Pulmonary hypertension in connective tissue diseases, new evidence and challenges.

40 Furthermore, the French nationwide SLE‐PAH study showed a trend towards better survival in patients who received hydroxychloroquine. 20 8 TREAT TO TARGET Risk status for mortality of Idiopathic PAH patients can be assessed by a comprehensive tool described by the ESC and the ERS guidelines. 33 , 41 Risk criteria are based on the presence of clinical signs of right heart failure, progression of symptoms, the WHO functional class, the 6MWT, parameters of cardiopulmonary exercise testing, NT‐pro‐BNP level, presence of right atrial dilatation and pericardial effusion on imaging, and (invasive) haemodynamics.

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