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Pulmonary hypertension (PH) is a possible complication of connective tissue diseases (CTDs), especially systemic sclerosis (SSc), systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD). It is defined by an elevation of the mean pulmonary arterial pressure above 20 mmHg documented during a right heart catheterization (RHC). Due to their multiorgan involvement, CTDs can induce PH by several mechanisms, that are sometimes intricated: pulmonary vasculopathy (group 1) affecting arterioles (pulmonary arterial hypertension, PAH) and possibly venules (pulmonary veno-occlusive-like disease), left-heart disease (group 2), chronic lung disease (group 3) and/or chronic thromboembolic PH (group 4). PH suspicion is often raised by clinical manifestations (dyspnea, fatigue), echocardiographic data (increased peak tricuspid regurgitation velocity), isolated decrease in DLCO in pulmonary function tests, and/or unexplained elevation of BNP/NT-proBNP. Its formal diagnosis always requires a hemodynamic confirmation by RHC. Strategies for PH screening and RHC referral have been extensively investigated for SSc-PAH but data are lacking in other CTDs. Therapeutic management of PH depends of the underlying mechanism(s): PAH-approved therapies in group 1 PH (with possible use of immunosuppressants, especially in case of SLE or MCTD); management of an underlying left-heart disease in group 2 PH; management of an underlying chronic lung disease in group 3 PH; anticoagulation, pulmonary endartectomy, PAH-approved therapies and/or balloon pulmonary angioplasty in group 4 PH. Regular follow-up is mandatory in all CTD-PH patients. [ABSTRACT FROM AUTHOR]

Résumé Introduction Aucune donnée n’est disponible concernant la tolérance du bévacizumab, anticorps anti- vascular endothelial growth factor-A (VEGF-A), chez le patient atteint d’hypertension artérielle pulmonaire (HTAP), pathologie dans laquelle le VEGF-A joue un rôle central et probablement protecteur. Observation Nous rapportons l’observation d’une patiente présentant une HTAP associée à une sclérodermie systémique, stable sous bosentan. La découverte d’un carcinome épidermoïde du col utérin métastatique a conduit à l’administration de deux lignes successives de chimiothérapie cytotoxique. Leur échec a motivé l’initiation d’un traitement anti-angiogénique : trois injections de bévacizumab 15 mg/kg ont ainsi été administrées. Tout au long du traitement, aucune modification clinique ni échographique de l’HTAP n’a été constatée. Conclusion Cette observation suggère que, sous surveillance clinique et échographique rapprochée, le bévacizumab peut être bien toléré en cas d’HTAP stable et modérée. La décision de traitement doit être prise de manière individuelle et adaptée au rapport bénéfice–risque, le risque d’aggravation de l’HTAP n’étant pas exclu. Introduction No data is available regarding the safety of bevacizumab, an anti-vascular endothelial growth factor-A (VEGF-A) antibody, in patients with pulmonary arterial hypertension (PAH), a condition in which VEGF seems to play a significant and probably protective role. Case report We report a patient with a history of systemic sclerosis-associated PAH, stable under bosentan therapy. She was diagnosed with metastatic cervical epidermoid carcinoma and treated by two successive cytotoxic chemotherapy regimens. As these treatments failed to control disease progression, she was started on anti-angiogenic therapy: 3 infusions of bevacizumab 15 mg/kg were administered. Over the course of this treatment, no change in the clinical status or echocardiography parameters was noted. Conclusion This observation suggests that, under careful clinical and echocardiographic follow-up, bevacizumab therapy can be well tolerated in case of stable and moderate PAH. Decision of treatment should be taken cautiously, as the possibility of PAH worsening is not excluded. [ABSTRACT FROM AUTHOR]