Session: SUN 29-49-Congenital Adrenal Hyperplasia & Ectopic Cushing's
Poster Board SUN-35
Glucocorticoids immunossuppressive properties are well known and they are a common treatment option for autoimmune diseases. Endogenous hypercortisolism is the hallmark of Cushing‘s syndrome. We present a case of rheumatoid arthritis (RA) unmasked after Cushing’s syndrome cure.
A 53 year old female with past history of hypertension presented with a 3 year history of abdominal obesity, facial plethora, easy bruising, proximal muscle weakness and buffalo hump. Laboratory results showed urinary cortisol 303μg/24h (20-90) and ACTH 13.7pg/dL (<46). She was admitted in the Endocrinology department for functional testing: 48hour low dose dexamethasone suppression test showed no suppression of cortisol (baseline 34.7μg/dL (5-25); 48hr: 22.3μg/dL). Pituitary MRI revealed a microadenoma and inferior petrosal sinus sampling was done and no evidence of central/peripheric gradient or CRH stimulation response was observed. Adrenal CT showed enlargement of both the adrenals. As the initial differential diagnosis was not clear she started ketoconazole to a maximum dose of 800mg/day. After 1 year of medical therapy blood results showed ACTH< 5pg/dL and the CT revealed adrenal nodules with 4cm on right adrenal and 3cm on the left. Independent ACTH Cushing´s syndrome was assumed and the patient was submited to bilateral adrenalectomy. Replacement with hydrocortisone and fludrocortisone was started. Pathology confirmed adrenal adenomas. Two months latter she developed severe poliarthargias and morning stiffness and was admitted in rheumatologic department. Blood results revealed ACTH 18.3pg/dL, high sedimentation rate (83mm/h) and high levels of rheumatoid factor (2170U/mL). Rheumatoid arthritis was diagnosed. Her glucocorticoid replacement dose was increased and metrothrexate was added with resolution of the symptoms a week later.
This patient represents a natural illustration of the antiinflammatory effects of supraphysiological levels of endogenous glucocorticoids. Development of RA symptoms occurred only after the surgical cure of Cushing´s syndrome. This suggests that it is important to follow the development and/or course of autoimmune diseases after the treatment of Cushing's syndrome.
Nothing to Disclose: FS, FA, RF, CSM, RF, HS, SD, CS, AMS
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