Lymphocytic Hypophysitis

Abstract

Background:Lymphocytic hypophysitis is a disorder of the pituitary gland that presents as a sellar mass lesion and/or hypopituitarism. It causes pituicyte destruction and hypopituitarism and is speculated to have an autoimmune basis. Diagnosis: Lymphocytic hypophysitis should be considered in the differential diagnosis of pituitary masses and/or hypopituitarism in females who are pregnant or in the early postpartum period, especially in cases associated with other autoimmune diseases or unusual patterns of hormone deficiencies. A definitive diagnosis requires tissue biopsy. A presumptive clinical diagnosis can be made based on a history of gestational or postpartum hypopituitarism, a contrast-enhancing sellar mass with imaging features characteristic of lymphocytic hypophysitis, a pattern of pituitary hormone deficiency with early loss of adrenocorticotrophic hormone and thyroid-stimulating hormone unlike that typically found with macroadenomas, relatively rapid development of hypopituitarism and a degree of pituitary failure disproportionate to the size of the mass.Symptoms resulting from partial or panhypopituitarism occur in approximately 80% of cases and multiple deficiencies are found in approximately 75% of cases. Management:Appropriate management remains controversial. Corticosteroid therapy has been advocated as a means of attenuating inflammation, but given the uncertainty of its efficacy and the known adverse effects, such therapy does not seem justified for most patients. The optimal surgical strategy involves partial resection of the mass to decompress the surrounding structures. All patients with lymphocytic hypophysitis require appropriate replacement therapy for deficient hormones. Long-term follow-up is mandatory to monitor for the development of other hormonal deficits.

Transient Polyuria Related to Central Diabetes Insipidus Caused by Lymphocytic Infundibulo-neurohypophysitis in a Patient Treated for Graves' Disease

“…Lymphocytic hypophysitis (LYH), a rare chronic inflammatory disorder of the pituitary gland developed mainly by autoimmune processes, causes pituicyte destruction and hypopituitarism (1). LYH is usually classified in lymphocytic adenohypophysitis, which is characterized by lymphocytic infiltration limited to the anterior lobe of pituitary, and lymphocytic infundibulo-neurohypophysitis (LINH), which involves the infundibulum, stalk and neurohypophysis.…”

Lymphocytic Hypophysitis Accompanied by Aseptic Meningitis Mimics Subacute Meningoencephalitis

“…The patient had polyuria for three months before admission, which suggested that inflammation may have spread from the posterior to the anterior pituitary accompanied by aseptic meningitis. Unlike LAH, some cases of LINH have not shown any autoimmune disorders, and middle-aged or oldaged male subjects can be affected with LINH (11,12). A diagnosis of comorbid hypophysitis and aseptic meningitis was challenging because of the lack of a typical mass effect, such as headaches and visual impairment, and because of the gradual clinical course that mimicked subacute meningoencephalitis, including tuberculous, fungal and carcinomatous meningitis.…”

Long-term clinical course of IgG4-related systemic disease accompanied by hypophysitis

“…It is therefore unclear whether improvements in the clinical course of this disease can be directly attributed to glucocorticoid treatment or simply reflect a natural progression [30]. Because of the uncertainty regarding the efficacy of glucocorticoid treatment and its known adverse effects, the justification for this approach typically requires a mass effect due to a swollen pituitary [31]. In the case of IgG4-related hypophysitis (Table 5), patients were treated with various doses of glucocorticoid, from a replacement dose for adrenal insufficiency to a pharmacological dose.…”