Recommendations for pulmonary arterial hypertension(PAH) associated with connective tissue disease (CTD)
| Statement | Class# | Level¶ |
| In patients with PAH associated with CTD the same treatment algorithm as in patients with IPAH is recommended | I | A |
| Echocardiographic screening for the detection of PH is recommended in symptomatic patients with scleroderma spectrum of diseases | I | B |
| Echocardiographic screening for the detection of PH is recommended in symptomatic patients with all other CTDs | I | C |
| RHC is indicated in all cases of suspected PAH associated with CTD, in particular if specific drug therapy is considered | I | C |
| Oral anticoagulation should be considered on an individual basis | IIa | C |
| Echocardiographic screening for the detection of PH may be considered in asymptomatic patients with the scleroderma spectrum of disease | IIb | C |
IPAH: idiopathic PAH; PH: pulmonary hypertension; RHC: right heart catheterisation. #: class of recommendation; ¶: level of evidence.