Reversible pulmonary hypertension associated with multivisceral Whipple's disease

Abstract

To the Editor: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][6][7][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.A 54-year-old Caucasian man, a technician for phonelines and former smoker (18 pack-years), with a medical history of gastro-oesophageal reflux and systemic hypertension treated by beta-blockers, was hospitalised for acute right heart failure in the respiratory intensive care unit. He reported a deterioration in general state over the previous year with fatigue, a weight loss of 15 kg, diarrhoea, a change in mood and episodes of acute left red eye with spontaneous resolution. In April 2019, he presented to the emergency department with progressive dyspnoea that had been worsening for a few weeks. Clinical examination showed signs of right heart failure, biology showed a microcytic anaemia and increased N-terminal pro-B-type natriuretic peptide (NT-proBNP), and transthoracic echocardiography showed dilation of the right heart chambers, increased estimated systolic pulmonary artery pressure (65 mmHg) and right ventricular dysfunction (tricuspid annular plane systolic excursion 12 mm). Computed tomography pulmonary angiography revealed right heart dilation, with no evidence of acute pulmonary embolism and normal lung parenchyma (figure 1). He had no family history or exposures to drugs and toxins. Right heart catheterisation (RHC) confirmed severe precapillary PH without acute pulmonary vasodilator response to inhaled nitric oxide: mean pulmonary artery pressure (mPAP) 40 mmHg, cardiac output 3.1 L•min −1 , cardiac index 2 L•min −1 •m −2 , pulmonary artery wedge pressure 13 mmHg and pulmonary vascular resistance (PVR) 8 WU. Treatment with intravenous dobutamine and diuretics was initiated together with oral dual combination of pulmonary arterial h...

Whipple's disease of the respiratory system: A case report

“…The treatment of WD is mainly antibiotic anti-infection treatment; commonly used drugs include penicillin, tetracycline, streptomycin, ceftriaxone, meropenem, hydroxychloroquine, doxycycline and cotrimoxazole (4). Initial treatment with ceftriaxone and meropenem for 2 weeks followed by maintenance treatment with cotrimoxazole for ~1 year is the typical treatment protocol, but in recent years, some studies have shown that T. whipplei is resistant to cotrimoxazole, which may increase the chances of recurrence of WD (19)(20)(21). In this situation, doxycycline combined with hydroxychloroquine as an alternative treatment can also achieve improved efficacy (19)(20)(21).…”