Ketoconazole for the treatment of refractory hypercalcemic sarcoidosis.

Hypercalcemia is a recognised complication of sarcoidosis due to excess 1,25-hydroxyvitamin D3 production by macrophages. Systemic corticosteroids inhibit 1,25-hydroxyvitamin D3 production, but long term therapy is often required to maintain normocalcemia. Ketoconazole is an imidazole antifungal that inhibits macrophage 1 alpha-hydroxylation of 25-hydroxyvitamin D3 and has been used in paraneoplastic hypercalcemia. We report a case series of four patients with relative contraindications to corticosteroids in whom treatment 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 corticosteroids are relatively contraindicated.