What Is Sarcoidosis?

Abstract

Irecently consulted on a young woman who had acquired fever, profuse pulmonary shadowing, and liver function abnormalities following a 1997 visit from her residence in upstate New York to the Mississippi Valley. An open-lung biopsy at that time revealed noncaseating epithelioid granulomas (NCG) consistent with sarcoidosis; fungal elements were not evident. The treating physician initiated prednisone therapy, but after a week, a histoplasmoFor editorial comment see page 6 sis complement fixation titer of 512, later supplemented by isolation of Histoplasma capsulatum organisms from the biopsy sample, led to its replacement with a short course of amphotericin B, followed by months of itraconazole therapy, with the expectation that her progressive disseminated histoplasmosis would resolve. She was unavailable for follow-up until 2001, when she was re-referred for evaluation of increasingly severe shortness of breath. Prednisone had been provided by her primary physician. A chest radiograph showed profuse pulmonary shadowing; high-resolution CT demonstrated findings characteristic of advanced stage IV sarcoidosis; pulmonary function tests revealed a severe restrictive defect accompanied by a marked reduction in diffusing capacity. Prednisone was discontinued because of the concern that her histoplasmosis