autoimmune pancreatitis presenting with jaundice
TRANSCRIPT
Salil Garg, HMS IIIDr. Gillian Lieberman
Autoimmune Pancreatitis Presenting with Jaundice
Salil Garg, Harvard Medical School, Year IIIGillian Lieberman, M.D.
Salil Garg, HMS IIIDr. Gillian Lieberman
Our patient: A 73 yo male presenting with obstructive
jaundice, history of autoimmune pancreatitis
Salil Garg, HMS IIIDr. Gillian Lieberman
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.
Salil Garg, HMS IIIDr. Gillian Lieberman
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)
Salil Garg, HMS IIIDr. Gillian Lieberman
Causes of Pancreatitis• Acute
– Alcohol– Gallstones– Metabolic– Drugs– Infection
• Chronic– Alcohol– Cigarette smoking– Hereditary/Congenital– Obstruction– Tropical pancreatitis– Idiopathic
• Autoimmune
Salil Garg, HMS IIIDr. Gillian Lieberman
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
In contrast, autoimmune pancreatitis is “dry” and often lacks fat stranding. Borders are regular.
BIDMC PACS, CT
Salil Garg, HMS IIIDr. Gillian Lieberman
Our patient’s 1st presentation: “Dry” Autoimmune Pancreatitis
Our patient presented with obstructive jaundice….
BIDMC PACS, C+ CTBIDMC PACS, C+ CT
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.
Salil Garg, HMS IIIDr. Gillian Lieberman
Biliary Tree Anatomy
http://www.hopkins-gi.org
Salil Garg, HMS IIIDr. Gillian Lieberman
Our patient, 1st presentation: Endoscopic Retrograde Cholangiopancreatography (ERCP) reveals stricture of the distal
common bile duct with proximal dilatation
Likely explains obstructive jaundice BIDMC PACS, ERCP
Salil Garg, HMS IIIDr. Gillian Lieberman
Our patient, 1st presentation: Guidewires during ERCP allowed placement of a stent across the stricture
Patient’s jaundice resolved, discharged on glucocorticoids
BIDMC PACS, ERCP BIDMC PACS, ERCP
Salil Garg, HMS IIIDr. Gillian Lieberman
Our patient, 2nd 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
Salil Garg, HMS IIIDr. Gillian Lieberman
Stricture of the left hepatic duct… …leading to dilation of the left hepatics
Our patient, 2nd presentation: ERCP revealed new proximal biliary strictures
BIDMC PACS, ERCP BIDMC PACS, ERCP
Salil Garg, HMS IIIDr. Gillian Lieberman
Our patient, 2nd presentation: The old stricture in the common bile duct which was previously stented has resolved
BIDMC PACS, ERCP
Salil Garg, HMS IIIDr. Gillian Lieberman
Our patient, follow up to second presentation: Stenting effectively removed stricture in left hepatic duct and cleared jaundice
Stricture of left hepatic duct at second presentation
Resolution of stricture after stent removal two months later
BIDMC PACS, ERCP BIDMC PACS, ERCP
Salil Garg, HMS IIIDr. Gillian Lieberman
Our patient, 3rd presentation: A year later, patient presented a third time with obstructive jaundice….
ERCP confirms stricture of the common hepatic duct with proximal dilation
Stricture of common hepatic duct with marked dilation of proximal hepatics.
BIDMC PACS, ERCP
BIDMC PACS, C+ CT
Salil Garg, HMS IIIDr. Gillian Lieberman
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 (2nd presentation) and common hepatic duct (3rd presentation)
• These were also resolved with ERCP stent placement
Salil Garg, HMS IIIDr. Gillian Lieberman
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**
Salil Garg, HMS IIIDr. Gillian Lieberman
…luckily for our patient, cytological studies of bile duct cells were
normal. What might malignant transformation look like?
Testing for Malignancy: Scrapings of all of the above biliary strictures were taken during ERCP
and sent for cytology……
Salil Garg, HMS IIIDr. Gillian Lieberman
Benign Bile Duct Cells
“Honeycomb” organization
Round, regular nuclei
Relatively high amount of cytoplasm
Salil Garg, HMS IIIDr. Gillian Lieberman
Reactive Bile Duct Cells
“Honeycomb” organization
Round, regular nuclei
Relatively low amount of cytoplasm
Salil Garg, HMS IIIDr. Gillian Lieberman
Malignant Ductal Cells
Single sheet organization lost, “3-D” like appearance
Irregular enlarged nuclei, with marked hyperchromatism
Very little cytoplasm
Salil Garg, HMS IIIDr. Gillian Lieberman
Recent progress in understanding the pathophysiology of Autoimmune Pancreatitis….
Frulloni et al, NEJM 2009.
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!!
Salil Garg, HMS IIIDr. Gillian Lieberman
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
Salil Garg, HMS IIIDr. Gillian Lieberman
References
• Finkelberg, DL et al. Autoimmune Pancreatitis. New Engl J of Med (2006) 355: 2670-6.
• Frulloni, L et al. Identification of a Novel Antibody Associated with Autoimmune Pancreatitis. New Engl J of Med (2009) 361: 2135-42.
• Sahani, DV et al. Autoimmune Pancreatitis: Disease Evolution, Staging, Response, Assessment, and CT Features that Predict Response to Corticosteroid Therapy. Radiology (2009) 250:118- 129.
• Saeki, T et al. Lymphoplasmacytic infiltration of multiple organs with immunoreactivity for IgG4: IgG4-related systemic disease. Intern Med (2006) 45:163-167.
• 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) 445:552-563
Salil Garg, HMS IIIDr. Gillian Lieberman
Acknowledgements
• Dr. Gillian Lieberman, Course Director• Dr. Jean-Marc Gauguet, Radiology• Dr. Robert Najarian, GI-Pathology• Larry Barbaras, webmaster• Emily Hanson, coordinator