A case of relapsed autoimmune hypothalamitis successfully treated with methylprednisolone and azathioprine

Autoimmune hypothalamitis is a rare autoimmune neuroendocirne disease. A case of a 70-year-old female with autoimmune hypothalamitis was reported. The chief clinical characteristics were diabetes insipidus and adenopituitary function deficiency. Cranial magnetic resonance imaging (MRI) scan indicated a mass in the hypothalamus. The diagnosis of autoimmune hypothalamitis was presumed. After treatment with prednisone, there was a marked reduction in the mass and the hypothalamus-adenopituitary function partially improved. However, after glucocorticoid therapy was withdrawn, the hypothalamic lesion relapsed progressively. High dose methylprednisolone pulse therapy (HDMPT) in combination with azathioprine was initiated thereafter. During follow-up, MRI scan indicated the lesion shrank strikingly, and the patient's clinical condition improved as well. In view of the good response of the hypothalamic lesion to glucocorticoid and immunodepressant, the putative diagnosis of autoimmune hypothalamitis was confirmed. This case report suggested that HDMPT in combination with azathioprine therapy might be an effective trial for autoimmune hypothalamitis treatment.