Résumé
Le syndrome pneumorénal est une entité nosologique associant une atteinte pulmonaire et rénale secondaire à une vascularite. Les principales causes sont les vascularites à autoanticorps anticytoplasme des polynucléaires neutrophiles (ANCA) et le syndrome de Goodpasture, mais d’autres pathologies auto-immunes sont parfois responsables de ce tableau clinique caractérisé par une hémoptysie avec dyspnée, une anémie parfois sévère et une insuffisance rénale aiguë par glomérulonéphrite rapidement progressive. Le diagnostic positif repose sur le bilan biologique d’autoimmunité, le lavage bronchoalvéolaire et la biopsie rénale, qui permettent également d’éliminer les autres causes de détresse respiratoire ou d’insuffisance rénale aiguë. L’identification rapide de la pathologie sous-jacente est essentielle, car elle permet l’initiation précoce du traitement spécifique, ce qui est essentiel pour améliorer le pronostic vital (25 à 50 % de mortalité pour les cas nécessitant la réanimation) et limiter les séquelles rénales. Les progrès thérapeutiques de ces dernières années ont permis de définir le traitement de référence de ces pathologies, associant corticoïdes, immunosuppresseurs (cyclophosphamide ou rituximab) et échanges plasmatiques dans la majorité des cas.
Abstract
Pulmonary-renal syndrome is characterized by lung and kidney diseases due to small-vessel vasculitis. It is mainly caused by antineutrophil cytoplasmic antibodies (ANCA)-related vasculitis and Goodpasture’s syndrome, but several other auto-immune diseases can be associated with this clinical presentation. Diffuse alveolar haemorrhage is caused by haemoptysis, dyspnoea, and anaemia, whereas acute renal failure with proteinuria and haematuria occurs due to rapidly progressive glomerulonephritis. Diagnosis is based on positivity of specific auto-antibodies, presence of blood on bronchoscopic alveolar lavage, and presence of specific glomerular lesions on the renal biopsy. The underlying auto-immune disorder must be identified as soon as possible in order to initiate specific treatment. Recent therapeutic protocols involving corticosteroids and immunosuppressive drugs such as cyclophosphamide or rituximab, associated with plasma exchanges have significantly improved the prognosis of this syndrome, which is still a difficult diagnostic and therapeutic challenge in the intensive care unit.
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Cet article correspond à la conférence faite par l’auteur au congrès de la SRLF 2012 dans la session: Ces maladies que l’on croit connaître.
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Karras, A. Syndromes pneumorénaux. Réanimation 21 (Suppl 2), 339–346 (2012). https://doi.org/10.1007/s13546-011-0332-1
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DOI: https://doi.org/10.1007/s13546-011-0332-1
Mots clés
- Vascularite
- Hémorragie intra-alvéolaire
- Glomérulonéphrite extracapillaire
- Anticorps anticytoplasme des polynucléaires neutrophiles (ANCA)
- Anticorps antimembrane basale glomérulaire