Arlt A, Harbeck B, Anlauf M, Alkatout I, Klöppel G, Fölsch U R, Bewig B, Mönig H
1st Department of Medicine, University Hospital Schleswig-Holstein, Campus Kiel, Kiel, Germany.
Exp Clin Endocrinol Diabetes. 2008 Oct;116(9):515-9. doi: 10.1055/s-2008-1062729. Epub 2008 Apr 1.
Immunosuppression with subsequent opportunistic infections is a well-recognized complication of severe hypercortisolism. We report a case of fatal pneumocystis jirovecii pneumonia (formerly pneumocystis carinii pneumonia) in a case of ectopic Cushing's syndrome caused by a neuroendocrine carcinoma of the kidney. The 36-year old male patient had consulted a physician because of weight gain. Further endocrine diagnostic work-up revealed ACTH-dependent hypercortisolism of non-pituitary origin. Because of rapid clinical deterioration therapy with metyrapone was initiated. A neuroendocrine carcinoma of the right kidney with regional lymph node infiltration was identified and was suspected to be the source of the ACTH excess. Before any causal therapy could be initiated, the patient developed severe pneumocystis jirovecii pneumonia and died shortly thereafter from multiorgan failure one month after he first consulted a physician. Pneumocystosis has been reported in only a few cases of Cushing's syndrome. There seems to be a relationship between the degree of hypercortisolism and the susceptibility to opportunistic infections. Since ACTH concentrations may be excessively high in ectopic Cushing's syndrome and pneumocystosis may deteriorate as a consequence of decreasing circulating cortisol levels under adrenolytic therapy, prophylaxis against pneumocystis jirovecii infection should be considered.
免疫抑制及随后的机会性感染是严重皮质醇增多症公认的并发症。我们报告一例由肾神经内分泌癌引起的异位库欣综合征患者发生致命性肺孢子菌肺炎（原称卡氏肺孢子虫肺炎）的病例。该36岁男性患者因体重增加就诊。进一步的内分泌诊断检查显示为非垂体来源的促肾上腺皮质激素（ACTH）依赖性皮质醇增多症。由于临床迅速恶化，开始使用甲吡酮治疗。发现右肾神经内分泌癌伴区域淋巴结浸润，怀疑是ACTH过量的来源。在能够开始任何病因治疗之前，患者发生了严重的肺孢子菌肺炎，首次就诊后1个月因多器官功能衰竭不久后死亡。仅在少数库欣综合征病例中报告过肺孢子菌病。皮质醇增多症的程度与机会性感染的易感性之间似乎存在关联。由于异位库欣综合征中ACTH浓度可能过高，且在肾上腺溶解疗法下循环皮质醇水平降低可能导致肺孢子菌病恶化，因此应考虑预防肺孢子菌感染。
