Materiały źródłowe

• #1 Autoimmune pancreatitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/autoimmune-pancreatitis/diagnosis-treatment/drc-20369804

  Autoimmune pancreatitis is hard to diagnose, because its symptoms are a lot like those of pancreatic cancer. However, an accurate diagnosis is extremely important. Undiagnosed cancer may result in delaying or not receiving necessary treatment. […] To pinpoint the diagnosis and determine the type of AIP, blood and imaging tests are necessary. […] No single test or characteristic feature identifies autoimmune pancreatitis. Recommended guidelines for diagnosis use a combination of imaging, blood tests and biopsy results. […] Specific tests may include: Imaging tests. Tests of your pancreas and other organs may include CT, MRI, endoscopic ultrasound (EUS) and endoscopic retrograde cholangiopancreatography (ERCP). […] Blood tests. You may have a test to check for elevated levels of an immunoglobulin called IgG4. IgG4 is produced by your immune system. People with type 1 AIP often have high levels of IgG4 in their blood. People with type 2 AIP usually do not.

• #2 Autoimmune Pancreatitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560769/

  Autoimmune pancreatitis is a clinical condition characterized by inflammation of the pancreas from an autoimmune etiology. It is an uncommon cause of pancreatitis but can present with significant morbidity and mortality because of repeated episodes of acute pancreatitis. […] Timely diagnosis and management are important to achieve good patient outcomes. […] The international consensus diagnostic criteria (ICDC) for autoimmune pancreatitis include imaging of the pancreas and pancreatic duct, serologic studies including IgG4 levels, histopathology, and other associated conditions. […] A histopathologic examination can help and is usually necessary to distinguish autoimmune pancreatitis from pancreatic cancer. […] Histopathological examination can be of yield and is preferred as a part of diagnostic criteria before initiating treatment of AIP. […] After a thorough evaluation and after ruling out differential diagnoses such as pancreatic malignancy, patients need continuous follow-up with their providers to assess their response to immunosuppressant therapy.

• #3 Diagnosis of autoimmune pancreatitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4248199/

  Autoimmune pancreatitis (AIP) is a distinct form of chronic pancreatitis that is increasingly being reported. […] Therefore, accurate diagnosis is necessary for cases of AIP. […] These findings have led several countries to propose diagnostic criteria for AIP, which consist of essentially similar diagnostic items; however, several differences exist for each country, mainly due to differences in the definition of AIP and the modalities used to diagnose this disease. […] An attempt to unite the diagnostic criteria worldwide was made with the publication in 2011 of the international consensus diagnostic criteria for AIP, established at the 2010 Congress of the International Association of Pancreatology (IAP). […] The first diagnostic criteria for AIP were established in Japan in 2002, revised in 2006, and revised again in 2011. […] AIP was later revealed to consist of two distinct subtypes: type 1 AIP, which is characterized by histology resembling that of lymphoplasmacytic sclerosing pancreatitis (LPSP), and type 2 AIP or idiopathic duct-centric pancreatitis (IDCP) with granulocytic epithelial lesion (GEL). […] The proportions of type 1 and type 2 AIP vary substantially in western and eastern countries. […] The ICDC are presently evaluated as the most sensitive and specific criteria for diagnosing AIP.

• #4 Diagnosis of autoimmune pancreatitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4248199/

  Autoimmune pancreatitis (AIP) is a distinct form of chronic pancreatitis that is increasingly being reported. […] Therefore, accurate diagnosis is necessary for cases of AIP. […] These findings have led several countries to propose diagnostic criteria for AIP, which consist of essentially similar diagnostic items; however, several differences exist for each country, mainly due to differences in the definition of AIP and the modalities used to diagnose this disease. […] An attempt to unite the diagnostic criteria worldwide was made with the publication in 2011 of the international consensus diagnostic criteria for AIP, established at the 2010 Congress of the International Association of Pancreatology (IAP). […] The first diagnostic criteria for AIP were established in Japan in 2002, revised in 2006, and revised again in 2011. […] AIP was later revealed to consist of two distinct subtypes: type 1 AIP, which is characterized by histology resembling that of lymphoplasmacytic sclerosing pancreatitis (LPSP), and type 2 AIP or idiopathic duct-centric pancreatitis (IDCP) with granulocytic epithelial lesion (GEL). […] The proportions of type 1 and type 2 AIP vary substantially in western and eastern countries. […] The ICDC are presently evaluated as the most sensitive and specific criteria for diagnosing AIP.

• #5 Diagnosis of autoimmune pancreatitis

  https://www.wjgnet.com/1007-9327/full/v20/i44/16559.htm

  Autoimmune pancreatitis (AIP) is a distinct form of chronic pancreatitis that is increasingly being reported. […] Therefore, accurate diagnosis is necessary for cases of AIP. […] An attempt to unite the diagnostic criteria worldwide was made with the publication in 2011 of the international consensus diagnostic criteria for AIP, established at the 2010 Congress of the International Association of Pancreatology (IAP). […] The first diagnostic criteria for AIP were established in Japan in 2002, revised in 2006, and revised again in 2011. […] AIP was later revealed to consist of two distinct subtypes: type 1 AIP, which is characterized by histology resembling that of lymphoplasmacytic sclerosing pancreatitis (LPSP), and type 2 AIP or idiopathic duct-centric pancreatitis (IDCP) with granulocytic epithelial lesion (GEL).

• #6 Diagnosis Autoimmune Pancreatitis | Pancreapedia

  https://pancreapedia.org/reviews/diagnosis-autoimmune-pancreatitis

  In 2011 a multinational group met to develop diagnostic criteria that would be meaningful for both clinical and research purposes. They achieved a consensus that recognizes our current understanding of AIP, permits flexibility in diagnostic evaluation (e.g., reliance on histology vs. pancreatography), and acknowledges the two AIP subtypes. Importantly, although the typical clinical presentation of patients with AIP is obstructive jaundice, occasionally with a mass, the criteria also permit diagnosis in those with less common disease presentations and indeterminate imaging findings. These criteria are referred to as the International Consensus Diagnostic Criteria (ICDC) for AIP. The cardinal clinical features of AIP in the ICDC are based on pancreatic parenchymal imaging, pancreatic ductal imaging (i.e., ERP), serum IgG4 level, other organ involvement, histology of the pancreas, and response to steroid treatment.

• #7 The Role of Endoscopic Ultrasound and Ancillary Techniques in the Diagnosis of Autoimmune Pancreatitis: A Comprehensive Review

  https://www.mdpi.com/2075-4418/14/12/1233

  Autoimmune pancreatitis (AIP) is a unique form of chronic pancreatitis with a multifactorial pathogenesis. The diagnosis of AIP is challenging and relies on a combination of clinical, histopathologic, serologic, and imaging characteristics. In the available guidelines, the imaging hallmarks of AIP are based on cross-sectional imaging and cholangiopancreatography retrograde endoscopic findings. Endoscopic ultrasound (EUS) is generally used for pancreatic tissue acquisition to rule out pancreatic cancer and diagnose AIP with limited accuracy. Several papers reported the reliability of EUS for providing informative morphologic features of AIP. […] The diagnosis of AIP is challenging and relies on a comprehensive work-up that includes different data. Several groups from different countries proposed diagnostic criteria over the years. In 2012, the International Association of Pancreatology proposed the International Consensus Diagnostic Criteria (ICDC), which are the most widely used. The ICDC combine the five cardinal features of AIP: (1) the imaging of the pancreatic parenchyma and pancreatic duct, (2) serology (serum IgG4), (3) other organ involvement, (4) the histology and IgG4 immunostaining of the pancreas, and (5) the response to corticosteroids.

• #8 Autoimmune pancreatitis – Wikipedia

  https://en.wikipedia.org/wiki/Autoimmune_pancreatitis

  Autoimmune pancreatitis (AIP) is an increasingly recognized type of chronic pancreatitis that can be difficult to distinguish from pancreatic carcinoma but which responds to treatment with corticosteroids, particularly prednisone. […] AIP is characterized by the following features: […] Histopathologic examination of the pancreas reveals a characteristic lymphoplasmacytic infiltrate of CD4- or CD8-positive lymphocytes and IgG4-positive plasma cells, and exhibits interstitial fibrosis and acinar cell atrophy in later stages. […] Most recently the fourteenth Congress of the International Association of Pancreatology developed the International Consensus Diagnostic Criteria (ICDC) for AIP. The ICDC emphasizes five cardinal features of AIP which includes the imaging appearance of pancreatic parenchyma and the pancreatic duct, serum IgG4 level, other organ involvement with IgG4-related disease, pancreatic histology and response to steroid therapy.

• #9 Autoimmune pancreatitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/autoimmune-pancreatitis/diagnosis-treatment/drc-20369804

  Autoimmune pancreatitis is hard to diagnose, because its symptoms are a lot like those of pancreatic cancer. However, an accurate diagnosis is extremely important. Undiagnosed cancer may result in delaying or not receiving necessary treatment. […] To pinpoint the diagnosis and determine the type of AIP, blood and imaging tests are necessary. […] No single test or characteristic feature identifies autoimmune pancreatitis. Recommended guidelines for diagnosis use a combination of imaging, blood tests and biopsy results. […] Specific tests may include: Imaging tests. Tests of your pancreas and other organs may include CT, MRI, endoscopic ultrasound (EUS) and endoscopic retrograde cholangiopancreatography (ERCP). […] Blood tests. You may have a test to check for elevated levels of an immunoglobulin called IgG4. IgG4 is produced by your immune system. People with type 1 AIP often have high levels of IgG4 in their blood. People with type 2 AIP usually do not.

• #10 Autoimmune Pancreatitis – National Pancreas Foundation

  https://pancreasfoundation.org/pancreas-disease/autoimmune-pancreatitis/

  Autoimmune pancreatitis is difficult to diagnose because its signs and symptoms are very similar to those of pancreatic cancer. However, an accurate diagnosis is extremely important. Undiagnosed cancer may result in delaying or not receiving necessary treatment. […] People with AIP tend to have a general enlargement of the pancreas, but they may also have a mass in the pancreas. To pinpoint the diagnosis and determine which type of AIP you have, blood and imaging tests are necessary. […] No single test or characteristic feature identifies autoimmune pancreatitis. Consensus guidelines for diagnosis use a combination of imaging, blood tests and biopsy results. […] Specific tests may include: Imaging tests. Tests of your pancreas and other organs may include CT, MRI, endoscopic ultrasound (EUS) and endoscopic retrograde cholangiopancreatography (ERCP).

• #11 Diagnosis of autoimmune pancreatitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4248199/

  Autoimmune pancreatitis (AIP) is a distinct form of chronic pancreatitis that is increasingly being reported. […] Therefore, accurate diagnosis is necessary for cases of AIP. […] These findings have led several countries to propose diagnostic criteria for AIP, which consist of essentially similar diagnostic items; however, several differences exist for each country, mainly due to differences in the definition of AIP and the modalities used to diagnose this disease. […] An attempt to unite the diagnostic criteria worldwide was made with the publication in 2011 of the international consensus diagnostic criteria for AIP, established at the 2010 Congress of the International Association of Pancreatology (IAP). […] The first diagnostic criteria for AIP were established in Japan in 2002, revised in 2006, and revised again in 2011. […] AIP was later revealed to consist of two distinct subtypes: type 1 AIP, which is characterized by histology resembling that of lymphoplasmacytic sclerosing pancreatitis (LPSP), and type 2 AIP or idiopathic duct-centric pancreatitis (IDCP) with granulocytic epithelial lesion (GEL). […] The proportions of type 1 and type 2 AIP vary substantially in western and eastern countries. […] The ICDC are presently evaluated as the most sensitive and specific criteria for diagnosing AIP.

• #12 Autoimmune pancreatitis: Clinical manifestations and diagnosis – UpToDate

  https://www.uptodate.com/contents/autoimmune-pancreatitis-clinical-manifestations-and-diagnosis

  Autoimmune pancreatitis: Clinical manifestations and diagnosis […] AIP can appear radiographically as a focal mass that can be difficult to distinguish from pancreatic cancer or as a diffuse pancreatic process that can be pathognomonic for AIP. […] The discovery of an elevated serum immunoglobulin G4 (IgG4) as a biomarker of AIP helped establish the disorder as distinct from other forms of chronic pancreatitis; although, later studies demonstrated that IgG4 levels are often normal in AIP and that other diseases such as pancreatic cancer may be associated with moderately elevated IgG4 levels. […] AIP is a syndrome defined by clinical, laboratory, and pathologic criteria. […] Type 1 AIP is defined histologically as lymphoplasmacytic sclerosing pancreatitis (LPSP). […] The International Consensus Diagnostic Criteria (ICDC) defined type 1 AIP as: dense infiltration of plasma cells and lymphocytes, particularly periductal; storiform (a swirling, „cartwheel” pattern) fibrosis; venulitis with lymphocytes and plasma cells, often leading to obliteration of the affected veins; and abundant (>10 cells per high-power field [HPF]) immunoglobulin G4 (IgG4)-positive plasma cells. […] Type 2 AIP is defined histologically as idiopathic duct-centric pancreatitis (IDCP) or AIP with granulocyte epithelial lesions (GELs). […] IDCP usually has very few IgG4-positive plasma cells (<10 cells/HPF).

• #13 Diagnosis of autoimmune pancreatitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4248199/

  Autoimmune pancreatitis (AIP) is a distinct form of chronic pancreatitis that is increasingly being reported. […] Therefore, accurate diagnosis is necessary for cases of AIP. […] These findings have led several countries to propose diagnostic criteria for AIP, which consist of essentially similar diagnostic items; however, several differences exist for each country, mainly due to differences in the definition of AIP and the modalities used to diagnose this disease. […] An attempt to unite the diagnostic criteria worldwide was made with the publication in 2011 of the international consensus diagnostic criteria for AIP, established at the 2010 Congress of the International Association of Pancreatology (IAP). […] The first diagnostic criteria for AIP were established in Japan in 2002, revised in 2006, and revised again in 2011. […] AIP was later revealed to consist of two distinct subtypes: type 1 AIP, which is characterized by histology resembling that of lymphoplasmacytic sclerosing pancreatitis (LPSP), and type 2 AIP or idiopathic duct-centric pancreatitis (IDCP) with granulocytic epithelial lesion (GEL). […] The proportions of type 1 and type 2 AIP vary substantially in western and eastern countries. […] The ICDC are presently evaluated as the most sensitive and specific criteria for diagnosing AIP.

• #14 Type 2 Autoimmune Pancreatitis: Consensus and Controversies

  https://www.gutnliver.org/journal/view.html?doi=10.5009/gnl210241

  Autoimmune pancreatitis (AIP) has attracted much attention in the last two decades, and due to the diagnostic value of immunoglobulin G4 (IgG4), the number of cases diagnosed in clinical practice has markedly increased. However, in contrast to prototypic IgG4-related type 1 AIP, a minor subtype of AIP, referred to as type 2 AIP, is less widely known and has thus not yet been characterized in detail. Type 2 AIP is unrelated to IgG4 and is a completely distinct entity from type 1 AIP. One confusing factor is that the two types of AIP share patterns of clinical presentation (e.g., acute pancreatitis and painless jaundice) and imaging abnormalities (e.g., diffuse or segmental enlargement). Since there are currently no established serum markers, the diagnosis of type 2 AIP is highly challenging and requires the tissue confirmation of neutrophilic injury to the pancreatic ducts, a finding designated as a granulocytic epithelial lesion.

• #15 Autoimmune pancreatitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/autoimmune-pancreatitis/diagnosis-treatment/drc-20369804

  Autoimmune pancreatitis is hard to diagnose, because its symptoms are a lot like those of pancreatic cancer. However, an accurate diagnosis is extremely important. Undiagnosed cancer may result in delaying or not receiving necessary treatment. […] To pinpoint the diagnosis and determine the type of AIP, blood and imaging tests are necessary. […] No single test or characteristic feature identifies autoimmune pancreatitis. Recommended guidelines for diagnosis use a combination of imaging, blood tests and biopsy results. […] Specific tests may include: Imaging tests. Tests of your pancreas and other organs may include CT, MRI, endoscopic ultrasound (EUS) and endoscopic retrograde cholangiopancreatography (ERCP). […] Blood tests. You may have a test to check for elevated levels of an immunoglobulin called IgG4. IgG4 is produced by your immune system. People with type 1 AIP often have high levels of IgG4 in their blood. People with type 2 AIP usually do not.

• #16 Autoimmune Pancreatitis: Symptoms, Risk Factors and Treatment

  https://my.clevelandclinic.org/health/diseases/17936-autoimmune-pancreatitis

  Autoimmune pancreatitis is an uncommon disorder. It causes your immune system to attack healthy cells in your pancreas. […] Symptoms are similar to pancreatic cancer, which is why a thorough evaluation is essential. […] Many tests are necessary to diagnose autoimmune pancreatitis and rule out pancreatic cancer. Your evaluation may include: […] Blood tests. These tests detect abnormal levels of IgG4. They may also check liver function and the levels of certain types of blood cells. […] Imaging tests. These tests check the size of your pancreas and detect signs of scarring and narrowed ducts. Imaging tests may include CT scans and MRIs. […] Biopsy. This involves taking a sample of pancreas tissue and examining it under a microscope. A biopsy is usually obtained via a minimally invasive endoscopic procedure called endoscopic ultrasound (EUS). […] Corticosteroid trial. This can help confirm a diagnosis. You take a short-term dose of steroids and undergo lab tests to see if it lowers IgG4 levels and improves imaging findings.

• #17 Autoimmune Pancreatitis – National Pancreas Foundation

  https://pancreasfoundation.org/pancreas-disease/autoimmune-pancreatitis/

  Autoimmune pancreatitis is difficult to diagnose because its signs and symptoms are very similar to those of pancreatic cancer. However, an accurate diagnosis is extremely important. Undiagnosed cancer may result in delaying or not receiving necessary treatment. […] People with AIP tend to have a general enlargement of the pancreas, but they may also have a mass in the pancreas. To pinpoint the diagnosis and determine which type of AIP you have, blood and imaging tests are necessary. […] No single test or characteristic feature identifies autoimmune pancreatitis. Consensus guidelines for diagnosis use a combination of imaging, blood tests and biopsy results. […] Specific tests may include: Imaging tests. Tests of your pancreas and other organs may include CT, MRI, endoscopic ultrasound (EUS) and endoscopic retrograde cholangiopancreatography (ERCP).

• #18

  https://link.springer.com/article/10.1007/s11604-020-00971-z

  The International Consensus Diagnosis Criteria for autoimmune pancreatitis (AIP) has been published internationally for the diagnosis of AIP. […] The Clinical Diagnostic Criteria for Autoimmune Pancreatitis 2018 have been published. […] The criteria were revised based the Clinical Diagnostic Criteria 2011, and included descriptions of characteristic imaging findings such as (1) pancreatic enlargement and (2) distinctive narrowing of the main pancreatic duct. […] The guideline explains some characteristic imaging findings, but does not contain descriptions of the imaging methods, such as detailed imaging parameters and optimal timings of dynamic contrast-enhanced computed tomography/magnetic resonance imaging. […] Although other characteristic findings have been reported, these findings were not described in the guideline.

• #19

  https://link.springer.com/article/10.1007/s11604-020-00971-z

  Optimal imaging methods and scan timings for dynamic contrast-enhanced CT (DCE-CT) are important for accurate assessment of pancreatic findings. […] Whenever possible, DCE-CT should be performed. […] Multidirectional and appropriate image acquisition including MRCP and dynamic contrast-enhanced MRI (DCE-MRI) is necessary for accurate assessment of a pancreatic lesion. […] Whenever possible, DCE-MRI should be performed. […] FDG-PET and Ga-67 scintigram are useful for diagnosis of AIP because they can evaluate the lesion function, which cannot be evaluated by CT and MRI. […] The degrees of drug accumulation in AIP and extrapancreatic lesions depend on the lesion activity. […] The drug accumulation decreases if treatment is effective. […] Specific CT and MR findings for AIP, such as the capsule-like rim, duct-penetrating sign, speckled/dotted enhancement, and homogeneous delayed enhancement, are adopted as useful findings for differentiation of AIP from pancreatic cancer in the JPS2018.

• #20 Autoimmune pancreatitis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/autoimmune-pancreatitis?lang=us

  Autoimmune pancreatitis is a rare, benign pancreatic disease associated with autoimmune manifestations on clinical, histological, and laboratory grounds 1. Three types (AIP-1, AIP-2, AIP-3) have been described, differing in presentation, pathogenesis, and natural history 23. […] Distinguishing this entity from other forms of chronic pancreatitis (such as alcohol-induced) is important as steroid treatment is effective both in reversing morphologic changes and also in returning pancreatic function to normal 2. […] AIP-1 and AIP-2: see the article diagnostic criteria autoimmune pancreatitis for further details 3,8. […] AIP-3: appropriate medication history; no other cause of pancreatitis 23. […] Combination of Ca 19-9 74 U/ml and IgG4 1.0 g/l distinguishes patients with autoimmune pancreatitis from those patients with pancreatic carcinoma with 94% sensitivity and 100% specificity 20.

• #21 Type 1 autoimmune pancreatitis | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-6-82

  Elevated serum levels of IgG4 ( 135 mg/dL) are seen in more than 90% of patients. […] This is the most sensitive and specific diagnostic test for type 1 AIP, with 95% sensitivity, 97% specificity, and 97% accuracy for discrimination from pancreatic cancer. […] It is also useful for determining disease activity and predicting relapse. […] Other commonly detectable immunological features include elevation of IgG (70%), -globulin (60%), and IgE (33%). […] However, disease-specific autoantibodies such as anti SS-A/Ro and SS-B/La or anti-mitochondrial antibodies are extremely uncommon. […] These findings suggest that patients with type 1 AIP can produce various autoantibodies but these antibodies lack disease specificity. […] An important radiological feature is irregular narrowing of the pancreatic duct which can be detected by endoscopic retrograde cholangiopancreatography (ERCP) or magnetic resonance cholangiopancreatography (MRCP).

• #22 Chronic Pancreatitis: Diagnosis and Treatment | AAFP

  https://www.aafp.org/pubs/afp/issues/2018/0315/p385.html

  The diagnosis of chronic pancreatitis is made based on a patient’s history, clinical presentation, and imaging findings. […] There is no single test that is diagnostic of chronic pancreatitis, especially in the early stages. […] If chronic pancreatitis is suspected based on history and physical examination, a diagnostic workup should be initiated. […] Diagnostic tests should be chosen based on their availability after consideration of risks and benefits. […] Laboratory tests used in the evaluation of patients with suspected chronic pancreatitis are summarized in Table 3. […] The radiologic evaluation of a patient with suspected chronic pancreatitis should progress from least invasive to more invasive. […] If CT findings are equivocal, patients may require referral for more focused pancreatic imaging, such as MRI or magnetic resonance cholangiopancreatography, or for endoscopic procedures, such as EUS or endoscopic retrograde cholangiopancreatography. […] A small study of 25 patients suggests that combining EUS and pancreatic function tests can improve the sensitivity of diagnosis.

• #23 Autoimmune pancreatitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/autoimmune-pancreatitis/diagnosis-treatment/drc-20369804

  Autoimmune pancreatitis is hard to diagnose, because its symptoms are a lot like those of pancreatic cancer. However, an accurate diagnosis is extremely important. Undiagnosed cancer may result in delaying or not receiving necessary treatment. […] To pinpoint the diagnosis and determine the type of AIP, blood and imaging tests are necessary. […] No single test or characteristic feature identifies autoimmune pancreatitis. Recommended guidelines for diagnosis use a combination of imaging, blood tests and biopsy results. […] Specific tests may include: Imaging tests. Tests of your pancreas and other organs may include CT, MRI, endoscopic ultrasound (EUS) and endoscopic retrograde cholangiopancreatography (ERCP). […] Blood tests. You may have a test to check for elevated levels of an immunoglobulin called IgG4. IgG4 is produced by your immune system. People with type 1 AIP often have high levels of IgG4 in their blood. People with type 2 AIP usually do not.

• #24 Autoimmune Pancreatitis: Symptoms, Risk Factors and Treatment

  https://my.clevelandclinic.org/health/diseases/17936-autoimmune-pancreatitis

  Autoimmune pancreatitis is an uncommon disorder. It causes your immune system to attack healthy cells in your pancreas. […] Symptoms are similar to pancreatic cancer, which is why a thorough evaluation is essential. […] Many tests are necessary to diagnose autoimmune pancreatitis and rule out pancreatic cancer. Your evaluation may include: […] Blood tests. These tests detect abnormal levels of IgG4. They may also check liver function and the levels of certain types of blood cells. […] Imaging tests. These tests check the size of your pancreas and detect signs of scarring and narrowed ducts. Imaging tests may include CT scans and MRIs. […] Biopsy. This involves taking a sample of pancreas tissue and examining it under a microscope. A biopsy is usually obtained via a minimally invasive endoscopic procedure called endoscopic ultrasound (EUS). […] Corticosteroid trial. This can help confirm a diagnosis. You take a short-term dose of steroids and undergo lab tests to see if it lowers IgG4 levels and improves imaging findings.

• #25 Autoimmune Pancreatitis – National Pancreas Foundation

  https://pancreasfoundation.org/pancreas-disease/autoimmune-pancreatitis/

  Blood tests. You will be tested for elevated levels of an immunoglobulin called IgG4, produced by your immune system. People with type 1 AIP, but usually not with type 2 AIP, will have highly elevated blood levels of IgG4. […] However a positive test doesn’t necessarily mean that you have the disease. A small number of people who don’t have autoimmune pancreatitis, including some with pancreatic cancer, also have high blood levels of IgG4. […] Endoscopic core biopsy. In this test, pathologists analyze a sample of pancreatic tissue in the laboratory. AIP has a distinctive appearance that can be easily recognized under a microscope by an expert pathologist. Doctors insert a small tube (endoscope) through the mouth into the stomach and guided by ultrasound, remove some tissue from the pancreas using a special needle.

• #26 Diagnosis of autoimmune pancreatitis

  https://www.wjgnet.com/1007-9327/full/v20/i44/16559.htm

  The ICDC are presently evaluated as the most sensitive and specific criteria for diagnosing AIP. […] Diagnostic criteria, either nationwide or international, consist mostly of common diagnostic items such as image findings of the pancreatic parenchyma, pancreatography, and extrapancreatic lesions; serological findings; histology of the pancreatic lesion; and response to steroid therapy. […] The most sensitive and specific serum marker for type 1 AIP is IgG4 ( 135 mg/dL, sensitivity: 86%, specificity to AIP against PC: 96%). […] A response to steroid is a commonly evaluated diagnostic item for AIP in almost all diagnostic criteria. […] AIP is often associated with sclerosing cholangitis, which needs differential diagnosis from bile duct cancer.

• #27 Type 1 autoimmune pancreatitis | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-6-82

  Elevated serum levels of IgG4 ( 135 mg/dL) are seen in more than 90% of patients. […] This is the most sensitive and specific diagnostic test for type 1 AIP, with 95% sensitivity, 97% specificity, and 97% accuracy for discrimination from pancreatic cancer. […] It is also useful for determining disease activity and predicting relapse. […] Other commonly detectable immunological features include elevation of IgG (70%), -globulin (60%), and IgE (33%). […] However, disease-specific autoantibodies such as anti SS-A/Ro and SS-B/La or anti-mitochondrial antibodies are extremely uncommon. […] These findings suggest that patients with type 1 AIP can produce various autoantibodies but these antibodies lack disease specificity. […] An important radiological feature is irregular narrowing of the pancreatic duct which can be detected by endoscopic retrograde cholangiopancreatography (ERCP) or magnetic resonance cholangiopancreatography (MRCP).

• #28 Autoimmune pancreatitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/autoimmune-pancreatitis/diagnosis-treatment/drc-20369804

  However, a positive test doesn’t necessarily mean that you have the disease. A small number of people who don’t have autoimmune pancreatitis, including some with pancreatic cancer, also have high levels of IgG4 in their blood. […] Endoscopic core biopsy. In this test, a medical professional called a pathologist studies a sample of pancreatic tissue in the laboratory. AIP has a distinctive appearance that can be easily recognized under a microscope by an expert pathologist. […] A small tube called an endoscope is inserted through the mouth into the stomach, guided by ultrasound. A sample of tissue is then removed from the pancreas using a special needle. […] The challenge is getting a sample of tissue large enough to study, rather than just a few cells. This procedure is not widely available, and results may not be conclusive.

• #29 Autoimmune Pancreatitis – National Pancreas Foundation

  https://pancreasfoundation.org/pancreas-disease/autoimmune-pancreatitis/

  Blood tests. You will be tested for elevated levels of an immunoglobulin called IgG4, produced by your immune system. People with type 1 AIP, but usually not with type 2 AIP, will have highly elevated blood levels of IgG4. […] However a positive test doesn’t necessarily mean that you have the disease. A small number of people who don’t have autoimmune pancreatitis, including some with pancreatic cancer, also have high blood levels of IgG4. […] Endoscopic core biopsy. In this test, pathologists analyze a sample of pancreatic tissue in the laboratory. AIP has a distinctive appearance that can be easily recognized under a microscope by an expert pathologist. Doctors insert a small tube (endoscope) through the mouth into the stomach and guided by ultrasound, remove some tissue from the pancreas using a special needle.

• #30 Review of Diagnostic Biomarkers in Autoimmune Pancreatitis: Where Are We Now?

  https://www.mdpi.com/2075-4418/11/5/770

  Autoimmune pancreatitis (AIP) is a pancreatic manifestation of an IgG4-related disease (IgG4-RD). AIP lacks disease-specific biomarkers, and therefore, it is difficult to distinguish AIP from malignancies, especially pancreatic cancer. In this review, we have summarized the latest findings on potential diagnostic biomarkers for AIP. Many investigations have been conducted, but no specific biomarkers for AIP are identified. Therefore, further studies are required to identify accurate diagnostic biomarkers for AIP. […] Elevation of serum IgG4 levels is not specific for the diagnosis of AIP because this happens in a variety of diseases, including cancers, infections, and other autoimmune diseases. It is essential to differentiate between AIP and pancreatic cancer before initiating steroid therapy.

• #31 Serological characteristics of autoimmune pancreatitis and its differential diagnosis from pancreatic cancer by using a combination of carbohydrate antigen 19-9, globulin, eosinophils and hemoglobin | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0174735

  Elevated serum eosinophils, globulin, together with decreased hemoglobin level can be used as a preoperative indicator for AIP and can help to initiate diagnosis of AIP in time. […] In this study, we reported concentration differences of the four serum markers, Ca 199, eosinophil percentage, globulin and hemoglobin, in patients with AIP and pancreatic adenocarcinoma. […] In conclusion, MicroRNAs regulate expression of more than 60% of all mammalian protein-coding genes. Serum miRNAs have been suggested as promising biomarkers to distinguish patients with pancreatic cancer from non-malignant diseases. Further investigations are needed to clarify expression profiles of miRNAs in AIP and differential diagnostic capacity of circulating miRNAs in AIP/PAC.

• #32 Autoimmune pancreatitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/autoimmune-pancreatitis/diagnosis-treatment/drc-20369804

  However, a positive test doesn’t necessarily mean that you have the disease. A small number of people who don’t have autoimmune pancreatitis, including some with pancreatic cancer, also have high levels of IgG4 in their blood. […] Endoscopic core biopsy. In this test, a medical professional called a pathologist studies a sample of pancreatic tissue in the laboratory. AIP has a distinctive appearance that can be easily recognized under a microscope by an expert pathologist. […] A small tube called an endoscope is inserted through the mouth into the stomach, guided by ultrasound. A sample of tissue is then removed from the pancreas using a special needle. […] The challenge is getting a sample of tissue large enough to study, rather than just a few cells. This procedure is not widely available, and results may not be conclusive.

• #33 Autoimmune Pancreatitis – National Pancreas Foundation

  https://pancreasfoundation.org/pancreas-disease/autoimmune-pancreatitis/

  Blood tests. You will be tested for elevated levels of an immunoglobulin called IgG4, produced by your immune system. People with type 1 AIP, but usually not with type 2 AIP, will have highly elevated blood levels of IgG4. […] However a positive test doesn’t necessarily mean that you have the disease. A small number of people who don’t have autoimmune pancreatitis, including some with pancreatic cancer, also have high blood levels of IgG4. […] Endoscopic core biopsy. In this test, pathologists analyze a sample of pancreatic tissue in the laboratory. AIP has a distinctive appearance that can be easily recognized under a microscope by an expert pathologist. Doctors insert a small tube (endoscope) through the mouth into the stomach and guided by ultrasound, remove some tissue from the pancreas using a special needle.

• #34 Autoimmune Pancreatitis – UChicago Medicine

  https://www.uchicagomedicine.org/conditions-services/gastroenterology/pancreatitis/autoimmune-pancreatitis

  Sometimes the bodys immune system mistakenly attacks the pancreas, causing the organ to swell. This is known as autoimmune pancreatitis. […] Diagnosing autoimmune pancreatitis requires high-quality imaging and, in many cases, a biopsy. For that, our highly-skilled interventional gastroenterologists thread a thin tube (endoscope) through the mouth to the pancreas to take a sample of pancreatic tissue. Then our pathologists examine this tissue under a microscope. Modern laboratory approaches, including some special stains, are used to make it easier to see the hallmark cellular features of autoimmune pancreatitis. […] Given that pancreatic cancer is a very serious disease, it is understandable that patients would want to ensure they are given an accurate diagnosis before undergoing treatment. However, its important to recognize that autoimmune disease is a rare disease. Pancreatic cancer is much more common and always needs to be ruled out before a diagnosis of autoimmune pancreatitis can be established.

• #35 Autoimmune pancreatitis: Clinical manifestations and diagnosis – UpToDate

  https://www.uptodate.com/contents/autoimmune-pancreatitis-clinical-manifestations-and-diagnosis

  Autoimmune pancreatitis: Clinical manifestations and diagnosis […] AIP can appear radiographically as a focal mass that can be difficult to distinguish from pancreatic cancer or as a diffuse pancreatic process that can be pathognomonic for AIP. […] The discovery of an elevated serum immunoglobulin G4 (IgG4) as a biomarker of AIP helped establish the disorder as distinct from other forms of chronic pancreatitis; although, later studies demonstrated that IgG4 levels are often normal in AIP and that other diseases such as pancreatic cancer may be associated with moderately elevated IgG4 levels. […] AIP is a syndrome defined by clinical, laboratory, and pathologic criteria. […] Type 1 AIP is defined histologically as lymphoplasmacytic sclerosing pancreatitis (LPSP). […] The International Consensus Diagnostic Criteria (ICDC) defined type 1 AIP as: dense infiltration of plasma cells and lymphocytes, particularly periductal; storiform (a swirling, „cartwheel” pattern) fibrosis; venulitis with lymphocytes and plasma cells, often leading to obliteration of the affected veins; and abundant (>10 cells per high-power field [HPF]) immunoglobulin G4 (IgG4)-positive plasma cells. […] Type 2 AIP is defined histologically as idiopathic duct-centric pancreatitis (IDCP) or AIP with granulocyte epithelial lesions (GELs). […] IDCP usually has very few IgG4-positive plasma cells (<10 cells/HPF).

• #36 Autoimmune Pancreatitis and Immunoglobulin G4-related Sclerosing Cholangitis: Past, Present, and Future

  https://www.kjg.or.kr/journal/view.html?doi=10.4166/kjg.2022.102

  The emergence of glucocorticoid-responsive autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis (IgG4-SC), a new disease entity, has attracted considerable interest within the international gastroenterology community. […] The diagnostic criteria used to diagnose AIP include several cardinal features of AIP that can be detected via pancreatic parenchymal imaging, ductal imaging, serum IgG4 levels, histopathology, other organ involvement, and response to glucocorticoid therapy. […] The ICDC for AIP was established so that there would be unifying criteria available for worldwide application. […] The ICDC for AIP was developed after existing criteria from Japan, the United States, Korea, and Italy were reviewed. […] The key histopathological features of type 1 AIP include 1) diffuse lymphoplasmacytic infiltration and fibrosis, 2) numerous IgG4-positive cells, 3) storiform fibrosis, 4) obliterative phlebitis, and 5) ductal lesion (periductal infiltrates with fibrosis).

• #37 Autoimmune pancreatitis: Clinical manifestations and diagnosis – UpToDate

  https://www.uptodate.com/contents/autoimmune-pancreatitis-clinical-manifestations-and-diagnosis

  Autoimmune pancreatitis: Clinical manifestations and diagnosis […] AIP can appear radiographically as a focal mass that can be difficult to distinguish from pancreatic cancer or as a diffuse pancreatic process that can be pathognomonic for AIP. […] The discovery of an elevated serum immunoglobulin G4 (IgG4) as a biomarker of AIP helped establish the disorder as distinct from other forms of chronic pancreatitis; although, later studies demonstrated that IgG4 levels are often normal in AIP and that other diseases such as pancreatic cancer may be associated with moderately elevated IgG4 levels. […] AIP is a syndrome defined by clinical, laboratory, and pathologic criteria. […] Type 1 AIP is defined histologically as lymphoplasmacytic sclerosing pancreatitis (LPSP). […] The International Consensus Diagnostic Criteria (ICDC) defined type 1 AIP as: dense infiltration of plasma cells and lymphocytes, particularly periductal; storiform (a swirling, „cartwheel” pattern) fibrosis; venulitis with lymphocytes and plasma cells, often leading to obliteration of the affected veins; and abundant (>10 cells per high-power field [HPF]) immunoglobulin G4 (IgG4)-positive plasma cells. […] Type 2 AIP is defined histologically as idiopathic duct-centric pancreatitis (IDCP) or AIP with granulocyte epithelial lesions (GELs). […] IDCP usually has very few IgG4-positive plasma cells (<10 cells/HPF).

• #38 Autoimmune pancreatitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/autoimmune-pancreatitis/diagnosis-treatment/drc-20369804

  However, a positive test doesn’t necessarily mean that you have the disease. A small number of people who don’t have autoimmune pancreatitis, including some with pancreatic cancer, also have high levels of IgG4 in their blood. […] Endoscopic core biopsy. In this test, a medical professional called a pathologist studies a sample of pancreatic tissue in the laboratory. AIP has a distinctive appearance that can be easily recognized under a microscope by an expert pathologist. […] A small tube called an endoscope is inserted through the mouth into the stomach, guided by ultrasound. A sample of tissue is then removed from the pancreas using a special needle. […] The challenge is getting a sample of tissue large enough to study, rather than just a few cells. This procedure is not widely available, and results may not be conclusive.

• #39 Autoimmune Pancreatitis – National Pancreas Foundation

  https://pancreasfoundation.org/pancreas-disease/autoimmune-pancreatitis/

  The challenge is obtaining a sample of tissue large enough to analyze, rather than just a few cells. This procedure is not widely available, and results may not be conclusive. […] Steroid trial. Autoimmune pancreatitis generally responds to steroids; doctors sometimes use a trial course of this drug to confirm a diagnosis. However, this strategy should ideally be under expert guidance, be used sparingly and only be done when there is strong evidence to support a diagnosis of autoimmune pancreatitis. Response to corticosteroids is measured by CT and improvement in serum IgG4 levels.

• #40 Autoimmune Pancreatitis and Immunoglobulin G4-related Sclerosing Cholangitis: Past, Present, and Future

  https://www.kjg.or.kr/journal/view.html?doi=10.4166/kjg.2022.102

  EUS-guided sampling from the pancreas can provide a definite diagnosis of AIP. […] A recent meta-analysis showed that the pooled diagnostic yields for level 1 or 2 histology criteria of AIP were 55.8% for FNA and 87.2% for FNB (p=0.030). […] Glucocorticoids are the first-line therapy for AIP and IgG4-RD. […] The treatment goal may be to achieve symptomatic and radiological remission. […] AIP tends to be both underdiagnosed and overdiagnosed. […] A thorough history taking and a detailed review of past medical issues often reveal unrecognized manifestations of IgG4-RD.

• #41 Autoimmune pancreatitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/autoimmune-pancreatitis/diagnosis-treatment/drc-20369804

  Steroid trial. Autoimmune pancreatitis generally responds to steroids; healthcare professionals sometimes use a trial course of this drug to confirm a diagnosis. However, this strategy should ideally happen under expert guidance. It should be used sparingly and only be done when there is strong evidence to support a diagnosis of autoimmune pancreatitis. Response to corticosteroids is measured by CT and improvement in serum IgG4 levels.

• #42 Autoimmune Pancreatitis – National Pancreas Foundation

  https://pancreasfoundation.org/pancreas-disease/autoimmune-pancreatitis/

  The challenge is obtaining a sample of tissue large enough to analyze, rather than just a few cells. This procedure is not widely available, and results may not be conclusive. […] Steroid trial. Autoimmune pancreatitis generally responds to steroids; doctors sometimes use a trial course of this drug to confirm a diagnosis. However, this strategy should ideally be under expert guidance, be used sparingly and only be done when there is strong evidence to support a diagnosis of autoimmune pancreatitis. Response to corticosteroids is measured by CT and improvement in serum IgG4 levels.

• #43 Autoimmune pancreatitis | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/autoimmune-pancreatitis

  You may have a test to check for elevated levels of an immunoglobulin called IgG4. IgG4 is produced by your immune system. People with type 1 AIP often have high levels of IgG4 in their blood. People with type 2 AIP usually do not. […] In this test, a medical professional called a pathologist studies a sample of pancreatic tissue in the laboratory. AIP has a distinctive appearance that can be easily recognized under a microscope by an expert pathologist. […] Autoimmune pancreatitis generally responds to steroids; healthcare professionals sometimes use a trial course of this drug to confirm a diagnosis. However, this strategy should ideally happen under expert guidance. It should be used sparingly and only be done when there is strong evidence to support a diagnosis of autoimmune pancreatitis. Response to corticosteroids is measured by CT and improvement in serum IgG4 levels.

• #44 Autoimmune Pancreatitis: Differential Diagnosis With Pancreatic Adenocarcinoma | Cirugía Española (English Edition)

  https://www.elsevier.es/es-revista-cirugia-espanola-english-edition–436-articulo-autoimmune-pancreatitis-differential-diagnosis-with-S2173507717301667

  AIP is a disease that responds to treatment with corticosteroids, and accurate diagnosis is crucial to avoid unnecessary major surgeries. The diagnosis is based on five factors: histology, imaging, serology, involvement of other organs and response to corticoid treatment (HISORt criteria). […] Radiological characteristics can be a determining factor in the differential diagnosis. The complementary tests that best guide the definitive diagnosis are computed tomography (CT) and endoscopic retrograde cholangiopancreatography (ERCP). […] Serum elevation of IgG4 (immunoglobulin G4) above normal levels (140mg/dL) is another of the basic diagnostic criteria. […] Treatment with corticosteroids should only be initiated after adenocarcinoma has been ruled out. The recommended dose is 40mg of prednisone every 24 hours. After 2 weeks, the response to treatment should be evaluated with IgG4, Ca 19-9 and imaging tests.

• #45 Autoimmune pancreatitis – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/autoimmune-pancreatitis/

  Steroid trial. Autoimmune pancreatitis generally responds to steroids; doctors sometimes use a trial course of this drug to confirm a diagnosis. However, this strategy should ideally be under expert guidance, be used sparingly and only be done when there is strong evidence to support a diagnosis of autoimmune pancreatitis. Response to corticosteroids is measured by CT and improvement in serum IgG4 levels.

• #46 Autoimmune Pancreatitis: Differential Diagnosis With Pancreatic Adenocarcinoma | Cirugía Española (English Edition)

  https://www.elsevier.es/es-revista-cirugia-espanola-english-edition–436-articulo-autoimmune-pancreatitis-differential-diagnosis-with-S2173507717301667

  AIP is a disease that responds to treatment with corticosteroids, and accurate diagnosis is crucial to avoid unnecessary major surgeries. The diagnosis is based on five factors: histology, imaging, serology, involvement of other organs and response to corticoid treatment (HISORt criteria). […] Radiological characteristics can be a determining factor in the differential diagnosis. The complementary tests that best guide the definitive diagnosis are computed tomography (CT) and endoscopic retrograde cholangiopancreatography (ERCP). […] Serum elevation of IgG4 (immunoglobulin G4) above normal levels (140mg/dL) is another of the basic diagnostic criteria. […] Treatment with corticosteroids should only be initiated after adenocarcinoma has been ruled out. The recommended dose is 40mg of prednisone every 24 hours. After 2 weeks, the response to treatment should be evaluated with IgG4, Ca 19-9 and imaging tests.

• #47 Treatment approaches in autoimmune pancreatitis (Review)

  https://www.spandidos-publications.com/10.3892/br.2023.1714

  Autoimmune pancreatitis (AIP) is a rare disease. There are two distinct types of AIP: AIP type 1 (AIP-1), a pancreatic manifestation of a multi-organ disease linked to immunoglobulin (Ig)G4, and AIP type 2 (AIP-2), a pancreas-specific disease unrelated to IgG4. […] The diagnosis of AIP is markedly different between the two main types of the disease. In the case of AIP-1, diagnosis is based on a combination of clinical, imaging and serological criteria, including increased levels of serum IgG4 and distinctive radiological features, such as diffuse or focal thickening of the pancreas and the presence of a peripancreatic halo in magnetic resonance imaging or computed tomography. On the other hand, the diagnosis of AIP-2 requires a multidisciplinary approach, including histological evaluation through endoscopically guided biopsy, the presence of associated IBD and a positive response to steroid therapy in the absence of specific serological markers such as IgG4. […] The diagnosis of AIP, an uncommon form of pancreatitis with autoimmune characteristics, is frequently challenging. The two type of AIP, AIP-1 and -2, which are clearly distinguished by ICDC, must also be distinguished when AIP is suspected.

• #48 Autoimmune pancreatitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/autoimmune-pancreatitis/diagnosis-treatment/drc-20369804

  Autoimmune pancreatitis is hard to diagnose, because its symptoms are a lot like those of pancreatic cancer. However, an accurate diagnosis is extremely important. Undiagnosed cancer may result in delaying or not receiving necessary treatment. […] To pinpoint the diagnosis and determine the type of AIP, blood and imaging tests are necessary. […] No single test or characteristic feature identifies autoimmune pancreatitis. Recommended guidelines for diagnosis use a combination of imaging, blood tests and biopsy results. […] Specific tests may include: Imaging tests. Tests of your pancreas and other organs may include CT, MRI, endoscopic ultrasound (EUS) and endoscopic retrograde cholangiopancreatography (ERCP). […] Blood tests. You may have a test to check for elevated levels of an immunoglobulin called IgG4. IgG4 is produced by your immune system. People with type 1 AIP often have high levels of IgG4 in their blood. People with type 2 AIP usually do not.

• #49 Autoimmune Pancreatitis – National Pancreas Foundation

  https://pancreasfoundation.org/pancreas-disease/autoimmune-pancreatitis/

  Autoimmune pancreatitis is difficult to diagnose because its signs and symptoms are very similar to those of pancreatic cancer. However, an accurate diagnosis is extremely important. Undiagnosed cancer may result in delaying or not receiving necessary treatment. […] People with AIP tend to have a general enlargement of the pancreas, but they may also have a mass in the pancreas. To pinpoint the diagnosis and determine which type of AIP you have, blood and imaging tests are necessary. […] No single test or characteristic feature identifies autoimmune pancreatitis. Consensus guidelines for diagnosis use a combination of imaging, blood tests and biopsy results. […] Specific tests may include: Imaging tests. Tests of your pancreas and other organs may include CT, MRI, endoscopic ultrasound (EUS) and endoscopic retrograde cholangiopancreatography (ERCP).

• #50 Autoimmune Pancreatitis – UChicago Medicine

  https://www.uchicagomedicine.org/conditions-services/gastroenterology/pancreatitis/autoimmune-pancreatitis

  Sometimes the bodys immune system mistakenly attacks the pancreas, causing the organ to swell. This is known as autoimmune pancreatitis. […] Diagnosing autoimmune pancreatitis requires high-quality imaging and, in many cases, a biopsy. For that, our highly-skilled interventional gastroenterologists thread a thin tube (endoscope) through the mouth to the pancreas to take a sample of pancreatic tissue. Then our pathologists examine this tissue under a microscope. Modern laboratory approaches, including some special stains, are used to make it easier to see the hallmark cellular features of autoimmune pancreatitis. […] Given that pancreatic cancer is a very serious disease, it is understandable that patients would want to ensure they are given an accurate diagnosis before undergoing treatment. However, its important to recognize that autoimmune disease is a rare disease. Pancreatic cancer is much more common and always needs to be ruled out before a diagnosis of autoimmune pancreatitis can be established.

• #51 Untypical autoimmune pancreatitis and pancreatic cancer: differential diagnosis experiences extracted from misdiagnose of two cases | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/s13023-019-1217-z

  Differentiation between pancreatic cancer (PC) and focal form of autoimmune pancreatitis (AIP) is very challenging, with similar clinical presentations, laboratory results and morphologic imagings of US, CT, EUS, MRI, ERCP, PET-CT. […] Considering the totally different management strategy of the two diseases, accurate diagnostic value is urgently needed to remind the clinicians of the rare diagnosis of untypical AIP among frequent PC-suspected patients. […] We find that mild fluctuating jaundice with abdominal pain, young age, tumor marker of TPS, TPA and diverse results between variable radiological tests can help to differentiate AIP mass from PC, through retrospectively analyzing work-up process of AIP in two patients who underwent laparotomy for suspected PC. […] However, several studies report pancreatic masses in patients with 1.6 times the upper limit of normal serum IgG4 levels (135mg/dL) histopathologically proven to be PC.

• #52

  https://link.springer.com/article/10.1007/s11604-020-00971-z

  Optimal imaging methods and scan timings for dynamic contrast-enhanced CT (DCE-CT) are important for accurate assessment of pancreatic findings. […] Whenever possible, DCE-CT should be performed. […] Multidirectional and appropriate image acquisition including MRCP and dynamic contrast-enhanced MRI (DCE-MRI) is necessary for accurate assessment of a pancreatic lesion. […] Whenever possible, DCE-MRI should be performed. […] FDG-PET and Ga-67 scintigram are useful for diagnosis of AIP because they can evaluate the lesion function, which cannot be evaluated by CT and MRI. […] The degrees of drug accumulation in AIP and extrapancreatic lesions depend on the lesion activity. […] The drug accumulation decreases if treatment is effective. […] Specific CT and MR findings for AIP, such as the capsule-like rim, duct-penetrating sign, speckled/dotted enhancement, and homogeneous delayed enhancement, are adopted as useful findings for differentiation of AIP from pancreatic cancer in the JPS2018.

• #53 The Role of Endoscopic Ultrasound and Ancillary Techniques in the Diagnosis of Autoimmune Pancreatitis: A Comprehensive Review

  https://www.mdpi.com/2075-4418/14/12/1233

  Guidelines support the preoperative differentiation of AIP with pancreaticobiliary malignancies that is crucial to avoid the consequences of progressive disease and unnecessary surgery. […] In the available guidelines, the imaging features of AIP are depicted using cross-sectional imaging systems and endoscopic retrograde cholangiography, while endoscopic ultrasound (EUS) is mainly performed to obtain tissue samples to rule out PC. […] Nevertheless, several papers have reported the reliability of EUS in providing the morphological features of pancreatic disease in patients with AIP. However, there is still a lack of standardization. […] The main goal for clinicians is to distinguish between AIP, specifically the focal form, and pancreatic malignancies as PC. As reported, in some cases, the clinical and radiological findings of AIP and PC may overlap making it arduous to reach a diagnosis. For this purpose, conventional EUS can display important information during the initial evaluation of this subgroup of patients but is often not enough despite high sensitivity and specificity over cross-sectional imaging in the detection of pancreatic malignancies.

• #54

  https://link.springer.com/article/10.1007/s11604-020-00971-z

  The ADC value of AIP is lower than that of pancreatic cancer, but it is difficult to differentiate between pancreatic cancer and pancreatitis accompanying the tumor. […] It should be noted that pathological diagnosis by EUS-fine needle aspiration should be considered if any radiologic findings suggestive of pancreatic cancer are seen.

• #55 Diagnosis of autoimmune pancreatitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4248199/

  Autoimmune pancreatitis (AIP) is a distinct form of chronic pancreatitis that is increasingly being reported. […] Therefore, accurate diagnosis is necessary for cases of AIP. […] These findings have led several countries to propose diagnostic criteria for AIP, which consist of essentially similar diagnostic items; however, several differences exist for each country, mainly due to differences in the definition of AIP and the modalities used to diagnose this disease. […] An attempt to unite the diagnostic criteria worldwide was made with the publication in 2011 of the international consensus diagnostic criteria for AIP, established at the 2010 Congress of the International Association of Pancreatology (IAP). […] The first diagnostic criteria for AIP were established in Japan in 2002, revised in 2006, and revised again in 2011. […] AIP was later revealed to consist of two distinct subtypes: type 1 AIP, which is characterized by histology resembling that of lymphoplasmacytic sclerosing pancreatitis (LPSP), and type 2 AIP or idiopathic duct-centric pancreatitis (IDCP) with granulocytic epithelial lesion (GEL). […] The proportions of type 1 and type 2 AIP vary substantially in western and eastern countries. […] The ICDC are presently evaluated as the most sensitive and specific criteria for diagnosing AIP.

• #56 Diagnosis of autoimmune pancreatitis

  https://www.wjgnet.com/1007-9327/full/v20/i44/16559.htm

  The ICDC are presently evaluated as the most sensitive and specific criteria for diagnosing AIP. […] Diagnostic criteria, either nationwide or international, consist mostly of common diagnostic items such as image findings of the pancreatic parenchyma, pancreatography, and extrapancreatic lesions; serological findings; histology of the pancreatic lesion; and response to steroid therapy. […] The most sensitive and specific serum marker for type 1 AIP is IgG4 ( 135 mg/dL, sensitivity: 86%, specificity to AIP against PC: 96%). […] A response to steroid is a commonly evaluated diagnostic item for AIP in almost all diagnostic criteria. […] AIP is often associated with sclerosing cholangitis, which needs differential diagnosis from bile duct cancer.

• #57

  https://link.springer.com/article/10.1007/s11604-020-00971-z

  The present paper describes the imaging methods for obtaining optimal images and the characteristic imaging findings with the aim of standardizing image quality and improving diagnostic accuracy when radiologists diagnose AIP in actual clinical settings. […] In the JPS2018, pancreatic duct evaluation by MRCP alone is permitted because the quality of MRCP has improved and useful MRCP findings for diagnosing AIP were recently reported. […] However, there is a problem in AIP diagnosis with equivalent accuracy among individual radiologists. […] There are no descriptions about the imaging methods, such as detailed imaging parameters and optimal timings of dynamic contrast-enhanced computed tomography (CT)/magnetic resonance imaging (MRI), in the JPS2018 or in the 2013 amendment of the Japanese Consensus Guidelines for AIP.

• #58

  https://link.springer.com/article/10.1007/s11604-020-00971-z

  Because the JPS2018 is not sufficient to enable all radiologists to achieve accurate diagnosis of AIP, there is a need for further guidance to make this possible and for easy-to-understand manuals that can be compared with the diagnostic images of AIP patients. […] We aimed to standardize the image examination methods and improve the diagnostic accuracy so that radiologists can accurately diagnose AIP. […] Optimal imaging methods to obtain good diagnostic images that cannot be covered by the guidelines and characteristic radiologic findings were described in a comprehensive way, together with radiologic images of AIP patients. […] In this guidance, AIP means type 1 AIP, if there is no other particular explanation. […] Comprehensive diagnosis using multiple modalities is mandatory for accurate diagnosis of AIP, and ultrasound (US) including abdominal US and endoscopic ultrasonography (EUS), CT including non-contrast CT and contrast-enhanced CT, nuclear medicine including fluorodeoxyglucose-positron-emission tomography (FDG-PET) and Ga-67 scintigraphy, and ERP are recommended.

• #59

  https://link.springer.com/article/10.1007/s11604-020-00971-z

  Because the JPS2018 is not sufficient to enable all radiologists to achieve accurate diagnosis of AIP, there is a need for further guidance to make this possible and for easy-to-understand manuals that can be compared with the diagnostic images of AIP patients. […] We aimed to standardize the image examination methods and improve the diagnostic accuracy so that radiologists can accurately diagnose AIP. […] Optimal imaging methods to obtain good diagnostic images that cannot be covered by the guidelines and characteristic radiologic findings were described in a comprehensive way, together with radiologic images of AIP patients. […] In this guidance, AIP means type 1 AIP, if there is no other particular explanation. […] Comprehensive diagnosis using multiple modalities is mandatory for accurate diagnosis of AIP, and ultrasound (US) including abdominal US and endoscopic ultrasonography (EUS), CT including non-contrast CT and contrast-enhanced CT, nuclear medicine including fluorodeoxyglucose-positron-emission tomography (FDG-PET) and Ga-67 scintigraphy, and ERP are recommended.

• #60 Diagnosis Autoimmune Pancreatitis | Pancreapedia

  https://pancreapedia.org/reviews/diagnosis-autoimmune-pancreatitis

  AIP is an increasingly recognized clinical entity. Although an elevated serum IgG4 level is an important diagnostic clue, this is inadequate to establish a diagnosis independently and other collateral evidence is needed. The ICDC are recently published diagnostic criteria that help both the clinician and researcher accurately identify those with AIP using one of several combinations of key diagnostic features (pancreatic parenchymal imaging, pancreatic ductal imaging (i.e. ERP), serum IgG4 level, other organ involvement, histology of the pancreas, and response to steroid treatment). Since AIP is rare the clinicians diagnostic approach must primarily focus on the exclusion of malignancy, and then on solidifying the AIP diagnosis.

• #61 Review of Diagnostic Biomarkers in Autoimmune Pancreatitis: Where Are We Now?

  https://www.mdpi.com/2075-4418/11/5/770

  Autoimmune pancreatitis (AIP) is a pancreatic manifestation of an IgG4-related disease (IgG4-RD). AIP lacks disease-specific biomarkers, and therefore, it is difficult to distinguish AIP from malignancies, especially pancreatic cancer. In this review, we have summarized the latest findings on potential diagnostic biomarkers for AIP. Many investigations have been conducted, but no specific biomarkers for AIP are identified. Therefore, further studies are required to identify accurate diagnostic biomarkers for AIP. […] Elevation of serum IgG4 levels is not specific for the diagnosis of AIP because this happens in a variety of diseases, including cancers, infections, and other autoimmune diseases. It is essential to differentiate between AIP and pancreatic cancer before initiating steroid therapy.

• #62 Review of Diagnostic Biomarkers in Autoimmune Pancreatitis: Where Are We Now?

  https://www.mdpi.com/2075-4418/11/5/770

  We previously identified laminin 511-E8 as a pathogenic antigen for AIP and suggested that anti-laminin 511-E8 may be a useful biomarker for AIP. However, various studies, including ours, could not identify specific biomarkers for the diagnosis of AIP. […] Therefore, combining IgG4 with other biomarkers could be useful for the diagnosis of AIP. […] The presence of diffuse pancreatic swelling and IAC-complicated AIP are considered clinical predictors of AIP relapse; however, objective evaluation using biomarkers is desirable. […] The presence of diffuse pancreatic swelling and IAC-complicated AIP are considered clinical predictors of AIP relapse; however, objective evaluation using biomarkers is desirable. […] The usefulness of MST for preventing AIP relapse has been controversial, but some reports in Japan support using MST following the remission of AIP. […] However, the appropriate therapy and follow-up management of AIP following remission is not established.

• #63 Serological characteristics of autoimmune pancreatitis and its differential diagnosis from pancreatic cancer by using a combination of carbohydrate antigen 19-9, globulin, eosinophils and hemoglobin | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0174735

  Elevated serum eosinophils, globulin, together with decreased hemoglobin level can be used as a preoperative indicator for AIP and can help to initiate diagnosis of AIP in time. […] In this study, we reported concentration differences of the four serum markers, Ca 199, eosinophil percentage, globulin and hemoglobin, in patients with AIP and pancreatic adenocarcinoma. […] In conclusion, MicroRNAs regulate expression of more than 60% of all mammalian protein-coding genes. Serum miRNAs have been suggested as promising biomarkers to distinguish patients with pancreatic cancer from non-malignant diseases. Further investigations are needed to clarify expression profiles of miRNAs in AIP and differential diagnostic capacity of circulating miRNAs in AIP/PAC.

• #64 Review of Diagnostic Biomarkers in Autoimmune Pancreatitis: Where Are We Now?

  https://www.mdpi.com/2075-4418/11/5/770

  We previously identified laminin 511-E8 as a pathogenic antigen for AIP and suggested that anti-laminin 511-E8 may be a useful biomarker for AIP. However, various studies, including ours, could not identify specific biomarkers for the diagnosis of AIP. […] Therefore, combining IgG4 with other biomarkers could be useful for the diagnosis of AIP. […] The presence of diffuse pancreatic swelling and IAC-complicated AIP are considered clinical predictors of AIP relapse; however, objective evaluation using biomarkers is desirable. […] The presence of diffuse pancreatic swelling and IAC-complicated AIP are considered clinical predictors of AIP relapse; however, objective evaluation using biomarkers is desirable. […] The usefulness of MST for preventing AIP relapse has been controversial, but some reports in Japan support using MST following the remission of AIP. […] However, the appropriate therapy and follow-up management of AIP following remission is not established.

• #65 EUS in the Diagnosis of Autoimmune Pancreatitis | Pancreapedia

  https://pancreapedia.org/reviews/eus-in-diagnosis-of-autoimmune-pancreatitis

  Autoimmune pancreatitis (AIP) has historically been considered a rare disorder, but is increasingly recognized due to an improved understanding of its diverse nature and proper means of diagnosis. The current international consensus diagnostic criteria (ICDC) for the diagnosis of AIP incorporate 5 cardinal features: imaging characteristics of the pancreas (parenchyma and duct), serology, other organ involvement, pancreatic histology, and response to steroids. Imaging techniques recognized in the guidelines include computed tomography (CT), magnetic resonance imaging (MRI), magnetic resonance cholangiopancreatography (MRCP), and endoscopic retrograde cholangiopancreatography (ERCP). Endoscopic ultrasound (EUS) is notably absent from the diagnostic algorithms. […] There are emerging data suggesting the potential utility of EUS in the diagnosis of AIP. EUS not only has the ability to provide high-definition imaging of the pancreas, but it also has the ability to acquire tissue through either fine needle aspiration (FNA) or trucut biopsy (TCB). These characteristics help to make it one of the most useful techniques in the diagnosis of pancreatic cancer and chronic pancreatitis. Therefore, EUS has the potential to play a role in the both diagnosis of AIP and exclusion of other pancreatic diseases.

• #66 EUS in the Diagnosis of Autoimmune Pancreatitis | Pancreapedia

  https://pancreapedia.org/reviews/eus-in-diagnosis-of-autoimmune-pancreatitis

  Autoimmune pancreatitis (AIP) has historically been considered a rare disorder, but is increasingly recognized due to an improved understanding of its diverse nature and proper means of diagnosis. The current international consensus diagnostic criteria (ICDC) for the diagnosis of AIP incorporate 5 cardinal features: imaging characteristics of the pancreas (parenchyma and duct), serology, other organ involvement, pancreatic histology, and response to steroids. Imaging techniques recognized in the guidelines include computed tomography (CT), magnetic resonance imaging (MRI), magnetic resonance cholangiopancreatography (MRCP), and endoscopic retrograde cholangiopancreatography (ERCP). Endoscopic ultrasound (EUS) is notably absent from the diagnostic algorithms. […] There are emerging data suggesting the potential utility of EUS in the diagnosis of AIP. EUS not only has the ability to provide high-definition imaging of the pancreas, but it also has the ability to acquire tissue through either fine needle aspiration (FNA) or trucut biopsy (TCB). These characteristics help to make it one of the most useful techniques in the diagnosis of pancreatic cancer and chronic pancreatitis. Therefore, EUS has the potential to play a role in the both diagnosis of AIP and exclusion of other pancreatic diseases.

• #67 EUS in the Diagnosis of Autoimmune Pancreatitis | Pancreapedia

  https://pancreapedia.org/reviews/eus-in-diagnosis-of-autoimmune-pancreatitis

  To our knowledge, there are no studies that have directly compared EUS to other imaging modalities such as CT scan, MRI, or ERCP for the diagnosis of AIP. Therefore, it is unclear as to the additive value of EUS imaging to the other imaging techniques. However, a cohort of 48 patients seen at Mayo Clinic Rochester with a diagnosis of AIP using HISORt criteria underwent EUS with TCB. The diagnosis of AIP was strongly suspected in 14 patients prior to EUS based on their clinical, laboratory, and imaging findings. In 22 patients, the diagnosis was considered as a part of a broader differential prior to EUS and in remaining 12 patients the EUS appearance alone led to the initial suspicion of AIP. Therefore, this suggests that EUS imaging alone may increase the diagnostic accuracy of AIP in patients who underwent other imaging modalities without a definitive diagnosis.

• #68 EUS in the Diagnosis of Autoimmune Pancreatitis | Pancreapedia

  https://pancreapedia.org/reviews/eus-in-diagnosis-of-autoimmune-pancreatitis

  Although the role of EUS-guided tissue acquisition has not been extensively studied, pancreatic histology is recognized as an important diagnostic criterion in the ICDC. Despite a few reports on the ability to diagnose AIP using FNA only, there are no broadly accepted consensus cytological diagnostic criteria for AIP and most pathologists are reluctant to rely solely on FNA specimens. FNA commonly yields small specimen samples and results in a loss of tissue architecture, making its interpretation challenging. […] EUS TCB appears to be safe and may provide sufficient material to aid in the diagnosis of AIP, thereby guiding treatment and avoiding surgical intervention. Some suggest the use of EUS TCB as a rescue technique to obtain adequate tissue samples if EUS FNA failed. The current ICDC guidelines recommend a pancreatic core biopsy in patients presenting with a focal mass and/or obstructive jaundice if cancer has been excluded and the diagnosis remains elusive.

• #69 Endoscopic Ultrasound–Guided Core Biopsy for Diagnosis of Autoimmune Pancreatitislogo-32logo-40logo-60NEJM Journal WatchnejmJW_1L_RGB-b

  https://www.jwatch.org/na51918/2020/07/01/endoscopic-ultrasound-guided-core-biopsy-diagnosis

  Endoscopic Ultrasound–Guided Core Biopsy for Diagnosis of Autoimmune Pancreatitis […] This approach seems safe and effective for achieving histological diagnosis. […] However, the diagnosis can be difficult to confirm. […] To evaluate the role of endoscopic ultrasound (EUS)–guided core biopsy in diagnosing suspected AIP, researchers conducted a prospective, multicenter study in 56 patients. […] Key pathologic findings for type 1 AIP — lymphoplasmacytic infiltration, obliterative phlebitis, storiform fibrosis, and >10 IgG4-positive plasma cells/high-power field — were identified in 55 (100%), 24 (44%), 40 (73%), and 36 (66%) of patients, respectively. […] This study shows that EUS-guided core biopsy for suspected AIP is safe and has a high diagnostic accuracy. […] In my experience, most patients with AIP can be accurately diagnosed without a biopsy, but these findings suggest that biopsy may be a good option in those with an unclear diagnosis.

• #70 Practical Applications of EUS Elastography: Evidence and Challenges | IntechOpen

  https://www.intechopen.com/online-first/1204926

  Autoimmune pancreatitis (AIP) is less common and is underdiagnosed. It has a wide spectrum of radiological features. When there is a focal mass-like lesion, it can be easily confused with pancreatic cancer (PC). This diagnostic dilemma could lead to unnecessary surgery. On elastographic assessment, there is increased hardness in the tumoral area, whereas the entire pancreas appears homogenously stiff in AIP. It thus helps us in differentiating PC from AIP. […] Normal pancreas appears homogenously green on qualitative elastography, while in AIP, the inflamed pancreatic parenchyma appears mainly green with some smaller areas of red or yellow. However, this is not a consistent feature and is not a reliable pattern to diagnose AIP. […] There is no reliable cut-off for diagnosis of AIP, nor there is consensus in differentiating features between focal AIP and PC on quantitative SWE elastography. […] As of today, elastography is not a mandatory investigation in diagnosing AIP, nor it is a useful stand-alone test to differentiate between PC and focal AIP. However, serial imaging when utilised with EUS elastography and FNB will help us in establishing a diagnosis.

• #71 Autoimmune pancreatitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/autoimmune-pancreatitis/diagnosis-treatment/drc-20369804

  Autoimmune pancreatitis is hard to diagnose, because its symptoms are a lot like those of pancreatic cancer. However, an accurate diagnosis is extremely important. Undiagnosed cancer may result in delaying or not receiving necessary treatment. […] To pinpoint the diagnosis and determine the type of AIP, blood and imaging tests are necessary. […] No single test or characteristic feature identifies autoimmune pancreatitis. Recommended guidelines for diagnosis use a combination of imaging, blood tests and biopsy results. […] Specific tests may include: Imaging tests. Tests of your pancreas and other organs may include CT, MRI, endoscopic ultrasound (EUS) and endoscopic retrograde cholangiopancreatography (ERCP). […] Blood tests. You may have a test to check for elevated levels of an immunoglobulin called IgG4. IgG4 is produced by your immune system. People with type 1 AIP often have high levels of IgG4 in their blood. People with type 2 AIP usually do not.

• #72 Autoimmune Pancreatitis – National Pancreas Foundation

  https://pancreasfoundation.org/pancreas-disease/autoimmune-pancreatitis/

  Autoimmune pancreatitis is difficult to diagnose because its signs and symptoms are very similar to those of pancreatic cancer. However, an accurate diagnosis is extremely important. Undiagnosed cancer may result in delaying or not receiving necessary treatment. […] People with AIP tend to have a general enlargement of the pancreas, but they may also have a mass in the pancreas. To pinpoint the diagnosis and determine which type of AIP you have, blood and imaging tests are necessary. […] No single test or characteristic feature identifies autoimmune pancreatitis. Consensus guidelines for diagnosis use a combination of imaging, blood tests and biopsy results. […] Specific tests may include: Imaging tests. Tests of your pancreas and other organs may include CT, MRI, endoscopic ultrasound (EUS) and endoscopic retrograde cholangiopancreatography (ERCP).

• #73 Diagnosis Autoimmune Pancreatitis | Pancreapedia

  https://pancreapedia.org/reviews/diagnosis-autoimmune-pancreatitis

  ICDC are organized to allow the user to diagnose AIP along several potential pathways; i.e., characteristic imaging, characteristic histology, or response to steroid therapy. However, it all starts with review of pancreatic findings on cross-sectional imaging (CT or MRI). These are classified as typical of AIP (level 1) or atypical/indeterminate for AIP (Level 2). Next, available data supporting the diagnosis of AIP (i.e., collateral evidence) is considered in combination with the imaging findings. Collateral evidence (i.e., pancreatic ductal imaging, serum IgG4, other organ involvement, and response to treatment) is assigned one of two levels based on the strength of association with AIP. To establish a definitive diagnosis of type 1 AIP varying strengths of collateral evidence are needed depending on imaging findings. For example, in patients with typical parenchymal imaging for AIP (which is a relatively specific finding) any level of non-pancreatic collateral evidence secures a definitive diagnosis. Conversely, stronger collateral evidence is required when imaging is indeterminate for AIP. Using these criteria several combinations can be used to establish an AIP diagnosis, even without the need for histology or ERP.

• #74 Autoimmune Pancreatitis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560769/

  Autoimmune pancreatitis is a clinical condition characterized by inflammation of the pancreas from an autoimmune etiology. It is an uncommon cause of pancreatitis but can present with significant morbidity and mortality because of repeated episodes of acute pancreatitis. […] Timely diagnosis and management are important to achieve good patient outcomes. […] The international consensus diagnostic criteria (ICDC) for autoimmune pancreatitis include imaging of the pancreas and pancreatic duct, serologic studies including IgG4 levels, histopathology, and other associated conditions. […] A histopathologic examination can help and is usually necessary to distinguish autoimmune pancreatitis from pancreatic cancer. […] Histopathological examination can be of yield and is preferred as a part of diagnostic criteria before initiating treatment of AIP. […] After a thorough evaluation and after ruling out differential diagnoses such as pancreatic malignancy, patients need continuous follow-up with their providers to assess their response to immunosuppressant therapy.

• #75 Diagnosis of autoimmune pancreatitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4248199/

  Diagnostic criteria, either nationwide or international, consist mostly of common diagnostic items such as image findings of the pancreatic parenchyma, pancreatography, and extrapancreatic lesions; serological findings; histology of the pancreatic lesion; and response to steroid therapy. […] The diagnostic items are very similar, but the method or approach for analyzing each finding varies depending on the country. […] AIP is often associated with sclerosing cholangitis, which needs differential diagnosis from bile duct cancer. […] Today, a response to steroid is a commonly evaluated diagnostic item for AIP in almost all diagnostic criteria.

• #76 Autoimmune Pancreatitis: An Update

  https://www.medscape.com/viewarticle/708921

  Autoimmune pancreatitis (AIP) is the pancreatic manifestation of a systemic fibroinflammatory disorder. […] There are established diagnostic criteria to diagnose AIP, most of which rely on a combination of clinical presentation, imaging of the pancreas and other organs (by CT scan, MRI and endoscopic retrograde pancreatography), serology, pancreatic histology and response to steroids to make the diagnosis. […] Diagnosing AIP can be a challenge. […] Multiple diagnostic criteria have been proposed. For example, the Japanese Pancreas Society (2002 and 2006) and Histology, Imaging, Serology, Other Organ Involvement and Response to therapy (HISORt). […] There is a classic presentation, which includes obstructive jaundice, sausage-shaped pancreatic enlargement with a low-density rim, serum IgG4 elevation and dramatic response to steroids, but AIP can present in more subtle ways without classical symptoms. […] As the collective experience with this condition increases, better diagnostic and therapeutic protocols will emerge.

• #77 Diagnosis Autoimmune Pancreatitis | Pancreapedia

  https://pancreapedia.org/reviews/diagnosis-autoimmune-pancreatitis

  AIP is an increasingly recognized clinical entity. Although an elevated serum IgG4 level is an important diagnostic clue, this is inadequate to establish a diagnosis independently and other collateral evidence is needed. The ICDC are recently published diagnostic criteria that help both the clinician and researcher accurately identify those with AIP using one of several combinations of key diagnostic features (pancreatic parenchymal imaging, pancreatic ductal imaging (i.e. ERP), serum IgG4 level, other organ involvement, histology of the pancreas, and response to steroid treatment). Since AIP is rare the clinicians diagnostic approach must primarily focus on the exclusion of malignancy, and then on solidifying the AIP diagnosis.

• #78 Autoimmune pancreatitis: Management – UpToDate

  https://www.uptodate.com/contents/autoimmune-pancreatitis-management

  Autoimmune pancreatitis (AIP) is an uncommon but well-established form of pancreatic inflammation. Unlike other pancreatic diseases, it typically has a dramatic response to glucocorticoid therapy. AIP can appear radiographically as a focal mass that is indistinguishable from pancreatic cancer or as a diffuse pancreatic process that can be pathognomonic for AIP. If untreated, AIP can lead to pancreatic insufficiency, fibrosis, and other complications. The initial discovery of elevated immunoglobulin G4 (IgG4) as a biomarker of AIP helped establish the disorder as distinct from other forms of chronic pancreatitis. However, it is now known that IgG4 levels are often normal in AIP and that other diseases such as pancreatic cancer may also have moderately elevated IgG4 levels. […] The approach to management of autoimmune pancreatitis (AIP) is based upon the manifestations of AIP and the presence of immunoglobulin G4-related disease (IgG4-RD). The goal of management is to alleviate the immediate symptoms of AIP and to prevent irreversible liver fibrosis and pancreatic exocrine and endocrine failure. Treatment may prevent progression to chronic pancreatitis. However, the fibroatrophic changes that accompany the initial presentation of the disease are typically permanent. Management of AIP is largely based upon observational studies since there have been few randomized controlled trials. Our recommendations are largely consistent with the guidelines of the International Association of Pancreatology consensus conference for the treatment of AIP and the Japanese consensus guidelines for treatment of AIP. […] Treatment for AIP is indicated in patients with any one of the following: Pancreatic symptoms including obstructive jaundice, abdominal pain, or back pain.

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