[Systemic lupus erythematosus presenting as severe alveolar hypoventilation and the shrinking lung syndrome]. / Hypoventilation alvéolaire sévère révélant un shrinking lung syndrome lupique.
Rev Mal Respir
; 34(5): 571-575, 2017 May.
Article
em Fr
| MEDLINE
| ID: mdl-28189434
ABSTRACT
INTRODUCTION:
The shrinking lung syndrome (SLS) is a rare complication of systemic lupus erythematosus. CASE REPORT A 69-year-old man presented with exertional dyspnoea, muscle weakness, and weight loss of 15kg in 6months. Pulmonary function tests revealed a restrictive lung disorder, with a dramatic decrease in maximal inspiratory pressure (17% of theoretical value), and alveolar hypoventilation (pH 7.43; PaCO2 55mmHg). A thoracic CT-scan showed bilateral diaphragmatic elevation. The creatinine phophokinase level was increased at 280U/L. Progress was marked by a rapidly increasing respiratory acidosis (pH 7.24, PaCO2 109mmHg) requiring invasive ventilation. Auto-immune studies revealed positive anti-nuclear antibodies (1/800) and positive anti-native DNA antibody at 45U/L. Treatment with systemic corticosteroids led to an initial improvement but it was not possible to discontinue mechanical ventilation. The outcome was fatal. Autopsy did not reveal any other cause and a diagnosis of the SLS associated with lupus was confirmed.CONCLUSION:
The interesting features of this case report consist of 1) the presentation of the SLS as an alveolar hypoventilation with a fatal outcome, 2) the presentation of systemic lupus as SLS.Palavras-chave
Texto completo:
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Base de dados:
MEDLINE
Assunto principal:
Hipoventilação
/
Pneumopatias
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Lúpus Eritematoso Sistêmico
Idioma:
Fr
Ano de publicação:
2017
Tipo de documento:
Article