Utility of rituximab treatment for exophthalmos, myxedema, and osteoarthropathy syndrome resistant to corticosteroids due to Graves' disease: a case report.

BACKGROUND: Exophthalmos, myxedema, and osteoarthropathy syndrome is a very rare condition that is associated with Graves' disease. The presence of dermopathy and the involvement of joint/bone tissues indicate that it seems to be related with the severity of the autoimmune process. Owing to its low incidence, there is a lack of information regarding its treatment and clinical follow-up. Some cases improved after use of high doses of steroids; however, some patients do not respond to this treatment. Recently, the effectiveness of rituximab for treatment of Graves' ophthalmopathy resistant to corticosteroids has been demonstrated. However, it has never been used for the treatment of exophthalmos, myxedema, and osteoarthropathy syndrome (particularly for the treatment of osteoarticular manifestations). CASE PRESENTATION: We present the case of a 54-year-old Mexican woman previously treated for Graves' disease who developed post-iodine hypothyroidism and exophthalmos, myxedema, and osteoarthropathy that did not improve after high doses of steroids (intravenous and oral). Her exophthalmos, myxedema, and osteoarthropathy syndrome symptoms improved as early as 6 months after treatment with rituximab. CONCLUSION: Exophthalmos, myxedema, and osteoarthropathy syndrome is a non-classical presentation of Graves' disease, whose clinical manifestations could improve after treatment with rituximab, particularly in those patients with lack of response to high doses of corticosteroids.

A Rare Presentation of Primary Hyperparathyroidism with Concurrent Aldosterone-Producing Adrenal Carcinoma.

Aldosterone-producing adrenocortical carcinomas are an extremely rare cause of hyperaldosteronism (<1%). Coexistence of different endocrine tumors warrants additional screening for multiple endocrine neoplasia syndromes, especially in young patients with large or malignant masses. We present the case of a 40-year-old man with a history of hypertension that presented with an incidental left adrenal tumor during an ultrasound performed for nephrolithiasis. Biochemical assessment showed a mildly elevated calcium (11.1 mg/dL), high parathyroid hormone, and a plasma aldosterone concentration/plasma renin activity ratio of 124.5 (normal < 30), compatible with primary hyperparathyroidism with a concomitant primary hyperaldosteronism. A Tc99m-MIBI scintigraphy showed an abnormally increased tracer uptake in the right superior parathyroid and abdominal computed tomography confirmed a left adrenal tumor of 20 cm. The patient underwent parathyroidectomy and adrenalectomy with final pathology reports of parathyroid hyperplasia and adrenal carcinoma with biochemical remission of both endocrinopathies. He was started on chemotherapy, but the patient developed a frontal cortex and an arm metastasis and finally died less than one year later.