J Rhinol JR Journal of Rhinology 1229-1498 2384-4361 The Korean Rhinologic Society 10.18787/jr.2017.24.2.138 Case Report A Case of Immunoglobulin G4-Related Sclerosing Disease not Responded to Steroid in Nasal Cavity Ko Seok Hwa MD Yoon Hyung Joon MD Zheng Tao MD Jeong Jin Hyeok MD PhD Department of Otolaryngology-Head and Neck Surgery, College of Medicine, Hanyang University, Seoul, Korea. Corresponding author (ent@hanyang.ac.kr) 11 2017 30 11 2017 24 2 138 142 13 03 2017 06 04 2017 22 04 2017 Copyright © 2017 Journal of Rhinology 2017 Journal of Rhinology

IgG4-related sclerosing disease was first discovered when researching autoimmune pancreatitis. It is an autoimmune disease that causes fibrosis on the pancreas, bile duct, gall bladder, salivary glands, and lungs. It is rarely reported in the nasal cavity. The diagnosis of IgG4-related sclerosing disease is based on biopsy findings demonstrating the characteristic histopathologic findings and immunohistochemical staining. IgG4-related sclerosing disease shows good response to high-dose steroid therapy. However, some patients do not respond to this treatment. Azathioprine or rituximab can be helpful in such case. We report our experience with IgG4-related sclerosing disease treated with azathioprine for 2 years, along with a related literature review.

 IgG4-related sclerosing disease Plasma cell Nasal cavity Paranasal sinus Umehara H Okazaki K Masaki Y Kawano M Yamamoto M Saeki T Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011 Mod Rheumatol 2012 22 1 21 30 Chen Y Zhao JZ Feng RE Shi JH Li XM Fei YY Types of Organ Involvement in Patients with Immunoglobulin G4-related Disease Chin Med J (Engl) 2016 129 13 1525 1532 Zen Y Fujii T Harada K Kawano M Yamada K Takahira M Th2 and regulatory immune reactions are increased in immunoglobin G4-related sclerosing pancreatitis and cholangitis Hepatology 2007 45 6 1538 1546 Ishida M Hotta M Kushima R Shibayama M Shimizu T Okabe H Multiple IgG4-related sclerosing lesions in the maxillary sinus, parotid gland and nasal septum Pathol Int 2009 59 9 670 675 Sasaki T Takahashi K Mineta M Fujita T Aburano T Immunoglobulin G4-Related Sclerosing Disease Mimicking Invasive Tumor in the Nasal Cavity and Paranasal Sinuses AJNR Am J neuroradiol 2012 33 2 E19 E20 Lee KK Cho HP Lee YM Park JH A case of immunoglobulin G4-related sialadenitis and dacryoadenitis Korean J Otorhinolaryngol-Head Neck Surg 2013 56 7 444 447 Yoon HJ Park WS Ji YB Lee SH Two Cases of Immunoglobulin G4-Related Sclerosing Disease Mimicking Nasopharyngeal Carcinoma Korean J Otorhinolaryngol-Head Neck Surg 2016 59 4 304 308 Suzuki M Nakamaru Y Akazawa S Mizumachi T Maeda M Takagi D Nasal manifestations of immunoglobulin G4-related disease Laryngoscope 2013 123 4 829 834 Okazaki K Uchida K Miyoshi H Ikeura T Takaoka M Nishio A Recent concepts of autoimmune pancreatitis and IgG4-related disease Clin Rev Allergy Immunol 2011 41 2 126 138 Ikeda R Awataguchi T Shoji F Oshima T A case of paranasal sinus lesions in IgG4-related sclerosing disease Otolaryngol Head Neck Surg 2010 142 3 458 459 Pace C Ward S A rare case of IgG4-related sclerosing disease of the maxillary sinus associated with bone destruction J Oral Maxillofac Surg 2010 68 10 2591 2593 Lee JR Lee BJ Chung YS Three Cases of IgG4-Related Sclerosing Disease in Nasal Cavity J Rhinol 2016 23 1 44 48 Kitagawa S Zen Y Harada K Sasaki M Sato Y Minato H Abundant IgG4-positive plasma cell infiltration characterizes chronic sclerosing sialadenitis (Küttner's tumor) Am J Surg Pathol 2005 29 6 783 791 Masaki Y Kurose N Umehara H IgG4-related disease: a novel lymphoproliferative disorder discovered and established in Japan in the 21st century J Clin Exp Hematop 2011 51 1 13 20 Lindau RH Su YB Kobayashi R Smith RB Immunoglobulin G4-related sclerosing disease of the paranasal sinus Head Neck 2013 35 10 E321 E324 Endoscopic findings of nasal cavity (2014.11.13). A: Large sized perforation (arrow) with very thin mucosal membrane and severe inflammation in right nasal cavity after crust removal. B: Left Nasal cavity before crust removal. White material is a Merocel packing to protect blood aspiration during deep biopsy. C: Ulcerative lesion (arrow) around left lateral nasal wall. U: Uncinate process, M: Middle turbinate, S: Septum. Radiologic findings. CT image showing mucosal thickening of left maxillary sinus, Coronal view (A). Axial view (B). Coronal MR image showing mucosal thickening of supero-anterior portion of left lateral nasal wall with left maxillary mucosal thickening, T1-weighted image (C). T1-weighted with gadolinium enhancement (D). T2-weighted image (E). Pathologic findings. It reveals fibrosclerotic change with marked lymphoplasmacytic inflammation (H&E, ×400) (A). It shows large Immunoglobulin G4 (IgG4)-positive plasma cell in fibrosclerotic stroma, measuring 35/HPF and the ratio of IgG4 with IgG is about 40% (IgG4 immunoperoxidase stain, ×400) (B). Endoscopic findings during treatment. Septal perforation with crust and ulcerative mucosal lesion was seen during treatment (After using azathioprine 1month, 14.3.31) (A). Well healed mucosa of nasal septum was seen after stop medication (16.2.14) (B). Treatment protocol and laboratory finding during follow-up

PDS: Prednisolone, MTX: Methotrexate, AZA: Azathioprine