The prevalence of IgG4-related hypophysitis

IgG4-related disease (RD) is a newly recognized clinical condition that was first proposed following the close observation of patients with autoimmune pancreatitis in 2001. It is characterized by IgG4+ plasma cell and lymphocyte infiltration and an elevated serum IgG4 concentration. Owing to the limited number of reported cases, the prevalence and clinical characteristics of IgG4-related hypophysitis remains unknown.

Bando et al. screened 170 consecutive outpatients with hypopituitarism and/or diabetes insipidus (DI) to estimate its prevalence. They detected seven new cases of IgG4-related hypophysitis  in patients with hypopituitarism and/or DI, and outlined the clinical characteristics of this disease.

Six of the seven cases showed a thickened pituitary stalk and/or pituitary mass. Except in one patient, these abnormal structures shrank significantly in response to glucocorticoid therapy. However, pituitary function was not restored following steroid therapy in any of the patients.

Their study reveals a higher than expected prevalence of IgG4-related hypophysitis in patients with hypopituitarism/DI. As IgG4-RD may cause hypopituitarism/DI associated with IgG4-related hypophysitis and they suggest that patients with IgG4-RD should be carefully followed up. Additionally, IgG4-RD should be considered in all cases presenting with hypophysitis, especially as it responds well to treatment and also as it is a systemic disease.

Read full article at Bando et al. (2014) European Journal of Endocrinology 170; 161-172; DOI: 10.1530/EJE-13-0642