A case of Whipple's disease mimicking auto-inflammatory disease and revealed by severe right cardiac failure under anakinra

Brevet, P.; Rottenberg, Pascal; Viacroze, Catherine; Schleifer, D.; Lequerré, Thierry; Vittecoq, Olivier

doi:10.1016/j.jbspin.2019.11.004

Cited by 5 publications

(6 citation statements)

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“…In the literature, we found 10 case reports of PH associated with WD, not always confirmed by RHC [5][6][7]. Six cases were well documented with evidence of precapillary PH [7].…”

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confidence: 94%

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confidence: 98%

“…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.…”

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confidence: 99%

“…Six cases were well documented with evidence of precapillary PH [7]. Among these 10 patients, 80% had favourable evolution with antibiotic therapy (intravenous beta-lactam antibiotics followed by trimethoprimsulfamethoxazole in six cases and doxycycline associated with hydroxychloroquine in the remaining two) [6][7][8]. Three patients were prescribed calcium channel blockers (associated with phosphodiesterase type 5 inhibitor in one), but this therapy failed with worsening of symptoms, even in the case with acute response to vasodilators [7].…”

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confidence: 99%

“…Alice Camboulive 1,2,3,9 , Etienne-Marie Jutant 1,2,3,9 , Laurent Savale 1,2,3 , Xavier Jaïs 1,2,3 , Olivier Sitbon 1,2,3 , Charlotte Mussini 4,5 , Jérémie Bénichou 5,6 , Jean-Christophe Lagier 7,8 , Marc Humbert 1,2,3 and David Montani 1,2,3 1 Assistance Publique -Hôpitaux de Paris (AP-HP), Dept of Respiratory and Intensive Care Medicine, Pulmonary Hypertension National Referral Center, Hôpital Bicêtre, Le Kremlin-Bicêtre, France. 2 Université Paris-Saclay, School of Medicine, Le Kremlin-Bicêtre, France.…”

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confidence: 99%

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Reversible pulmonary hypertension associated with multivisceral Whipple's disease

et al. 2020

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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...

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“…In the literature, we found 10 case reports of PH associated with WD, not always confirmed by RHC [5][6][7]. Six cases were well documented with evidence of precapillary PH [7].…”

mentioning

confidence: 94%

mentioning

confidence: 98%

mentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

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