Pulmonary hypertension in diffuse scleroderma and the CREST syndrome

: In 22 patients with systemic sclerosis (SS) i.e. 16 patients with diffuse scleroderma and 6 patients with CREST syndrome the presence of pulmonary hypertension (PH) was investigated by microcatheter. Manifest PH was found in 3 patients with diffuse scleroderma (19%) and in 3 patients with CREST syndrome (50%). Latent PH was found in 8 patients with diffuse scleroderma (50%) and in 2 patients with CREST syndrome (33%). In our patients the occurrence of PH in CREST syndrome was more frequent than in diffuse scleroderma which is in accordance with the data from the literature. Isolated PH (without simultaneous involvement of pulmonary interstitium) was found in most patients with CREST syndrome (but in 1 case) and in 3 out of 11 patients with diffuse scleroderma.