Balloon pulmonary angioplasty in sarcoid-related pulmonary hypertension

Jelco Tramper, Esther J. Nossent, Rutger J. Lely, Frans H. Krouwels, Lilian J. Meijboom, Anton Vonk Noordegraaf

Source: Eur Respir J, 51 (1) 1701502; 10.1183/13993003.01502-2017
Journal Issue: January
Disease area: Interstitial lung diseases, Thoracic oncology

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Abstract

A 43-year-old, never-smoking, African-American female was referred to our outpatient clinic for progressive dyspnoea. She had a medical history of sarcoidosis with lymph-node, pulmonary, skin and ocular involvement. Diagnosis was based on a previously conducted chest high-resolution computed tomography (CT) that showed bilateral hilar and mediastinal lymphadenopathy containing calcifications and multiple small lung nodules with peri-lymphatic distribution along the pleura and fissures A histological analysis of biopsy specimens from a lymph node located in the left side of the neck revealed large granulomas with multinucleated foreign body giant cells without necrosis. Microbiological cultures were negative. Extrapulmonary manifestations consisted of anterior uveitis and several cutaneous lesions and the reason for initiating corticosteroid therapy, which ameliorated the sarcoidosis. After discontinuation of corticosteroid therapy, the patient's condition deteriorated and she developed progressive dyspnoea (New York Heart Association (NYHA) functional class 3). Thoracic CT showed bilateral pleural and pericardial effusion. There were no signs of pulmonary fibrosis or cardial signs of pulmonary hypertension (PH). Analysis of the pleural fluid showed exudation with lymphocytosis. An infectious cause was excluded.



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Jelco Tramper, Esther J. Nossent, Rutger J. Lely, Frans H. Krouwels, Lilian J. Meijboom, Anton Vonk Noordegraaf. Balloon pulmonary angioplasty in sarcoid-related pulmonary hypertension. Eur Respir J, 51 (1) 1701502; 10.1183/13993003.01502-2017

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