[01] Amber Hoang, University of Central Florida College of Medicine, Orlando, Florida, United States. [02] Sarina M. Amin, University of Florida College of Medicine, Department of Ophthalmology, Gainesville, Florida, United States. [03] Syed Gibran Khurshid, University of Florida College of Medicine, Department of Ophthalmology, Gainesville, Florida, United States.

Objective: The objective of this paper is to present a case report of IgG4 disease with associated hypertrophic pachymeningitis and panuveitis that primarily presented to an eye clinic for evaluation of symptoms. The purpose of this paper is to help clinicians realize the broad clinical differential of panuveitis when presenting with concurrent systemic complaints. Methods: A 52-year-old Hispanic female with a history of panuveitis of the left eye presents with left eye pain, severe headaches, confusion and dizziness. Ophthalmic examination of the left eye revealed nongranulomatous panuveitis and optic disc edema. She was started on topical corticosteroids, however her vision continued to deteriorate and her systemic symptoms became more prevalent. She was admitted for further evaluation and for initiation of intravenous corticosteroids due to the severity of presentation. Results: She was diagnosed with idiopathic hypertrophic pachymeningitis after clinical, radiological, and laboratory evaluation. Dural biopsy was performed and histopathologic analysis revealed IgG4 cell population predominance consistent with IgG4-related disease. Conclusion: We report a case of uveitis and hypertrophic pachymeningitis as manifestations of systemic IgG4 disease with elevated p-ANCA and myeloperoxidase antibodies.

IgG4 Disease, p-ANCA, Myeloperoxidase, Pachymeningitis, Uveitis

[01] Lindstrom KM, Cousar JB, Lopes MB. IgG4-related meningeal disease: clinico-pathological features and proposal for diagnostic criteria. Acta Neuropathol. 2010 Dec; 120(6): 765-76. doi: 10.1007/s00401-010-0746-2. Epub 2010 Sep 16. [02] Mattoo H, Mahajan VS, Della-Torre E, et al. De novo oligoclonal expansions of circulating plasmablasts in active and relapsing IgG4-related disease. J Allergy Clin Immunol. 2014 Sep; 134(3): 679-87. doi: 10.1016/j.jaci.2014.03.034. Epub 2014 May 6. [03] Mahajan VS, Mattoo H, Deshpande V, et al. IgG4-related disease. Annu Rev Pathol. 2014; 9: 315-347. [04] Jeannin P, Lecoanet S, Delneste Y, et al. IgE versus IgG4 production can be differentially regulated by IL-10. J Immunol. 1998; 160(7) :3555-3561. [05] Satoguina JS,Weyand E, Larbi J, et al.T regulatory-1 cells induce IgG4 production by B cells: role of IL-10. J Immunol. 2005; 174(8): 4718-4726. [06] Umehara H, Nakajima A, Nakamura T, et al. IgG4-related disease and its pathogenesis - cross-talk between innate and acquired immunity. Int Immunol. 2014; 26(11): 585-595. [07] Wallace ZS, Carruthers MN, Khosroshahi A, et al. IgG4-related disease and hypertrophic pachymeningitis. Medicine (Baltimore). 2013 Jul; 92(4):206-16. doi: 10.1097/MD.0b013e31829cce35. [08] Deshpande V, Zen Y, Chan JK, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol. 2012 Sep; 25(9):1181-92. doi: 10.1038/modpathol.2012.72. Epub 2012 May 18. [09] Lu LX, Della-Torre E, Stone JH, et al. IgG4-related hypertrophic pachymeningitis: clinical features, diagnostic criteria, and treatment. JAMA Neurol. 2014 Jun; 71(6):785-93. doi: 10.1001/jamaneurol.2014.243. [10] Carruthers MN, Khosroshahi A, Augustin T, et al. The diagnostic utility of serum IgG4 concentrations in IgG4-related disease. Ann Rheum Dis. 2014. [11] Khosroshahi A, Stone JH. Treatment approaches to IgG4-related systemic disease. Curr Opin Rheumatol. 2011 Jan; 23(1):67-71. doi: 10.1097/BOR.0b013e328341a240. [12] Ghazale A, Chari ST, Zhang L, et al. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology 2008; 134: 706–715. [13] Khosroshahi A, Carruthers MN, Deshpande V, et al. Rituximab for the treatment of IgG4-related disease: lessons from 10 consecutive patients. Medicine (Baltimore). 2012 Jan; 91(1):57-66. doi: 10.1097/MD.0b013e3182431ef6. [14] Khosroshahi A, Bloch DB, Deshpande V, et al. Rituximab therapy leads to rapid decline of serum IgG4 levels and prompt clinical improvement in IgG4-related systemic disease. Arthritis Rheum. 2010 Jun; 62(6):1755-62. doi: 10.1002/art.27435. [15] Kamisawa T, Shimosegawa T, Okazaki K, et al. Standard steroid treatment for autoimmune pancreatitis. Gut. 2009 Nov; 58(11):1504-7. doi: 10.1136/gut.2008.172908. Epub 2009 Apr 26. [16] Lee CS, Harocopos GJ, Kraus CL, et al. IgG4-associated orbital and ocular inflammation. J Ophthalmic Inflamm Infect. 2015 May 29;5:15. doi: 10.1186/s12348-015-0047-y. eCollection 2015. [17] Iguchi A, Wada Y, Kobayashi D, et al. A case of MPO- and PR3-ANCA-positive hypertrophic cranial pachymeningitis with elevated serum IgG4. Mod Rheumatol. 2013 Jan; 23(1):151-5. doi: 10.1007/s10165-012-0613-1. Epub 2012 Feb 15. [18] Popkirov S, Kowalski T, Schlegel U, et al. Immunoglobulin-G4-related hypertrophic pachymeningitis with antineutrophil cytoplasmatic antibodies effectively treated with rituximab. J Clin Neurosci. 2015 Jun; 22(6):1038-40. doi: 10.1016/j.jocn.2014.12.020. Epub 2015 Apr 7.