Lymphocytic hypophysitis: modern day management with limited role for surgery.

Abstract

We set out to describe the modern day management of Lymphocytic hypophysitis (LH) with respect to its diagnosis, treatment and long-term endocrine and visual outcomes. We deliberately included patients with a clinical diagnosis of LH and compared them with those with a histological diagnosis following surgery. A multi-centre observational study was performed. Twenty-two patients were included and studied over a mean follow-up period of 8.6years (range 4-26years). Patients presented with headache (N=15; 68%), visual field disturbances (N=7; 32%) or a combination of these symptoms (N=5; 23%). The time lag between onset of symptoms and diagnosis was <1month, 1-6months or >6months in approximately a third of the patients each, respectively. In two-thirds of the patients (N=14) LH was diagnosed based on their clinical, biochemical and radiological findings. The surgical cohort (N=8) was statistically more likely to have presented with larger lesions, with suprasellar extension and greater visual field defects. During follow-up there was improvement in headaches and visual symptoms, but the rate of hypopituitarism as reflected by the need for on-going pituitary hormone replacement persisted. At the 1, 5 and 10year follow-up there was no significant difference between the medically and surgically managed cohorts in terms of ongoing symptomatology or need for pituitary hormone replacement. In the modern era, most patients with LH are diagnosed by non-surgical means and managed medically. There remains a significant time lag between the onset of symptoms and the eventual diagnosis of LH. Despite the considerable morbidity attached to LH, overall prognosis is good.