Autoimmune Pancreatitis in Germany: Rare but Relevant

Petzold, Golo; Ellenrieder, Volker; Neeße, Albrecht

doi:10.1159/000481251

Cited by 5 publications

(5 citation statements)

References 5 publications

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“…There is an ongoing debate whether type 1 AIP is more frequent in East Asia, whereas type 2 AIP prevails in Western countries 20. The relative proportion of type 2 AIP among total AIP is generally known to be higher in the West when compared to the East.…”

Section: Discussionmentioning

confidence: 99%

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

Song

Moon

et al. 2019

Gut and Liver

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Background/Aims 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. Methods Type 2 AIP patients were classified according to International Consensus Diagnostic Criteria. Radiological findings were compared between type 2 AIP presenting as acute pancreatitis and gallstone pancreatitis. Results 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. Conclusions 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.

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Section: Discussionmentioning

confidence: 99%

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

Song

Moon

et al. 2019

Gut and Liver

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“…AIP-2 is predominant in western countries and is characterized by an equal incidence between men and women, without a specific sex deviation ( 15 ). It has also been noted that 16-30% of cases of AIP-2 are associated with IBD, such as UC ( 16 , 17 ).…”

Section: Introductionmentioning

confidence: 99%

Treatment approaches in autoimmune pancreatitis (Review)

Pădureanu,

Drăgoescu,

Pădureanu

et al. 2023

Biomed Rep

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Autoimmune pancreatitis (AIP) is a rare disease. There are two distinct types of AIP: AIP type 1 (AIP-1), a pancreatic manifestation of a multi-organ disease linked to immunoglobulin (Ig)G4, and AIP type 2 (AIP-2), a pancreas-specific disease unrelated to IgG4. The 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.

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“…Autoimmune pancreatitis (AIP) is a rare form of chronic pancreatitis with characteristic clinicopathological features. Type I AIP (lymphoplasmacytic sclerosing pancreatitis) describes the pancreatic manifestation of IgG4-related diseases (IgG4-RDs), a systemic disease characterized by an abundant infiltration of IgG4 + plasma cells which can affect multiple organs 1 – 3 . In contrast, type II AIP exclusively affects the pancreas and is termed idiopathic duct-centric pancreatitis (IDCP) based on a characteristic infiltration pattern of neutrophil granulocytes to small pancreatic ducts known as granulocytic epithelial lesion (GEL).…”

Section: Introductionmentioning

confidence: 99%

Functional IKK/NF-κB signaling in pancreatic stellate cells is essential to prevent autoimmune pancreatitis

et al. 2022

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Pancreatic stellate cells (PSCs) are resident cells in the exocrine pancreas which contribute to pancreatic fibrogenesis and inflammation. Studies on NF-κB in pancreatitis so far focused mainly on the parenchymal and myeloid compartments. Here we show a protective immunomodulatory function of NF-κB in PSCs. Conditional deletion of NEMO (IKKγ) in PSCs leads to spontaneous pancreatitis with elevated circulating IgM, IgG and antinuclear autoantibodies (ANA) within 18 weeks. When further challenged with caerulein, NEMOΔCol1a2 mice show an exacerbated autoimmune phenotype characterized by increased infiltration of eosinophils, B and T lymphocytes with reduced latency period. Transcriptomic profiling shows that NEMOΔCol1a2 mice display molecular signatures resembling autoimmune pancreatitis patients. Mechanistically, we show that PSCΔNEMO cells produce high levels of CCL24 ex vivo which contributes to eosinophil recruitment, as neutralization with a CCL24 antibody abolishes the transwell migration of eosinophils. Our findings uncover an unexpected immunomodulatory role specifically of NF-κB in PSCs during pancreatitis.

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Autoimmune Pancreatitis in Germany: Rare but Relevant

Cited by 5 publications

References 5 publications

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

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

Treatment approaches in autoimmune pancreatitis (Review)

Functional IKK/NF-κB signaling in pancreatic stellate cells is essential to prevent autoimmune pancreatitis

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