AIP is type of chronic pancreatitis characterized by an autoimmune inflammatory process in which prominent lymphocye infiltration with associated fibrosis of the pancreas cuases organ dysfunction.
AIP is one component of multisystem IgG4 associated fibro-inflammatory disease.
The diagnosis is based on combination of clinical, radiological and pathological criteria.
Clinical features include:
-painless jaundice
-upper abdominal discomfort
-Wt loss
-DM i in 75%
-other associaed autoimmune disorders
Radiological features:
-CT: sausage-shaped pancreas
-ERCP: low CBD stricture, diffuse pancreatic stricturing.
histologicaly, there are 2 types:
1-Lymphoplasmacytic LPSP
IgG4 positive,
fibrosis > inflammation
older age, M > F
2-Idiopathic duct centric IDCP
IgG4 negative ( i.e no serum marker)
inflammation > fibrosis
younger age, M=F
Treatment:
corticosteroids
The failure to differentiate AIP from malignancy may lead to unnecessary pancreatic resection.
AIP is one component of multisystem IgG4 associated fibro-inflammatory disease.
The diagnosis is based on combination of clinical, radiological and pathological criteria.
Clinical features include:
-painless jaundice
-upper abdominal discomfort
-Wt loss
-DM i in 75%
-other associaed autoimmune disorders
Radiological features:
-CT: sausage-shaped pancreas
-ERCP: low CBD stricture, diffuse pancreatic stricturing.
histologicaly, there are 2 types:
1-Lymphoplasmacytic LPSP
IgG4 positive,
fibrosis > inflammation
older age, M > F
2-Idiopathic duct centric IDCP
IgG4 negative ( i.e no serum marker)
inflammation > fibrosis
younger age, M=F
Treatment:
corticosteroids
The failure to differentiate AIP from malignancy may lead to unnecessary pancreatic resection.
sausage-shaped pancreas
The CT image displays the characteristic pattern of autoimmune pancreatitis. The pancreas is "sausage" shaped and has a characteristic inflammatory "halo." The initial mode of therapy is oral prednisone which is effective in over 60-70% of cases.
The CT image displays the characteristic pattern of autoimmune pancreatitis. The pancreas is "sausage" shaped and has a characteristic inflammatory "halo." The initial mode of therapy is oral prednisone which is effective in over 60-70% of cases.
Type 1 AIP accounts for the majority of patients described in the world literature. The pancreatic manifestations of type 1 AIP represent only one component of a systemic disease process referred to as IgG4-associated systemic disease which may also affect the biliary tree, retroperitoneum, kidney, lymph nodes, and salivary glands. There is a male predominance and most patients present in the sixth decade with obstructive jaundice. The characteristic histologic pattern is termed lymphoplasmacytic sclerosing pancreatitis (LPSP) defined by the presence of periductal lymphoplasmacytic infiltration, obliterative venulitis (phlebitis), and storiform fibrosis. Tissue staining most often reveals abundant (>10) IgG4-positive plasma cells/high power field (HPF). Pancreatic imaging usually demonstrates diffuse pancreatic enlargement with delayed (rim) enhancement and a diffusely irregular, attenuated main pancreatic duct. The serologic hallmark is elevation in the IgG4 level, typically ≥2x/ULN. In comparison to type 1 disease, patients with type 2 AIP are more often male and younger (fourth decade) at the time of diagnosis. Most patients with type 2 disease present with obstructive jaundice resulting from either direct distal bile duct involvement and/or extrinsic biliary compression secondary to pancreatic head swelling. Type 2 patients have a distinct histologic lesion termed granulocyte epithelial lesion (GEL) manifested by pancreatic duct epithelial neutrophil infiltration that leads to ductal obstruction and obliteration. (Figure A) The presence of GEL is considered diagnostic for type 2 AIP. While clinical, laboratory, imaging, and histological features are utilized for diagnosing type 1 AIP, currently type 2 AIP can only be diagnosed by histology. Tissue immunostaining is typically negative, but may reveal mild (1-10/HPF) or moderate (11-20/HPF) numbers of IgG4-positive plasma cells. IgG4 serology is of minimal diagnostic utility because levels are typically normal in type 2 AIP. The only known extra-pancreatic manifestation in type 2 AIP is inflammatory bowel disease.
ReplyDeleteWhich of the following is most accurate with regard to autoimmune pancreatitis?
ReplyDelete1-It is more common in women than in men.
2-It is associated with sclerosis of extrapancreatic sites such as the salivary glands and retroperitoneum.
3-It typically presents between the ages of 40 and 55.
4-When sclerosing cholangitis occurs in association with autoimmune pancreatitis, it tends to be poorly responsive to corticosteroid treatment.
Autoimmune pancreatitis is most commonly diagnosed in men (2:1) older than age 50. In addition to fibrosis in the pancreas, other sites may be affected including the bile ducts, salivary and lacrimal glands, retroperitoneum, and kidneys. The disease tends to be steroid responsive with resolution of abnormalities in both the pancreatic and extrapancreatic locations.
Very well written and informative article, thank you :-)
ReplyDeleteToshiba PVT-375BT
Hi,
ReplyDeleteTook Onglyza off and on for a year. I have an enlarged adrenal gland. Still I await the outcome of that CT, but I know that much. Will find out more.
I had the CT because of chronic pancreatic pain that started out as "attacks" from a couple of times a month to finally after 3 months of use without interruption, "attacks" 2-3 times a week. My PA put Onglyza on my allergies list.
In the meantime, I lost almost 50 lbs in 5 months due to illness. Loss of appetite, pancreatic pain, chronic diarrhea, then eventually, inability to move my bowels. Severe back pain from the pancreas, and severe chest pain sent me to the ER where I was worked up for cardiac pain. I was cardiac cleared, but told my amylase was very low.
Still seeking a diagnosis, but I lay the blame squarely on Onglyza. I'd had pancreatic issues in the past, and argued with the PA that prescribed it, she was calling me non-compliant, and I feared repercussion from my insurance company.
I even took an article about the dangers of Onglyza, particularly in patients with a history, and she made me feel foolish.
I wish I had listened to my instincts, I fear not only damage to my pancreas that is irreversible, but also severe damage to my left kidney, though I have bilateral kidney pain.
I was off all diabetes meds, and control sugars strictly low to no carb. I can barely eat anymore, I have severe anorexia.
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I have been off Onglyza now, for 7 months, and simply 100% improvement with the help of Dr Itua. I had none of these issues except a history of pancreatitis in my distant past.
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