Pneumocystis carinii pneumonia associated with a rapid reduction of cortisol level in a patient with ectopic ACTH syndrome treated by octreotide and ketoconazole

A case is herein reported of pneumocystis carinii pneumonia in a 60-year-ol d female patient with ectopic production of ACTH at a position 2 cm superio r to her right clavicle, revealed in an octreotide scan. Her extremely high plasma ACTH and cortisol levels (460 pg/ml and 80 mu g/dl, respectively) w ere markedly decreased with the combined treatment of octreotide (300 mu g/ d) and ketoconazole (600 mg/d). As her serum cortisol concentration decreas ed, pneumocystis carinii pneumonia occurred on the third day of treatment. A secondary E. coli infection was superimposed and the patient died of diss eminated intravascular coagulation and adult respiratory distress syndrome. This case suggests that primary prophylaxis fur pneumocystis carinii infec tion should be initiated before cortisol lowering therapy, especially when the plasma cortisol concentration is excessively high, and that early adjun ctive glucocorticoid therapy can reduce the acute mortality in patients wit h endogenous Gushing's syndrome and Pneumocystis carinii pneumonia. This ca se study would also like to point out that plasma ACTH and cortisol levels were decreased effectively by the combination of octreotide and ketoconazol e in this instance of ectopic ACTH syndrome.