A Curious Case Of “Double Cushing Syndrome ”

ABSTRACT Objective: Chronic exogenous steroid therapy can lead to iatrogenic Cushing syndrome (CS). However, concomitant presence of endogenous CS in a patient on exogenous steroids has not been previously reported. We report a case of a 23-year-old who had manifestations of both exogenous as well as endogenous CS. Methods: We present a case report and literature review. Results: A 23-year-old woman presented to us with severely deforming polyarthritis of both small and large joints since childhood, associated with recurrent bouts of uveitis. She was on triamcinolone therapy for the last 16 years, with cushingoid features and severely reduced bone mineral density, presumably due to exogenous CS. Anthropometry revealed severely retarded height for age and weight for age. She underwent hormonal profile assessment for evaluation of her hypothalamic-pituitary-adrenal axis status, which revealed the concurrent presence of adrenocorticotropic hormone-independent endogenous hypercortisolemia. Abdominal imaging showed bulky and nodular adrenal glands. A diagnosis of primary pigmented nodular adrenal disease in the background of juvenile idiopathic arthritis was considered, as was therapy with chronic glucocorticoid treatment. She was started on methotrexate and analgesics. Surgery was also recommended, however she refused bilateral adrenalectomy. Conclusion: This case highlights that both exogenous and endogenous CS can coexist in an individual. Furthermore, diagnosis of endogenous CS in a patient on chronic steroid therapy requires a high degree of clinical suspicion. Abbreviations: ACTH = adrenocorticotropic hormone; CECT = contrast-enhanced computed tomography; CS = Cushing syndrome; JIA = juvenile idiopathic arthritis; PPNAD = primary pigmented nodular adrenal disease