LBMON134 Igg4 Related Hypophysitis And Skull Base Lesion: Retrospective Analysis Of 10 Years’ Experience Of A Tertiary Care Hospital Of Northern India.

Abstract

Abstract Introduction 'IgG4-related' disease is a spectrum of autoimmune phenomenon that clinically manifests with multisystem disorder in nearly 50% of the cases. The nervous system involvement is noted only in 4% of cases. Hypophysitis is considered as rare inflammation of the pituitary gland and/or stalk that results in pituitary enlargement, that may or may not be associated with hypopituitarism. Design and setting: Retrospective study from a tertiary care hospital of North India. Methods and patients Biopsy proven IgG4-RD patients with various systemic manifestations registered in Postgraduate Institute of Medical Education and Research, Chandigarh between 2012 and 2022 were retrospectively analyzed. Results Among 3006 operated sellar and parasellar SOL since 2012, only 5 patients had IgG4 related hypophysitis and skull base lesion. After considering all organ systems, nearly 154 biopsy proven IgG4-RD had been reported from this institute. Three of those five patients were male and two were female. Mean age of presentation 32.2±9.49 years. Median duration of illness at presentation was 1 year. Two out of 5 cases reported with Headache, diminution of vision was the presenting complaints in rest of the three cases. Two of the 5 patients presented with some form of hypopituitarism. MRI showing Sellar space occupying lesion in 2 patients, although posterior pituitary bright-spot was absent in one case, none of them had diabetes insipidus at presentation. Three cases had stalk thickening on MRI. A female patient had retroperitoneal fibrosis leading to obstructive uropathy after four years of follow up of hypophysitis, one had associated craniopharyngioma, and one with testicular failure. Except one patient, all 3 patients had normal serum IgG4 level, data of one patient was missing. Methylprednisolone was the cornerstone of therapy for almost all the cases. Discussion Hypophysitis and inflammatory CNS lesions are not uncommon phenomena but relatively rare with IgG4-RD. IgG4-RD is diagnosed based on clinical and pathological criteria. The proposed diagnostic criteria include (1) characteristic diffuse or localized swelling or masses in single or multiple organs; (2) elevated serum IgG4 level; (3) histopathology demonstrating infiltration of lymphocytes and plasmacytes with the demonstration of IgG4 plasma cells (ratio of IgG4 to IgG cells > 40% and > 10 IgG4 plasma cells/high power field) and storiform fibrosis and /or obliterative phlebitis. The diagnosis is considered definite in the presence of all findings, probable if 1 and 3 are present, possible if 1 and 2 are present. Since they are inflammatory excellent steroid responsiveness has been documented in the literature. In this study we reported various presentation of hypophysial and skull base IgG4-RD which often confused as tumorous lesion, warrant high index of suspicion for diagnosis. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.