Intravenous methylprednisolone pulse therapy in the treatment of Graves' ophthalmopathy.

Graves' ophthalmopathy (GO) is a specific immune-mediated disorder, whose treatment is sometimes difficult. In order to investigate the efficacy of intravenous methylprednisolone (MP) pulse therapy in GO, we studied eight patients with GO, followed up for at least 6 months by clinical patient self-assessment, ophthalmological examination and orbital computed tomography (OCT). A 12.5 mg/kg dose of MP was administered intravenously over a 10 hour period, once every month. Three to six MP pulse administrations were performed in each patient. All patients were outpatients. A 0.5 mg/kg/day oral prednisone dose was given to each patient as interpulse therapy. Clinical assessment of MP pulse therapy showed a good response in 87.5% and no response in 12.5% of patients. The treatment was rapidly efficient, mostly on patient self-assessment, soft tissue inflammation, ophthalmoplegia, corneal involvement, visual acuity and extraocular muscle enlargement on OCT. Post-treatment ophthalmic index was significantly improved (6.75 +/- 3.06 vs. 2.5 +/- 1.41: p < 0.05). MP pulse therapy had less effect on proptosis (22.94 +/- 2.32 mm vs. 21.56 +/- 2.22 mm: p < 0.05). No adverse effects were noted with MP pulse therapy. Patients showed no relapse of eye involvement during a mean follow up of 31.8 months (2-77 months). In conclusion, our results suggest that intravenous MP pulse therapy is a good immunosuppressive therapy for GO. Moreover, in comparison with the previous studies, the MP dose used in our present study appears to be optimal with high efficacy. MP pulse therapy represents a safe and efficient treatment in GO, which can easily be performed in outpatients.

[Food-dependent Cushing syndrome: a new entity of organic hypercorticism]. / Le syndrome de Cushing alimentaire: une nouvelle entité d'hypercorticisme organique.

Diagnosis of Cushing's syndrome is quite difficult in endocrinology. Spontaneous Cushing's syndrome is usually divided into two subgroups, one which is dependent on corticotropin (ACTH) and another one which is not. In the first class are Cushing's disease, the ectopic corticotropin syndrome and the rare ectopic corticotropin-releasing hormone (CRH) syndrome; these ACTH-dependent Cushing's syndrome have usually diffusely enlarged adrenal glands. In the second class are cortisol producing unilateral adrenocortical adenomas or carcinomas, and the recent Cushing's syndrome with food dependent periodic hormonogenesis. This food dependent Cushing's syndrome is an ACTH-independent Cushing's syndrome with multinodular enlargement of both adrenal glands. Pathogenesis is an aberrant adrenal sensitivity to physiologic secretion of gastric inhibitory peptide (GIP). Ectopic expression of GIP receptors on adrenal cells involve pathologic food induced cortisol secretion. Food dependent Cushing's syndrome is a new cause of Cushing's syndrome. Food induced cortisol secretion may have to be explored in the ACTH-independent Cushing's syndrome.