Reversible pulmonary hypertension associated with multivisceral Whipple's disease

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.