[Pulmonary hypertension in diffuse scleroderma and the CREST syndrome]. 1989

M Presetnik, and E Music, and B Kolsek, and B Rozman

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.

UI MeSH Term Description Entries
D006976 Hypertension, Pulmonary Increased VASCULAR RESISTANCE in the PULMONARY CIRCULATION, usually secondary to HEART DISEASES or LUNG DISEASES. Pulmonary Hypertension
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D011928 Raynaud Disease An idiopathic vascular disorder characterized by bilateral Raynaud phenomenon, the abrupt onset of digital paleness or CYANOSIS in response to cold exposure or stress. Cold Fingers, Hereditary,Raynaud Phenomenon,Raynaud's Disease,Raynauds Disease
D002114 Calcinosis Pathologic deposition of calcium salts in tissues. Calcification, Pathologic,Calcinosis, Tumoral,Microcalcification,Microcalcinosis,Pathologic Calcification,Calcinoses,Calcinoses, Tumoral,Microcalcifications,Microcalcinoses,Tumoral Calcinoses,Tumoral Calcinosis
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly
D012595 Scleroderma, Systemic A chronic multi-system disorder of CONNECTIVE TISSUE. It is characterized by SCLEROSIS in the SKIN, the LUNGS, the HEART, the GASTROINTESTINAL TRACT, the KIDNEYS, and the MUSCULOSKELETAL SYSTEM. Other important features include diseased small BLOOD VESSELS and AUTOANTIBODIES. The disorder is named for its most prominent feature (hard skin), and classified into subsets by the extent of skin thickening: LIMITED SCLERODERMA and DIFFUSE SCLERODERMA. Sclerosis, Systemic,Systemic Scleroderma,Systemic Sclerosis

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