Sarcoidosis-related pulmonary veno-occlusive disease presenting with recurrent haemoptysis

Abstract

To the Editors: A 52-yr-old female nonsmoker presented with haemoptysis consisting of streaks of bright red blood daily for 3 weeks. She gave a 3-yr history of dry cough, episodic wheeze and mild exertional dyspnoea. The symptoms had not improved with inhaled corticosteroids (fluticasone 250 μg b.i.d ). The patient denied fever, sweats or weight loss and had no significant past medical history. Physical examination was unremarkable. Chest radiograph showed no focal pulmonary abnormality or obvious lymphadenopathy, and electrocardiogram was normal. Pulmonary function tests recorded a forced expiratory volume in 1 s (FEV1) of 2.22 L (86% predicted), a forced vital capacity (FVC) of 2.93 L (96% pred) and a diffusing capacity of the lung for carbon monoxide ( D L,CO) of 7.23 mmol·m−1·kPa−1 (88% pred). Laboratory investigations showed a haemoglobin level of 12.9 g·dL−1, and a normal coagulation screen, metabolic screen and erythrocyte sedimentation rate (21 mm·h−1). Serum auto-antibodies were negative and the angiotensin converting enzyme (ACE) level was 53 IU·L−1 (normal range 0–52 IU·L−1). High-resolution computed tomography (HRCT) of the chest showed thickening of central bronchovascular bundles and scattered, nodular, ground-glass and airspace opacities (fig. 1a⇓). Fibreoptic bronchoscopy was requested. At 1 h prior to the procedure, the patient had a large haemoptysis of 200 mL bright red blood. Bronchoscopy showed diffuse fresh blood and grossly hyperaemic bronchial mucosa with generalised oozing from major and lobar bronchi (fig. 1b⇓). Fig. 1— a) Axial high-resolution computed tomography (HRCT) image showing nodular thickening extending along central bronchovascular bundles in the apical segment of the left lower lobe. Elsewhere, there were scattered nodular, ground-glass and airspace opacities consistent with pulmonary sarcoidosis. b) Bronchoscopic view of the right main and right upper lobe bronchus (arrow) showing diffuse mucosal erythema. c) HRCT thorax image taken 15 months after initial presentation showing thickened interlobular septa and patchy ground-glass attenuation. The subsegmental …