Case Reports : Unusual Involvement of IgG4-Related Sclerosing Disease in Lacrimal and Submandibular Glands and Extraocular Muscles

( Yong Un Shin ),( Young Ha Oh ),( Yoon Jung Lee ) 대한안과학회 2012 Korean Journal of Ophthalmology Vol.26 No.3

Chronic sclerosing sialadenitis, also known as Kuttner tumor, is a chronic inflammatory disease of the salivary glands that is reported in a few cases in medical literature. Recent reports suggest that certain aspects of sclerosing diseases, including chronic sclerosing sialadenitis or dacryoadenitis, should be classified under immunoglobulin G4 (IgG4)-related sclerosing disease based on immunohistochemical studies. This study reports an unusual case of IgG4-related sclerosing disease appearing simultaneously in the lacrimal glands, submandibular glands, and extraocular muscles. A 56-year-old male presented with complaints of bilateral eyelid swelling and proptosis that began two years ago. Computed tomography confirmed that bilateral submandibular enlargements also existed five years ago in the subject. Orbital computed tomography and magnetic resonance imaging revealed bilateral lacrimal gland enlargement and thickening of extraocular muscles. Typical findings of chronic sclerosing dacryoadenitis were revealed upon pathologic exam of the right lacrimal gland. Immunostaining revealed numerous IgG4-positive plasma cells. Through these clinical features, we make a diagnosis of IgG4-relataed sclerosing disease in the subject.

특발성 경화성외안근염 환자에서 발생한 눈꺼풀의 IgG4 연관경화병증 1예

윤제환,정지웅,지미정,Je Hwan Yoon,Jee Woong Jung,Mi Jung Chi 대한안과학회 2013 대한안과학회지 Vol.54 No.1

Purpose: To report a case of IgG4-related sclerosing disease involving the eyelid in an idiopathic sclerosing myositis patient. Case summary: A 51-year-old woman presented with swelling, redness, and tenderness of the left lower eyelid of 1 month duration had taken an immunosuppressant for idiopathic sclerosing myositis. Eye movements showed limitation all directions but there was no exophthalmos. A palpable mass was noted in the left lower eyelid. The left extraocular muscles were hypertrophied but the lacrimal gland was normal on orbital magnetic resonance imaging. IgG4-related sclerosing disease was confirmed by immunostained biopsy from the left lower eyelid, showing infiltration of IgG4-positive lymphoplasmacytic cells. The patient was given oral steroids and an immunosuppressant and the symptoms did not recur for at least 7 months. Conclusions: IgG4-related sclerosing disease involving ocular adnexa usually consists of bilateral lacrimal gland involvement. Additionally, the orbital soft tissue involvement without dacryoadenitis is rare. The authors of the present study report a case of IgG4-related sclerosing disease involving the left lower eyelid in an idiopathic sclerosing myositis patient and should be considered in the differential diagnosis of eyelid masses.

Sclerosing Cholangitis: Clinicopathologic Features, Imaging Spectrum, and Systemic Approach to Differential Diagnosis

Seo, Nieun,Kim, So Yeon,Lee, Seung Soo,Byun, Jae Ho,Kim, Jin Hee,Kim, Hyoung Jung,Lee, Moon-Gyu The Korean Society of Radiology 2016 KOREAN JOURNAL OF RADIOLOGY Vol.17 No.1

<P>Sclerosing cholangitis is a spectrum of chronic progressive cholestatic liver disease characterized by inflammation, fibrosis, and stricture of the bile ducts, which can be classified as primary and secondary sclerosing cholangitis. Primary sclerosing cholangitis is a chronic progressive liver disease of unknown cause. On the other hand, secondary sclerosing cholangitis has identifiable causes that include immunoglobulin G4-related sclerosing disease, recurrent pyogenic cholangitis, ischemic cholangitis, acquired immunodeficiency syndrome-related cholangitis, and eosinophilic cholangitis. In this review, we suggest a systemic approach to the differential diagnosis of sclerosing cholangitis based on the clinical and laboratory findings, as well as the typical imaging features on computed tomography and magnetic resonance (MR) imaging with MR cholangiography. Familiarity with various etiologies of sclerosing cholangitis and awareness of their typical clinical and imaging findings are essential for an accurate diagnosis and appropriate management.</P>

랑게르한스세포 조직구증 환아에서 발생한 속발성 경화성 담관염 1례

김자형,최보화,김경모,문형남,Kim, Ja-Hyung,Choi, Bo-Hwa,Kim, Kyung-Mo,Moon, Hyung-Nam 대한소아소화기영양학회 2001 Pediatric gastroenterology, hepatology & nutrition Vol.4 No.1

Sclerosing cholangitis is a chronic cholestatic disease characterized by inflammation and obliterative fibrosis of the bile ducts, leading to biliary cirrhosis and ultimately to liver failure. In children, sclerosing cholangitis is known to be associated with Langerhans cell histiocytosis, as well as with congenital immunodeficiencies and cystic fibrosis. Secondary sclerosing cholangitis is suspected in Langerhans cell histiocytosis with chronic cholestasis, liver dysfunction and portal hypertension. Unlike primary sclerosing cholangitis, the cholangitis associated Langerhans cell histiocytosis is destructive in nature and progresses more rapidly to biliary cirrhosis, therefore uniformly the prognosis is poor. In this setting, liver transplantation should be considered early in children with sclerosing cholangitis complicating Langerhans cell histiocytosis before end-stage liver failure and variceal bleeding. We experienced a case of secondary sclerosing cholangitis in Langerhans cell histiocytosis in a 2-year-old boy. We report this case with brief review of the related literatures. 저자들은 복부 팽만과 지속적인 황달을 주소로 내원한 랑게르한스세포 조직구증 환아에서 간조직 검사 및 내시경적 역행성 담도조영술로 진단한 속발성 경화성 담관염 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

폐 경화성 혈관종의 전산화단층촬영 소견 : 2례보고

한영민 대한영상의학회 1993 대한영상의학회지 Vol.29 No.1

Sclerosing hemangiomas are rare benign neopla는 in pulmonary parenchyme, We analyzed CT findings of two cases of sclerosing hemangiomas of the lung in two adult females. Chest radiograph showed well-circumscuribed, round soft tissue mass with calcification in right lower lobe of one patient and without calcification and moderate or marked contrast enhancement was clearly demonstrated with CT scan. CT findings of contratst enhancement and contour of calcification allow differentiation of sclerosing hemangioma from other venign neoplasms or postinflammotory pseudotusrs of the lung. Sclerosing hemangiomas are rare benign neopla는 in pulmonary parenchyme, We analyzed CT findings of two cases of sclerosing hemangiomas of the lung in two adult females. Chest radiograph showed well-circumscuribed, round soft tissue mass with calcification in right lower lobe of one patient and without calcification and moderate or marked contrast enhancement was clearly demonstrated with CT scan. CT findings of contratst enhancement and contour of calcification allow differentiation of sclerosing hemangioma from other venign neoplasms or postinflammotory pseudotusrs of the lung.

Sclerosing Cholangitis: Clinicopathologic Features, Imaging Spectrum, and Systemic Approach to Differential Diagnosis

서니은,김소연,이승수,변재호,김진희,김형중,이문규 대한영상의학회 2016 Korean Journal of Radiology Vol.17 No.1

Sclerosing cholangitis is a spectrum of chronic progressive cholestatic liver disease characterized by inflammation, fibrosis, and stricture of the bile ducts, which can be classified as primary and secondary sclerosing cholangitis. Primary sclerosing cholangitis is a chronic progressive liver disease of unknown cause. On the other hand, secondary sclerosing cholangitis has identifiable causes that include immunoglobulin G4-related sclerosing disease, recurrent pyogenic cholangitis, ischemic cholangitis, acquired immunodeficiency syndrome-related cholangitis, and eosinophilic cholangitis. In this review, we suggest a systemic approach to the differential diagnosis of sclerosing cholangitis based on the clinical and laboratory findings, as well as the typical imaging features on computed tomography and magnetic resonance (MR) imaging with MR cholangiography. Familiarity with various etiologies of sclerosing cholangitis and awareness of their typical clinical and imaging findings are essential for an accurate diagnosis and appropriate management.

Unusual Involvement of IgG4-Related Sclerosing Disease in Lacrimal and Submandibular Glands and Extraocular Muscles

신용운,오영하,이윤정 대한안과학회 2012 Korean Journal of Ophthalmology Vol.26 No.3

Chronic sclerosing sialadenitis, also known as Kuttner tumor, is a chronic inflammatory disease of the salivary glands that is reported in a few cases in medical literature. Recent reports suggest that certain aspects of sclerosing diseases, including chronic sclerosing sialadenitis or dacryoadenitis, should be classified under immunoglobulin G4 (IgG4)-related sclerosing disease based on immunohistochemical studies. This study reports an unusual case of IgG4-related sclerosing disease appearing simultaneously in the lacrimal glands, submandibular glands, and extraocular muscles. A 56-year-old male presented with complaints of bilateral eyelid swelling and proptosis that began two years ago. Computed tomography confirmed that bilateral submandibular enlargements also existed five years ago in the subject. Orbital computed tomography and magnetic resonance imaging revealed bilateral lacrimal gland enlargement and thickening of extraocular muscles. Typical findings of chronic sclerosing dacryoadenitis were revealed upon pathologic exam of the right lacrimal gland. Immunostaining revealed numerous IgG4-positive plasma cells. Through these clinical features, we make a diagnosis of IgG4-relataed sclerosing disease in the subject.

Risks of colorectal cancer and biliary cancer according to accompanied primary sclerosing cholangitis in Korean patients with ulcerative colitis: a nationwide population-based study

오은혜,Kim Ye-Jee,Minju Kim,박승하,김태오,박상형 대한장연구학회 2023 Intestinal Research Vol.21 No.2

Background/Aims: We conducted a nationwide population-based study to investigate incidence rates of colorectal and biliary cancers according to accompanying primary sclerosing cholangitis in Korean ulcerative colitis patients. Methods: We used the Health Insurance Review and Assessment claim database from January 2007 to April 2020. Standardized incidence ratios of colorectal and biliary cancers in ulcerative colitis patients were calculated. Results: Among 35,189 newly diagnosed ulcerative colitis patients, 1,224 patients were diagnosed with primary sclerosing cholangitis. During the study period, 122 and 52 patients were diagnosed with colorectal and biliary cancers, respectively. Incidences of colorectal cancer were not higher in ulcerative colitis patients than those in the general population (standardized incidence ratios, 0.83; 95% confidence interval, 0.69–0.99), regardless of accompanied primary sclerosing cholangitis (standardized incidence ratio, 0.73; 95% confidence interval, 0.24–1.71). While incidences of biliary cancer were not higher in ulcerative colitis patients than those in the general population (standardized incidence ratio, 1.14; 95% confidence interval, 0.80–1.58), these were much higher with accompanied primary sclerosing cholangitis (standardized incidence ratio, 10.07; 95% confidence interval, 5.75–16.36). Cumulative incidences of colorectal and biliary cancers increased in patients who were diagnosed with ulcerative colitis at an older age. Conclusions: In Korean ulcerative colitis patients, colorectal cancer incidences were not higher than those in the general population regardless of accompanied primary sclerosing cholangitis. However, biliary cancer incidences were much higher in ulcerative colitis patients with primary sclerosing cholangitis than in those without, or in the general population.

Sclerosing Adenosis Diagnosed by Needle Biopsy

Heon Jin Jung(정헌진),Won Shik Han(한원식),Hyeong-Gon Moon(문형곤),Soo Kyung Ahn(안수경),Ji-Sun Kim(김지선),Dong-Yong Noh(노동영),Jung-Min Chang(장정민),Nariya Cho(조나리야),Woo Kyung Moon(문우경),In-Ae Park(박인애),Hee-Chul Shin(신 대한종양외과학회 2012 Korean Journal of Clinical Oncology Vol.8 No.2

목적 : 본 연구는 침생검을 통해 진단 받은 경화성 선증에 대해 추가적인 수술 없이 경과 관찰했을 때의 잠재적 안정성을 알아보고자 하였다. 방법 : 1996년 1월부터 2010년 5월까지 침생검을 통해 경화성 선증으로 진단된 143명의 환자의 145개 병변을 대상으로 (core-needle biopsies 118례, vacuum-assisted biopsies 27례) 의무기록 검토를 토대로 한 후향적 고찰이다. 결과 : 환자들의 BI-RADS(The American College of Radiology Breast Imaging Reporting and Data System) 분류는 다음과 같다: C3(n=8), C4a(n=123), C4b(n=11), C4c(n=e), C5(n=1). 그 중 14명은 조직검사를 재시행하였고 BI-RADS 분류 C5이면서 영상의학적 소견과 병리학적 소견의 불일치를 보인 1명의 환자가 침윤성 유방암으로 진단되어 수술을 시행하였다. 나머지 환자들은 정기적인 초음파 검사 및 유방촬영술을 통해 경과 관찰하였고, 평균 추적관찰 기간 40개월 동안 144개의 병변의 최종 BI-RADS 분류는 다음과 같다: C1(n=5), C2(n=107), C3(n=32), 경과 관찰기간 동안 142명의 환자에서 악성 유방질환이 발견된 환자는 없었다. 결론 : 침생검을 통해 경화성 선증으로 진단된 환자에 있어서 추가적인 수술 없이 정기적인 검진을 통하여 경과 관찰하는 것은 잠재적 안전성을 가지고 있다. 하지만 조직검사와 영상검사 결과의 불일치 소견을 보이는 경우 수술적 생검을 통한 최종 진단이 필요하다. Background : The aim of this study was to assess the potential safety of follow up for patients diagnosed with sclerosing adenosis through needle biopsies without additional surgery. Patients and Methods : From January 1996 to May 2010, 145 lesions in 143 sclerosing adenosis patients who underwent core-needle biopsies (n=118), vacuum-assisted biopsies (n=27) in our institution were followed. Results : The American College of Radiology Breast Imaging Reporting and Data System (BI-BADS) classification in these patients was as follows : C3(n=8), C4a(n=123), C4b(n=11), C4c(n=e) and C5(n=1). After sclerosing adenosis was diagnosed by needle biopsy, 14 patients underwent re-biopsy. Only 1 patient who had BIRAD C5 sonographic features was diagnosed with invasive ductal carcinoma and surrounding ductal carcinoma in situ. The remaining 142 patients received regular medical exam with ultrasonography and/or mammography. 144 lesions’ BI-RADS category were C1(n=5), C2(n=107) and C3(n=32). With the median follow-up period of 40 months, none of the patients developed breast malignancy. Conclusions : Our study suggest that patients with sclerosing adenosis diagnosed by needle biopsies can be safely followed without additional surgery. However, in case of discordance with images and biopsy result, surgical excision for definite diagnosis is recommended.

Prednisolone에 치료반응을 보인 경화담관염 1예

신요식 ( Yo Sig Shin ),장용석 ( Yong Seok Jang ),강민경 ( Min Kyoung Kang ),장병국 ( Byoung Kuk Jang ),박경식 ( Kyung Sik Park ),황재석 ( Jae Seok Hwang ),권중혁 ( Jung Hyeok Kwon ),강유나 ( Yu Na Kang ) 대한소화기학회 2007 대한소화기학회지 Vol.50 No.6

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by progressive fibrosis and destruction of intra- and extrahepatic bile ducts resulting in hepatic failure and death. Only the liver transplantation is the possible treatment for patients to survive. There has been a few reports that steroid is an effective treatment in autoimmune variant sclerosing cholangitis, which is thought to be a familial diseases with different etiology, and steroid responsive biliary strictures be named as immunoglobulin G4 (IgG4)-associated cholangitis (IAC). There is no reliable data regarding effective steroid treatment in autoimmue variant sclerosing cholangitis in Korea. We report a case of 32-year-old male with sclerosing cholangitis, who was diagnosed by endoscopic retrograde cholangiopancreatography (ERCP) and liver biopsy, showing favorable response to prednisolone therapy. (Korean J Gastroenterol 2007;50:402-406)