RESUMO
BACKGROUND: The evolution in pulmonary arterial hypertension (PAH) management has been summarised in three iterations of the European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines. No study has assessed whether changes in management, as reflected in the changing guidelines, has translated to improved long-term survival in PAH. METHODS: We performed a mixed retrospective/prospective analysis of treatment-naïve, incident PAH patients (n=392) diagnosed at three major centres in Canada from 2009 to 2021. Patients were divided into two groups based on their diagnosis date and in accordance with the 2009 and 2015 ESC/ERS guideline iterations. Overall survival was assessed based on date of diagnosis and initial treatment strategy (i.e. monotherapy versus combination therapy). RESULTS: There was a shift towards more aggressive upfront management with combination therapy in Canada after the publication of the 2015 ESC/ERS guidelines (10.4% and 30.8% in patients from 2009 to 2015 and 36.0% and 57.4% in patients diagnosed after 2015 for baseline and 2-year follow-up, respectively). A key factor associated with combination therapy after 2015 was higher pulmonary vascular resistance (p=0.009). The 1-, 3- and 5-year survival rates in Canada were 89.2%, 75.6% and 56.0%, respectively. Despite changes in management, there was no improvement in long-term survival before and after publication of the 2015 ESC/ERS guidelines (p=0.53). CONCLUSIONS: There was an increase in the use of initial and sequential combination therapy in Canada after publication of the 2015 ESC/ERS guidelines, which was not associated with improved long-term survival. These data highlight the continued difficulties of managing this aggressive pulmonary disease in an era without a cure.
Assuntos
Cardiologia , Hipertensão Arterial Pulmonar , Hipertensão Pulmonar Primária Familiar/terapia , Humanos , Estudos Retrospectivos , Taxa de SobrevidaRESUMO
Rivaroxaban is commonly used for prevention of thromboembolic diseases in patients with atrial fibrillation. We report a case of an 86-year-old man with hypertension, chronic kidney disease, type 2 diabetes mellitus, dyslipidemia, and atrial fibrillation who developed bullous eruptions 1 week after a rivaroxaban dose increase. He was subsequently hospitalized, and direct immunofluorescence confirmed bullous pemphigoid (BP). After switching to apixaban, the patient's skin eruptions stabilized and improved. This is the first reported case of immunofluorescence-confirmed BP associated with rivaroxaban use. Prompt discontinuation of rivaroxaban and a switch to other anticoagulants is important for patients with suspected drug-associated BP.
Le rivaroxaban est couramment utilisé pour la prévention des maladies thromboemboliques chez les patients atteints de fibrillation auriculaire. Nous rapportons le cas d'un homme de 86 ans atteint d'hypertension, de néphropathie chronique, de diabète de type 2, de dyslipidémie et de fibrillation auriculaire, qui a présenté des éruptions bulleuses une semaine après l'augmentation de sa dose de rivaroxaban. Il a par la suite été hospitalisé, et une analyse par immunofluorescence directe a permis de confirmer le diagnostic de pemphigoïde bulleuse. Après le passage à l'apixaban, les éruptions cutanées du patient se sont stabilisées et atténuées. Il s'agit du premier cas rapporté de pemphigoïde bulleuse confirmée par immunofluorescence associé à l'utilisation du rivaroxaban. Chez les patients présentant une pemphigoïde bulleuse possiblement associée au rivaroxaban, il est important d'arrêter rapidement l'administration du médicament et de le remplacer par d'autres anticoagulants.