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• #1 Autoimmune pancreatitis // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/autoimmune-pancreatitis

  Autoimmune pancreatitis, also called AIP, is difficult to diagnose. Often, it doesn’t cause any symptoms. Symptoms of type 1 AIP are like those of pancreatic cancer. […] The most common sign of type 1 AIP is painless jaundice. About 80% of people with type 1 AIP have painless jaundice. This is caused by blocked bile ducts. People with type 2 AIP can have repeat episodes of acute pancreatitis. Pain in the upper abdomen, a common symptom of pancreatic cancer, is often absent in autoimmune pancreatitis. […] Autoimmune pancreatitis often doesn’t cause any symptoms. See a healthcare professional, however, if you have unexplained weight loss, belly pain, jaundice or other symptoms that bother you.

• #2 Autoimmune Pancreatitis – National Pancreas Foundation

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

  Autoimmune pancreatitis (AIP) is difficult to diagnose. Often, it doesn’t cause any symptoms. Symptoms and signs of type 1 AIP are similar to those of pancreatic cancer. […] The most common sign of type 1 AIP, present in about 80% of people, is painless jaundice, caused by blocked bile ducts. Type 2 AIP can present with recurrent episodes of acute pancreatitis. Pain in the upper abdomen, a common symptom of pancreatic cancer, is frequently absent in autoimmune pancreatitis. […] Autoimmune pancreatitis often doesn’t cause any symptoms. See your doctor, however, if you experience unexplained weight loss, abdominal pain, jaundice, or other signs and symptoms that bother you. […] Autoimmune pancreatitis can cause a variety of complications. […] Symptoms of autoimmune pancreatitis often improve after a short course of prednisolone or prednisone. Many people respond quickly, even dramatically. Sometimes people get better without any treatment.

• #3 Autoimmune pancreatitis | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/autoimmune-pancreatitis?content_id=CON-20369783

  Autoimmune pancreatitis is a relatively uncommon disease of the pancreas that may be mistaken for pancreatic cancer. […] Autoimmune pancreatitis, also called AIP, is difficult to diagnose. Often, it doesn’t cause any symptoms. Symptoms of type 1 AIP are like those of pancreatic cancer. […] The most common sign of type 1 AIP is painless jaundice. About 80% of people with type 1 AIP have painless jaundice. This is caused by blocked bile ducts. People with type 2 AIP can have repeat episodes of acute pancreatitis. Pain in the upper abdomen, a common symptom of pancreatic cancer, is often absent in autoimmune pancreatitis. […] Autoimmune pancreatitis often doesn’t cause any symptoms. See a healthcare professional, however, if you have unexplained weight loss, belly pain, jaundice or other symptoms that bother you.

• #4 Autoimmune pancreatitis – Wikipedia

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

  Autoimmune pancreatitis may cause a variety of symptoms and signs, which include pancreatic and biliary (bile duct) manifestations, as well as systemic effects of the disease. Two-thirds of patients present with either painless jaundice due to bile duct obstruction or a „mass” in the head of the pancreas, mimicking carcinoma. […] Type 1 AIP typically presents in a 60-70-year-old male with painless jaundice. In some cases, imaging reveals a mass in the pancreas or diffuse pancreatic enlargement. Narrowing in the pancreatic duct called strictures may occur. Rarely, Type 1 AIP presents with acute pancreatitis. Type 1 AIP presents with manifestations of autoimmune disease (IgG4 related) in at least half of cases. The most common form of systemic involvement is cholangitis, which occurs in up to 80 percent of cases of Type 1 AIP. Additional manifestations include inflammation in the salivary glands (chronic sclerosing sialadenitis), in the lungs resulting in scarring (pulmonary fibrosis) and nodules, scarring within the chest cavity (mediastinal fibrosis) or in the anatomic space behind the abdomen (retroperitoneal fibrosis) and inflammation in the kidneys (tubulointerstitial nephritis).

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

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

  Autoimmune pancreatitis (AIP) is difficult to diagnose. Often, it doesn’t cause any symptoms. Symptoms and signs of type 1 AIP are similar to those of pancreatic cancer. […] The most common sign of type 1 AIP, present in about 80% of people, is painless jaundice, caused by blocked bile ducts. Type 2 AIP can present with recurrent episodes of acute pancreatitis. Pain in the upper abdomen, a common symptom of pancreatic cancer, is frequently absent in autoimmune pancreatitis. […] Autoimmune pancreatitis often doesn’t cause any symptoms. See your doctor, however, if you experience unexplained weight loss, abdominal pain, jaundice, or other signs and symptoms that bother you.

• #6 Autoimmune Pancreatitis: Symptoms, Types, Treatment, More

  https://www.healthline.com/health/autoimmune-pancreatitis

  The symptoms of autoimmune pancreatitis can include: jaundice, or yellowing of the skin and eyes […] upper abdominal pain […] fatigue […] loss of appetite […] unintentional weight loss […] nausea or vomiting […] dark urine […] pale or clay-colored stools. Abdominal pain is generally more common in type 2 disease. When it does happen in type 1 disease, its often mild and comes and goes. Autoimmune pancreatitis can also cause enlargement of the pancreas as well as the appearance of noncancerous growths. These findings, along with the symptoms of autoimmune pancreatitis, can sometimes be mistaken for pancreatic cancer. […] Autoimmune pancreatitis is an uncommon type of pancreatitis in which your immune system attacks healthy tissue in your pancreas. This can lead to symptoms like jaundice, fatigue, and pain in your upper abdomen. Corticosteroids are typically very effective at treating autoimmune pancreatitis. However, its still possible for relapses to happen after treatment, particularly with type 1 autoimmune pancreatitis. If its not treated, autoimmune pancreatitis can cause complications. Additionally, pancreatic cancer has very similar symptoms. As such, its important to see your doctor if you have symptoms like jaundice, abdominal pain, or unexplained weight loss.

• #7 Autoimmune Pancreatitis: Symptoms, Causes and Treatment

  https://www.medicoverhospitals.in/diseases/autoimmune-pancreatitis/

  The symptoms of autoimmune pancreatitis can vary but generally include abdominal pain, jaundice, and unexplained weight loss. […] Common Symptoms: Abdominal Pain: Often mild and persistent, it can sometimes be mistaken for other gastrointestinal disorders. […] Jaundice: Yellowing of the skin and eyes, usually due to bile duct obstruction. […] Weight Loss: Unintentional weight loss is common and may be due to malabsorption of nutrients. […] Diabetes: Some patients develop diabetes as a result of pancreatic damage. […] Autoimmune pancreatitis can disrupt the pancreas’s endocrine function, leading to the development of diabetes. […] Symptoms may include abdominal pain, jaundice, and weight loss.

• #8 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. […] In its early stages, autoimmune pancreatitis doesnt always cause symptoms. As it progresses, your pancreas ducts may narrow, preventing the release of enzymes. […] Blockage of the pancreas duct can result in: Upper abdominal pain. Middle back pain. Diarrhea. Nausea/vomiting. Weight loss. […] Autoimmune pancreatitis can also cause swelling and non-cancerous growths in your pancreas and fluid collections (cysts) in or around your pancreas. If the swelling, growths or cysts occur in the head of your pancreas, they can block the portion of the bile duct (tube) that runs through your pancreas, preventing passage of bile into your small intestine.

• #9 Autoimmune Pancreatitis: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/autoimmune-pancreatitis-causes-symptoms-and-treatment?srsltid=AfmBOoqRuD9VwPcbou2rjHLXJsrPQJwiOgeiD4H1jydvEq8fFF5O775S

  Autoimmune pancreatitis does not present any prominent signs in the majority of cases, making it quite challenging to diagnose. When the indications exhibit obviously, they resemble those of pancreatic cancer and consist of the following: […] Dark and discoloured urine […] Irregular light-coloured stools […] Discomfort in the abdomen and back pain […] Noticeable yellowing of skin and eyes, like in jaundice […] Nausea, dizziness and vomiting […] Sudden and immense weight loss […] Excessive lethargy, fatigue and weak feeling in the body […] In addition, being a persistent illness, autoimmune pancreatitis often triggers consequences of diabetes and pancreatic calcifications or stones.

• #10 Autoimmune pancreatitis symptoms, treatments & forums | PatientsLikeMe

  https://www.patientslikeme.com/conditions/autoimmune-pancreatitis

  Autoimmune pancreatitis is a chronic inflammatory characterized as masses and strictures within the pancreas. This non cancerous condition resembles pancreatic cancer. […] Common symptom: Fatigue. […] 14 autoimmune pancreatitis patients report severe fatigue (45%). […] 11 autoimmune pancreatitis patients report moderate fatigue (35%). […] 6 autoimmune pancreatitis patients report mild fatigue (19%). […] 0 autoimmune pancreatitis patients report no fatigue (0%). […] Common symptom: Pain. […] 11 autoimmune pancreatitis patients report severe pain (35%). […] 11 autoimmune pancreatitis patients report moderate pain (35%). […] 7 autoimmune pancreatitis patients report mild pain (22%). […] 2 autoimmune pancreatitis patients report no pain (6%). […] Common symptom: Stress. […] 2 autoimmune pancreatitis patients report severe stress (13%).

• #11 Autoimmune Pancreatitis | Digestive Health | Loyola Medicine

  https://www.loyolamedicine.org/services/digestive-health-program/digestive-health-conditions/autoimmune-pancreatitis

  Many of the signs and symptoms of autoimmune pancreatitis mirror that of pancreatic cancer (cancer of the pancreas), so it is important to identify underlying causes of inflammation in the pancreas. […] Individuals with autoimmune pancreatitis often experience the following: […] An enlarged or abnormally-shaped and inflamed pancreas […] Dark brown urine […] Diabetic symptoms or signs of pancreatic failure, such as high and insulin-resistant blood sugar levels (specifically, type 2 diabetes mellitus) […] Enlarged salivary glands […] Jaundice or yellowing of the skin […] Mild or moderate elevation of pancreas-derived enzymes in the blood […] The presence or formation of stones in the pancreatic duct […] Weight loss.

• #12 Autoimmune pancreatitis » Global Autoimmune Institute

  https://www.autoimmuneinstitute.org/autoimmune-resources/autoimmune-diseases-list/autoimmune-pancreatitis/

  Dark urine, pale and/or floating stool, nausea and vomiting, loss of appetite, unexplained weight loss, painless jaundice, upper abdomen and/or middle back pain, weakness, and extreme fatigue. […] AIP is caused by an immune system attack on the pancreas which causes chronic inflammation, enlargement, or a mass in the pancreas.

• #13 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. […] Jaundice (yellow eyes and skin) is a common symptom of autoimmune pancreatitis, which is caused by blocked bile ducts. Other possible symptoms include unexplained weight loss, stomach pain, and dark urine. Imaging tests often reveal a swollen pancreas (and possibly swelling in other organs) or mass-like growths on the pancreas. […] The symptoms are very similar to those experienced by people with pancreatic cancer and certain patients with chronic pancreatitis. […] Autoimmune pancreatitis responds well to prescription medicine. But a significant number of patients experience repeat attacks of the disease. Reducing recurrences is important because uncontrolled autoimmune pancreatitis can lead to chronic pancreatitis and irreversible damage to pancreatic tissue.

• #14 Autoimmune Pancreatitis – UChicago Medicine

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

  The risk of recurrence depends, in part, on the type of autoimmune pancreatitis a patient has. There are two types: Type 1 (IgG4-related) autoimmune pancreatitis tends to recur in more than 50 percent of patients. […] Type 2 autoimmune pancreatitis only affects the pancreas. After being controlled by drugs, type 2 only recurs in about 10-20 percent of patients.

• #15 Autoimmune Pancreatitis: What’s Really Behind Those Symptoms | MDedge

  https://medauth2.mdedge.com/content/autoimmune-pancreatitis-whats-really-behind-those-symptoms

  Type 1 AIP typically affects men aged 50 years or older and is often associated with jaundice, pseudotumor formation, diabetes, and exocrine pancreatic insufficiency. […] Diagnosing type 1 AIP is challenging because it can affect multiple organs, often with few symptoms, leading to significant clinical variability. […] Symptomatic patients and those at risk for organ failure, particularly lung and kidney failure, are eligible for induction treatment. […] Remission was achieved in 99% of type 1 and 92% of type 2 cases. […] The risk for recurrence (in case of nonresponse or recurrence before 12 months posttreatment) is higher in type 1 (one third of cases) than in type 2 (15%).

• #16

  https://journals.lww.com/ajg/fulltext/2018/10001/a_sausage_shaped_pancreas_that_isn_t_autoimmune.1425.aspx

  Autoimmune pancreatitis has two distinct phenotypes, type I (systemic IgG4 disease) and type II (duct-centric lymphoplasmacytic sclerosing pancreatitis, IgG4 negative). […] Diffuse parenchymal enlargement giving sausage-shaped appearance is the characteristic CT finding in autoimmune pancreatitis. […] Other abnormalities include low-attenuating rim around the pancreas, focal enlargement mimicking a tumor and diffuse pancreatic atrophy. […] The involved area has decreased enhancement on arterial phase and increased enhancement on delayed phase.

• #17 Challenges for clinicians treating autoimmune pancreatitis: Current perspectives

  https://www.wjgnet.com/2307-8960/full/v11/i1/30.htm

  Autoimmune pancreatitis (AIP) is a rare disease clinically characterized by obstructive jaundice, unintentional weight loss, acute pancreatitis, focal pancreatic mass, and diabetes. […] Clinical manifestations of AIP mainly include obstructive jaundice, weight loss, acute pancreatitis, focal pancreatic mass, abdominal pain, diabetes mellitus (DM), and inflammatory bowel disease. The most common clinical symptom of type 1 AIP is obstructive jaundice, whereas that of type 2 AIP is acute pancreatitis. […] According to a systematic review, the pooled estimate for the prevalence of DM at the time of diagnosis of type 1 AIP was 44%, which was 11% higher than that of type 2 AIP. In addition, the pooled estimated prevalence of exocrine insufficiency at the time of AIP diagnosis was 45%. Thus, the presence of DM and pancreatic exocrine insufficiency in AIP indicates that more than one-third of patients with AIP develop significant impairment of their pancreatic function.

• #18 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. Approximately one-third of cases are associated with inflammatory bowel disease, particularly ulcerative colitis; however, the pathological relationship between these two conditions has not yet been clarified. Unanswered questions relate to its pathophysiology, the potential development of a similar granulocytic injury in other organs, and the characteristics of pediatric cases. This review summarizes consensus and controversies surrounding type 2 AIP, with the aim of increasing awareness and highlighting the unmet needs of this underrecognized condition.

• #19 Autoimmune Pancreatitis: What’s Really Behind Those Symptoms | MDedge

  https://www.mdedge.com/gihepnews/article/272496/pancreas-and-biliary-tract/autoimmune-pancreatitis-whats-really-behind-those-symptoms

  Autoimmune pancreatitis [AIP] remains a diagnostic challenge. Type 1 AIP typically affects men aged 50 years or older and is often associated with jaundice, pseudotumor formation, diabetes, and exocrine pancreatic insufficiency. It is a systemic disease where lymphoplasmacytic infiltration can affect multiple organs, with the pancreas and lymph nodes most commonly involved. Diagnosing type 1 AIP is challenging because it can affect multiple organs, often with few symptoms, leading to significant clinical variability. The risk for recurrence (in case of nonresponse or recurrence before 12 months posttreatment) is higher in type 1 (one third of cases) than in type 2 (15%). This disease, which is associated with the risk for underlying cancer, requires extensive imaging (CT, MRI, and endoscopic ultrasound) to differentiate between AIP and cancer. […] Type 2 AIP generally affects younger individuals, with no gender preference. It is pathophysiologically distinct and is linked to IBD in 87% of cases.

• #20 Type 2 Autoimmune Pancreatitis: Consensus and Controversies

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

  The majority of patients with type 2 AIP present with features of acute pancreatitis (60%) or painless jaundice (30%). Episodes of acute pancreatitis in patients with type 2 AIP are clinically mild without the need for intensive care unit admission, the development of organ failure, or peripancreatic fluid collection. Other less common patterns of presentation include liver dysfunction, non-specific abdominal symptoms, and the incidental detection of a pancreatic mass on images taken for other purposes. […] Type 2 AIP is a steroid-responsive disorder, and rapid improvements in symptoms and imaging abnormalities are expected within 2 or 3 weeks of the commencement of corticosteroids. Spontaneous regression without immunosuppression has also been documented. In contrast to type 1 AIP, in which relapse is relatively common (30% to 50%), disease relapse is uncommon in type 2 AIP (10%); therefore, maintenance therapy is unnecessary in most patients.

• #21 Type 2 Autoimmune Pancreatitis (Idiopathic Duct-Centric Pancreatitis) Highlighting Patients Presenting as Clinical Acute Pancreatitis: A Single-Center Experience

  https://www.gutnliver.org/journal/view.html?doi=10.5009/gnl18429

  Type 2 autoimmune pancreatitis (AIP) has been considered extremely rare in East Asia. This study aimed to clarify the prevalence, clinical characteristics and radiological findings of type 2 AIP highlighting patients presenting as acute pancreatitis in a single center. Among 244 patients with AIP, 27 (11.1%) had type 2 AIP (definite, 15 [55.5%] and probable 12 [44.5%]). The median age of patients with type 2 AIP was 29 years (interquartile range, 20 to 39 years). Acute pancreatitis was the most common initial presentation (n=17, 63%) while obstructive jaundice was present in only one patient. Ulcerative colitis (UC) was associated with type 2 AIP in 44.4% (12/27) of patients. Radiological pancreatic imaging such as delayed enhancement of diffusely enlarged pancreas, homogeneous enhancement of focal enlargement/mass, absent/minimal peripancreatic fat infiltration or fluid collection, and multifocal main pancreatic duct narrowings were helpful for differentiating type 2 AIP from gallstone pancreatitis. During follow-up (median, 32.3 months), two patients (2/25, 8%) experienced relapse. In South Korea, type 2 AIP is not as rare as previously thought. Overall, the clinical profile of type 2 AIP was similar to that of Western countries. Type 2 AIP should be considered in young UC patients with acute pancreatitis of uncertain etiology. The most common clinical presentation of type 2 AIP was clinical acute pancreatitis (17/27, 63%). Like ours, a European study showed that the most common clinical presentation was acute pancreatitis (80%), followed by abdominal pain (11%), and obstructive jaundice (7%). Among patients with coexisting type 2 AIP and UC in the present study, type 2 AIP most occurred after the diagnosis of UC. The relapse rate of type 2 AIP is generally considered to be significantly lower than in type 1 AIP. In our study, the relapse rate of type 2 AIP was about 8%.

• #22 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. […] Clinically, autoimmune pancreatitis can manifest with symptoms related to biliary or pancreatic involvement. Patients can present with recurrent episodes of abdominal pain, with or without attacks of pancreatitis. Obstructive jaundice is a common presentation that can be accompanied by non-specific symptoms such as nausea, vomiting, loss of appetite, or weight loss, depending on the degree of biliary or pancreatic strictures and involvement. […] Autoimmune pancreatitis responds generally well to treatment with corticosteroids and has a favorable prognosis. A majority of cases exhibit remission with steroids, and the rate of complications is also reported to improve significantly with treatment.

• #23 Clinical Aspects of Autoimmune Pancreatitis in Sjogren’s Syndrome

  https://www.primescholars.com/articles/clinical-aspects-of-autoimmune-pancreatitis-in-sjogrens-syndrome-98499.html

  Nishimori I et al. presented the clinical pictures of the 54 AIP cases successfully treated by means of steroid therapy as the clinical features of autoimmune pancreatitis. […] The age of onset occurs 10 to 20 years later than that of chronic pancreatitis of other etiologies. Abdominal pain and jaundice of the cholestatic type were the two most common complaints and both were observed in one third of the two groups. Abdominal pain was less frequent and severe in autoimmune pancreatitis than in other types of chronic pancreatitis. […] Serum liver enzyme abnormality of a mild cholestatic type was observed in about 40-60% of the 54 AIP cases. Elevated levels of serum gamma-globulin was present in 43% of the 54 AIP cases, IgG in 62% and non-specific autoantibodies in 36%. […] Exocrine pancreatic insufficiency compatible with definite or probable chronic pancreatitis was found in all 10 patients studied by secretin test in the 54 AIP cases and in eight of the nine patients studied by secretin test in the 33 CP-SjS cases. Recovery of exocrine pancreatic function was reported after oral steroid therapy.

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

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

  Many of these symptoms also occur in pancreatic cancer, but Its important to realize that having this condition doesnt mean you have cancer. […] Chronic (long-term) inflammation of your pancreas can result in nausea, weight loss and chronic upper abdominal and back pain. Damage to the insulin-producing cells of the pancreas can lead to diabetes. […] Autoimmune pancreatitis often comes back (relapses). This is more common in people with Type 1. Relapses are likely in the first three years after diagnosis. A person with relapsing autoimmune pancreatitis is more likely to experience complications. […] With successful management, people with relapsing autoimmune pancreatitis can live active, healthy lives.

• #25 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. […] Symptoms of autoimmune pancreatitis often improve after a short course of prednisolone or prednisone. Many people respond quickly, even dramatically. Sometimes people get better without any treatment. […] The disease can relapse. For type 1 AIP, the disease relapses about 30% to 50% of the time. For type 2 AIP, it relapses less than 10% of the time. Cases of relapse require additional treatment, sometimes long term. […] Type 1 AIP often is associated with other organ involvement, including enlarged lymph nodes and salivary glands, scarring of the bile ducts, liver inflammation, and kidney disease. Although these signs may lessen or disappear completely with steroid therapy, your care team will continue to monitor you.

• #26 Long-term outcomes of autoimmune pancreatitis: a multicentre, international analysis | Gut

  https://gut.bmj.com/content/62/12/1771

  Autoimmune pancreatitis (AIP) is a treatable form of chronic pancreatitis that has been increasingly recognised over the last decade. […] The majority of patients with type 1 (99%) and type 2 (92%) AIP who were treated with steroids went into clinical remission. […] Relapses were more common in patients with type 1 (31%) versus type 2 AIP (9%, p0.001), especially those with IgG4-related sclerosing cholangitis (56% vs 26%, p0.001). […] Relapses typically occurred in the pancreas or biliary tree. […] AIP is a global disease which uniformly displays a high response to steroid treatment and tendency to relapse in the pancreas and biliary tree. […] Potential long-term sequelae include pancreatic duct stones and malignancy, however they were uncommon during the study period and require additional follow-up.

• #27

  https://link.springer.com/article/10.1007/s00535-022-01891-7

  It has been provided that 62.8% of patients with type 1 AIP are symptomatic in the 2016 Japanese nationwide survey; among these, 48.6% showed jaundice, 25.6% experienced abdominal pain, and 12% reported other symptoms involving extrapancreatic lesions. […] Among the asymptomatic cases of type 1 AIP, 57.8% exhibited abnormal imaging findings, 23.4% abnormal laboratory data, and 18.8% had new-onset or exacerbated diabetes mellitus. […] A rapid response to glucocorticoids is a primary feature of type 1 AIP, and the 2016 Japanese nationwide survey has reported that 98.6% of cases with type 1 AIP respond to steroid therapy. […] In spite of the high initial remission rates, it has been suggested that 15-60% of patients will experience relapse after cessation of steroid therapy or during weaning.

• #28 Long-term outcomes of autoimmune pancreatitis: a multicentre, international analysis | Gut

  https://gut.bmj.com/content/62/12/1771

  Disease relapses are common after steroid discontinuation, and typically occur in the pancreas and/or biliary tract. […] Pancreatic duct stones are relatively uncommon, but are seen more frequently in patients with at least one disease relapse. […] The occurrence of incident cancers following AIP diagnosis appears to be uncommon. […] The majority (74%) of subjects with type 1 AIP were initially treated with steroids, rather than surgical or conservative treatments, in comparison with type 2 subjects in which only 62% were treated with steroids (p=0.01). […] Of the 978 subjects with type 1 disease, a total of 302 (31%) subjects experienced at least one disease relapse during the study period, compared with 8 (9%, p0.001) subjects with type 2 AIP. […] Most relapses occurred in the biliary system or pancreas for type 1 AIP, while relapses in type 2 AIP were limited to the pancreas. […] The occurrence of pancreatic duct stones in this study is low with higher prevalence in those with at least one relapse. […] Additionally, we report the first systematic collection of malignancies in patients with AIP. Importantly, there were only five cases of pancreatic cancer in this study.

• #29 Autoimmune pancreatitis can develop into chronic pancreatitis | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-9-77

  Autoimmune pancreatitis (AIP) has been recognized as a distinct type of pancreatitis that is possibly caused by autoimmune mechanisms. AIP is characterized by high serum IgG4 and IgG4-positive plasma cell infiltration in affected pancreatic tissue. Acute phase AIP responds favorably to corticosteroid therapy and results in the amelioration of clinical findings. However, the long-term prognosis and outcome of AIP remain unclear. […] We have proposed a working hypothesis that AIP can develop into ordinary chronic pancreatitis resembling alcoholic pancreatitis over a long-term course based on several clinical findings, most notably frequent pancreatic stone formation. […] Approximately 40% of AIP patients experience pancreatic stone formation over a long-term course, for which a primary risk factor is narrowing of both Wirsungs and Santorinis ducts; and nearly 20% of AIP patients progress to confirmed chronic pancreatitis according to the revised Japanese Clinical Diagnostic Criteria, with independent risk factors being pancreatic head swelling and non-narrowing of the pancreatic body duct.

• #30 Autoimmune pancreatitis can develop into chronic pancreatitis | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-9-77

  We evaluated the long-term outcomes of patients with AIP and observed that some individuals experienced pancreatic calcification. […] We subsequently examined 42 AIP patients who had been followed for at least 12 months (median follow-up period: 54.5 months, range: 13111 months). Eight patients (19%) developed pancreatic calcification. […] These studies suggested that pancreatic calcification was closely associated with relapse and that AIP could transform into ordinary chronic pancreatitis after multiple recurrences. […] The frequency of pancreatic calcification in AIP ranges from 4% to 40%. […] The relapse rate of AIP has been estimated at 30% to 50%. […] We have postulated two mechanisms for pancreatic stone formation in AIP: 1) severe tissue injury attributed to the specific inflammatory disease processes; and 2) pancreatic juice stasis due to pancreatic duct narrowing.

• #31 Autoimmune pancreatitis can develop into chronic pancreatitis | Orphanet Journal of Rare Diseases | Full Text

  https://ojrd.biomedcentral.com/articles/10.1186/1750-1172-9-77

  Approximately 40% of patients with AIP experienced pancreatic stone formation over a long-term course. […] The long-term outcome of pancreatic function in AIP remains unclear, especially with respect to pancreatic stone formation. […] This cumulative study has clarified the following points: 1) pancreatic calcification in AIP is closely associated with disease recurrence; 2) advanced stage AIP might have earlier been included in ordinary chronic pancreatitis; 3) approximately 40% of patients with AIP experience pancreatic stone formation over a long-term course, for which a primary risk factor is narrowing of both Wirsungs and Santorinis ducts; and 4) nearly 20% of AIP patients progress to confirmed chronic pancreatitis according to the revised JCDC, with independent risk factors of pancreatic head swelling and MPD non-narrowing in the pancreatic body. Finally, AIP can lead to severe pancreatic stone formation and progress to confirmed chronic pancreatitis over a long-term period, which may be most presumably caused by disease recurrence and pancreatic juice stasis.

• #32

  https://link.springer.com/article/10.1007/s00535-022-01891-7

  The rate of patients with type 1 AIP to develop chronic pancreatitis are reported from 7 to 40%, with pancreatic head swelling and non-narrowing MPD reported to be risk factors. […] Understanding the relationship of type 1 AIP with pancreatic cancer is important because chronic pancreatitis has been reported to be a risk factors for pancreatic cancer. […] Among patients with type 1 AIP, it was also found that the K-ras mutation occurred not only in the pancreas but also in the bile duct and the gallbladder, frequently and significantly. […] They speculated that type 1 AIP may be a manifestation of paraneoplastic syndrome, but the definitive risk of malignancy remains unclear.

• #33 Prognosis and Long Term Outcomes of Autoimmune Pancreatitis | Pancreapedia

  https://pancreapedia.org/reviews/prognosis-and-long-term-outcomes-of-autoimmune-pancreatitis

  Autoimmune pancreatitis (AIP) is characterized by pancreatic swelling and irregular narrowing of the main pancreatic duct, which often mimic pancreatic cancer (1, 17, 18, 20). Most patients with type 1 AIP (referred to as AIP in this section) respond favorably to corticosteroid therapy, which results in the amelioration of symptomatic, radiographic, serologic, and pathologic findings. It is possible for patients to have a spontaneous recovery. However, during long-term follow-up, some patients with AIP are noted to progress to the advanced stage of pancreatic stone formation after recurrence, which may be similar to the findings of chronic pancreatitis (29, 30, 40). AIP is characterized by high serum IgG4 concentration, IgG4-positive staining plasma cell infiltration in affected pancreatic tissue, and a favorable response to corticosteroid therapy. Imaging analyses by ultrasonography (US), computed tomography (CT), and endoscopic retrograde cholangiopancreatography (ERCP) show sonolucent (i.e., hypoechoic) swelling and irregular narrowing of the main pancreatic duct, both of which are due to lymphoplasmacytic inflammation at the acute stage. Although most patients have a favorable response to corticosteroid therapy, some develop pancreatic atrophy and stone formation with irregular dilatation of the main pancreatic duct (MPD) (30, 40). These imaging findings mimic those of chronic pancreatitis, suggesting that in some cases AIP may progress into chronic pancreatitis. If this is the case, ordinary chronic pancreatitis could also include the advanced stage of AIP. This is supported by the observation that serum IgG4 remains elevated in over 60% of patients after clinical improvement (19). A long-term follow up study showed 16 of 73 (22%) AIP patients progressed to chronic pancreatitis that fulfilled the revised Japanese clinical diagnostic criteria for chronic pancreatitis in the chronic stage (29). However, other studies have indicated a lower prevalence of pancreatic stone formation during long term follow-up (41, 42). A recent multicenter, international analysis estimated that pancreatic stones occurred in only 7% of subjects with follow-up imaging permitting evaluation for stone disease (8). AIP is a chronic disease that can have a relapsing clinical course. Twenty-eight of the 84 patients (33%) experienced a total of 60 recurrences, including autoimmune pancreatitis (n=26 times), sclerosing cholangitis (n=18), lacrimal and salivary gland lesions (n=5), and retroperitoneal fibrosis (n=4). During clinical follow-up, the development of pancreatic stones was more frequent in the relapse group (14 patients, 50%) than in the non-relapse group (13 patients, 23%). Collectively, one-third of patients with AIP developed a pancreatic stone. Although spontaneous remission occurs in some patients with AIP, these patients are usually good candidates for corticosteroid therapy (9, 13, 16, 24, 26). Type 1 AIP is a chronic, relapsing disease. Although the acute inflammatory phase is very responsive to corticosteroid therapy, there are several potential long term complications that can develop. Endocrine and exocrine pancreatic dysfunction is more typical during the acute phase. They may resolve with corticosteroid therapy, but occur later when the pancreas has atrophied. Disease relapses are common and can develop in the pancreas, biliary tree, or other distant sites associated with IgG4-RD. Pancreatic duct stones can develop, and are more likely in those with relapsing disease. There is a theoretical increased risk for developing pancreatic cancer, but the actual risk is not fully understood. In contrast, in type 2 AIP disease relapse and other long term complications are uncommon.

• #34 Pancreatitis: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/8103-pancreatitis

  Over time, you may notice weight loss. […] Chronic pancreatitis is a long-term, progressive condition. It doesn’t go away and gets worse over time. […] Chronic pancreatitis will eventually do lasting damage to your pancreas, although it may take many years. […] Constant inflammation causes scarring of your pancreas tissues (fibrosis), which stops them from making enzymes and hormones. […] Repeat episodes of acute pancreatitis can lead to chronic pancreatitis. […] Chronic pancreatitis will also cause your pancreas to produce less of the hormones that regulate your blood sugar (glucose). […] Chronic pancreatitis is a slow-progressing disease. […] Pain management can become a challenge for some people, and it can also affect your mental health and quality of life.

• #35 Challenges for clinicians treating autoimmune pancreatitis: Current perspectives

  https://www.wjgnet.com/2307-8960/full/v11/i1/30.htm

  After induction of remission, the recurrence rate of type 1 AIP was higher than that of type 2 AIP (31% vs 9%), and the need for treatment maintenance was suggested owing to the high recurrence of type 1 AIP. […] In patients with AIP, the prevalence of endocrine and exocrine insufficiency at the time of AIP diagnosis is high. In a recent meta-analysis, the pooled estimate rate for the overall prevalence of DM in patients at the time of AIP (combined type 1 and type 2 AIP) diagnosis was 37%.

• #36

  https://www.nhs.uk/conditions/chronic-pancreatitis/

  The most common symptom of chronic pancreatitis is repeated episodes of severe pain in your tummy (abdomen). […] As the condition progresses, the painful episodes may become more frequent and severe. […] Other symptoms develop as the damage to the pancreas progresses and it becomes unable to produce digestive juices, which help to break down food. […] The pancreas usually only loses these functions many years after the first symptoms started. […] You may also experience weight loss, loss of appetite, yellowing of the skin and eyes (jaundice), symptoms of diabetes such as feeling very thirsty, needing to pee more often than usual and feeling very tired, ongoing nausea and sickness (vomiting).

• #37 Hardhik Gastro and Liver Hospital 4048573044, 6305939545 | Best gastro and hepatology clinic in NacharamHyderabadIndia| ravisankargastro.com

  https://ravisankargastro.com/autoimmune-pancreatitis.php

  Autoimmune pancreatitis can cause a variety of complications. […] AIP may affect the ability of your pancreas to make enough enzymes. Signs and symptoms may include diarrhea, weight loss, metabolic bone disease, and vitamin or mineral deficiency. […] Because the pancreas is the organ that produces insulin, damage to it may cause diabetes, and you may need treatment with oral medication or insulin.

• #38 Chronic pancreatitis – Guts UK

  https://gutscharity.org.uk/advice-and-information/conditions/chronic-pancreatitis/

  The major symptoms of chronic pancreatitis are: Pain, Nausea, Vomiting, Diarrhoea, Weight loss, Fatigue. […] Some people with chronic pancreatitis have only one or two of these symptoms. Symptoms of diabetes may happen late in the disease. […] The pain of chronic pancreatitis can range from mild to severe and is felt across the upper abdomen. Chronic pancreatitis causes upper abdominal pain (just below the ribs) that can range in intensity from mild to severe. Additionally, the pain could radiate to the back. […] The number of people with chronic pancreatitis who are diagnosed with diabetes increases over time. 25 people in 100 to 80 people in 100 will develop diabetes within 10 to 20 years. […] 8 in 10 people with chronic pancreatitis will develop pancreatic exocrine insufficiency (PEI) over time. This is poor absorption of food due to a lack of digestive enzymes resulting from the damaged pancreas being unable to produce enough.

• #39 Autoimmune pancreatitis | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/autoimmune-pancreatitis?content_id=CON-20369783

  Symptoms may include diarrhea, weight loss, metabolic bone disease, and vitamin or mineral deficiency. […] Symptoms of autoimmune pancreatitis often improve after a short course of prednisolone or prednisone. Many people respond quickly, even dramatically. Sometimes people get better without any treatment. […] For type 1 AIP, the disease relapses about 30% to 50% of the time. For type 2 AIP, it relapses less than 10% of the time. Cases of relapse require additional treatment, sometimes long term.

• #40 Pancreatitis: Symptoms, Causes, and Treatments

  https://www.healthline.com/health/pancreatitis

  The condition can also develop into chronic pancreatitis, especially if you smoke or drink alcohol heavily. […] Chronic pancreatitis is an inflammation of the pancreas that comes back consistently or occurs over a long period of time. […] People with chronic pancreatitis can have permanent damage to their pancreas and other complications. Scar tissue develops from this continuing inflammation. […] Pancreatitis can damage cells that produce insulin. Insulin is a hormone the pancreas releases to regulate the amount of sugar in your blood. When pancreatitis damages these cells, it leads to diabetes in about 45 percent of people with chronic pancreatitis. […] Long-term heavy alcohol use can cause pancreatitis in adults. Autoimmune and genetic diseases, such as cystic fibrosis, can also cause chronic pancreatitis in some people.

• #41 Pancreatitis: Symptoms, Causes, Diagnosis, Treatments, Tests

  https://www.webmd.com/digestive-disorders/digestive-diseases-pancreatitis

  Symptoms of acute pancreatitis include moderate to severe pain in the upper part of your belly that goes into your back. Eating may make it worse, especially foods high in fat. […] Symptoms of chronic pancreatitis are similar to those of the acute form. But you may also have constant pain in your upper belly that radiates to your back. This pain may be disabling. […] Diarrhea and weight loss because your pancreas isn’t releasing enough enzymes to break down food. […] Fatty, oily stools that smell especially bad and leave a film in the toilet.

• #42 Autoimmune Pancreatitis – National Pancreas Foundation

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

  Type 1 AIP often is associated with other organ involvement, including enlarged lymph nodes and salivary glands, scarring of the bile ducts, liver inflammation, and kidney disease. Although these signs may lessen or disappear completely with steroid therapy, your doctor will continue to monitor you.

• #43 Autoimmune pancreatitis symptoms, treatments & forums | PatientsLikeMe

  https://www.patientslikeme.com/conditions/autoimmune-pancreatitis

  11 autoimmune pancreatitis patients report moderate stress (73%). […] 1 autoimmune pancreatitis patient reports mild stress (6%). […] 1 autoimmune pancreatitis patient reports no stress (6%). […] Common symptom: Anxious mood. […] 3 autoimmune pancreatitis patients report severe anxious mood (10%). […] 14 autoimmune pancreatitis patients report moderate anxious mood (46%). […] 8 autoimmune pancreatitis patients report mild anxious mood (26%). […] 5 autoimmune pancreatitis patients report no anxious mood (16%). […] Common symptom: Depressed mood. […] 3 autoimmune pancreatitis patients report severe depressed mood (10%). […] 10 autoimmune pancreatitis patients report moderate depressed mood (33%). […] 11 autoimmune pancreatitis patients report mild depressed mood (36%). […] 6 autoimmune pancreatitis patients report no depressed mood (20%).

• #44 Autoimmune pancreatitis – Wikipedia

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

  AIP often completely resolves with steroid treatment. The failure to differentiate AIP from malignancy may lead to unnecessary pancreatic resection, and the characteristic lymphoplasmacytic infiltrate of AIP has been found in up to 23% of patients undergoing pancreatic resection for suspected malignancy who are ultimately found to have benign disease. […] Autoimmune pancreatitis responds dramatically to corticosteroid treatment. […] AIP does not affect long-term survival.

• #45 Autoimmune pancreatitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3364658/

  Autoimmune pancreatitis occurs in twice as many men as women. The initial presentation usually occurs between ages 50-60, but patients can also develop autoimmune pancreatitis as early as age 30 as well as late in life. […] Common symptoms of autoimmune pancreatitis include jaundice, weight loss, and mild abdominal pain. Severe abdominal pain or other symptoms of acute pancreatitis are unusual. However, autoimmune pancreatitis can also cause a wide variety of symptoms that tend to occur as a relapsing-remitting type of disease, with periods of symptoms alternating with periods of remission. […] The evaluation of the therapy effect includes the improvement of clinical signs and symptoms: 1) decreased jaundice, abdominal and back pain, 2) decreased pancreatic size on imaging, 3) decreased levels of pancreatic enzymes, hapatobiliary enzymes and total bilirubin, and 4) recovery of blood glucose and insulin level.

• #46 Treatment approaches in autoimmune pancreatitis (Review)

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

  Autoimmune pancreatitis (AIP) is a rare disease. The usual course of treatment for AIP is oral corticosteroid medication. Clinically, obstructive jaundice with or without a pancreatic mass is a common symptom of AIP, which is also defined histologically by a lymphoplasmacytic infiltration and fibrosis, and therapeutically by a notable reaction to steroids. IgG4+ plasma cell infiltration, lymphoplasmacytic sclerosing pancreatitis, pancreatic edema, pancreatic duct constriction and obliterative phlebitis are the characteristics of AIP-1. AIP-2 starts earlier than AIP-1, and is frequently aggravated by inflammatory bowel disease (IBD), especially ulcerative colitis. 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. After beginning steroid medication, symptoms and radiographic remission can be seen 2-4 weeks later. Relapses occur more frequently in patients with AIP-1 than in patients with AIP-2. The most notable risk factors for AIP relapse are deemed to be high IgG4 levels, jaundice and involvement of retroperitoneal fibrosis, chronic periaortitis, autoimmune hypophysitis, sclerosing cholangitis and Riedel’s thyroiditis. The relapse rate in AIP-1 is notably higher than that in AIP-2, which has generated a debate on how to effectively treat AIP-1 relapse.

• #47 Autoimmune pancreatitis | ABC Medical Center

  https://centromedicoabc.com/en/padecimientos/autoimmune-pancreatitis/

  Autoimmune pancreatitis is classified as: […] It generally has no symptoms, but when they appear, they can be similar in both types: Enlarged pancreas. Sore abdomen. Back pain. Lack of appetite. Extreme fatigue. Pale or floating stools. Jaundice. Dark urine. Nausea. Weight loss. Vomit. […] The treatment of autoimmune pancreatitis type 1 and 2 is very different, so it is essential to have an accurate diagnosis that allows them to be distinguished and treated promptly since failure to do so can trigger various complications such as the formation of pancreatic stones or calcifications, diabetes, narrowing of the bile ducts and insufficient enzyme production.

• #48 Chronic pancreatitis – Guts UK

  https://gutscharity.org.uk/advice-and-information/conditions/chronic-pancreatitis/

  The two main complications of chronic pancreatitis are diabetes and PEI. […] Diabetes related to pancreatitis is called Type 3c diabetes. It occurs because of damage to the pancreatic cells that produce insulin. […] PEI can cause maldigestion, weight loss, vitamin deficiencies and lead to malnutrition.

• #49 Final Diagnosis — Case 800

  https://path.upmc.edu/cases/case800/dx.html

  Type 1 autoimmune pancreatitis usually affects males over the age of 50, but the clinical presentation can vary. The most common symptoms are painless jaundice and abdominal pain. Additional symptoms can include weight loss, diabetes, and in rare cases severe abdominal pain and acute pancreatitis. Because this disease can be systemic, patients may also present with extra-pancreatic manifestations, such as renal insufficiency, and pulmonary infiltrates. […] The foundation treatment for type 1 autoimmune pancreatitis is corticosteroids. Steroid regimens usually begin with a week of prednisone 40 mg daily followed by tapering of the dose by 5 mg per week. Prior to and during treatment, a patient should undergo imaging and lab testing in order to monitor response. Steroid response consists of the pancreas returning to normal size and a normalizing of pancreatic function. Patients with discrete masses undergoing corticosteroid therapy should have complete resolution of the mass as determined by imaging. Carcinoma of the pancreas can have some response to steroids, but complete resolution of the mass will not occur. Patients who fail to respond to steroids should have a surgical biopsy.

• #50 Clinical Aspects of Autoimmune Pancreatitis in Sjogren’s Syndrome

  https://www.primescholars.com/articles/clinical-aspects-of-autoimmune-pancreatitis-in-sjogrens-syndrome-98499.html

  Oral steroid therapy (oral prednisolone 30-40 mg/day for 3-4 weeks) is effective in the relief of pain and jaundice, as well as in the reduction of the pancreatic enlargement and irregular narrowing of the pancreatic duct. However, contribution of steroid therapy to long-term prognosis requires further confirmation.

• #51 Autoimmune Pancreatitis in Two Adolescent Boys | Saade | International Journal of Clinical Pediatrics

  https://www.theijcp.org/index.php/ijcp/article/view/303/258

  Although AIP is rarely seen in children as compared to adults, and is usually underdiagnosed, the introduction of the ICDC helped raising the index of suspicion for AIP thus allowing earlier diagnosis possibly without the full spectrum of pancreatic manifestations. Patients with AIP can present with variable symptoms including recurrent episodes of epigastric pain with rarely signs of acute pancreatitis such as nausea, vomiting and weight loss, or symptoms of other associated diseases. Jaundice, acholia and choluria may be present in up to 80% of patients. Although jaundice is present in a high percentage of patients, our two patients did not present any jaundice, but only abdominal pain, nausea, or vomiting. […] Steroids help improve clinical symptoms in first place, then biological disorders in about 2 weeks and finally morphological anomalies of the pancreas. Response to steroids treatment is included in the ICDC and is considered an important criterion when it shows improvement or resolution of radiological features obtained at baseline. The recommended starting dose is 1 mg/kg/day followed by progressive tapering of the dose. Our first patient had a good control for ulcerative colitis with no recurrence of AIP features after steroid treatment. Our second patient presented resolution of signs and symptoms of AIP after receiving steroids.

• #52 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 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. […] Symptomatic patients — Treatment for AIP is indicated in patients with any one of the following: Pancreatic symptoms including obstructive jaundice, abdominal pain, or back pain.

• #53 Autoimmune pancreatitis: Management – UpToDate

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

  Autoimmune pancreatitis (AIP) is an uncommon but well-established form of pancreatic inflammation. […] If untreated, AIP can lead to pancreatic insufficiency, fibrosis, and other complications. […] 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. […] Symptomatic patients—Treatment for AIP is indicated in patients with any one of the following: Pancreatic symptoms including obstructive jaundice, abdominal pain, or back pain.

• #54 Autoimmune Pancreatitis: A Surgical Dilemma | Cirugía Española (English Edition)

  https://www.elsevier.es/en-revista-cirugia-espanola-english-edition–436-articulo-autoimmune-pancreatitis-a-surgical-dilemma-S217350771400458X

  The existence of prominent lymphoid aggregates and follicles in parenchyma and peripancreatic fat is another characteristic fact of AIP (100% for type 1 and 47% for type 2); however, it is also observed in approximately half of alcoholic chronic pancreatitis and obstructive chronic pancreatitis cases. […] The most frequent clinical manifestation of AIP, in particular type 1, is painless obstructive jaundice related to a pancreatic mass (68%84%). […] In many cases, a pancreatic head mass lesion and involvement of the distal biliary duct caused by AIP produce symptoms matching pancreatic cancer.

• #55 Autoimmune Pancreatitis | Padmavathi Gastro and Liver Hospital | Best liver transplant hospital in Hyderabad

  https://padmavathigastro.com/pancreas/autoimmune-pancreatitis.html

  Autoimmune pancreatitis (AIP) is difficult to diagnose. Often, it doesn’t cause any symptoms. When it does, its symptoms and signs are similar to those of pancreatic cancer. […] The most common sign of autoimmune pancreatitis, present in about 80 percent of people, is painless jaundice, caused by blocked bile ducts. AIP can also cause weight loss. Many people with autoimmune pancreatitis have masses in the pancreas and other organs, which can be misdiagnosed as cancer. […] Autoimmune pancreatitis often doesn’t cause any symptoms. See your doctor, however, if you experience unexplained weight loss, abdominal pain, jaundice, or other signs and symptoms that bother you.

• #56 An unusual case of autoimmune pancreatitis presenting as pancreatic mass and obstructive jaundice: a case report and review of the literature | Journal of Medical Case Reports | Full Text

  https://jmedicalcasereports.biomedcentral.com/articles/10.1186/1752-1947-5-253

  Autoimmune pancreatitis is a rare chronic inflammatory pancreatic disease that is increasingly being diagnosed worldwide. […] The clinical manifestations are protean. Symptoms include abdominal pain, obstructive jaundice, weight loss, steatorrhea and new-onset diabetes mellitus. […] The diagnosis of AIP is challenging, as this disorder closely mimics pancreatic cancer. […] AIP has been shown to be responsive to corticosteroid therapy. […] A thorough evaluation by experienced multi-disciplinary team is critical in diagnosing AIP, especially in young patients. Clinical suspicion of the entity is critical.

• #57 Autoimmune pancreatitis – Wikipedia

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

  AIP is characterized by the following features: Scleral Icterus (yellow eyes), jaundice (yellow skin) which is usually painless, usually without acute attacks of pancreatitis. Relatively mild symptoms, such as minimal weight loss or nausea. Increased serum levels of gamma globulins, immunoglobulin G (IgG) or IgG4. The presence of serum autoantibodies such as anti-nuclear antibody (ANA), anti-lactoferrin antibody, anti-carbonic anhydrase II antibody, and rheumatoid factor (RF). Contrast-enhanced CT demonstrates a diffusely enlarged (sausage-shaped) pancreas. Diffuse irregular narrowing of the main pancreatic duct, and stenosis of the intrapancreatic bile duct on endoscopic retrograde cholangiopancreatography (ERCP). Rare pancreatic calcification or cyst formation. Marked responsiveness to treatment with corticosteroids.

• #58 Autoimmune Pancreatitis in Two Adolescent Boys | Saade | International Journal of Clinical Pediatrics

  https://www.theijcp.org/index.php/ijcp/article/view/303/258

  In conclusion, AIP is a rare disease in children, and a high index of suspicion must be present to diagnose it and differentiate it from other conditions. Established criteria help confirming the diagnosis, keeping in mind that IgG4 antibodies are associated with type 1 and autoantibodies are not specific. The pancreas is usually enlarged on imaging studies and the main pancreatic duct is narrow. Steroid therapy is the main treatment and response to steroids is included in the diagnostic criteria. It is important to highlight that an accurate diagnosis should be made in order to treat the condition and to avoid unnecessary surgical procedures when AIP is mistaken for pancreatic cancer, especially in adult patients, where both conditions can present as a pancreatic mass.

• #59 Autoimmune pancreatitis

  https://ask-ahd.ahdubai.com/con-20369783

  Autoimmune pancreatitis is a relatively uncommon disease of the pancreas that may be mistaken for pancreatic cancer. Autoimmune pancreatitis (AIP) is a chronic inflammation that is thought to be caused by the body’s immune system attacking the pancreas and that responds to steroid therapy. Symptoms and signs of type 1 AIP are similar to those of pancreatic cancer. The most common sign of type 1 AIP, present in about 80% of people, is painless jaundice, caused by blocked bile ducts. Type 2 AIP can present with recurrent episodes of acute pancreatitis. Pain in the upper abdomen, a common symptom of pancreatic cancer, is frequently absent in autoimmune pancreatitis. Autoimmune pancreatitis often doesn’t cause any symptoms. See your doctor, however, if you experience unexplained weight loss, abdominal pain, jaundice, or other signs and symptoms that bother you. Autoimmune pancreatitis can cause a variety of complications. Symptoms of autoimmune pancreatitis often improve after a short course of prednisolone or prednisone. Many people respond quickly, even dramatically. However, even with these complications, people who are treated for autoimmune pancreatitis have a normal life expectancy. 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.

• #60 Causes of Pancreatitis | Columbia Surgery

  https://columbiasurgery.org/pancreas/causes-pancreatitis

  In approximately 5-6% of patients with chronic pancreatitis, the disease is caused by autoimmune inflammation (in which the immune system attacks the pancreas). Symptoms may be mild, but patients with autoimmune pancreatitits (AIP) tend to show elevated levels of immunoglobulin G4 (IgG4) and a high rate of pancreatic stone formation. Other indicators of autoimmune pancreatitis include narrowing of the main pancreatic duct, scarring of the pancreatic tissue, and infiltration with inflammatory cells. […] AIP can occur by itself or in association with other autoimmune diseases such as primary sclerosing cholangitis (PSC), primary biliary cirrhosis, retroperitoneal fibrosis, rheumatoid arthritis, sarcoidosis, and Sjgren’s syndrome.

• #61 Autoimmune Pancreatitis: A Surgical Dilemma | Cirugía Española (English Edition)

  https://www.elsevier.es/en-revista-cirugia-espanola-english-edition–436-articulo-autoimmune-pancreatitis-a-surgical-dilemma-S217350771400458X

  Autoimmune pancreatitis (AIP) is defined as a particular form of pancreatitis that often manifests as obstructive jaundice associated with a pancreatic mass or an obstructive bile duct lesion, and that has an excellent response to corticosteroid treatment. […] The most frequent manifestation is obstructive jaundice caused by a pancreatic mass (up to 59% of cases) or by enlargement of the common bile duct wall. […] It may also appear as single or recurrent acute pancreatitis or progress into chronic pancreatitis with exocrine and endocrine pancreatic calcification and failure. […] AIP shows well-defined histopathological changes in the pancreas that are easily differentiated from changes occurring in other types of pancreatitis (alcoholic or chronic obstructive). […] Storiform fibrosis, obliterative phlebitis, prominent lymphoid follicles and IgG4+ plasma cells are findings highly characteristic of type 1 AIP, although they are also found in lower proportions in type 2.

• #62 Autoimmune pancreatitis

  http://www.scielo.org.co/scielo.php?script=sci_arttext&pid=S0121-81232014000100008

  Autoimmune pancreatitis is a rare disease that presents with clinical, serologic, and histologic characteristics of a defined autoimmune process that usually responds to the use of steroids. […] Autoimmune pancreatitis presents with chronic abdominal pain accompanied by acute, chronic or recurrent pancreatitis. […] Up to 85% of patients present with a pancreatic mass that might be mistaken for carcinoma or lymphoma with stenosis pancreatic ducts. […] The diagnosis of autoimmune pancreatitis should always be considered in patients that present with symptoms that suggest cholangitis or inflammation of the pancreas, especially when there is history of autoimmune disease or recurrent episodes of pancreatitis. […] Even though it is a rare disease, its opportune diagnosis and treatment limit the progression of the disease, and can reduce the need of surgery and further complications.

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