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IgG4-Seronegative Autoimmune Pancreatitis and Sclerosing Cholangitis.

Kahn A, Yadav AD, Harrison ME - Case Rep Gastrointest Med (2015)

Bottom Line: The presence of elevated serum IgG4 is suggestive but not essential for the diagnosis of type 1 AIP and is a pervasive feature of the proposed diagnostic criteria.The differential diagnosis of type 1 AIP includes malignant conditions, emphasizing the importance of a deliberate, comprehensive evaluation.Management of patients with a suggestive clinical presentation, but without serum IgG4 elevation, is difficult.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine, Mayo Clinic, Scottsdale, AZ, USA.

ABSTRACT
IgG4-related disease is a relatively novel clinical entity whose gastrointestinal manifestations include type 1 autoimmune pancreatitis (AIP) and IgG4-associated sclerosing cholangitis. The presence of elevated serum IgG4 is suggestive but not essential for the diagnosis of type 1 AIP and is a pervasive feature of the proposed diagnostic criteria. The differential diagnosis of type 1 AIP includes malignant conditions, emphasizing the importance of a deliberate, comprehensive evaluation. Management of patients with a suggestive clinical presentation, but without serum IgG4 elevation, is difficult. Here we present three cases of IgG4-seronegative AIP and sclerosing cholangitis that responded to empiric steroid therapy and discuss approach considerations. These cases demonstrate the value of meticulous application of existing diagnostic algorithms to achieve a clinical diagnosis and avoid surgical intervention.

No MeSH data available.


Related in: MedlinePlus

MRCP findings: Case 2. (a) Extrahepatic bile duct narrowing. (b) Resolution after steroid therapy. (c) Diffusion restriction shows hyperintensity and edema in the pancreatic tail. (d) Resolution after steroid therapy.
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fig2: MRCP findings: Case 2. (a) Extrahepatic bile duct narrowing. (b) Resolution after steroid therapy. (c) Diffusion restriction shows hyperintensity and edema in the pancreatic tail. (d) Resolution after steroid therapy.

Mentions: A 45-year-old gentleman with prior history of small duct primary sclerosing cholangitis and Crohn's disease presented with symptoms of abdominal pain, pruritus, dark urine, and jaundice. Laboratory analysis showed an AST of 152, ALT of 158, alkaline phosphatase of 531, total bilirubin of 5.6, direct bilirubin of 3.8, total protein of 7.4, albumin of 4.0, lipase of 157, amylase of 82, CA of 19-9 194, CEA of 2.9, total IgG of 1620, and IgG4 of 53.3. MRI of the abdomen demonstrated diffuse enlargement of the body and the tail of the pancreas. No focal enhancing pancreatic mass was identified. MRCP revealed new irregular narrowing of the common hepatic duct, upper common bile duct beaded dilatation, and narrowing of the intrahepatic bile ducts (Figures 2(a) and 2(c)). Several prominent porta hepatis lymph nodes were noted.


IgG4-Seronegative Autoimmune Pancreatitis and Sclerosing Cholangitis.

Kahn A, Yadav AD, Harrison ME - Case Rep Gastrointest Med (2015)

MRCP findings: Case 2. (a) Extrahepatic bile duct narrowing. (b) Resolution after steroid therapy. (c) Diffusion restriction shows hyperintensity and edema in the pancreatic tail. (d) Resolution after steroid therapy.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4561869&req=5

fig2: MRCP findings: Case 2. (a) Extrahepatic bile duct narrowing. (b) Resolution after steroid therapy. (c) Diffusion restriction shows hyperintensity and edema in the pancreatic tail. (d) Resolution after steroid therapy.
Mentions: A 45-year-old gentleman with prior history of small duct primary sclerosing cholangitis and Crohn's disease presented with symptoms of abdominal pain, pruritus, dark urine, and jaundice. Laboratory analysis showed an AST of 152, ALT of 158, alkaline phosphatase of 531, total bilirubin of 5.6, direct bilirubin of 3.8, total protein of 7.4, albumin of 4.0, lipase of 157, amylase of 82, CA of 19-9 194, CEA of 2.9, total IgG of 1620, and IgG4 of 53.3. MRI of the abdomen demonstrated diffuse enlargement of the body and the tail of the pancreas. No focal enhancing pancreatic mass was identified. MRCP revealed new irregular narrowing of the common hepatic duct, upper common bile duct beaded dilatation, and narrowing of the intrahepatic bile ducts (Figures 2(a) and 2(c)). Several prominent porta hepatis lymph nodes were noted.

Bottom Line: The presence of elevated serum IgG4 is suggestive but not essential for the diagnosis of type 1 AIP and is a pervasive feature of the proposed diagnostic criteria.The differential diagnosis of type 1 AIP includes malignant conditions, emphasizing the importance of a deliberate, comprehensive evaluation.Management of patients with a suggestive clinical presentation, but without serum IgG4 elevation, is difficult.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine, Mayo Clinic, Scottsdale, AZ, USA.

ABSTRACT
IgG4-related disease is a relatively novel clinical entity whose gastrointestinal manifestations include type 1 autoimmune pancreatitis (AIP) and IgG4-associated sclerosing cholangitis. The presence of elevated serum IgG4 is suggestive but not essential for the diagnosis of type 1 AIP and is a pervasive feature of the proposed diagnostic criteria. The differential diagnosis of type 1 AIP includes malignant conditions, emphasizing the importance of a deliberate, comprehensive evaluation. Management of patients with a suggestive clinical presentation, but without serum IgG4 elevation, is difficult. Here we present three cases of IgG4-seronegative AIP and sclerosing cholangitis that responded to empiric steroid therapy and discuss approach considerations. These cases demonstrate the value of meticulous application of existing diagnostic algorithms to achieve a clinical diagnosis and avoid surgical intervention.

No MeSH data available.


Related in: MedlinePlus