Cryptococcosis and sarcoidosis: strange bed-fellows. A report of five cases

Background and Aim: Sarcoidosis is known to predispose to cryptococcal infection. In this case series. variations in presentation, diagnostic dilemmas and responses to treatment are highlighted. Methods: Of several hundred patients referred to the sarcoidosis clinic at The Prince Charles Hospital, Brisbane and to the private clinic of the co-author between 1990 and 2002, five subsequently developed cryptococcal infections as a complication of sarcoidosis. All five subjects were treated by the co-author (RKAA). A review of the literature was also performed. Results: Cryptococcal infection occurred in 4 patients with sarcoidosis being treated with steroids and one patient who was not on treatment. All responded to antifungal therapy. Cryptococcosis was diagnosed by transbronchial (2), bronchial (1) and cutaneous (2) biopsies. Fluconazole was used in those with pulmonary infections but not in cutaneous disease where excision sufficed. One patient with pulmonary cryptococcosis from immunosuppressants also developed a nocardial brain abscess. Conclusions: Our series of patients with sarcoidosis and cryptococcal infections is unique in Australia. Although an unusual infection, cryptococcosis should always be considered in patients with sarcoidosis as it may be overlooked particularly in the lungs and can be fatal if untreated. Further immune dysregulation through steroid use may contribute significantly to the disease manifestations. Understanding why cryptococcosis and not other infections is more common in patients with sarcoidosis may reveal more about the mechanisms of granuloma formation and the nature of sarcoidosis itself.