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JUVENILE WILSON'S DISEASE: HISTOLOGICAL AND FUNCTIONAL CORRELATIONS DURING PENICILLAMINE THERAPY

Clinical improvement and cupruresis occur during penicillamine(PCN) therapy for Wilson's Disease(WD), but long-term effects of the drug on hepatic morphology are virtually unknown. We studied 6 patients(pts) with WD (diagnosed at 7-24 yrs) who were clinically asymptommatic at follow-up(f/u) liver biopsy 2-7 yrs after onset of treatment. By comparison to pre-treatment biopsies (3 pts), there was marked reduction in portal fibrosis in 1 (7 yr f/u) and significant but less impressive decreases in 2 (2 yr f/u). Portal inflammation was greatly diminished in all 3, with periportal necrosis still present in 1 (2 yr f/u). None had complete restitution of normal architecture. At f/u, 3 pts on therapy for 3,5 or 7 yrs (without initial biopsy) had dense portal cirrhosis; in 2, portal lymphocytic infiltration was prominent; in 1, there was marked fatty change. Hepatocellular non-bilirubin pigment was abundant in 5/6 f/u specimens. Hepatic Cu++ levels were 80-750 μg/g (n1<50) and did not correlate with the degree of pigment deposition or healing. Liver function reverted to normal in 5/6 pts; serum Cu++ levels fell 50% and remained low. Hypoceruloplasminemia persisted in all. K-F rings faded or disappeared. One pt has hepatic dysfunction, cirrhosis and K-F rings despite therapy. The data show that morphological improvement occurs in some PCN-treated pts with WD, but that this is not predicted by function tests. Sequential biopsies are needed to evaluate fully the extent of healing.