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Abstract
Objective: to assess the efficacy, tolerance and determinants for relapse in patients
with porphyria cutanea tarda treated with low-dose oral chloroquine. Design: open
trial with a median follow-up of 3 years. Setting: outpatient referral unit of a university
hospital. Patients: 53 patients with low-moderate iron overload or intolerance to
phlebotomies. Intervention: 250 mg twice weekly oral chloroquine diphosphate until
remission or failure to respond. Measurements: porphyrin excretion, biochemical changes
and development of side effects. Results: after administration of a median dose of
23.5 g of chloroquine (limits 12.6–56 g) during a median time of 8 months (limits:
1–26 months), 50 patients (94%) reached a metabolic remission (urinary uroporphyrin
excretion < 100 μg/l). In 14 of these patients (28%), porphyrin excretion further
decreased after finishing chloroquine therapy. Metabolic remission persisted during
24 months (limits 6–97 months). Side-effects (severe pruritus) appeared only in one
patient. Twenty-two patients relapsed, the relapses being associated with greater
basal values of serum AST, ALT, gammaglobulin, urinary uroporphyrin and to the time
needed to achieve remission. One year and three years after finishing therapy the
probabilities of relapse were 12% (95% C.I.: 5–27%) and 49% (95% C.I.: 34–67%), respectively.
Time to achieve remission was the only independent predictor of relapse (hazard ratio:
1.2, 95% C.I.: 1.05–1.21, P < 0.01). Conclusion: low-dose oral chloroquine is a safe therapy that promotes a
high proportion of remission and sustained control of porphyria cutanea tarda associated
with low-moderate iron overload.
Keywords
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Article info
Publication history
Accepted:
December 24,
1993
Received in revised form:
December 15,
1993
Received:
July 26,
1993
Identification
Copyright
© 1994 Published by Elsevier Inc.
