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Published on: July 11, 2013
Treatment of generalized scleroderma: updated results
Acta Dermato-Venereologica
|January 1, 1979
Summary
Long-term treatment using connective-tissue biosynthesis inhibitors can significantly regress scleroderma. Factors like age and disease duration influence treatment success, with untreated scleroderma progressing relentlessly.
Area of Science:
- Biochemistry
- Dermatology
- Pharmacology
Background:
- Scleroderma is a progressive connective tissue disease characterized by dermal sclerosis.
- Untreated scleroderma typically shows inexorable progression.
Purpose of the Study:
- To evaluate the efficacy of long-term treatment with connective-tissue biosynthesis inhibitors in generalized progressive scleroderma.
- To identify factors influencing treatment outcomes.
Main Methods:
- Patients received long-term treatment with various inhibitors including D-penicillamine, hydralazine, and corticosteroids.
- Disease activity was monitored using urinary fractions of hydroxyproline, hydroxylysine, and uronic acid.
Main Results:
- Total or subtotal regression of dermal sclerosis was observed in 40.8% of patients.
- Partial regression occurred in 33.1%, and disease progression arrested in 14.8%.
- Younger patients, those with shorter disease duration, and those receiving higher total doses showed better prospects.
Conclusions:
- Connective-tissue biosynthesis inhibitors offer a viable treatment option for generalized progressive scleroderma, achieving regression in a significant proportion of patients.
- Treatment outcomes are influenced by patient age, disease duration, and cumulative drug dosage.
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