{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T00:01:58Z","timestamp":1772150518060,"version":"3.50.1"},"reference-count":0,"publisher":"Jaypee Brothers Medical Publishing","issue":"11","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JAPI"],"published-print":{"date-parts":[[2025,11,10]]},"abstract":"<jats:p>Introduction: Immunoglobulin G4-related disease (IgG4-RD) is a rare autoimmune \nfibroinflammatory condition that can affect multiple organs. Central nervous system (CNS) \ninvolvement is seen in only 2\u20134% of cases. Due to its rarity and heterogeneous presentation, \nit often mimics malignancies, infections, or other inflammatory conditions, leading to delayed \ndiagnosis. We report two cases illustrating the spectrum of CNS IgG4-RD and highlight diagnostic \nand therapeutic considerations.\nCase 1: A 29-year-old male presented with new-onset generalized tonic-clonic seizures. Brain \nmagnetic resonance imaging (MRI) revealed a left-sided, extra-axial dural-based enhancing lesion \nwith vasogenic edema. Serum IgG4 was elevated (3.25 gm\/dL), but whole body positron emission \ntomography\u2013computed tomography (PET-CT) ruled out systemic involvement. Surgical resection \nof the lesion was performed. Histopathology revealed a lymphoplasmacytic infiltrate with fibrosis \nand an IgG4:IgG plasma cell ratio of 20%. The patient was treated with tapering corticosteroids \nand methotrexate, leading to complete radiological resolution and seizure control.\nCase 2: A 44-year-old woman with longstanding hypothyroidism presented with headache, \ntinnitus, polydipsia, and polyuria. Laboratory investigations revealed panhypopituitarism. Imaging \nrevealed an enlarged pituitary with systemic fluorodeoxyglucose (FDG)-avid lesions on PET-CT. \nSerum IgG4 was elevated (3.01 gm\/L). A diagnosis of probable IgG4-related hypophysitis with \nmultisystem involvement was made. She was managed with pulse methylprednisolone followed \nby oral steroids, methotrexate, and desmopressin. Follow-up showed clinical and radiological \nimprovement, and serum IgG4 levels normalized.\nDiscussion: These cases demonstrate the clinical heterogeneity of CNS IgG4-RD, ranging from \nisolated pachymeningitis mimicking neoplasia to multisystem hypophysitis with systemic uptake. \nWhile the 2020 diagnostic criteria emphasize an IgG4:IgG ratio \u226540%, case 1 underscores that lower \nratios (e.g., 20%) may still be diagnostically relevant, particularly in meningeal disease. Both patients \nresponded well to corticosteroids and methotrexate, supporting their role as effective first-line \ntreatment. These cases add to the growing evidence base for CNS-specific IgG4-RD and emphasize \nthe need for organ-specific diagnostic flexibility and long-term immunosuppressive strategies.<\/jats:p>","DOI":"10.59556\/japi.73.1244","type":"journal-article","created":{"date-parts":[[2025,12,18]],"date-time":"2025-12-18T09:17:13Z","timestamp":1766049433000},"page":"54-56","source":"Crossref","is-referenced-by-count":1,"title":["Intracranial IgG4-related Disease: Insights from Two Cases"],"prefix":"10.59556","volume":"73","author":[{"given":"Shaurav","family":"Khanna","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sandeep","family":"Yadav","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C","family":"Balakrishnan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"2914","published-online":{"date-parts":[[2025,11,10]]},"container-title":["Journal of the Association of Physicians of India"],"original-title":[],"deposited":{"date-parts":[[2025,12,18]],"date-time":"2025-12-18T09:17:14Z","timestamp":1766049434000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.japi.org\/article\/japi-73-11-s1-54"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,11,10]]},"references-count":0,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2025,11,10]]},"published-print":{"date-parts":[[2025,11,10]]}},"URL":"https:\/\/doi.org\/10.59556\/japi.73.1244","relation":{},"ISSN":["0004-5772"],"issn-type":[{"value":"0004-5772","type":"print"}],"subject":[],"published":{"date-parts":[[2025,11,10]]}}}