TY - JOUR T1 - Pulmonary hypertension associated with Whipple disease JF - European Respiratory Review JO - EUROPEAN RESPIRATORY REVIEW SP - 533 LP - 536 DO - 10.1183/09059180.00005714 VL - 23 IS - 134 AU - Adolfo Baloira AU - Marta Núñez AU - Manuel Tumbeiro AU - Isaura Parente-Lamelas AU - María Bastos AU - Mario Gutiérrez Y1 - 2014/12/01 UR - http://err.ersjournals.com/content/23/134/533.abstract N2 - To the Editor:Whipple disease is a rare multi-systemic disorder caused by Thropheryma whipplei, a Gram-positive bacillus. Gastrointestinal manifestations are the most frequent, but many other organs may be involved [1, 2]. Pulmonary hypertension (PH) associated with Whipple disease is extremely rare, and only a few isolated cases have been reported. We present two patients with PH-Whipple disease with a positive vasodilator test and excellent response to antibiotic therapy. A 72-year-old Caucasian male with an 8-month history of intermittent abdominal pain and diarrhoea presented with dyspnoea on exertion that had progressed in the last 4 months (functional class III) and two-pillow orthopnoea to keep his head at 30°. He reported weight loss of 6 kg during the preceding 8 months. A colonoscopy performed 15 days before showed only nonspecific inflammation. Past medical history included mild chronic obstructive pulmonary disease treated with ipratropium. Physical examination revealed hypotension (systolic arterial pressure 90 mmHg) and bibasilar rales but not lower leg oedema. Laboratory data showed haemoglobin of 94 g·L−1 and albumin of 19 g·L−1. Antinuclear antibodies, rheumatoid factor and a HIV test were negative. A chest computed tomography (CT) with embolism protocol demonstrated bilateral pleural and pericardial effusions and mediastinal adenopathy. No emboli were found in the pulmonary arteries. Abdominal CT showed mild ascites and multiple small lymph nodes. Gastroduodenoscopy demonstrated diffuse inflammation in the duodenum. Small bowel biopsies showed typical periodic acid–Schiff (PAS)-positive diastase-resistant histiocytes. Several days later PCR confirmed the presence of T. whipplei. Transthoracic echocardiography showed normal left cavities with a dilated right ventricle (55 mm). The velocity of the tricuspid jet was 3.68 m·s−1 with an estimated systolic pulmonary artery pressure (sPAP) of 64 mmHg. A ventilation/perfusion scan excluded thromboembolic disease. Right heart catheterisation confirmed the diagnosis of PH (table … ER -