Progressive systemic sclerosis (PSS, scleroderma) dermal fibroblasts synthesize increased amounts of...

Fibroblast monolayers were established by explant culture of dermis from seven patients with early, rapidly advancing PSS (scleroderma) and from six normal individuals. Net GAG synthesis was measured as [3H]glucosamine incorporation into the polymer and also as total uronic acid accumulation in the cultures. Cultures from patients with PSS accumulated up to fivefold more GAG than did normal cultures. Evidence suggests that this is a result of increased synthesis rather than decreased degradation. PSS cultures simultaneously accumulated increased amounts of CSP as well as GAG. Data obtained from studies of several monolayers, followed for as many as 10 generations, show that the difference between PSS and normal cultures can be propagated. The total quantity of GAGs was increased in PSS cultures, and there were differences in the distribution of individual GAGs on a percentage basis in PSS and normal cell cultures. GAG, as well as collagen, may be important in the development of connective tissue thickening and induration in scleroderma.