Article Text
Summary
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.
- valvar diseases
- lung function
- mechanical ventilation
- systemic lupus erythematosus
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Footnotes
Contributors All authors contributed to the production of this case report. JFS worked on research and initial write-up. AB and CM directly worked on the concept of the case report as well as repeat corrections and final critique on the paper.
Competing interests None declared.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.