INTRODUCTION: Cushing Syndrome, characterized by endogenous over-production of glucocorticoids, is a rare risk factor for the development of Pneumocystis Jiroveci pneumonia (PJP). We report the case of a patient who developed PJP as well as a fungal cavity in the setting of Cushing Syndrome due to ectopic adrenocorticotropin (ACTH) production.
CASE PRESENTATION: A 72 y/o male with hypertension (HTN), diabetes mellitus (DM), and hypothyroidism initially presented to the hospital with 2-3 months of worsening lower extremity edema, hyperglycemia, hypokalemia and a 12-pound weight loss. He had had increased emotional lability over the past month, confusion and decreased generalized strength. The patient denied fevers, chills, shortness of breath, cough, abdominal pain, nausea, vomiting, or diarrhea. The social history was significant for a 30 pack-year smoking history, quit 25 years prior to presentation. Vital signs were significant for moderate hypertension and mild hypoxia. Physical exam revealed decreased but clear breath sounds bilaterally,significant pitting edema in the both lower extremities to the mid-thigh, ecchymoses, thin skin and slight confusion. Laboratory values were significant for a sodium of 147mmol/L, a potassium of 2.3mmol/L, and a bicarbonate of 37mmol/L. Morning cortisol was significantly elevated at 320 mcg/dL, as was the ACTH at >1700 pg/dL. A dexamethasone suppression test failed to show suppression and ectopic ACTH production was diagnosed. HIV testing was negative. A chest computed tomography (CT) scan initially demonstrated a nodule in the right middle lobe. A positron emission tomography-CT (PET-CT) obtained two weeks after the initial CT revealed diffuse ground glass opacities with intense diffuse metabolic activity as well as a cavitary lesion in the left lower lobe that was new. Shortly after the PET scan was obtained the patient developed hypoxemic respiratory failure and ultimately required intubation. Bronchoscopy at this time was positive for PJP, and eventually grew a mold of the Exophalia species. Other bacterial, mycobacterial, and fungal work-up was negative. The patient improved on trimethoprim/sulfamethoxazole and antifungal therapy and was extubated. Eventually, the patient elected to pursue palliative care due to his increasing debility.
DISCUSSION: Traditionally, the development of PJP in non-HIV patients is associated with exogenous glucocorticoid use. This case reminds us that endogenous hyper-cortisolism also can create an immunocompromised state. Previous case series have shown that Cushing Syndrome, particularly that associated with ectopic ACTH production, is associated with the development of PJP and fungal infections. Not surprisingly these reports have shown that higher levels of cortisol are associated with a greater risk of developing these opportunistic infections. Our patient’s cortisol level was extremely high and was comparable to the highest levels in previous case series. As for the Exophalia mold isolated from the patient, it is unclear whether this was causative of the cavitary lesion or if it was an airway contaminant. However, there have been reports of it causing invasive pulmonary disease.
CONCLUSIONS: When taking care of patients with endogenous hypercortisolism it is important to mindful of the potential for opportunistic infections similar to those found in patients on pharmacologic immunosuppression.
Reference #1 Sarlis NJ, Chanock SJ and Neiman LK. Corisolemic indices predict severe infections in Cushing syndrome due to ectopic production of adrenocorticotropin. J Clin Endocrinol Metab. 2000 Jan; 85(1): 42-7.
DISCLOSURE: The following authors have nothing to disclose: Armand Ryden, Matthew Leibowitz, Roger Lee, Arin Aboulian, M. Iain Smith
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