Clinical and serological comparison of 17 chronic progressive systemic sclerosis (PSS) and 17 CREST syndrome patients matched for sex, age, and disease duration.

Abstract

SUMMARY This study compares the clinical and serological differences between 17 PSS and 17 carefully matched CREST patients. Patients were matched for sex, age by decade, and, importantly, disease duration (112± 9.2 vs. 12*0±9-3 years). Muscular and skin involvement were greater for the PSS groups (p<002) and pulmonary involvement was also greater (p<0O05), at least for non-smoking PSS patients. On the other hand no clinically significant differences were found between groups for other visceral involvement -including comparisons of gastrointestinal, cardiovascular, and renal involvement. There were also no laboratory differences except in anti-RNP antibody (p<0-04).

Prevalence of Barrett's esophagus in systemic sclerosis

“…However, other data suggest that it can occur regardless of the cutaneous subtype and regardless of disease duration (44,45).…”

Immunosuppression with chlorambucil, versus placebo, for scleroderma. Results of a three‐year, parallel, randomized, double‐blind study

“…Skin score was determined by palpation of the skin over 10 surface areas (the face, back, chest, abdomen, arms, forearms, hands, thighs, legs, and feet) and was graded as follows: 0 = skin not bound down or tethered; 1 = minimal tethering; 2 = moderate tethering; and 3 = severe tethering (maximum possible index of skin scores 30). This method has a coefficient of variation of 0.08 for interobserver error (22).…”

“…This large number of individual variables mandated some form of summarization. We therefore used modifications of 10 summary indices, the rationale and formulations for which have previously been published (22,23), as methods of summarizing and semiquantitating $ Count ofjoints tender to palpation, maximum score 29. 8 Sum of 10 categorical variables representing degree of "hide binding" or "tethering," as described in Patients and Methods.…”

“…A semiquantative method (skin score) was used to assess skin "hide binding" or "tethering," as previously reported (21,22). Skin score was determined by palpation of the skin over 10 surface areas (the face, back, chest, abdomen, arms, forearms, hands, thighs, legs, and feet) and was graded as follows: 0 = skin not bound down or tethered; 1 = minimal tethering; 2 = moderate tethering; and 3 = severe tethering (maximum possible index of skin scores 30).…”

Scleroderma esophagus

“…This subset of scleroderma is characterized by limited visceral involvement, an excellent prognosis, and a high incidence of positive anticentromere antibodies [7][8][9]. In a recent carefully matched comparison between patients with scleroderma and CREST, incidence of upper gastrointestinal involvement did not differ between patients in the two groups [10].In this review, an illustrative case presentation will be followed by a summary of the clinical manifestations, pathophysiology, and treatment of esophageal scleroderma. …”