Successful treatment of far-advanced progressive systemic sclerosis by D-penicillamine

A 52-yr-old man had chronic recurrent pseudo-obstruction of the colon, along with multisystem disease secondary to progressive systemic sclerosis (PSS). D-Penicillamine treatment reversed the PSS, which involved the duodenum, small and large intestines, lungs, myocardium, and proximal and peripheral skin. The improvement was documented by electrocardiogram pulmonary function studies, roentgenography of gastrointestinal tract, and by repeated skin biopsies. Hand blood-volume studies using 99m Tc also revealed improvement of PSS in both hands. A variable degree of improvement was documented in seven additional patients with PSS. Follow-up periods were 18 mo to 4 yr. The most common side effect of the drug was transient hypogeusia, and no patient showed a serious side effect. This article includes a review of the literature on penicillamine therapy for PSS.