| Literature DB >> 29122894 |
Jeremy Fleri Soler1, Andrew Borg2, Cecilia Mercieca2.
Abstract
A 32-year-old woman suffering from systemic lupus erythematosus presented with a 6-week history of progressive dyspnoea and pleuritic chest pain. Examination was normal apart from reduced air entry at the lung bases.Arterial blood gases showed hypoxaemia and chest X-ray revealed raised hemidiaphragms without any pleural effusions. Lung function showed a restrictive pathology while high-resolution chest CT and CT pulmonary angiogram were negative. Echocardiography showed normal ventricular diameters and no pericardial effusion. Reduced lung volumes and a positive fluoroscopic sniff test lead to a diagnosis of shrinking lung syndrome. Symptoms improved following treatment with glucocorticoids and non-invasive ventilation, but there was no change in lung function.A year later, our patient presented again with worsening dyspnoea. This time echocardiography revealed severe mitral stenosis with pulmonary hypertension. Mitral valve replacement was performed and dyspnoea resolved. Histology showed Libman-Sachs endocarditis. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.Entities:
Keywords: lung function; mechanical ventilation; systemic lupus erythematosus; valvar diseases
Mesh:
Year: 2017 PMID: 29122894 PMCID: PMC5695324 DOI: 10.1136/bcr-2017-220162
Source DB: PubMed Journal: BMJ Case Rep ISSN: 1757-790X