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[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.

Adult↗
PubMed1984

Myocardial function and perfusion in the CREST syndrome variant of progressive systemic sclerosis. Exercise radionuclide evaluation and comparison with diffuse scleroderma.

Myocardial function and perfusion were evaluated in 22 patients with progressive systemic sclerosis with the CREST syndrome using exercise and radionuclide techniques, pulmonary function testing, and chest roentgenography. The results were compared with a similar study of 26 patients with progressive systemic sclerosis with diffuse scleroderma. The prevalence of thallium perfusion abnormalities was similar in the groups with CREST syndrome and diffuse scleroderma, (64 percent versus 77 percent), but the defects were significantly smaller in the CREST syndrome (p less than 0.01). Reperfusion thallium defects in the absence of extramural coronary artery disease were seen in 38 percent of patients with diffuse scleroderma. This finding was not seen in any of the patients with the CREST syndrome. In diffuse scleroderma, abnormalities of both right and left ventricular function were related to larger thallium perfusion defects. In the CREST syndrome, abnormalities of left ventricular function were minor, were seen only during exercise, and were unrelated to thallium perfusion defects. Abnormal resting right ventricular function was seen in 36 percent of the patients with the CREST syndrome and was associated with an isolated decrease in diffusing capacity of carbon monoxide. It is concluded that the cardiac manifestations of the CREST syndrome are distinct from those found in diffuse scleroderma. Unlike diffuse scleroderma, abnormalities of left ventricular function in the CREST syndrome are minor and are unrelated to abnormalities of coronary perfusion. Right ventricular dysfunction in the CREST syndrome appears to be primarily related to pulmonary vascular disease.

Adult↗
PubMed1985

Scl-86, a marker antigen for diffuse scleroderma.

More than 300 sera from patients with a connective tissue disease were analyzed with the immunoblotting technique. The presence of autoantibodies against an 86,000-mol wt marker antigen for diffuse scleroderma (Scl-86) was found in 14 out of 33 patients with scleroderma. The presence of anti-Scl-86 antibodies seemed to correlate with the diagnosis of diffuse scleroderma since they were found in 13 out of 22 diffuse scleroderma patients and in only one out of 11 patients with limited scleroderma. All scleroderma sera (33 patients' sera and 13 reference sera) were also tested for the presence of anti-Scl-70 antibodies. It was found that all anti-Scl-70 positive sera (n = 25) contained anti-Scl-86 antibody as well, suggesting a relationship between these two antigens. However, the Scl-86 antigen was shown to be an extremely insoluble nonchromosomal protein, resistant to boiling in sodium dodecyl sulfate. This contrasts with the Scl-70 antigen, which has been described as a thermolabile, soluble antigen present in the chromatin fraction. Together, our results are consistent with the idea that Scl-70 is a degradation product of Scl-86. The Scl-86 antigen is present in freshly prepared rabbit thymus, spleen, and liver nuclei as well as in nuclei from various cultured cell lines, but is not detectable in extractable nuclear antigen from rabbit thymus. In a limited retrospective study, the anti-Scl-86 antibodies were found in two sera from patients with Raynaud's phenomenon before the development of diffuse scleroderma. Therefore, it is possible that screening of patients' serum for this antibody might predict the development of diffuse scleroderma.

Animals↗
PubMed1989

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

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'.

Adult↗
PubMed1981

Acute diffuse scleroderma.

A case of acute diffuse scleroderma without contemporaneous visceral involvement is described. Antibodies to endothelial cells have been found in the skin.

Acute Disease↗