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Painless obstructive jaundice - think beyond malignancy

Painless obstructive jaundice - think beyond malignancy. Lorraine Ma Ruttonjee Hospital. Patient. 56/M , Good past health , Non-smoker , non-drinker Presented with on and off epigastric discomfort, bloating sensation for 2 weeks No abdominal pain

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Painless obstructive jaundice - think beyond malignancy

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  1. Painless obstructive jaundice- think beyond malignancy Lorraine Ma Ruttonjee Hospital

  2. Patient • 56/M , Good past health , Non-smoker , non-drinker • Presented with on and off epigastric discomfort, bloating sensation for 2 weeks • No abdominal pain • Seen private practitioners and given antacids with no relieve • Clinically jaundice, vague epigastric ?mass • WCC normal • Bil 102 / ALP 629 / ALT 313 / Amylase 247 ?malignant biliary obstruction - ?cholangiocarcinoma ?Ca pancreas

  3. CT Arterial Portal-venous

  4. Diffuse marked enlargement of the pancreaswith compression and marked narrowing of upper SMV and medial splenic vein • Obliteration of the distal CBD with moderate dilatation of the biliary tree. • Minimal rind of enhancing soft-tissue densities are also seen around the lower abdominal aorta just above IMA origin. • Represent IgG4-related disease with autoimmune pancreatitisand minimal periaortitis / retroperitoneal fibrosis.

  5. Autoimmune pancreatitis • First described in 1961 • Raised gammaglobin in chronic idiopathic pancreatitis • Suspecting autoimmune involvement • 2 distinctive forms denoted in 2003 • Type 1 AIP – lymphoplasmacytic sclerosing pancreatitis • Part of IgG4-related diseases • Type 2 AIP – idiopathic duct-centric chronic pancreatitis Sarles H, et al. Chronic inflammatory sclerosing of the pancreas-An autonomous pancreatic disease? Am J Dis 1961;6:688-98 Notohara K, et al. Idiopathic chronic pancreatitis with periductallymphoplamacytic infiltration. Clinicopathologic features of 35 cases. Am J SurgPathol2003;27:1119-27

  6. Autoimmune pancreatitis (AIP) • Frequently presents as painless obstructive jaundice with or without epigastric mass • Mimicking malignancy biliary obstruction, specifically cancer of the pancreas • Others may present as acute/ chronic pancreatitis

  7. KamisawaT, et al. Recent advances in autoimmune pancreatitis: type1 and type . Gut 2013;62:1373-80 KannoA, et al, and the Research Committee on Intractable Diseases of Pancreas. Nationwide epidemiological survey of autoimmune pancreatitis in Japan. In 2011 Pancreas 2015; 44: 535–39.

  8. Diagnosing of AIP – type 1 • Imaging • Parenchymal – CT / MRI • Ductal – ERCP / MRCP • Serology – IgG / IgG4 • Histology • FNAC • Core biopsy • Resection • Other organ involvement • Response to steroids Shimosegawa T, Chari ST, Frulloni L, et al.Guidelines of the International Association of Pancreatology International Consensus Diagnostic Criteria for Autoimmune Pancreatitis.Pancreas 2011; 40:352-358

  9. Imaging – Parenchymal - CT / MRI • Diffuse enlargement -‘sausage-shaped’ pancreas • Delayed enhancement • >90% AIP • Low levels of enhancement in adenocarcinomas / lymphomas (>70%) • Pancreatic ‘envelope’ • Hypoattenuating / hypointense capsule –like peripheral rim Proctor RD, et al. Autoimmune pancreatitis: An illustrated guide to diagnosis. Clinical Radiology 68 (2013) 422-432

  10. CT Arterial Portal-venous Proctor RD, et al. Autoimmune pancreatitis: An illustrated guide to diagnosis. Clinical Radiology 68 (2013) 422-432

  11. MRI T1 T2 Proctor RD, et al. Autoimmune pancreatitis: An illustrated guide to diagnosis. Clinical Radiology 68 (2013) 422-432

  12. Imaging – Ductal – ERCP / MRCP • Long Irregular narrowing of the main pancreatic duct (MPD) • Lack of upstream dilatation from narrowing • Multiple strictures • Side branches from narrowing • MRCP preferred in the absence of symptomatic biliary obstruction that requires intervention • But ERCP shows the pancreatic strictures clearer Sugumar A, Levy MJ, Kamisawa T, et al. Endoscopic retrograde pancreatography criteria to diagnose autoimmune pancreatitis: an international multicentre study. Gut 2011; 60:666-670

  13. Lack upstream dilatation ERCP Long stricture Side branching Multiple strictures Sugumar A, Levy MJ, Kamisawa T, et al. Endoscopic retrograde pancreatography criteria to diagnose autoimmune pancreatitis: an international multicentre study. Gut 2011; 60:666-670

  14. Serology – IgG4 2001; 344:732-8 • High IgG4 levels in autoimmune pancreatitis • Sensitivity 95% specificity 97% • Levels corresponds to disease activity

  15. J Gastroenterol Hepatol. 2009 Jan;24(1):15-36 • AIP vs control (n=1258) • Sensitivity: 82.4% (P=0.078) • Specificity: 94.6% (P<0.001) • AIP vs pancreatic cancers (n=434) • Sensitivity: 82.3% (P=0.043) • Specificity: 95.4% (P=0.001)

  16. et al. Am J Gastroenterol 2007;102:1646–1653) ICDC recommends 2x upper normal limit as evidence for definite AIP

  17. Typical parenchymal imaging & significantly elevated IgG4sufficient in making diagnosis in Type 1 autoimmune pancreatitis In the absence of histology / ductal imaging (ERP/MRP)

  18. ? Pancreatic cancer ?Autoimmune pancreatitis

  19. Histology • Collection method: • Ampullary biopsies • Useful in diffuse type / pancreatic head AIP • EUS-guided core biopsy / FNA • Focal type AIP • Resection • Findings: • Periductal lymphoplasmacytic infiltrate without granulocytic infiltration • Obliterative phlebitis • Storiform fibrosis • Abundant (>10cells/HPF) IgG4-postives cells K Terumi. et al. Role of endoscopy in the diagnosis of autoimmune pancreatitis and immunoglobulin G4-related sclerosing cholangitis Digestive Endoscopy 2014; 26: 627–635

  20. Other organ involvement

  21. IgG4-associated cholangitis • Intrapancreatic biliary strictures - resembles Ca Pancreas • Proximal extrahepatic biliary strictures -mimick cholangiocarcinoma • Intrahepatic strictures - similar to primary sclerosing cholangitis • Multifocal strictures. Ghazale A, et al. Immunoglobulin G4-Associated Cholangitis: Clinical Profile and Response to Therapy. Gastroenterology 2008;134:706-715

  22. Treatment for AIP Steroids (Prednisolone) • Induction: • 0.6mg/kg/day for 2-4 weeks initially • then tapered by 5 mg every 1–2 weeks • Maintenance • 2.5-5mg/day for 6-12 months • Withdrawal • Depends on disease activity • Within 3 years Kamisawa T, Okazaki K, Kawa S, et al. Research Committee for Intractable Pancreatic Disease and Japan Pancreas Society Japanese consensus guidelines for management of autoimmune pancreatitis. III. Treatment and prognosis of AIP. J Gastroenterol 2010;45:471-7.

  23. Treatment of AIP • Relapses • Immunomodulators • Azathioprine, mycophenolate mofetil (MMF), and methotrexate • Commonly used as maintenance but no clinical trials • Rituximab (CD-20 monoclonal antibody) • Usefulness still not proven Kamisawa T, et al. Japanese consensus guidelines for management of autoimmune pancreatitis. III. Treatment and prognosis of AIP. J Gastroenterol2010;45:471-7. KamisawaT, et al. IgG4-related diasease. Lancet 2015; 385:1460-71 Stone JH , et al. IgG4-Related Disease . N Engl J Med 2012;366:539-51.

  24. Back to our patient Steroid therapy Type 1 autoimmune pancreatitis • Fluctuating liver enzymes and bilirubin levels between 35-100 • Epigastric mass subsided • No enlarged salivary glands • Ca19.9 normal (18.6) , AFP/CEA also normal • IgG 21g/L (upper normal limit 15.6) • IgG4 1092mg/dL (upper normal limit 100)

  25. Take-home message • Autoimmune pancreatitis as an uncommon but important differential diagnosis of painless obstructive jaundice • Can be diagnosed with TYPICAL imaging findings with positive serology results • Look out for other organ involvement

  26. Thank you

  27. Shimosegawa T, et al. International Consensus Diagnostic Criteria for Autoimmune Pancreatitis. Pancreas 2011; 40:352-358

  28. Kanno A, et al, and the Research Committee on Intractable Diseases of Pancreas. Nationwide epidemiological survey of autoimmune pancreatitis in Japan. In 2011 Pancreas 2015; 44: 535–39

  29. Mikulicz’s disease Kamisawa T, et al. IgG4-related diasease. Lancet 2015; 385:1460-71

  30. PET-CT • Not as a diagnostic tool • Helps in locating other organ involvement • Monitor disease progresss K. Nakatani et al. Utility of FDG PET/CT in IgG4-related systemic disease Clinical Radiology 2012 67:297-305

  31. Relapses & Prognosis • Recurrence in 22.2 % patients (193/869) • 55% pancreas (107) • Same area 72% , different 28% • Sclerosing cholangitis • 14% porta hepatis • 14% intrapancreatic • 8% lacrimal/salivary glands inflammation • 5.7% retroperitoneal fibrosis • 17 of 903 patients died , only 7 (0.8%) died of pancreatic cancer

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