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