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The echocardiogram of a 62-year-old woman with a medical history of anti-synthetase syndrome associated with interstitial lung disease (ILD) suggested pulmonary hypertension. An etiological evaluation of pulmonary hypertension was performed. The ventilation/perfusion scan had the aspect of perfusion defects with dissociation. The CT scan showed features of active ILD (basal and subpleural pulmonary infiltration with regular septal line thickening and ground glass-like opacities). After multidisciplinary discussion, the pulmonary hypertension was considered to be secondary to either chronic thromboembolic pulmonary hypertension (CTEPH) and/or ILD. It was decided (i) to initiate an oral anticoagulant (rivaroxaban) and (ii) to initiate intravenous cyclophosphamide plus oral corticosteroids. One year later, while the patient was still taking rivaroxaban, right heart catheterisation showed the persistence of pre-capillary pulmonary hypertension with …
Contributors All authors have actively participated in the preparation of the article and have read and approved the text.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
Data availability statement There are no data in this work
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