Materiały źródłowe

• #1 Long-term outcomes of autoimmune pancreatitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5016376/

  Autoimmune pancreatitis (AIP) has been considered a favorable-prognosis disease; however, currently, there is limited information on natural course of AIP during long-term follow-up. […] Recently published studies regarding the long-term outcomes of AIP has demonstrated the developments of pancreatic stone formation, exocrine insufficiency, and endocrine insufficiency are observed in 5%-41%, 34%-82%, and 38%-57% of patients having the disease. […] Furthermore, the incidence rate of developing pancreatic cancer ranges from 0% to 4.8% during the long-term follow-up. […] The event of death from AIP-related complications other than accompanying cancer is likely to be rare. […] During follow-up of AIP patients, careful surveillance for not only relapse of the disease but also development of complications at regular intervals is needed.

• #2 Long-term outcomes of autoimmune pancreatitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5016376/

  Generally, the short-term prognosis of AIP is considered favorable based on the remarkable improvement of clinical and radiological findings after steroid therapy. […] This review provides a current overview of AIP regarding long-term outcomes such as pancreatic stone formation, pancreatic exocrine or endocrine dysfunction, associated malignancy, and mortality. […] The multivariate logistic regression analysis by Buijs et al demonstrated that the risk factors of the development of endocrine insufficiency were longer follow-up period (OR = 1.36; 95%CI: 1.11-1.68) and older age at onset (OR = 1.06; 95%CI: 1.01-1.11). […] Although the reported ameliorating effect of steroid therapy for pancreatic function varies across studies owing to differences in observation period and definition of pancreatic exocrine and endocrine dysfunctions, steroid therapy appears to induce improvement of pancreatic exocrine function, as assessed by the urine exocrine N-benzoyl-L-tyrosyl-p-aminobenzoic acid (BT-PABA) test, in 40%-73% of AIP patients and glycemic control in 15%-63% of AIP patients with preexistent diabetes mellitus.

• #3 Long-term outcomes of autoimmune pancreatitis: a multicentre, international analysis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3862979/

  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). […] 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. […] Disease relapses are common after steroid discontinuation, and typically occur in the pancreas and/or biliary tract.

• #4 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). […] 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. […] Disease relapses are common after steroid discontinuation, and typically occur in the pancreas and/or biliary tract.

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

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

  Many people feel better after corticosteroids and experience no complications. A bout of autoimmune pancreatitis doesn’t affect your life expectancy. […] 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.

• #6 Long-term outcomes of autoimmune pancreatitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5016376/

  However, it is still unknown whether maintenance steroid therapy leads to favorable long-term outcomes not only in terms of prevention of relapse and progression of AIP but also in terms of steroid-related side effects. […] Patients with AIP are less likely to die from AIP-related complications other than accompanying cancer, although mortality due to complications of IgG4-related diseases, such as liver and renal failure, has been reported in rare cases. […] No significant difference in survival was observed between patients with AIP and age- and sex-matched controls from the national population. […] Long-term use of maintenance steroid therapy to prevent relapse of the disease can cause serious side effects, which can be fatal and therefore requires considerable attention. […] Although the characteristics, magnitude, and sequelae of complications that occur during a long-term course of AIP are still poorly understood, careful surveillance for not only relapse of the disease but also development of complications at regular intervals during follow-up of AIP patients is important.

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

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

  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. […] 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. […] In addition, the possibility of an association with malignant conditions, such as pancreatic cancer or other malignancies has been reported. […] 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. […] Published series have reported similar relapse rates in AIP ranging from 30% to 50%.

• #8

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

  Steroid therapy can improve pancreatic exocrine function in type 1 AIP by suppressing inflammation and fibrosis, and regeneration by correcting aberrant localization of the cystic fibrosis transmembrane conductance regulator (CFTR) in the pancreatic duct cells, and stimulating the regeneration of acinar cells. Endocrine function is also improved by steroid therapy, and, as a result, the prognosis of type 1 AIP is usually good in the short term. […] However, the long-term prognosis is not clear due to the multiple unknown elements in particular, relapse, pancreatic exocrine/endocrine dysfunction, and accompanying malignancies as an example, pancreatic cancer. 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.

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

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

  The occurrence of incident cancers following AIP diagnosis appears to be uncommon. […] Since disease relapses are common, additional studies are needed to compare different treatment strategies for maintaining disease remission. […] The remission rate of treating patients following disease relapse remained high. […] Pancreatic duct stones were relatively uncommon, but occurred more frequently in patients with at least one disease relapse. […] Importantly, pancreatic cancer was diagnosed in five male patients at a median age of 77 years (range 65-80) at the time of cancer diagnosis. […] Pancreatic duct stones were uncommonly seen occurring in 46/659 (7%) subjects with follow-up imaging permitting evaluation for stone disease. […] Additional studies with longer follow-up will help refine our understanding of these long-term sequelae.

• #10 Long-term outcomes of autoimmune pancreatitis: a multicentre, international analysis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3862979/

  The occurrence of incident cancers following AIP diagnosis appears to be uncommon. […] Since disease relapses are common, additional studies are needed to compare different treatment strategies for maintaining disease remission. […] The proportion of subjects having a relapse was similar in those with persistently abnormal IgG4 levels following steroids compared with those with a normal level (32.7% vs 31.4%, respectively, p=0.77). […] The rates of relapse were similar in those with and without distal biliary disease (33.9% vs 31.1%, respectively, p=0.44). […] Pancreatic duct stones were uncommonly seen occurring in 46/659 (7%) subjects with follow-up imaging permitting evaluation for stone disease. […] Importantly, pancreatic cancer was diagnosed in five male patients at a median age of 77years (range 6580) at the time of cancer diagnosis.

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

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

  Corticosteroid therapy was reported to significantly increase the remission rate and reduce the relapse rate of AIP. […] According to the Japanese Consensus Guidelines for Management of AIP, maintenance therapy (2.55 mg/day) is recommended to prevent recurrence, and stopping of maintenance therapy should be planned within at least 3 years in cases with serologic and radiologic improvement. […] Previous studies indicated that various factors at diagnosis, including involvement of proximal biliary tract, diffuse pancreatic swelling, jaundice, IgG4, immune complex, soluble IL2 receptor, and complement are predictive factors of relapse. […] The long-term prognosis and outcome of type 2 AIP have not been fully clarified. […] However, the recurrence rate of type 2 AIP was significantly lower than that of type 1, and the site of type 2 AIP recurrence was limited to the pancreas.

• #12 Clinical characteristics and long-term prognosis of autoimmune pancreatitis with renal lesions | Scientific Reports

  https://www.nature.com/articles/s41598-020-79899-3

  Autoimmune pancreatitis (AIP) is recognized as the pancreatic manifestation of a systemic IgG4-related disease that can involve various organs, including the kidney. […] However, the way in which these renal lesions affect the treatment and long-term prognosis of AIP is still not well understood. […] The cumulative relapse rates were significantly higher in the KD group than in the non-KD group (22.1% vs. 7.5% in one year and 61% vs. 21.9% in 3 years, respectively, P<0.001). [...] The relapse rate was significantly higher in the KD group than in the non-KD group, and tailored maintenance therapy with a higher dosage or longer duration may need to be considered. [...] However, the risk of developing a malignancy appears to be low in these patients, and early therapeutic intervention may contribute to the preservation of renal function.

• #13 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

  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 relapse rate of type 2 AIP is generally considered to be significantly lower than in type 1 AIP. Due to the lower relapse rates in type 2 AIP, long-term maintenance therapy may not be necessary. In our study, the relapse rate of type 2 AIP was about 8%.

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

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

  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. […] 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.

• #15

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

  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. Ikeura et al. reported that patients with type 1 AIP have a potent of higher risk for pancreatic cancer, equivalent to patients with usual chronic pancreatitis. […] On the other hand, Shiokawa et al. reported the risk of developing malignancies to be highest during the first year after or before diagnosis of type 1 AIP. They speculated that type 1 AIP may be a manifestation of paraneoplastic syndrome, but the definitive risk of malignancy remains unclear. Recently, a Japanese nationwide epidemiological survey of IgG4-RD with malignancy reported the frequencies of type1 AIP, IgG4-related sialadenitis, IgG4-related eye disease, IgG4-related kidney disease, and IgG4-related retroperitoneal fibrosis to be 44.7, 20.8, 14.0, 5.16, and 5.12%, respectively. The overall prevalence of malignancy in IgG4-RD was estimated to be 10,900 per 100,000 cases, which was significantly higher compared with malignancy in the general population in Japan according to the National Cancer Center Japan (which reported 1,834 per 100,000 people). When we examine type 1 AIP patients, we always have to pay attention to malignancy include pancreatic cancer during and after steroid therapy.

• #16 Disease profile and long-term outcome of patients with autoimmune pancreatitis/IgG4 systemic disease | Gut

  https://gut.bmj.com/content/60/Suppl_1/A8.1

  Long-term follow-up in this large cohort shows that AIP/IgG4 systemic disease may be associated with multi-organ complications, which cause significant morbidity, and a small but significant mortality. […] Extrapancreatic involvement at presentation predicts a complicated course.

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