Pulmonary hypertension in connective tissue diseases: an update

Pulmonary hypertension ( PH ) is a relatively commoner complication of systemic sclerosis ( SS c) with estimated prevalence ranging between 8% and 12% as compared to much lower figures in other connective tissue diseases ( CTD ). It is a major cause of morbidity and mortality in CTDs. PH is classified into five major groups. CTD ‐associated PH belongs to group 1 PH , also known as pulmonary arterial hypertension ( PAH ). Around 30% of scleroderma‐related deaths are due to PAH . Underlying pathogenesis is related to pulmonary vasculopathy involving small vessels. The Evidence‐based Detection of Pulmonary Arterial Hypertension in Systemic sclerosis ( DETECT ) algorithm outperforms the current European Society of Cardiology/European Respiratory Society guidelines as a screening tool in SS c‐ PAH ; it can, therefore, suggest when to refer a patient for right heart catheterization. CTD ‐ PAH patients constitute at least 20% of patients included in all major trials of PH ‐specific therapy and the results are comparable to those of idiopathic PAH . The role of anticoagulation in CTD ‐ PAH is associated with a high risk‐benefit ratio with the caveat of its potential role in those with severe disease. There appears to be no role of immunosuppression in scleroderma‐ PAH ; however, immunosuppressive agents, namely the combination of glucocorticoids and pulse cyclophosphamide / possibly mycophenolate, may result in clinical improvement in a subset of patients with systemic lupus erythematosus and mixed connective tissue disease‐related PAH .