Lymphocytic hypophysitis is an uncommon autoimmune disease in which the pituitary gland is infiltrated by lymphocytes, plasma cells, and macrophages that could cause posterior pituitary dysfunction (diabetes insipidus) and/or anterior pituitary dysfunction. Clinicians should suspect lymphocytic hypophysitis in pregnant women and in women with recent delivery presenting with hyperprolactinemia, headache, visual field alterations, and deficiency of one or more pituitary hormones with secondary impairment of related peripheral target glands, especially when
Lymphocytic hypophysitis is an uncommon autoimmune disease in which the pituitary gland is infiltrated by lymphocytes, plasma cells, and macrophages that could cause posterior pituitary dysfunction (diabetes insipidus) and/or anterior pituitary dysfunction. Clinicians should suspect lymphocytic hypophysitis in pregnant women and in women with recent delivery presenting with hyperprolactinemia, headache, visual field alterations, and deficiency of one or more pituitary hormones with secondary impairment of related peripheral target glands, especially when122 It can also occur less frequently in prepubertal or post-menopausal women and in men.123
2.3.5.1 Prevalence and Incidence
There has been a recent increase in the number of reported cases of lymphocytic hypophysitis (from 1962–1981,16 cases; from 1982–2001, 290 cases, and from 2002-2004, 73 cases).124 The recent increase in the use of noninvasive pituitary imaging and transsphenoidal surgery likely contributes to the increased diagnosis of lymphocytic hypophysitis, as does the growing general awareness of the condition in the medical community. A 2001 study by Buxton and Robertson reported a 1 per 9 million per year incidence estimate.124 This may well be an underestimate of today’s incidence, especially since some lymphocytic hypophysitis cases may still go undiagnosed because of their indolent, subclinical course.125
2.3.5.2 Demographic Differences
There have been reported cases of lymphocytic hypophysitis in 27 of the 193 sovereign nations of the world, but principally in Japan (34%), the US (22%), the United Kingdom (7%), Germany (7%), and Canada (5%). This might reflect a geographic or ethnic variation in risk, but it is more likely due to variations in diagnosis and/or reporting.125
One study reported that lymphocytic hypophysitis is more common in women, with a female-to-male ratio of 6:1, and that woman tend to present at a younger age (35 ± 13 years) than males (45 ± 14 years). In a significant percentage of women lymphocytic hypophysitis manifests during pregnancy or postpartum.125
2.3.5.3 Key Trends and Health Outcomes
A retrospective analysis conducted from 1997 to 2014 at a single academic center identified 21 patients (13 women and eight men) with lymphocytic hypophysitis with a median diagnosis age of 37.4 years. Patients presented with various symptoms of expanding sellar mass with most common signs including headache (57%), polyuria/polydipsia (52%), vision changes (52%), and amenorrhea or decreased libido (48%). Pre-treatment endocrine evaluation revealed that 12 (57%) patients had complete anterior hypopituitarism, 11 patients (52%) had diabetes insipidus, 10 patients (48%) had mild hyperprolactinemia and three patients (14%) had isolated endocrine axis deficiencies with partial gland function.122
Therapy is directed at treating associated anterior and posterior hormone deficiencies and/or hyperprolactinemia and controlling symptoms of headache and visual disturbance that result from mass effect. A patient might need a neurosurgical biopsy to confirm the diagnosis and exclude other neoplastic and infiltrative stalk lesions, which would require different treatments.123 Despite lack of evidence-based studies, clinicians often prescribe steroids for patients with symptomatic/problematic cases.126
Spontaneous remission can occur; so, a careful follow-up is required in all patients including pituitary function testing and sellar imaging. Symptomatic patients will need medical treatments (immunosuppressive, replacement and antiprolactinemic) and neurosurgical treatments (decompression).123