Characteristic feautures of cholangitis with serum IgG4 elevation compared with primary sclerosing cholangitis

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1 Characteristic feautures of cholangitis with serum IgG4 elevation compared with primary sclerosing cholangitis Poster No.: C-2005 Congress: ECR 2011 Type: Scientific Paper Authors: T. Takeda, T. Ueda, K. Motoori, H. Ito; Chiba/JP Keywords: Abdomen, Biliary Tract / Gallbladder,, CT, MR, Contrast agentintravenous, Cholangiography DOI: /ecr2011/C-2005 Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 28

2 Purpose Purpose To compare radiologic features of IgG4-related sclerosing cholangitis (IgG4SC) in systemic IgG4-related disease and primary sclerosing cholangitis (PSC) in contrast-enhanced computed tomography (CECT) and magnetic resonance cholangiopancreatography (MRCP). IgG4-related disease [Overview] Etiology unknown, possibly due to autoimmune mechanizm Characterized by prominent infiltration of plasmacytes expressing IgG4 into exocrine glands or other extranodal tissues Comprehensive reassembly of previously-recognized existing diseases such as - Autoimmune pancreatitis - Pituitary disease - Retroperitoneal fibrosis - Mikulicz's disease (lacrimal/parotid gland) - Küttner tumor (submandibular gland) - Inflammatory pseudotumor (lung, breast, liver, etc.) - Interstitial nephritis - Inflammatory abdomina aortic aneurysm,... etc. Fig 1: Systemic manifestation of IgG4-related disease Page 2 of 28

3 Fig.: Systemic manifestation of IgG4-related disease References: Radiology, Chiba University Hospital - Chiba/JP Fig 2: Images of IgG4-related disease Page 3 of 28

4 Fig.: Images of IgG4-related disease References: Takahira et al.igg4-related Chronic Sclerosing Dacryoadenitis.Arch Ophthalmol.2007;125: , Saeki et al.clinicopathological characteristics of patients with IgG4-related tubulointerstitial nephritis. Kidney International 2010: 78, , Inoue et al. Immunoglobulin G4-related lung disease: CT findings with pathologic correlations. Radiology,2009: 251; a) IgG4-SC and periaortic fibrosis b) Lung manifestation c) Retroperitoneal fibrosis d) Autoimmune pancreatitis e) IgG4-related tubulointerstitial nephritis f) Mikulicz disease [Clinical Features ] Page 4 of 28

5 Nonspecific systemic symptoms such as fever, local edema, and pain, etc. Symptom depends on involved organs (Fig 1.2). Good response to corticosteroid therapy [Pathological Features] Prominent infiltration of IgG4-positive plasma cell and T lymphocyte infiltration into involved tissue. Fibrosis surround by the plasma cells. Arterial obliteration caused by inflammation. Mucosal epithelium is preserved. IgG4-related sclerosing cholangitis (IgG4-SC) A manifestation of IgG4-related disease in biliary tract Abundant IgG4-positive cells around bile duct Priviously recognized as a part of PSC before IgG4-related disease is proposed However, clear clinico-pathological distinction of IgG4-SC from PSC is gradually being recognized (Table 1) 1,2 Table 1: Clinico-pathological features of IgG4-SC and PSC IgG4-SC PSC Gender, male 80% 65% Age, years 60 y.o. or later around 40 y.o. Sympton abdominal pain, liver function abnormality abstructive jaundice Complication AIP, ulcerative colitis Retroperitoneal fibrosis, Mikulicz's disease, Küttner tumor, Inflammatory pseudo tumor, etc. Elevated serum IgG4 around 70% 7-9% Response to steroids effective basically invalid Histological findings Accumulation of IgG4 positive cells surrounding the bile duct Periductal concentric fibrosis ("onionskin") Fig3: Pathological differences between IgG4-SC and PSC Page 5 of 28

6 Fig.: Pathological difference between IgG4-SC and PSC. References: Deshpande et al. IgG4-associated cholangitis: a comparative histological and immunophenotypic study with primary sclerosing cholangitis on liver biopsy material. Modern Pathology 2009; 22: (a) Pathology of IgG4-sclerosing cholangitis shows fibrosis of bile duct surrounded by accumulation of IgG4 positive cells. (b) Pathology of PSC shows prominent periductal concentric fibrosis ("onion-skin"). Page 6 of 28

7 Methods and Materials Subjects: 18 patients (9 IgG4-SC: 9 PSC) Age yo. (median 57.7) Male: female = 9:9 Diagnostic criteria Diagnosis of IgG4-SC and PSC were made according to criteria belows; IgG4-SC: Following clinical diagnosis criteria of AIP 3 - H: Histology - I: (pancreatic) imaging - S: serology (IgG4>140 mg percent) - O: Other organ involvement - RT: Response to steroid Treatment PSC: American Association for the Study of Liver Diseases (AASLD) PRACTICE GUIDELINES 4 Image Assessment: [Modality] - Contrast enhanced CT (CECT) *Axial image *Curved MPR along the biliary tract - MR cholangiopancreatography (MRCP) Page 7 of 28

8 Radiological features of IgG4-SC and PSC were assessed categorized by Distribution of bile duct abnormality Constriction/dilatation pattern of bile duct Presence of mural thickening of bile duct Peribiliary lesions (permiative enhancement, mass formation) Wedge-shaped enhancement of liver Involvement of other organ Constriction/dilatation pattern of bile duct (#2) were evaluated with MRCP/CECT.Other findings (#1, 3, 4, 5, 6) were evaluated with CECT [Distribution of bile duct abnormality] evaluated with MRCP/CECT The distribution of bile duct abnormality was assessed categorized by following 4 anatomical region (Fig 5). Fig 5: Distribution of bile duct abnormality Page 8 of 28

9 Fig.: Evaluated four anatomical region of liver References: Radiology, Chiba University Hospital - Chiba/JP 1. Intrahepatic region 2. Hilar region 3. extrahepatic-extra pancreatic region 4. extrahepatic-intrapancreatic region [Constriction/dilatation pattern] evaluated with MRCP/CECT Constriction/dilatation patterns of abnormal bile ducts were classified into the four patterns according to the length and the frequency of constrictions( Fig6). Fig 6: Constriction/Dilatation pattern Fig.: Constriction / Dilatation pattern 1) band like short constriction, 2)band-like short constriction, 3)medium constriction, 4)long constriction Page 9 of 28

10 References: Radiology, Chiba University Hospital - Chiba/JP Band-like short constriction: Short (<5mm )constriction is seen at low frequency (one constriction with over 10mm interval, commonly over 15 mm). Beaded short constriction: Short constriction is seen at high frequency (one constriction at least in 5-6 mm). Bile duct sandwiched between constriction has been dilatated. Midium constricture(5-10mm) Long constricture(>10mm) [Mural thickening of bile duct ] evaluated with CECT Presence of wall thickening of bile duct and findings of thickened bile duct were assessed. Fig 7: Categorization of mural thickening of bile duct Page 10 of 28

11 Fig.: Mural thickening of bile duct. a)pencil-drawing margin, b)sumie-drawing margin References: Radiology, Chiba University Hospital - Chiba/JP Pencil-drawing margin(left): The margin of bile duct wall is clear as if the line is written by sharpen pencil. Sumie-drawing margin(right): The margin of bile duct wall is unclear and faded away outward as if the line is drawn with calligraphy-brush (Japanese sumie brush). [peribiliary mass formation] evaluated with CECT Fig 8: Peribiliary mass formation Fig.: Mural thickening of bile duct; mass formation References: Radiology, Chiba University Hospital - Chiba/JP The thickened bile duct wall is permeatively transit to soft-tissue mass surrounded the bile duct. Page 11 of 28

12 [Wedge-shaped enhancement of liver] evaluated with CECT Fig 9: Wedge-shaped enhancement of liver Fig.: Wedge-shaped enhancement of liver References: Radiology, Chiba University Hospital - Chiba/JP Wedge-shaped enhancement of the liver were assessed. The distribution of wedge-shape enhancement were devised into whole liver, lober, and segmental. [Involvement of other organs] evaluated with CECT Involvement to other organs was recorded (Fig 1). Page 12 of 28

13 Page 13 of 28

14 Results Results [Distribution of bile duct abnormality] Table2: Distribution of bile duct abnormality Fig.: Distribution of bile duct abnormality References: Radiology, Chiba University Hospital - Chiba/JP Distribution of bile duct abnormality showed no significant difference between IgG4-SC and PSC. [Constriction/dilatation pattern] Page 14 of 28

15 Table3: Constriction/dilatation pattern Fig.: Constriction/ Dilatation pattern References: Radiology, Chiba University Hospital - Chiba/JP IgG4-SC and PSC showed different constriction/dilatation patterns. 5 out of 9 patients (55%) with IgG4-SCs showed long constriction over 10 mm, commonly over 35 mm. 8 out of 9 (88%) patients with PSC showed short constriction less than 5 mm. [Mural thickening of bile duct and peribiliary mass formation] Table4: Mural thickening of bile duct and peribiliary mass formation Page 15 of 28

16 Fig.: Wall thickening of bile duct / peribiliary mass References: Radiology, Chiba University Hospital - Chiba/JP 8 out of 9 IgG4-SC patients (88%) and 7 out of 9 PSC patients (77%) showed mural thickening of bile duct wall. Findings of thickening were different between IgG4-SC and PSC. 8 IgG4-SC patients (88%) showed sumie-drawing margin. 7 PSC patients (77%) showed pencil-drawing margin. 4 out of 9 (44%) patients with IgG4-SCs showed peribiliary mass formation surrounding the thickened bile duct. No patients with PSC showed peribiliary mass formation Fig10: Wall thickening and peribiliary mass formation of IgG4-SC Page 16 of 28

17 Fig.: Wall thickening and peribiliary mass in IgG4 related sclerosing cholangitis References: Radiology, Chiba University Hospital - Chiba/JP [Wedge-shaped enhancement of liver] Table4: Wedge-shaped enhancement of liver Page 17 of 28

18 Fig.: Frequency of wedge-shaped enhancement of liver References: Radiology, Chiba University Hospital - Chiba/JP Wedge-shaped transient enhancement of live parenchyma, which seems like arterioportal shunt were observed in 6 patients. 2 out of 6 IgG4-SC patients (33%) with large mass formation depicted wedge-shaped enhancement. [Systemic involvement of IgG4-related disease] Table 5: Systemic involvement of IgG4-related disease Autoimmune pancreatitis 5/9 (55%) Retroperitoneal fibrosis 2/9 (22%) Rymph node swelling 2/9 (22%) Mikulicz disease (salivary gland) 1/9 (11%) Page 18 of 28

19 Tubulonterstitial nephritis 1/9 (11%) Page 19 of 28

20 Images for this section: Fig. 0: Evaluated four anatomical region of liver Radiology, Chiba University Hospital - Chiba/JP Page 20 of 28

21 Fig. 0: Constriction / Dilatation pattern 1) band like short constriction, 2)band-like short constriction, 3)medium constriction, 4)long constriction Radiology, Chiba University Hospital - Chiba/JP Page 21 of 28

22 Fig. 0: Radiographic difference between IgG4-SC and PSC Radiology, Chiba University Hospital - Chiba/JP Page 22 of 28

23 Fig. 0: Wedge-shaped enhancement of liver Radiology, Chiba University Hospital - Chiba/JP Page 23 of 28

24 Conclusion Conculusion: Table5: Summary of radiographic features of IgG4-SC and PSC IgG4-SC PSC Length of constriction longer (>10mm) shorter (<5m) Margin of bile duct unclear clear sumie-brush drawing pencil-drawing Peribiliary mass formation + - Wedge-shaped enhancement of liver ++ ± Fig 11: Radiographic difference between IgG4-SC and PSC Page 24 of 28

25 Fig.: Radiographic difference between IgG4-SC and PSC References: Radiology, Chiba University Hospital - Chiba/JP Long constriction over 10 mm are seen with IgG4-SC, in contrast to short constriction with PSC. Fig 12: Peribiliary mass formation with wedge-shaped enhancement in patients with IgG4-SC. Page 25 of 28

26 Fig.: Peribiliary mass formation with wedge-shaped enhancement in patients with IgG4-SC. References: Radiology, Chiba University Hospital - Chiba/JP Page 26 of 28

27 References Kamiwsawa et al. Sclerosing cholangitis associated with autoimmune pancreatitis differs from primary sclerosing cholangitis.world J Gastroenterol. 2009;15: Alderlieste et al. Immunoglobulin G4-associated cholangitis: one variant of immunoglobulin G4-related systemic disease.digestion. 2009;79(4):220-8 Chapman et al. Diagnosis and Management of Primary Sclerosing Cholangitis. Hepatology 2010; 51: Chiri et al. Diagnosis of autoimmune pancreatitis. Clin Gastroenterol Hepatol 2006; 4 :1010 Page 27 of 28

28 Personal Information Fig.: Chiba university hospital References: Radiology, Chiba University Hospital - Chiba/JP Tomoko Takeda, MD Inohana, Chuo-ku, Chiba, , Japan. Chiba University Hospital Department of Radiology takeda.hypers0mn1@gmail.com [Acknowledgment] We thank Ms. Risa Ishimura for medical illustrations. Page 28 of 28