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Background: Immunoglobulin (Ig)G4-related disease is a systemic fibroinflammatory disease characterized by dense infiltration of IgG4-positive plasma cells in the affected tissue(s) with or without elevated plasma levels of IgG4. Hypophysitis itself is a very rare disease with reported prevalence in the operative specimens are around 0.2–0.88%. IgG4-related hypophysitis (IgG4-RH) may account for a substantial percentage of cases previously regarded as idiopathic hypophysitis. Methods: This study is a registry-based, retrospective, and cohort study from a tertiary care hospital in North India. The medical records and clinical data of biopsy-proven and suspected IgG4-RH patients registered were retrospectively analyzed. Treatment outcome of cases was also explored during this analysis. Results: Two thousand and six sellar area space-occupying lesions have been operated-on since 2006, among them only four patients had IgG4-RH on histopathological specimen. One case was diagnosed on clinical suspicion. Mean age of the patients was 31.8 ± 6.32 years. Most frequent presenting complaint was headaches. Extracranial manifestations were present in four patients. The most common pituitary dysfunction was cortisol deficiency. 18 F-fluorodeoxyglucose positron emission tomography (18F FDG PET) was helpful in three cases for diagnosis of hypophysitis and other organ involvement. Classical histological findings with storiform fibrosis, obliterative phlebitis seen in two cases, and IgG4-positive plasma cell infiltration were positive in four cases. Surgery was the primary modality of treatment in all four cases. Only one patient received steroids as a primary therapeutic modality. Conclusion: IgG4-RH is rare. High index of suspicion is required to diagnosis the case precisely. FDG PET is helpful in diagnosing hypophysitis and extrapituitary lesions.

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.

Skin Involvement is seen nearly 76 per cent of Systemic lupus erythematosus (SLE) patients, but bullous lesion however accounting for less than one per cent of cutaneous manifestation of lupus erythematosus. A 21-year-old female presented with fever, polyarthalgia and vesicobullous skin lesion and acute renal failure. Clinico-pathological evaluation imparted she was having bullous systemic lupus erythematosus (BSLE) with lupus nephritis. She was put on rituximab after initial resuscitation. She had responded dramatically with resolution of skin lesion and recovery from acute renal failure. She was followed up for one year without complication with stable disease course.