Résumé
Le syndrome thoracique aigu (STA) est une des complications majeures des syndromes drépanocytaires et reste la principale cause de mortalité chez l’adulte. Il peut survenir chez des patients auparavant peu ou non symptomatiques, notamment chez ceux porteurs d’un syndrome drépanocytaire composite. Trois grandes causes dominent les étiologies : les infections, l’obstruction vasculaire pulmonaire (par des emboles graisseux et/ou des thrombi fibrinocruoriques) et les hypoventilations alvéolaires secondaires à des suites chirurgicales ou des infarctus osseux. La symptomatologie associe fièvre, dyspnée et douleur thoracique. Elle survient souvent après quelques jours d’hospitalisation pour crise vaso-occlusive simple ou en postopératoire. L’imagerie retrouve des lésions alvéolaires condensantes des deux bases pulmonaires et la biologie une hémolyse associée à un syndrome inflammatoire. Il faut savoir rechercher deux facteurs de mauvais pronostic : un taux de plaquettes bas et surtout une insuffisance ventriculaire droite.
Le traitement repose sur un trépied : hydratation, oxygénation et analgésie associés à une antibiothérapie couvrant les bactéries encapsulées et atypiques. Dans les formes sévères, le seul traitement spécifique est la transfusion ou l’exsanguinotransfusion suivant le taux d’hémoglobine. Une transfusion préventive ou un échange transfusionnel peuvent être discutés chez des patients à risque, notamment lors de circonstances particulières (grossesse, intervention chirurgicale). Cette décision sera prise au cas par cas en fonction des antécédents transfusionnels. Dans le cadre de la prévention, une spirométrie incitative doit toujours être prescrite pour les patients drépanocytaires hospitalisés, notamment pour crise vaso-occlusive.
Abstract
Acute chest syndrome is the second most common reason for hospitalization and the leading cause for mortality in adult patients with sickle cell disease. This complication is not only observed in most severe patients, but can appear in previously non symptomatic patients. Three main mechanisms are involved: infection, vascular obstruction (by fat embolism and/or pulmonary artery thrombus), and alveolar hypoventilation secondary to surgery or to bone infarcts. The most frequent symptoms are fever, dyspnea, and thoracic pain. In half of patients, vaso-occlusive crisis can precede these symptoms. Basal alveolar consolidation is observed on lung imaging. Hemolysis and systemic inflammation are frequent biological findings. Two prognostic markers have been suggested in the litterature: thrombopenia and right ventricular dysfunction. Supportive treatments (oxygenation, rehydration, and analgesia) are usually prescribed with antibiotics. Transfusion and/or partial blood exchange depending on the haemoglobin level are used in the severe forms. Preventive transfusion should be discussed in specific conditions such as pregnancy and surgical procedure. Preventive incentive spirometry should also be implemented in hospitalized sickle cell patients, especially during vaso-occlusive crisis.
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Maitre, B., Habibi, A., Colin, C. et al. Le syndrome thoracique aigu : complication pulmonaire aiguë des patients adultes atteints d’un syndrome drépanocytaire majeur. Réanimation 24, 71–77 (2015). https://doi.org/10.1007/s13546-015-1033-y
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DOI: https://doi.org/10.1007/s13546-015-1033-y