Materiały źródłowe

• #1 Autoimmune Pancreatitis: From Pathogenesis to Treatment

  https://www.mdpi.com/1422-0067/23/20/12667

  Autoimmune pancreatitis (AIP) is a rare disease. […] The standard therapy for AIP is oral administration of corticosteroids. […] Rituximab (RTX) has also been proposed for induction of remission and maintenance therapy in relapsing AIP-1. […] In selected patients, immunomodulators such as azathioprine are used to maintain remission. […] The UEG has given a similar indication but also recommends treatment of symptomatic patients for subclinical conditions that may lead to severe or irreversible organ failure. […] According to the international consensus for the treatment of autoimmune pancreatitis mentioned above, steroids are the first-line agent to use in all patients with active AIP. […] In the case of contraindications to the use of steroids, rituximab is the only agent with recognized efficacy in inducing remission as a single agent, whereas other steroid-sparing therapies (e.g., thiopurines) are not very effective when used as monotherapy.

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

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

  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. […] 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.

• #3 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. Rituximab has also been recommended for recurrent AIP-1 in order to initiate remission and provide ongoing treatment. Immunomodulators such as azathioprine are used to keep certain patients in remission. Evaluation also takes into account a number of pharmacological alternatives, including biologic drugs like anti-tumor necrosis factor therapy, a safe and efficient second-line treatment for AIP-2 relapse or steroid dependence. Corticosteroids and immunosuppressants, which are poorly tolerated due to considerable side effects, are being replaced by other biologic drugs, which may offer a beneficial therapeutic alternative. […] Steroids are the first-line treatment for all patients with active AIP, according to the international consensus for the condition. Rituximab (RTX) is the only steroid-sparing therapy with proven efficacy in leading to remission as a single agent in cases of contraindication to steroids, while other steroid-sparing medicines such as thiopurines are ineffective when administered as monotherapy.

• #4 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 characterized by obstructive jaundice, acute pancreatitis, and focal pancreatic mass. […] Fortunately, all subtypes of AIP show dramatic response to steroid treatment. This review discusses the current perspectives on the diagnosis and management of AIP in clinical practice. […] The standard treatment for AIP is steroid therapy, which can successfully induce remission for both type 1 and type 2 AIP. […] 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. […] Steroid therapy is recommended as the first-line treatment unless there is a contraindication for the use of steroids for induction of remission.

• #5 Autoimmune Pancreatitis: From Pathogenesis to Treatment

  https://www.mdpi.com/1422-0067/23/20/12667

  Autoimmune pancreatitis (AIP) is a rare disease. […] The standard therapy for AIP is oral administration of corticosteroids. […] Rituximab (RTX) has also been proposed for induction of remission and maintenance therapy in relapsing AIP-1. […] In selected patients, immunomodulators such as azathioprine are used to maintain remission. […] The UEG has given a similar indication but also recommends treatment of symptomatic patients for subclinical conditions that may lead to severe or irreversible organ failure. […] According to the international consensus for the treatment of autoimmune pancreatitis mentioned above, steroids are the first-line agent to use in all patients with active AIP. […] In the case of contraindications to the use of steroids, rituximab is the only agent with recognized efficacy in inducing remission as a single agent, whereas other steroid-sparing therapies (e.g., thiopurines) are not very effective when used as monotherapy.

• #6 Autoimmune Pancreatitis: From Pathogenesis to Treatment

  https://www.mdpi.com/1422-0067/23/20/12667

  Some patients with AIP-1 may require maintenance therapy with low-dose glucocorticoids or immunosuppressive agents or rituximab after induction of remission. […] Maintenance therapy is recommended in patients with AIP-1 who have diffuse enlargement of the pancreas, delayed radiographic remission, or persistently high serum IgG4 (>2xUNL) after treatment, or more than 2 OOIs (>2) or association with proximal IgG4-SC before treatment. […] Suggested medications for maintenance therapy are low dose steroids (2.5–7.5 mg/day), immunomodulators, or rituximab. […] Glucocorticoids (GC) have pleiotropic effects on the immune system. […] According to UEG and SGF evidence-based recommendations, the initial posology of prednisone should be 0.6–0.8 mg/kg per day (typically 30–40 mg/die) for one month, evaluating response to treatment after 2–4 weeks.

• #7 Steroid Therapy and Steroid Response in Autoimmune Pancreatitis

  https://www.mdpi.com/1422-0067/21/1/257

  The major indication for steroid treatment is the presence of symptoms. The schedule for standard oral steroid treatment is as follows: an initial prednisolone dose of 0.4–0.6 mg/kg/day for 2–4 weeks, reduced by 5 mg/d every 1–2 weeks until 10–15 mg/day, and then gradually tapered to a maintenance dose of 2.5–5 mg/day over a period of 2–3 months. Maintenance treatment with low-dose steroids reduces but does not eliminate the risk of relapses. […] The current guidelines recommend low-dose (5 mg/day) maintenance steroid treatment (MST) for 2–3 years to reduce the relapse rate (<30%). [...] The use of immunosuppressants, such as immunomodulators, rituximab, and corticosteroids, in patients with occult hepatitis B virus infection should be carefully monitored by quantification of serum hepatitis B virus-DNA, as the virus can be reactivated by immunosuppressive agents.

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

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

  To induce remission, an initial dose of 0.6-1.0 mg/kg/day of prednisone, which is maintained for 2-4 wk and then gradually reduced is recommended. […] Maintenance treatment to prevent AIP recurrence is not recommended for type 2 AIP or for type 1 AIP with low pre-treatment disease activity. […] However, maintenance treatment with low-dose glucocorticoids or steroid-sparing agents may be helpful in some patients with type 1 AIP. […] There are rare cases of steroid non-response in patients with AIP. Rituximab is recommended as an alternative treatment in the setting of steroid-refractory AIP. […] In patients with AIP with obstructive jaundice, biliary stenting is usually performed along with biopsy through ERCP. […] In cases of biliary stent insertion, early biliary stent removal after diagnosis of AIP according to the guidelines should be considered owing to stent related complications such as migration or stent dysfunction. […] In patients with recurrent AIP, steroids and steroid-sparing agents may be used.

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

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

  How Is Pancreatitis Treated? […] Treatment for acute pancreatitis […] If you have an attack of acute pancreatitis, you may receive strong drugs for pain. You may have to have your stomach drained through a tube placed through your nose. If the attack is prolonged, you may be fed and hydrated intravenously (through a vein). […] Treatment for chronic pancreatitis […] If you have this type, your doctor will focus on treating pain while guarding against possible addiction to prescription painkillers. They’ll also watch for complications that affect digestion. You may be placed on a pancreatic enzyme replacement therapy to restore the digestive tract’s ability to digest nutrients; this will also likely reduce the frequency of new attacks. […] You might need: […] Pancreatic enzymes to help your body get enough nutrients from your food.

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

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

  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. […] 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.

• #11 Autoimmune Pancreatitis – National Pancreas Foundation

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

  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. […] Treatments for autoimmune pancreatitis, such as long-term steroid use, also can cause complications. However, even with these complications, people who are treated for autoimmune pancreatitis have a normal life expectancy. […] 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. […] To help reduce the serious side effects associated with extended steroid use, doctors often add drugs that suppress or modify the immune system, sometimes called steroid-sparing medications, to the treatment. You may be able to stop taking steroids altogether depending on your response to steroid-sparing medication. […] Immunosuppressants and immunomodulators include mycophenolate (CellCept), mercaptopurine (Purinethol, Purixan), azathioprine (Imuran, Azasan) and rituximab (Rituxan). In general, they have been used in small sample groups and long-term benefits are still being studied.

• #12 Standard steroid treatment for autoimmune pancreatitis | Gut

  https://gut.bmj.com/content/58/11/1504

  Objective: To establish an appropriate steroid treatment regimen for autoimmune pancreatitis (AIP). […] Results: Of 563 patients with AIP, 459 (82%) received steroid treatment. The remission rate of steroid-treated AIP was 98%, which was significantly higher than that of patients without steroid treatment (74%, 77/104; p0.001). […] The major indication for steroid treatment in AIP is the presence of symptoms. An initial prednisolone dose of 0.6 mg/kg/day, is recommend, which is then reduced to a maintenance dose over a period of 36 months. Maintenance treatment with low-dose steroid reduces but does not eliminate relapses.

• #13 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. […] Retreatment with steroids remained effective at inducing remission with or without alternative treatment, such as azathioprine. […] AIP is a global disease which uniformly displays a high response to steroid treatment and tendency to relapse in the pancreas and biliary tree. […] Since disease relapses are common, additional studies are needed to compare different treatment strategies for maintaining disease remission. […] Although most subjects with jaundice required biliary intervention prior to steroid therapy, the need for diabetes treatment was unexpectedly low.

• #14 Autoimmune Pancreatitis: From Pathogenesis to Treatment

  https://www.mdpi.com/1422-0067/23/20/12667

  Some patients with AIP-1 may require maintenance therapy with low-dose glucocorticoids or immunosuppressive agents or rituximab after induction of remission. […] Maintenance therapy is recommended in patients with AIP-1 who have diffuse enlargement of the pancreas, delayed radiographic remission, or persistently high serum IgG4 (>2xUNL) after treatment, or more than 2 OOIs (>2) or association with proximal IgG4-SC before treatment. […] Suggested medications for maintenance therapy are low dose steroids (2.5–7.5 mg/day), immunomodulators, or rituximab. […] Glucocorticoids (GC) have pleiotropic effects on the immune system. […] According to UEG and SGF evidence-based recommendations, the initial posology of prednisone should be 0.6–0.8 mg/kg per day (typically 30–40 mg/die) for one month, evaluating response to treatment after 2–4 weeks.

• #15 Steroid Therapy and Steroid Response in Autoimmune Pancreatitis

  https://www.mdpi.com/1422-0067/21/1/257

  The major indication for steroid treatment is the presence of symptoms. The schedule for standard oral steroid treatment is as follows: an initial prednisolone dose of 0.4–0.6 mg/kg/day for 2–4 weeks, reduced by 5 mg/d every 1–2 weeks until 10–15 mg/day, and then gradually tapered to a maintenance dose of 2.5–5 mg/day over a period of 2–3 months. Maintenance treatment with low-dose steroids reduces but does not eliminate the risk of relapses. […] The current guidelines recommend low-dose (5 mg/day) maintenance steroid treatment (MST) for 2–3 years to reduce the relapse rate (<30%). [...] The use of immunosuppressants, such as immunomodulators, rituximab, and corticosteroids, in patients with occult hepatitis B virus infection should be carefully monitored by quantification of serum hepatitis B virus-DNA, as the virus can be reactivated by immunosuppressive agents.

• #16 Autoimmune Pancreatitis: From Pathogenesis to Treatment

  https://www.mdpi.com/1422-0067/23/20/12667

  Some patients with AIP-1 may require maintenance therapy with low-dose glucocorticoids or immunosuppressive agents or rituximab after induction of remission. […] Maintenance therapy is recommended in patients with AIP-1 who have diffuse enlargement of the pancreas, delayed radiographic remission, or persistently high serum IgG4 (>2xUNL) after treatment, or more than 2 OOIs (>2) or association with proximal IgG4-SC before treatment. […] Suggested medications for maintenance therapy are low dose steroids (2.5–7.5 mg/day), immunomodulators, or rituximab. […] Glucocorticoids (GC) have pleiotropic effects on the immune system. […] According to UEG and SGF evidence-based recommendations, the initial posology of prednisone should be 0.6–0.8 mg/kg per day (typically 30–40 mg/die) for one month, evaluating response to treatment after 2–4 weeks.

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

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

  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. […] 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.

• #18 Autoimmune pancreatitis | UM Health-Sparrow

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

  Immunosuppressants and immunomodulators. 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. To help reduce the serious side effects associated with extended steroid use, medicines that suppress or modify the immune system may be added to the treatment. These are sometimes called steroid-sparing medicines. You may be able to stop taking steroids altogether depending on your response to steroid-sparing medicine. […] Monitoring of other organ involvement. 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.

• #19 Autoimmune Pancreatitis – National Pancreas Foundation

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

  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. […] Treatments for autoimmune pancreatitis, such as long-term steroid use, also can cause complications. However, even with these complications, people who are treated for autoimmune pancreatitis have a normal life expectancy. […] 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. […] To help reduce the serious side effects associated with extended steroid use, doctors often add drugs that suppress or modify the immune system, sometimes called steroid-sparing medications, to the treatment. You may be able to stop taking steroids altogether depending on your response to steroid-sparing medication. […] Immunosuppressants and immunomodulators include mycophenolate (CellCept), mercaptopurine (Purinethol, Purixan), azathioprine (Imuran, Azasan) and rituximab (Rituxan). In general, they have been used in small sample groups and long-term benefits are still being studied.

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

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

  To help reduce the serious side effects associated with extended steroid use, medicines that suppress or modify the immune system may be added to the treatment. These are sometimes called steroid-sparing medicines. You may be able to stop taking steroids altogether depending on your response to steroid-sparing medicine. […] Immunosuppressants and immunomodulators include mycophenolate (CellCept), mercaptopurine (Purinethol, Purixan), azathioprine (Imuran, Azasan) and rituximab (Rituxan). In general, they have been used in small sample groups and long-term benefits are still being studied. […] 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.

• #21 Autoimmune Pancreatitis: From Pathogenesis to Treatment

  https://www.mdpi.com/1422-0067/23/20/12667

  In cases of refractory patients, a steroid mini-pulse treatment (2 courses of methyl-prednisolone 500 mg × 3 days with 4 days intervals) may be effective. […] Although remission is reached easily in most cases of AIP after corticosteroid induction treatment, AIP relapse after or during initial treatment is common and represents a challenging problem in clinical practice. […] To avoid long-term side effects of glucocorticoid therapy, conventional glucocorticoid-sparing agents, including azathioprine, 6-mercaptopurine, mycophenolate mofetil, cyclosporine A, tacrolimus, methotrexate, cyclophosphamide, may be considered. […] Rituximab (RTX) is a monoclonal antibody directed against the B-cell-specific antigen CD20 that acts as an efficient depleter of B cells in peripheral blood. […] According to European guidelines, RTX should be considered in patients with AIP-1 if they are resistant to or cannot tolerate high-dose GC or have not responded to immunomodulatory therapies (IM).

• #22 Maintenance therapy in autoimmune pancreatitis: a weak light into the darkness

  https://atm.amegroups.org/article/view/15862/html

  The study by Masamune et al. suggests long-term (3 years) low-dosage steroids keep in remission 76.7% of AIP patients. AZA at dosage of 2 mg/kg/day seems to have similar percentage of sustained remission at 3 years (75%) in two small retrospective studies. RTX administered in 2 doses (1.000 mg) at time 0 and 15 days seems to be effective in IgG4-related diseases both to induce and to maintain remission. […] Long-term low-dosage steroids, AZA or RTX are the possible choices to maintain remission high risk for disease relapse patients. Long-term low-dosage steroids approach (prednisone 5 mg/day) is probably the most largely applicable, safe, cheap and the only evidence-based treatment, even with the limitations previously stressed. RTX seems to be the most effective drug in maintaining remission but with significant limitations, especially old age and history of cancer. […] In conclusion, maintenance therapy in AIP should be tailored on single patient, considering age, personal history, comorbidities, risk of cancer, risk of disease relapse, and patients preference for care.

• #23 Autoimmune Pancreatitis – National Pancreas Foundation

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

  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. […] Treatments for autoimmune pancreatitis, such as long-term steroid use, also can cause complications. However, even with these complications, people who are treated for autoimmune pancreatitis have a normal life expectancy. […] 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. […] To help reduce the serious side effects associated with extended steroid use, doctors often add drugs that suppress or modify the immune system, sometimes called steroid-sparing medications, to the treatment. You may be able to stop taking steroids altogether depending on your response to steroid-sparing medication. […] Immunosuppressants and immunomodulators include mycophenolate (CellCept), mercaptopurine (Purinethol, Purixan), azathioprine (Imuran, Azasan) and rituximab (Rituxan). In general, they have been used in small sample groups and long-term benefits are still being studied.

• #24 Autoimmune Pancreatitis: From Pathogenesis to Treatment

  https://www.mdpi.com/1422-0067/23/20/12667

  In cases of refractory patients, a steroid mini-pulse treatment (2 courses of methyl-prednisolone 500 mg × 3 days with 4 days intervals) may be effective. […] Although remission is reached easily in most cases of AIP after corticosteroid induction treatment, AIP relapse after or during initial treatment is common and represents a challenging problem in clinical practice. […] To avoid long-term side effects of glucocorticoid therapy, conventional glucocorticoid-sparing agents, including azathioprine, 6-mercaptopurine, mycophenolate mofetil, cyclosporine A, tacrolimus, methotrexate, cyclophosphamide, may be considered. […] Rituximab (RTX) is a monoclonal antibody directed against the B-cell-specific antigen CD20 that acts as an efficient depleter of B cells in peripheral blood. […] According to European guidelines, RTX should be considered in patients with AIP-1 if they are resistant to or cannot tolerate high-dose GC or have not responded to immunomodulatory therapies (IM).

• #25 Autoimmune Pancreatitis: Clinical Presentation and Therapy | IntechOpen

  https://www.intechopen.com/chapters/65032

  Autoimmune pancreatitis is a relatively recently identified entity. […] However, all types of AIP respond quickly to steroid treatment with a complete recovery. […] The widely accepted dose of prednisone is relatively low, about 0.50-0.6 mg/kg/day. Others initiate the treatment with 40 mg prednisone/day. After 3-4 weeks with this treatment, the steroid dose is tapered, reducing it 5 mg/day every 2 weeks. Finally, the treatment can be stopped after the complete morphological recovery demonstrated by CT scan or magnetic resonance. […] The recurrent disease also responds to steroid treatment. However, in case of recurrence, it is advisable to initiate a longer treatment with some steroid-sparing agent, azathioprine (1.0-1.5 mg/kg/day) or mycophenolate (2 to 3 g/day) for several years. […] The effect of steroids is uniformly excellent. It means that if steroids fail to induce remission, one must have serious doubts in the diagnosis, whatever was the basis to establish it. […] In addition, pancreatic resection has surgical morbi-mortality and late metabolic consequences, which are hardly justifiable in a benign medically treatable disease.

• #26 Autoimmune Pancreatitis: From Pathogenesis to Treatment

  https://www.mdpi.com/1422-0067/23/20/12667

  In cases of refractory patients, a steroid mini-pulse treatment (2 courses of methyl-prednisolone 500 mg × 3 days with 4 days intervals) may be effective. […] Although remission is reached easily in most cases of AIP after corticosteroid induction treatment, AIP relapse after or during initial treatment is common and represents a challenging problem in clinical practice. […] To avoid long-term side effects of glucocorticoid therapy, conventional glucocorticoid-sparing agents, including azathioprine, 6-mercaptopurine, mycophenolate mofetil, cyclosporine A, tacrolimus, methotrexate, cyclophosphamide, may be considered. […] Rituximab (RTX) is a monoclonal antibody directed against the B-cell-specific antigen CD20 that acts as an efficient depleter of B cells in peripheral blood. […] According to European guidelines, RTX should be considered in patients with AIP-1 if they are resistant to or cannot tolerate high-dose GC or have not responded to immunomodulatory therapies (IM).

• #27 Treatment approaches in autoimmune pancreatitis (Review)

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

  Low dose steroids (2.5-7.5 mg/day), immunomodulators or RTX are recommended drugs for maintenance therapy. Japanese doctors advise the use of low-dose GC maintenance therapy for 3 years, while the duration of maintenance therapy is still debatable. Maintenance therapy should be scheduled to terminate in instances of radiographic and serological improvement. […] Conventional GC-sparing drugs, such as azathioprine (AZA), 6-mercaptopurine, mycophenolate mofetil (MMF), cyclosporine A, tacrolimus, methotrexate and cyclophosphamide, may be taken into consideration to prevent long-term side effects of GC therapy. […] According to European recommendations, patients with AIP-1 who are resistant to or cannot tolerate high-dose GC, or have not responded to immunomodulatory therapy (IM), should be given the option of RTX. This monoclonal antibody may be used as an alternate GC medication for induction therapy in patients with confirmed AIP-1. […] Biological therapy, such as anti-tumor necrosis factor (TNF) therapy, is a well-tolerated and effective second-line therapeutic option for AIP-2 relapse or steroid dependency.

• #28 Autoimmune Pancreatitis: From Pathogenesis to Treatment

  https://www.mdpi.com/1422-0067/23/20/12667

  In cases of refractory patients, a steroid mini-pulse treatment (2 courses of methyl-prednisolone 500 mg × 3 days with 4 days intervals) may be effective. […] Although remission is reached easily in most cases of AIP after corticosteroid induction treatment, AIP relapse after or during initial treatment is common and represents a challenging problem in clinical practice. […] To avoid long-term side effects of glucocorticoid therapy, conventional glucocorticoid-sparing agents, including azathioprine, 6-mercaptopurine, mycophenolate mofetil, cyclosporine A, tacrolimus, methotrexate, cyclophosphamide, may be considered. […] Rituximab (RTX) is a monoclonal antibody directed against the B-cell-specific antigen CD20 that acts as an efficient depleter of B cells in peripheral blood. […] According to European guidelines, RTX should be considered in patients with AIP-1 if they are resistant to or cannot tolerate high-dose GC or have not responded to immunomodulatory therapies (IM).

• #29 Autoimmune Pancreatitis: From Pathogenesis to Treatment

  https://www.mdpi.com/1422-0067/23/20/12667

  In cases of refractory patients, a steroid mini-pulse treatment (2 courses of methyl-prednisolone 500 mg × 3 days with 4 days intervals) may be effective. […] Although remission is reached easily in most cases of AIP after corticosteroid induction treatment, AIP relapse after or during initial treatment is common and represents a challenging problem in clinical practice. […] To avoid long-term side effects of glucocorticoid therapy, conventional glucocorticoid-sparing agents, including azathioprine, 6-mercaptopurine, mycophenolate mofetil, cyclosporine A, tacrolimus, methotrexate, cyclophosphamide, may be considered. […] Rituximab (RTX) is a monoclonal antibody directed against the B-cell-specific antigen CD20 that acts as an efficient depleter of B cells in peripheral blood. […] According to European guidelines, RTX should be considered in patients with AIP-1 if they are resistant to or cannot tolerate high-dose GC or have not responded to immunomodulatory therapies (IM).

• #30 Immunomodulators and Rituximab in the Management of Autoimmune Pancreatitis | Pancreapedia

  https://pancreapedia.org/reviews/immunomodulators-and-rituximab-in-management-of-autoimmune-pancreatitis

  Approximately half the patients with type 1 AIP develop disease relapse within the first three years following AIP diagnosis. […] Relapses can be treated with one of four strategies, i.) tapered high-dose steroids without maintenance treatment, ii.) tapered high-dose steroids with maintenance low-dose steroids iii) tapered high-dose steroids with a maintenance steroid-sparing immunomodulator, or iv.) rituximab monotherapy. […] A small portion of patients are unable to tolerate induction treatment with high-dose steroids due short-term severe adverse effects (e.g. severe hyperglycemia or emotional/mental instability). These subsets of difficult to treat patients are most likely to benefit from steroid-sparing immunomodulators or rituximab. […] Immunomodulators were initially considered as a steroid-sparing alternative to long-term steroid use for maintaining disease remission.

• #31 Maintenance therapy in autoimmune pancreatitis: a weak light into the darkness

  https://atm.amegroups.org/article/view/15862/html

  The study by Masamune et al. suggests long-term (3 years) low-dosage steroids keep in remission 76.7% of AIP patients. AZA at dosage of 2 mg/kg/day seems to have similar percentage of sustained remission at 3 years (75%) in two small retrospective studies. RTX administered in 2 doses (1.000 mg) at time 0 and 15 days seems to be effective in IgG4-related diseases both to induce and to maintain remission. […] Long-term low-dosage steroids, AZA or RTX are the possible choices to maintain remission high risk for disease relapse patients. Long-term low-dosage steroids approach (prednisone 5 mg/day) is probably the most largely applicable, safe, cheap and the only evidence-based treatment, even with the limitations previously stressed. RTX seems to be the most effective drug in maintaining remission but with significant limitations, especially old age and history of cancer. […] In conclusion, maintenance therapy in AIP should be tailored on single patient, considering age, personal history, comorbidities, risk of cancer, risk of disease relapse, and patients preference for care.

• #32 Autoimmune Pancreatitis: From Pathogenesis to Treatment

  https://www.mdpi.com/1422-0067/23/20/12667

  In cases of refractory patients, a steroid mini-pulse treatment (2 courses of methyl-prednisolone 500 mg × 3 days with 4 days intervals) may be effective. […] Although remission is reached easily in most cases of AIP after corticosteroid induction treatment, AIP relapse after or during initial treatment is common and represents a challenging problem in clinical practice. […] To avoid long-term side effects of glucocorticoid therapy, conventional glucocorticoid-sparing agents, including azathioprine, 6-mercaptopurine, mycophenolate mofetil, cyclosporine A, tacrolimus, methotrexate, cyclophosphamide, may be considered. […] Rituximab (RTX) is a monoclonal antibody directed against the B-cell-specific antigen CD20 that acts as an efficient depleter of B cells in peripheral blood. […] According to European guidelines, RTX should be considered in patients with AIP-1 if they are resistant to or cannot tolerate high-dose GC or have not responded to immunomodulatory therapies (IM).

• #33 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. Rituximab has also been recommended for recurrent AIP-1 in order to initiate remission and provide ongoing treatment. Immunomodulators such as azathioprine are used to keep certain patients in remission. Evaluation also takes into account a number of pharmacological alternatives, including biologic drugs like anti-tumor necrosis factor therapy, a safe and efficient second-line treatment for AIP-2 relapse or steroid dependence. Corticosteroids and immunosuppressants, which are poorly tolerated due to considerable side effects, are being replaced by other biologic drugs, which may offer a beneficial therapeutic alternative. […] Steroids are the first-line treatment for all patients with active AIP, according to the international consensus for the condition. Rituximab (RTX) is the only steroid-sparing therapy with proven efficacy in leading to remission as a single agent in cases of contraindication to steroids, while other steroid-sparing medicines such as thiopurines are ineffective when administered as monotherapy.

• #34 Autoimmune Pancreatitis: From Pathogenesis to Treatment

  https://www.mdpi.com/1422-0067/23/20/12667

  Autoimmune pancreatitis (AIP) is a rare disease. […] The standard therapy for AIP is oral administration of corticosteroids. […] Rituximab (RTX) has also been proposed for induction of remission and maintenance therapy in relapsing AIP-1. […] In selected patients, immunomodulators such as azathioprine are used to maintain remission. […] The UEG has given a similar indication but also recommends treatment of symptomatic patients for subclinical conditions that may lead to severe or irreversible organ failure. […] According to the international consensus for the treatment of autoimmune pancreatitis mentioned above, steroids are the first-line agent to use in all patients with active AIP. […] In the case of contraindications to the use of steroids, rituximab is the only agent with recognized efficacy in inducing remission as a single agent, whereas other steroid-sparing therapies (e.g., thiopurines) are not very effective when used as monotherapy.

• #35 Autoimmune Pancreatitis: From Pathogenesis to Treatment

  https://www.mdpi.com/1422-0067/23/20/12667

  In cases of refractory patients, a steroid mini-pulse treatment (2 courses of methyl-prednisolone 500 mg × 3 days with 4 days intervals) may be effective. […] Although remission is reached easily in most cases of AIP after corticosteroid induction treatment, AIP relapse after or during initial treatment is common and represents a challenging problem in clinical practice. […] To avoid long-term side effects of glucocorticoid therapy, conventional glucocorticoid-sparing agents, including azathioprine, 6-mercaptopurine, mycophenolate mofetil, cyclosporine A, tacrolimus, methotrexate, cyclophosphamide, may be considered. […] Rituximab (RTX) is a monoclonal antibody directed against the B-cell-specific antigen CD20 that acts as an efficient depleter of B cells in peripheral blood. […] According to European guidelines, RTX should be considered in patients with AIP-1 if they are resistant to or cannot tolerate high-dose GC or have not responded to immunomodulatory therapies (IM).

• #36 Autoimmune Pancreatitis: From Pathogenesis to Treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9604056/

  To avoid long-term side effects of glucocorticoid therapy, conventional glucocorticoid-sparing agents, including azathioprine, 6-mercaptopurine, mycophenolate mofetil, cyclosporine A, tacrolimus, methotrexate, cyclophosphamide, may be considered. […] Rituximab (RTX) is a monoclonal antibody directed against the B-cell-specific antigen CD20 that acts as an efficient depleter of B cells in peripheral blood. […] Rituximab has also been shown to be an excellent therapy for patients at high risk of relapse, such as those with multiorgan involvement, prior relapse, and failure of disease-modifying antirheumatic drug (DMARD) therapy. […] Although not among the therapies recommended in European and international guidelines, cases of patients with AIP treated with anti-tumor necrosis factor drugs (ANTI-TNF) have been described in the literature. […] Therefore, new therapeutic targets can be developed in patients with AIP and IgG4-RD.

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

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

  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. […] 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.

• #38 Autoimmune pancreatitis | UM Health-Sparrow

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

  Immunosuppressants and immunomodulators. 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. To help reduce the serious side effects associated with extended steroid use, medicines that suppress or modify the immune system may be added to the treatment. These are sometimes called steroid-sparing medicines. You may be able to stop taking steroids altogether depending on your response to steroid-sparing medicine. […] Monitoring of other organ involvement. 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.

• #39 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

  To prevent relapse, long-term maintenance therapy (approximately three years) with low-dose prednisolone can be applied. A dose of 5 mg/day or more may be preferred over a dose of 2.5 mg/day to prevent relapse. For relapsed AIP, the re-administration of a high dose of glucocorticoids with a slow steroid taper is effective. In patients with multiple relapses or severe steroid intolerance, steroid-sparing agents can be considered, including immunomodulators or rituximab.

• #40 Immunomodulators and Rituximab in the Management of Autoimmune Pancreatitis | Pancreapedia

  https://pancreapedia.org/reviews/immunomodulators-and-rituximab-in-management-of-autoimmune-pancreatitis

  Approximately half the patients with type 1 AIP develop disease relapse within the first three years following AIP diagnosis. […] Relapses can be treated with one of four strategies, i.) tapered high-dose steroids without maintenance treatment, ii.) tapered high-dose steroids with maintenance low-dose steroids iii) tapered high-dose steroids with a maintenance steroid-sparing immunomodulator, or iv.) rituximab monotherapy. […] A small portion of patients are unable to tolerate induction treatment with high-dose steroids due short-term severe adverse effects (e.g. severe hyperglycemia or emotional/mental instability). These subsets of difficult to treat patients are most likely to benefit from steroid-sparing immunomodulators or rituximab. […] Immunomodulators were initially considered as a steroid-sparing alternative to long-term steroid use for maintaining disease remission.

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

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

  The remission rate of treating patients following disease relapse remained high. […] Steroids were the most commonly used treatment for managing disease relapse in type 1 AIP, and inducing remission was successful in 201/210 (95%) of subjects. […] The addition of azathioprine was used for 68 subjects with successful induction in 56 (85%). […] Pancreatic duct stones were uncommonly seen occurring in 46/659 (7%) subjects with follow-up imaging permitting evaluation for stone disease. […] The occurrence of pancreatic duct stones in this study is low with higher prevalence in those with at least one relapse.

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

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

  The remission rate of treating patients following disease relapse remained high. […] Steroids were the most commonly used treatment for managing disease relapse in type 1 AIP, and inducing remission was successful in 201/210 (95%) of subjects. […] The addition of azathioprine was used for 68 subjects with successful induction in 56 (85%). […] Pancreatic duct stones were uncommonly seen occurring in 46/659 (7%) subjects with follow-up imaging permitting evaluation for stone disease. […] The occurrence of pancreatic duct stones in this study is low with higher prevalence in those with at least one relapse.

• #43 Autoimmune Pancreatitis: From Pathogenesis to Treatment

  https://www.mdpi.com/1422-0067/23/20/12667

  In cases of refractory patients, a steroid mini-pulse treatment (2 courses of methyl-prednisolone 500 mg × 3 days with 4 days intervals) may be effective. […] Although remission is reached easily in most cases of AIP after corticosteroid induction treatment, AIP relapse after or during initial treatment is common and represents a challenging problem in clinical practice. […] To avoid long-term side effects of glucocorticoid therapy, conventional glucocorticoid-sparing agents, including azathioprine, 6-mercaptopurine, mycophenolate mofetil, cyclosporine A, tacrolimus, methotrexate, cyclophosphamide, may be considered. […] Rituximab (RTX) is a monoclonal antibody directed against the B-cell-specific antigen CD20 that acts as an efficient depleter of B cells in peripheral blood. […] According to European guidelines, RTX should be considered in patients with AIP-1 if they are resistant to or cannot tolerate high-dose GC or have not responded to immunomodulatory therapies (IM).

• #44 Efficacy of Steroid Pulse Therapy for Autoimmune Pancreatitis Type 1: A Retrospective Study | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0138604

  Our study indicated no significant difference in the effect on disease activity between steroid pulse therapy and oral steroid therapy. However, steroid pulse therapy was more effective than oral steroid therapy in patients with diffuse pancreatic swelling, and these patients may be suitable candidates for steroid pulse therapy. […] Within these limitations, we conclude that steroid pulse therapy for AIP does not show a stronger effect than oral steroid therapy for prevention of relapse or on long-term prognosis, but pulse therapy may be an effective treatment for patients with diffuse swelling of the pancreas.

• #45 Treatment approaches in autoimmune pancreatitis (Review)

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

  Low dose steroids (2.5-7.5 mg/day), immunomodulators or RTX are recommended drugs for maintenance therapy. Japanese doctors advise the use of low-dose GC maintenance therapy for 3 years, while the duration of maintenance therapy is still debatable. Maintenance therapy should be scheduled to terminate in instances of radiographic and serological improvement. […] Conventional GC-sparing drugs, such as azathioprine (AZA), 6-mercaptopurine, mycophenolate mofetil (MMF), cyclosporine A, tacrolimus, methotrexate and cyclophosphamide, may be taken into consideration to prevent long-term side effects of GC therapy. […] According to European recommendations, patients with AIP-1 who are resistant to or cannot tolerate high-dose GC, or have not responded to immunomodulatory therapy (IM), should be given the option of RTX. This monoclonal antibody may be used as an alternate GC medication for induction therapy in patients with confirmed AIP-1. […] Biological therapy, such as anti-tumor necrosis factor (TNF) therapy, is a well-tolerated and effective second-line therapeutic option for AIP-2 relapse or steroid dependency.

• #46 Autoimmune Pancreatitis: From Pathogenesis to Treatment

  https://www.mdpi.com/1422-0067/23/20/12667

  Although not among the therapies recommended in European and international guidelines, cases of patients with AIP treated with anti-tumor necrosis factor drugs (ANTI-TNF) have been described in the literature. […] Therefore, new therapeutic targets can be developed in patients with AIP and IgG4-RD.

• #47 Autoimmune Pancreatitis: From Pathogenesis to Treatment

  https://www.mdpi.com/1422-0067/23/20/12667

  Although not among the therapies recommended in European and international guidelines, cases of patients with AIP treated with anti-tumor necrosis factor drugs (ANTI-TNF) have been described in the literature. […] Therefore, new therapeutic targets can be developed in patients with AIP and IgG4-RD.

• #48 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. Rituximab has also been recommended for recurrent AIP-1 in order to initiate remission and provide ongoing treatment. Immunomodulators such as azathioprine are used to keep certain patients in remission. Evaluation also takes into account a number of pharmacological alternatives, including biologic drugs like anti-tumor necrosis factor therapy, a safe and efficient second-line treatment for AIP-2 relapse or steroid dependence. Corticosteroids and immunosuppressants, which are poorly tolerated due to considerable side effects, are being replaced by other biologic drugs, which may offer a beneficial therapeutic alternative. […] Steroids are the first-line treatment for all patients with active AIP, according to the international consensus for the condition. Rituximab (RTX) is the only steroid-sparing therapy with proven efficacy in leading to remission as a single agent in cases of contraindication to steroids, while other steroid-sparing medicines such as thiopurines are ineffective when administered as monotherapy.

• #49 Colchicine Promising for Autoimmune Pancreatitis? | MedPage Today

  https://www.medpagetoday.com/gastroenterology/pancreaticdiseases/94956

  Treatment with the anti-inflammatory drug colchicine resolved recurrent type 2 autoimmune pancreatitis (AIP) in patients who previously received other drugs, a case series in Italy found. […] The researchers acknowledged that formal clinical studies are needed to confirm these „preliminary findings,” but concluded that based on these results, colchicine could be a promising treatment for type 2 AIP.

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

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

  Autoimmune pancreatitis treatment includes corticosteroids like prednisone or prednisolone. These medications reduce inflammation and calm an overactive immune system. Care usually begins with a high dose of steroids that’s gradually lowered (tapered). […] Additional treatments are typically not necessary unless you experience complications. The therapies you may need depend on the type of complication. If duct narrowing occurs, you may need a procedure to open and reinforce the duct with a hollow mesh device (stent). […] With successful management, people with relapsing autoimmune pancreatitis can live active, healthy lives. You may need to go back on corticosteroids. Some people continue steroid therapy for several years to keep symptoms from returning. Healthcare providers may also try other drugs that treat an overactive immune system. These include immunomodulating and immunosuppressive drugs. […] To get the most out of autoimmune pancreatitis treatment, its essential to continue taking steroids as prescribed. […] Most people make a full recovery after taking steroids. Some people need additional autoimmune pancreatitis treatment to achieve lasting relief.

• #51 Autoimmune pancreatitis | UM Health-Sparrow

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

  Treatments for autoimmune pancreatitis, such as long-term steroid use, can cause complications. However, even with these complications, people who are treated for autoimmune pancreatitis have a typical life expectancy. […] Biliary stenting. Before starting medicine, sometimes doctors or other healthcare professionals will insert a tube to drain the biliary ducts. This is called biliary stenting and is done in people with symptoms of obstructive jaundice. Often, though, jaundice improves with steroid treatment alone. Sometimes drainage is recommended if the diagnosis is not certain. Tissue samples and cells from the bile duct may be taken at the time of stent placement. […] Steroids. 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.

• #52 Autoimmune pancreatitis | UM Health-Sparrow

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

  Treatments for autoimmune pancreatitis, such as long-term steroid use, can cause complications. However, even with these complications, people who are treated for autoimmune pancreatitis have a typical life expectancy. […] Biliary stenting. Before starting medicine, sometimes doctors or other healthcare professionals will insert a tube to drain the biliary ducts. This is called biliary stenting and is done in people with symptoms of obstructive jaundice. Often, though, jaundice improves with steroid treatment alone. Sometimes drainage is recommended if the diagnosis is not certain. Tissue samples and cells from the bile duct may be taken at the time of stent placement. […] Steroids. 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.

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

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

  Most people with autoimmune pancreatitis respond well to treatment with corticosteroids. These drugs reduce inflammation by working to lower the activity of the immune system. Examples of corticosteroids include prednisone and prednisolone. […] An older study from 2013 included 1,064 participants with autoimmune pancreatitis. It found that 99 percent of those with type 1 disease and 92 percent of those with type 2 disease went into remission with corticosteroid treatment. […] If you have a narrowing or blockage in your pancreatic or bile ducts due to autoimmune pancreatitis, your doctor may place a stent in the duct. This is a narrow tube thats placed into the affected duct, allowing fluids to pass through more effectively. […] If a relapse happens, additional corticosteroid treatment may be necessary. Its also possible that a different immunomodulating or immunosuppressing drug may be used, such as azathioprine, methotrexate, or rituximab. […] 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.

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

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

  To induce remission, an initial dose of 0.6-1.0 mg/kg/day of prednisone, which is maintained for 2-4 wk and then gradually reduced is recommended. […] Maintenance treatment to prevent AIP recurrence is not recommended for type 2 AIP or for type 1 AIP with low pre-treatment disease activity. […] However, maintenance treatment with low-dose glucocorticoids or steroid-sparing agents may be helpful in some patients with type 1 AIP. […] There are rare cases of steroid non-response in patients with AIP. Rituximab is recommended as an alternative treatment in the setting of steroid-refractory AIP. […] In patients with AIP with obstructive jaundice, biliary stenting is usually performed along with biopsy through ERCP. […] In cases of biliary stent insertion, early biliary stent removal after diagnosis of AIP according to the guidelines should be considered owing to stent related complications such as migration or stent dysfunction. […] In patients with recurrent AIP, steroids and steroid-sparing agents may be used.

• #55 Diagnosis and Treatment of Autoimmune Pancreatitis in China: A Systematic Review | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0130466

  A total of 26 original articles involving 706 AIP patients were included with an estimated proportion of type 2 AIP as 4.7%. […] Nearly three tenths AIP patients received surgery (pooled rate: 29.7%, 95%CI: 18.1%42.8%) due to mimicked malignancy. Steroid treatment was given to 78.4% patients (95%CI: 65.3%89.1%) with a pooled remission rate of 96.2% (95%CI: 94.0%97.9%). […] Due to mimicked malignancy, nearly one third AIP patients received surgery (pooled rate: 29.7%, 95%CI: 18.1%42.8%). Surgical procedures included pancreaticoduodenectomy (43.0%, 52/121), distal pancreatectomy with or without splenectomy (9.1%, 11/121), choledojejunostomy with the resection of hilar mass (1.6%, 2/121), biliary-entericanastomosis (27.3%, 33/121) and exploratory laparotomy (19.0%, 23/121). […] Steroid treatment was given to most patients (pooled rate: 78.4%, 95%CI: 65.3%89.1%). In the 272 patients with detailed regimen, a fixed dose (30mg or 40mg prednisone per day) were given as the initial dose to 206 (75.7%) patients, while a personalized initial dose (0.6mg/kg per day) were given to other 66 (24.3%) patients. After steroid therapy, most patients got remission (pooled rate: 96.2%, 95%CI: 94.0%97.9%). In 269 patients receiving steroid treatment and having follow-up data, relapse occurred in 37 patients (pooled rate: 13.8%, 95%CI: 7.2%22.0%) with the mean follow-up time ranging from 12 to 45 months. Repeated steroid treatment was given to most patients with relapse (pooled rate: 95.8%, 95%CI: 89.8%99.3%) and obtained good remission outcome (pooled rate: 94.8%, 95%CI: 88.2%98.8%).

• #56 Diagnosis and Treatment of Autoimmune Pancreatitis in China: A Systematic Review | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0130466

  A total of 26 original articles involving 706 AIP patients were included with an estimated proportion of type 2 AIP as 4.7%. […] Nearly three tenths AIP patients received surgery (pooled rate: 29.7%, 95%CI: 18.1%42.8%) due to mimicked malignancy. Steroid treatment was given to 78.4% patients (95%CI: 65.3%89.1%) with a pooled remission rate of 96.2% (95%CI: 94.0%97.9%). […] Due to mimicked malignancy, nearly one third AIP patients received surgery (pooled rate: 29.7%, 95%CI: 18.1%42.8%). Surgical procedures included pancreaticoduodenectomy (43.0%, 52/121), distal pancreatectomy with or without splenectomy (9.1%, 11/121), choledojejunostomy with the resection of hilar mass (1.6%, 2/121), biliary-entericanastomosis (27.3%, 33/121) and exploratory laparotomy (19.0%, 23/121). […] Steroid treatment was given to most patients (pooled rate: 78.4%, 95%CI: 65.3%89.1%). In the 272 patients with detailed regimen, a fixed dose (30mg or 40mg prednisone per day) were given as the initial dose to 206 (75.7%) patients, while a personalized initial dose (0.6mg/kg per day) were given to other 66 (24.3%) patients. After steroid therapy, most patients got remission (pooled rate: 96.2%, 95%CI: 94.0%97.9%). In 269 patients receiving steroid treatment and having follow-up data, relapse occurred in 37 patients (pooled rate: 13.8%, 95%CI: 7.2%22.0%) with the mean follow-up time ranging from 12 to 45 months. Repeated steroid treatment was given to most patients with relapse (pooled rate: 95.8%, 95%CI: 89.8%99.3%) and obtained good remission outcome (pooled rate: 94.8%, 95%CI: 88.2%98.8%).

• #57 Autoimmune Pancreatitis: Clinical Presentation and Therapy | IntechOpen

  https://www.intechopen.com/chapters/65032

  Autoimmune pancreatitis is a relatively recently identified entity. […] However, all types of AIP respond quickly to steroid treatment with a complete recovery. […] The widely accepted dose of prednisone is relatively low, about 0.50-0.6 mg/kg/day. Others initiate the treatment with 40 mg prednisone/day. After 3-4 weeks with this treatment, the steroid dose is tapered, reducing it 5 mg/day every 2 weeks. Finally, the treatment can be stopped after the complete morphological recovery demonstrated by CT scan or magnetic resonance. […] The recurrent disease also responds to steroid treatment. However, in case of recurrence, it is advisable to initiate a longer treatment with some steroid-sparing agent, azathioprine (1.0-1.5 mg/kg/day) or mycophenolate (2 to 3 g/day) for several years. […] The effect of steroids is uniformly excellent. It means that if steroids fail to induce remission, one must have serious doubts in the diagnosis, whatever was the basis to establish it. […] In addition, pancreatic resection has surgical morbi-mortality and late metabolic consequences, which are hardly justifiable in a benign medically treatable disease.

• #58 Steroid Therapy and Steroid Response in Autoimmune Pancreatitis

  https://www.mdpi.com/1422-0067/21/1/257

  Autoimmune pancreatitis (AIP), a unique subtype of pancreatitis, is often accompanied by systemic inflammatory disorders. Standard therapy for AIP is oral corticosteroid administration. Steroid treatment is generally indicated for symptomatic cases and is exceptionally applied for cases with diagnostic difficulty (diagnostic steroid trial) after a negative workup for malignancy. More than 90% of patients respond to steroid treatment within 1 month, and most within 2 weeks. […] Short- and long-term corticosteroid treatment may induce adverse events, including chronic glycometabolism, obesity, an immunocompromised status against infection, cataracts, glaucoma, osteoporosis, and myopathy. […] With low-dose steroid treatment or treatment withdrawal, there is a high incidence of AIP recurrence (24–52%). Therefore, there is a need for long-term steroid maintenance therapy and/or steroid-sparing agents (immunomodulators and rituximab). Corticosteroids play a critical role in the diagnosis and treatment of AIP.

• #59 Autoimmune pancreatitis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/autoimmune-pancreatitis/symptoms-causes/syc-20369800

  Treatments for autoimmune pancreatitis, such as long-term steroid use, can cause complications. However, even with these complications, people who are treated for autoimmune pancreatitis have a typical life expectancy. […] Type 1 AIP can be mistakenly diagnosed as pancreatic cancer. The two conditions have overlapping symptoms, but very different treatments, so it is very important to distinguish one from the other. […] Autoimmune pancreatitis can cause a variety of complications. […] There is no established association between AIP and pancreatic cancer.

• #60 Autoimmune pancreatitis | UM Health-Sparrow

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

  Treatments for autoimmune pancreatitis, such as long-term steroid use, can cause complications. However, even with these complications, people who are treated for autoimmune pancreatitis have a typical life expectancy. […] Biliary stenting. Before starting medicine, sometimes doctors or other healthcare professionals will insert a tube to drain the biliary ducts. This is called biliary stenting and is done in people with symptoms of obstructive jaundice. Often, though, jaundice improves with steroid treatment alone. Sometimes drainage is recommended if the diagnosis is not certain. Tissue samples and cells from the bile duct may be taken at the time of stent placement. […] Steroids. 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.

• #61 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

  In such cases, treatment with PSL to prevent pancreatic stone formation and progression to chronic pancreatitis may be of limited use, even if the dose is increased. […] We nonetheless maintain that in early stage AIP, intensive PSL therapy has the potential to prevent pancreatic stone formation as well as the progression to chronic pancreatitis. […] 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.

• #62 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

  In such cases, treatment with PSL to prevent pancreatic stone formation and progression to chronic pancreatitis may be of limited use, even if the dose is increased. […] We nonetheless maintain that in early stage AIP, intensive PSL therapy has the potential to prevent pancreatic stone formation as well as the progression to chronic pancreatitis. […] 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.

• #63 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. […] Acute phase AIP responds favorably to corticosteroid therapy and results in the amelioration of clinical findings. […] Patients with AIP respond favorably to prednisolone (PSL) therapy from clinical, serological, imaging, and pathological perspectives. […] As corticosteroid therapy has been reported to significantly increase the remission rate and decrease the relapse rate of AIP, it is considered to be the standard treatment for inducing remission. […] We consider that patients with AIP who have residual pancreatic head swelling and/or narrowing of Wirsungs and Santorinis ducts after PSL therapy might be at a more advanced stage or active state of AIP.

• #64 Maintenance therapy in autoimmune pancreatitis: a weak light into the darkness

  https://atm.amegroups.org/article/view/15862/html

  The study by Masamune et al. suggests long-term (3 years) low-dosage steroids keep in remission 76.7% of AIP patients. AZA at dosage of 2 mg/kg/day seems to have similar percentage of sustained remission at 3 years (75%) in two small retrospective studies. RTX administered in 2 doses (1.000 mg) at time 0 and 15 days seems to be effective in IgG4-related diseases both to induce and to maintain remission. […] Long-term low-dosage steroids, AZA or RTX are the possible choices to maintain remission high risk for disease relapse patients. Long-term low-dosage steroids approach (prednisone 5 mg/day) is probably the most largely applicable, safe, cheap and the only evidence-based treatment, even with the limitations previously stressed. RTX seems to be the most effective drug in maintaining remission but with significant limitations, especially old age and history of cancer. […] In conclusion, maintenance therapy in AIP should be tailored on single patient, considering age, personal history, comorbidities, risk of cancer, risk of disease relapse, and patients preference for care.

• #65 Autoimmune pancreatitis | UM Health-Sparrow

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

  Immunosuppressants and immunomodulators. 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. To help reduce the serious side effects associated with extended steroid use, medicines that suppress or modify the immune system may be added to the treatment. These are sometimes called steroid-sparing medicines. You may be able to stop taking steroids altogether depending on your response to steroid-sparing medicine. […] Monitoring of other organ involvement. 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.

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

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

  To help reduce the serious side effects associated with extended steroid use, medicines that suppress or modify the immune system may be added to the treatment. These are sometimes called steroid-sparing medicines. You may be able to stop taking steroids altogether depending on your response to steroid-sparing medicine. […] Immunosuppressants and immunomodulators include mycophenolate (CellCept), mercaptopurine (Purinethol, Purixan), azathioprine (Imuran, Azasan) and rituximab (Rituxan). In general, they have been used in small sample groups and long-term benefits are still being studied. […] 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.

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

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

  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. […] 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.

• #68 Autoimmune Pancreatitis: Causes, Symptoms And Treatment

  https://www.netmeds.com/health-library/post/autoimmune-pancreatitis-causes-symptoms-and-treatment?srsltid=AfmBOordYq8-iFjf2fjox70BMiKDvijdOtTmopJPz2zKP3D_tms_Wsr2

  Once the diagnosis and type of AIP are confirmed in the patient, the pertinent treatment measures are initiated. There is no complete cure for AIP, but the symptoms can be alleviated and the functioning of the pancreas can be improved with timely medical intervention. […] The physician prescribes steroid medications, as well as immunosuppressant drugs and enzymatic supplements, to ensure the ceasing of the bodys immune system attacking the pancreatic cells and improve the regular activity of the pancreas. Medications for diabetes, to keep blood sugar in check, as well as surgical procedures like biliary stenting, to drain out surplus fluids in bile ducts are also carried out, to restore normal metabolism in the patient and help them regain optimal functioning of the pancreas.

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

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

  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. […] If untreated, AIP can lead to chronic pain, pancreatic insufficiency, fibrosis, and other complications. […] 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.

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