Organ manifestations in 100 patients with progressive systemic sclerosis: a comparison between the CREST syndrome and diffuse scleroderma. 1989

A Akesson, and F A Wollheim
Department of Rheumatology, University Hospital, Lund, Sweden.

Internal organ function, biochemical, and immunological variables were assessed in 100 patients with progressive systemic sclerosis (PSS), 37 with diffuse scleroderma and 63 with the CREST syndrome. The frequency of oesophageal symptoms and dysmotility did not differ between the two subsets, but patients with diffuse scleroderma had more pronounced motility disturbances. Calcinosis, Raynaud's phenomenon, and telangiectasia were equally frequent in the two groups. Vital capacity, total lung capacity, and static lung compliance were lower among patients with diffuse scleroderma (p less than 0.001), who also had a higher frequency of lung fibrosis (p less than 0.001) and cardiomegaly (p less than 0.01). Sixty eight per cent of the patients with diffuse scleroderma and 62% of those with the CREST syndrome had a positive antinuclear antibody test, which was associated with oesophageal hypomotility (p less than 0.001), fat malabsorption (p less than 0.01) and pulmonary dysfunction (p less than 0.05). Thus, the severity of organ manifestations varied not only with the form of PSS, but also with the absence/presence of antinuclear antibody. This study shows that the term 'CREST syndrome' could be replaced by 'limited cutaneous systemic sclerosis'.

UI MeSH Term Description Entries
D007408 Intestinal Absorption Uptake of substances through the lining of the INTESTINES. Absorption, Intestinal
D007410 Intestinal Diseases Pathological processes in any segment of the INTESTINE from DUODENUM to RECTUM. Disease, Intestinal,Diseases, Intestinal,Intestinal Disease
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
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
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

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