Pulmonary hypertension in patients with connective tissue diseases]

Pulmonary hypertension (PH) is said to be frequently associated with connective tissue diseases (CTD). The national surveillance of CTD associated PH (CTD-PH) by the study groups of the Ministry of Health and Welfare (MHW) revealed that mixed connective tissue disease (MCTD) had the highest incidence of PH (5.02%), next to systemic sclerosis (SSc) (2.64%). Patients with CTD-PH could be divided into two groups according to the prognosis: poor prognosis group and fairly good prognosis group. Poor prognosis was associated statistically with polyarthritis and high level of CK. Treatment of CTD-PH was still difficult, but adrenocoticosteroids could be tried in some patients, and drip infusion therapy of prostacyclin seemed very promising, but it has not been accepted by the MHW so far.