title: Treatment of generalized scleroderma: updated results.
abstract: Long-term treatment of patients with generalized progressive scleroderma by means of inhibitors of connective-tissue biosynthesis brings about total or subtotal regression of dermal sclerosis in 40.8%, partial regression in 33.1%, arrest of progression without regression in 14.8%, while in 11.3% it had no effect whatsoever. The drugs used were D-penicillamine, benzyl-penicillin-diethyl-aminoethylesterhydro-iodide, glutamine, hydralazine, chlorpromazine, L-dopa, diphenylhydantoin, and corticosteroids. Disease activity before, during and after treatment was indicated by the urinary fractions of high-molecular hydroxyproline and hydroxylysine containing peptides and of uronic acid, break-down products of collagen and acid glycosaminoglycans of connective-tissue ground substance. The prospects were better for young patients than for old, for those with a short history than for the longstanding disease cases, and for those having a large total dose than for those who had less. If left untreated, scleroderma progresses inexorably.
mesh_terms: D002746:Chlorpromazine / Q000627:therapeutic use; D003094:Collagen / Q000037:antagonists & inhibitors / Q000096:biosynthesis*; D004341:Drug Evaluation; D005973:Glutamine / Q000627:therapeutic use; D006025:Glycosaminoglycans / Q000037:antagonists & inhibitors*; D006801:Humans; D006830:Hydralazine / Q000627:therapeutic use; D007980:Levodopa / Q000627:therapeutic use; D010396:Penicillamine / Q000627:therapeutic use; D010400:Penicillin G / Q000627:therapeutic use; D010672:Phenytoin / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use; D012595:Scleroderma, Systemic / Q000188:drug therapy*
chemical_list: D006025:Glycosaminoglycans; D005973:Glutamine; D006830:Hydralazine; D007980:Levodopa; D010672:Phenytoin; D003094:Collagen; D010396:Penicillamine; D010400:Penicillin G; D002746:Chlorpromazine; D011241:Prednisone