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BMJ Case Rep ; 20172017 Nov 08.
Artigo em Inglês | MEDLINE | ID: mdl-29122894

RESUMO

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.


Assuntos
Dispneia/diagnóstico , Pulmão/fisiopatologia , Lúpus Eritematoso Sistêmico/complicações , Estenose da Valva Mitral/diagnóstico , Adulto , Diagnóstico Diferencial , Dispneia/etiologia , Ecocardiografia , Hipertensão Pulmonar Primária Familiar/diagnóstico , Hipertensão Pulmonar Primária Familiar/etiologia , Feminino , Humanos , Lúpus Eritematoso Sistêmico/patologia , Estenose da Valva Mitral/diagnóstico por imagem , Estenose da Valva Mitral/cirurgia , Doenças Raras , Testes de Função Respiratória/métodos , Resultado do Tratamento
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