Endocrine Abstracts

Introduction: IgG4-related disease is a newly recognized entity associated with autoimmune conditions involving almost every organ system, characterized by elevated serum IgG4 as well as mass like tissue infiltration by IgG4-positive plasma cells. Pituitary gland can be involved and can presented as hypophysitis.

Case description: We report a case of a 56-year-old man, who has been suffering for the nine last years of episodes of fatigue and headaches with spontaneous remission was admitted to our hospital for headaches, fatigue, myalgia, diplopia and visual disturbances. The endocrine assessment revealed a complete ante-hypopituitarism. Dynamic LHRH and CRH tests revealed a delayed but robust response of gonadotrophins and corticotrophin. MRI scan showed nodular thickening of the pituitary and pituitary stalk with minimal compression of the optic chiasm. Other pathology than sarcoidosis was suspected and further investigation showed high levels of blood IgG4. All these findings were highly suggestive for a diagnosis of IgG4-related hypophysitis. Steroids were administered and within 24 hours, the patient became completely asymptomatic. A month later, thyroid and gonadal function normalized. Cranial MRI scan showed a net decrease in the size of the pituitary gland compared to the previous examination.

Conclusions: We report a case of IgG4-related hypophysitis who responded completely and rapidly to steroids. Intriguingly, according to his pas medical history, he might have presented a cyclical evolution of his disease with periods of spontaneous remission. It’s a rare condition but increasingly recognized and should be sought in face of an acute swelling of the pituitary gland and/or its stalk, since its favorable response to steroid therapy should help to avoid unnecessary surgery.

Keywords: Hypophysitis, IgG4-disease, panhypopituitarism