비강내 악성종물로 오인된 부비동의 Immunoglobulin G4 연관 경화성 질환 1예 = A Case of Immunoglobulin G4-Related Sclerosing Disease of the Paranasal Sinus Mimicking Nasal Malignancy

다국어 초록 (Multilingual Abstract)

Immunoglobulin G4 (IgG4)-related sclerosing disease (RSD) is a chronic inflammatory disorder characterized by elevated serum IgG4 levels as well as an abundant infiltration of IgG4-positive plasmacytes. Involvement of the paranasal sinus is very rare, so it is difficult to make an accurate diagnosis. Hence, various differential diagnoses, which typically include elevated serum IgG4 levels and the infiltration of IgG4-postive cells into tissues, need to be excluded, especially for malignant diseases and mimicking disorders. Systemic corticosteroids are generally effective at inducing IgG4-RSD remission. Recently, a 43-yearold woman presented with a mass originating from the ethmoid sinus, suggesting malignancy. The patient underwent biopsy of the lesion. The pathologic specimen revealed the magnitude of IgG4-positive plasma cell infiltration, which was consistent with IgG4-RSD. In addition, serum IgG4 levels were markedly increased. After glucocorticoid treatment, the serum IgG4 levels decreased and the symptoms improved. We present a rare case of IgG4-RSD with isolated involvement of the paranasal sinuses.

참고문헌 (Reference)

1 이재령, "비강 내에 발생한 면역글로불린G4 연관 경화성 질환 3예" 대한비과학회 23 (23): 44-48, 2016

2 이경규, "면역글로불린 G4 연관 타액선 및 눈물샘염(Mikulicz 병) 1예" 대한이비인후과학회 56 (56): 444-447, 2013

3 Carruthers MN, "The diagnostic utility of serum IgG4 concentrations in IgG4-related disease" 74 : 14-18, 2015

4 Khosroshahi A, "Rituximab therapy leads to rapid decline of serum IgG4levels and prompt clinical improvement in IgG4-related systemic disease" 62 : 1755-1762, 2010

5 Takano K, "Recent advances in knowledge regarding the head and neck manifestations of IgG4-related disease" 44 (44): 7-17, 2017

6 Cheuk W, "Ocular adnexal lymphoma associated with IgG4+ chronic sclerosing dacryoadenitis: a previously undescribed complication of IgG4- related sclerosing disease" 32 (32): 1159-1167, 2008

7 Ishida M, "Multiple IgG4-related sclerosing lesions in the maxillary sinus, parotid gland and nasal septum" 59 (59): 670-675, 2009

8 Alt JA, "Locally destructive skull base lesion: IgG4-related sclerosing disease" 3 (3): e41-45, 2012

9 Vandjelovic ND, "Immunoglobulin G4-related sclerosing disease of the paranasal sinuses: A case report and literature review" 7 (7): 85-89, 2016

10 Zen Y, "IgG4-related sclerosing cholangitis with and without hepatic inflammatory pseudotumor, and sclerosing pancreatitis-associated sclerosing cholangitis: do they belong to a spectrum of sclerosing pancreatitis?" 28 (28): 1193-1203, 2004

11 Zen Y, "IgG4-positive plasma cells in inflammatory pseudotumor (plasma cell granuloma) of the lung" 36 (36): 710-717, 2005

12 Moteki H, "IgG4 related chronic rhinosinusitis: a new clinical entity of nasal disease" 131 (131): 518-526, 2011

13 Hamano H, "Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis" 359 (359): 1403-1404, 2002

14 Hamano H, "High serum IgG4 concentrations in patients with sclerosing pancreatitis" 344 : 732-738, 2001

15 Deshpande V, "Consensus statement on the pathology of IgG4-related disease" 25 : 1181-1192, 2012

16 Kitagawa S, "Abundant IgG4-positive plasma cell infiltration characterizes chronic sclerosing sialadenitis (Küttner’s tumor)" 29 (29): 783-791, 2005