Autoimmune lymphocytic hypophysitis in association with autoimmune eye disease and sequential treatment with...

Autoimmune lymphocytic hypophysitis associates predominantly with other autoimmune endocrinopathies and is most commonly treated with glucocorticoids and/or decompressive pituitary surgery. Here we report a new association and treatment modality for lymphocytic hypophysitis.

A 52-year-old woman presented with scleritis, uveitis, facial palsy, and central diabetes insipidus, accompanied by thickened pituitary stalk and enlarged pituitary on cranial MRI. Neurosarcoidosis was suspected and treatment with glucocorticoids and methotrexate initiated. Since symptoms persisted, infliximab (a monoclonal antibody that antagonizes tumor necrosis factor alpha) was added to her regimen. The patient initially improved but after 6 months developed recurrent pituitary enlargement, bilateral optic neuritis, and panhypopituitarism. To ascertain the nature of the pituitary lesion, she underwent transsphenoidal biopsy, which revealed lymphocytic hypophysitis with numerous CD20 positive B lymphocytes. The pathological finding suggested to us that administration of rituximab (a monoclonal antibody that lyzes B cells expressing CD20) could be useful. Following two courses of rituximab, the pituitary mass resolved and the corticotroph axis partially recovered. The patient has remained in remission during 3 years of follow up.

This is the first report of hypophysitis occurring with the triad of scleritis, uveitis, and optic neuritis, as well as the first immunotherapy based on the sequential use of infliximab and rituximab.