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A 61-year-old female presented with left side facial numbness for 4 months, with history of wax and wane lymphadenopathy for 12 years. Multiple lymph node biopsy revealed chronic nonspecific inflammation. Physical examination showed right cervical lymphadenopathy, zone II, size 3x4 cm, cranial nerve V: loss of pinprick sensation at left V2 dermatome. Investigation: MRI brain showed a thick enhancing dural lesion of the left lateral wall of the cavernous sinus and the adjacent medial part of the left middle cranial fossa, size 1.0x3.8x1.0 cm. Pathology results of the left juxtasellar mass biopsy indicated positive IgG staining for plasma cells, positive IgG4 with IgG4 count 50-70 cells/HPF and IgG4+/IgG+ cell ratio 60%. Blood test of serum IgG4 was 1,026 mg/dL (8-140 mg/dL). The tumor of the left juxtasellar mass was surgically removed and she received systemic corticosteroids (prednisolone 0.5 mg/kg/day). Her numbness improved and the right cervical lymph node was decreased to 2x3 cm.
2. Baptista B, Casian A, Gunawardena H, D’Cruz D, Rice CM. Neurological Manifestations of IgG4-Related Disease. Curr Treat Options Neurol. 2017;19:14.
3. Okano A, Nakatomi H, Shibahara J, Tsuchiya T, Saito N. Intracranial Inflammatory Pseudotumors Associated with Immunoglobulin G4-Related Disease Mimicking Multiple Meningiomas: A Case Report and Review of the Literature. World Neurosurg. 2015;83:1181e1-4.
4. Umehara H, Okazaki K, Kawano M, Mimori T, Chiba T. How to diagnose IgG4-related disease. Ann Rheum Dis. 2017;76:e46.
5. Khosroshahi A, Wallace ZS, Crowe JL, Akamizu T, Azumi A, Carruthers MN, et al. International Consensus Guidance Statement on the Management and Treatment of IgG4-Related Disease. Arthritis Rheumatol. 2015;67:1688-99.
6. Kamisawa T, Okazaki K, Kawa S, Shimosegawa T, Tanaka M, Research Committee for Intractable Pancreatic D, et al. Japanese consensus guidelines for management of autoimmune pancreatitis: III. Treatment and prognosis of AIP. J Gastroenterol. 2010;45:471-7.
7. Della-Torre E, Stone JH. “How I manage” IgG4-Related Disease. J Clin Immunol. 2016;36:754-63.
8. Carruthers MN, Topazian MD, Khosroshahi A, Witzig TE, Wallace ZS, Hart PA, et al. Rituximab for IgG4-related disease: a prospective, open-label trial. Ann Rheum Dis. 2015;74:1171-7.