Serum and urinary aminoterminal type III procollagen peptide in progressive systemic sclerosis: relationship to sclerodermal involvement, serum hyaluronan and urinary collagen metabolites.

Increased serum values of aminoterminal type III procollagen peptide and hyaluronan (hyaluronate) and enhanced urinary content of hydroxyproline and hydroxylsine containing polypeptides were demonstrated in patients with progressive systemic sclerosis (PSS). The serum propeptide level and the relative urinary excretion of hydroxyproline as polypeptides were related to the extent of cutaneous involvement. Elevated serum propeptide and hyaluronan values were seen in patients who progressed within the following 6 months.

Blister spreading, a diagnostic test in pemphigus. Loss of epidermal and dermoepidermal cohesion.

Differentiation of the pemphigus group of diseases from other bullous dermatoses can be established by spreading of blisters by gentle finger pressure. In combination with histopathology, the same test is diagnostic for every single pemphigus disease.

Scleroderma.

After a review of some pathogenetic and pathologic aspects of scleroderma, the experimental effects of a group of agents that can inhibit the formation of connective tissue, especially the biosynthesis of collagen, are mentioned. These substances were transferred to clinical therapy of scleroderma. Regular determinations of disease activity and guidance of therapy with quantitative and semiquantitative physical and biochemical technics are of utmost importance because treatment without guidance allows for no disclosure of therapeutic failure or recurrence of the disease in due time for readjustment of the treatment.

Bioavailability of D-penicillamine in relation to gastrointestinal involvement of generalized scleroderma.

The bioavailability of D-penicillamine was measured in 24 patients with generalized scleroderma (Progressive Systemic Sclerosis, PSS). Esophageal changes characteristic of generalized scleroderma were present in 15 of the patients, and 3 of those patients had duodenal involvements as well. The plasma concentrations of D-penicillamine were measured at 0 h, 1 h, 2 h, and 4 h after an oral dose of 300 mg D-penicillamine.

Bioavailability of D-penicillamine in a patient with gastrointestinal progressive systemic sclerosis.

D-penicillamine pharmacokinetics were studied in a patient with gastrointestinal progressive systemic sclerosis possibly complicated by malabsorption. D-penicillamine bioavailability was examined after oral, duodenal, intravenous and rectal administration. No D-penicillamine was detectable in plasma after administration to the gastrointestinal tract.