Autoimmune Pancreatitis Presenting with Jaundice

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Drusilla Richard
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1 Autoimmune Pancreatitis Presenting with Jaundice Salil Garg, Harvard Medical School, Year III Gillian Lieberman, M.D.

2 Our patient: A 73 yo male presenting with obstructive jaundice, history of autoimmune pancreatitis

3 Autoimmune Pancreatitis (AIP) Relatively rare, representing 5%-11% of chronic pancreatitis Twice as common in men as women Wide variance in age, most cases > 50 yo Most common presentation is jaundice or abdominal pain. Acute pancreatitis presentation is rare.

4 Criteria for AIP Diagnosis No gold standard, HISORT used most frequently in United States Histology Periductal infiltrate, T lymphocytes and plasma cells (IgG4+) Imaging Diffuse pancreatic enlargement, or focal mass Serology Hypergammaglobulinemia, Highly elevated IgG4 is specific but not sensitive Other organ involvement Gallbladder, bile ducts, kidney, lung, salivary glands Response to Steroid Treatment Glucocorticoids (prednisolone)

5 Acute Alcohol Gallstones Metabolic Drugs Infection Causes of Pancreatitis Chronic Alcohol Cigarette smoking Hereditary/Congenital Obstruction Tropical pancreatitis Idiopathic Autoimmune

6 Companion Patient I: Usual Signs of Pancreatitis on CT. Diffusely enlarged pancreas often with irregular borders Peripancreatic inflammation Fat stranding Heterogenously enhancing parenchyma Necrosis, Abscess BIDMC PACS, CT In contrast, autoimmune pancreatitis is dry and often lacks fat stranding. Borders are regular.

Pancreatic borders are regular and little to no fat stranding is apparent when compared to

7 Our patient s 1 st presentation: Dry Autoimmune Pancreatitis Our patient shows diffuse pancreatic enlargement, most easily visualized here in the head of the pancreas. Pancreatic borders are regular and little to no fat stranding is apparent when compared to other forms of pancreatitis. BIDMC PACS, C+ CT BIDMC PACS, C+ CT Our patient presented with obstructive jaundice.

8 Biliary Tree Anatomy

9 Our patient, 1 st presentation: Endoscopic Retrograde Cholangiopancreatography (ERCP) reveals stricture of the distal common bile duct with proximal dilatation BIDMC PACS, ERCP Likely explains obstructive jaundice

10 Our patient, 1 st presentation: Guidewires during ERCP allowed placement of a stent across the stricture BIDMC PACS, ERCP BIDMC PACS, ERCP Patient s jaundice resolved, discharged on glucocorticoids

11 Our patient, 2 nd presentation: Two years later our patient again presents with obstructive jaundice, now with biliuria Pancreatic head is smaller but a dilated pancreatic duct is visible

12 Our patient, 2 nd presentation: ERCP revealed new proximal biliary strictures BIDMC PACS, ERCP Stricture of the left hepatic duct BIDMC PACS, ERCP leading to dilation of the left hepatics

13 Our patient, 2 nd presentation: The old stricture in the common bile duct which was previously stented has resolved BIDMC PACS, ERCP

14 Our patient, follow up to second presentation: Stenting effectively removed stricture in left hepatic duct and cleared jaundice BIDMC PACS, ERCP Stricture of left hepatic duct at second presentation BIDMC PACS, ERCP Resolution of stricture after stent removal two months later

BIDMC PACS, ERCP BIDMC PACS, C+ CT Stricture of common hepatic duct

15 Our patient, 3 rd presentation: A year later, patient presented a third time with obstructive jaundice. BIDMC PACS, ERCP BIDMC PACS, C+ CT Stricture of common hepatic duct with marked dilation of proximal hepatics. ERCP confirms stricture of the common hepatic duct with proximal dilation

16 Our patient: Summary of clinical course Presented with obstructive jaundice due to common bile duct stricture. Stricture and jaundice resolved with ERCP placement of a stent Strictures recurred at the left hepatic duct (2 nd presentation) and common hepatic duct (3 rd presentation) These were also resolved with ERCP stent placement

17 What are other pathologies to worry about in this patient? Pancreatic Adenocarcinoma Primary Sclerosing Cholangitis Other autoimmune diseases: rheumatoid arthritis, Sjogren s syndrome, inflammatory bowel disease Lymphocytic infiltrates in other organ systems (Lung, Kidney, salivary glands, soft tissues near Aorta) **Cholangiocarcinoma**

18 Testing for Malignancy: Scrapings of all of the above biliary strictures were taken during ERCP and sent for cytology luckily for our patient, cytological studies of bile duct cells were normal. What might malignant transformation look like?

19 Benign Bile Duct Cells Honeycomb organization Round, regular nuclei Relatively high amount of cytoplasm

20 Reactive Bile Duct Cells Honeycomb organization Round, regular nuclei Relatively low amount of cytoplasm

21 Malignant Ductal Cells Single sheet organization lost, 3-D like appearance Irregular enlarged nuclei, with marked hyperchromatism Very little cytoplasm

22 Recent progress in understanding the pathophysiology of Autoimmune Pancreatitis. A monoclonal antibody which recognizes plasminogen binding protein from Heliobacter pylori is found specifically in patients with AIP. A very similar peptide is extensively expressed on pancreatic acinar cells!! Frulloni et al, NEJM 2009.

23 Summary Autoimmune pancreatitis is a relatively rare but important cause of pancreatitis The most common presenting symptom is jaundice Radiological appearance is of a dry pancreatitis, often with pancreatic enlargement. Constriction or dilatation of the pancreatic duct is also possible. Treatment (Steroids, Interventional Radiology) is efficacious in most patients though not curative as symptoms (such as strictures) can reoccur Important to distinguish AIP from pancreatic cancer, cholangiocarcinoma, and primary sclerosing cholangitis and to monitor for these complications Progress is being made in understanding the etiology of this disease

24 References Finkelberg, DL et al. Autoimmune Pancreatitis. New Engl J of Med (2006) 355: Frulloni, L et al. Identification of a Novel Antibody Associated with Autoimmune Pancreatitis. New Engl J of Med (2009) 361: Sahani, DV et al. Autoimmune Pancreatitis: Disease Evolution, Staging, Response, Assessment, and CT Features that Predict Response to Corticosteroid Therapy. Radiology (2009) 250: Saeki, T et al. Lymphoplasmacytic infiltration of multiple organs with immunoreactivity for IgG4: IgG4-related systemic disease. Intern Med (2006) 45: Zamboni, G et al. Histopathological features of diagnostic and clinical relevance in autoimmune pancreatitis: a study on 53 resection specimens and 9 biopsy specimens. Virchows Arch (2004) 445:

25 Acknowledgements, Course Director Dr. Jean-Marc Gauguet, Radiology Dr. Robert Najarian, GI-Pathology Larry Barbaras, webmaster Emily Hanson, coordinator

Autoimmune pancreatitis. Lars Aabakken Oslo University Hospital - Rikshospitalet Oslo, Norway

Autoimmune pancreatitis. Lars Aabakken Oslo University Hospital - Rikshospitalet Oslo, Norway Autoimmune pancreatitis Lars Aabakken Oslo University Hospital - Rikshospitalet Oslo, Norway Autoimmune pancreatitis Concept introduced in 1961 (Sarles) Re-invented in Japan 1995 (Yoshida) Increasingly