whipple's disease
Alice Camboulive, Etienne-Marie Jutant, Laurent Savale, Xavier Jaïs, Olivier Sitbon, Charlotte Mussini, Jérémie Bénichou, Jean-Christophe Lagier, Marc Humbert, David Montani
European Respiratory Journal Feb 2021, 57 (2) 2003132; DOI: 10.1183/13993003.03132-2020

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Reversible pulmonary hypertension associated with multivisceral Whipple's disease
Alice Camboulive, Etienne-Marie Jutant, Laurent Savale, Xavier Jaïs, Olivier Sitbon, Charlotte Mussini, Jérémie Bénichou, Jean-Christophe Lagier, Marc Humbert, David Montani
European Respiratory Journal Feb 2021, 57 (2) 2003132; DOI: 10.1183/13993003.03132-2020
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