Ketoconazole for the treatment of refractory hypercalcemic sarcoidosis

Hypercalcemia is a recognised complication of sarcoidosis due to excess 1,2 5-hydroxyvitamin D-3 production by macrophages. Systemic corticosteroids in hibit 1,25-hydroxyvitamin D-3 production, but long term therapy is often re quired to maintain normocalcemia. Ketoconazole is an imidazole antifungal t hat inhibits macrophage 1 alpha-hydroxylation of 25-hydroxyvitamin D-3 and has been used in paraneoplastic hypercalcemia. We report a case series of f our patients with relative contraindications to corticosteroids in whom tre atment with ketoconazole allowed cessation or reduction in the maintenance dose of corticosteroids. We conclude that ketoconazole should be considered as an alternative therapy for hypercalcemic sarcoidosis when corticosteroi ds are relatively contraindicated.