scholarly journals Kidney Involvement in Patients with Type 1 Autoimmune Pancreatitis

2019 ◽  
Vol 8 (2) ◽  
pp. 258
Author(s):  
Miroslav Vujasinovic ◽  
Raffaella Pozzi Mucelli ◽  
Roberto Valente ◽  
Caroline Verbeke ◽  
Stephan Haas ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
Lymphocytic Infiltration ◽  
Normal Serum ◽  
International Consensus ◽  
Kidney Involvement ◽  
Type 1 Autoimmune Pancreatitis ◽  
Elevated Creatinine ◽  
Imaging Signs

Introduction: Autoimmune pancreatitis (AIP) type 1 is a special form of chronic pancreatitis with a strong lymphocytic infiltration as the pathological hallmark and other organ involvement (OOI). IgG4-related kidney disease (IgG4-RKD) was first reported as an extrapancreatic manifestation of AIP in 2004. The aim of the present study was to determine the frequency and clinical impact of kidney lesions observed in patients with AIP type 1. Methods: We performed a single-centre retrospective study on a prospectively collected cohort of patients with a histologically proven or highly probable diagnosis of AIP according to the International Consensus Diagnostic Criteria (ICDC) classification. Results: Seventy-one patients with AIP were evaluated. AIP type 1 was diagnosed in 62 (87%) patients. Kidney involvement was present in 17 (27.4%) patients with AIP type 1: 15 (88.2%) males and 2 (11.8%) females. Laboratory and/or imaging signs of kidney involvement were presented at the time of AIP diagnosis in eight (47.1%) patients. In other patients, the onset of kidney involvement occurred between four months and eight years following diagnosis. At the time of the diagnosis of kidney involvement, eight (47.1%) patients showed elevated creatinine, and nine (52.9%) patients showed normal serum creatinine. None of the patients were treated with dialysis. Conclusions: IgG4-RKD was present in 27.4% of patients with AIP type 1, with male gender predominance. In cases of early diagnosis and cortisone treatment, the clinical course was mild in most cases. Regular laboratory control of renal function should be a part of the follow-up of patients with AIP type 1.

Clinical Evaluation of International Consensus Diagnostic Criteria for Type 1 Autoimmune Pancreatitis in Comparison With Japanese Diagnostic Criteria 2011

Pancreas ◽  
2013 ◽  
Vol 42 (8) ◽  
pp. 1238-1244 ◽  
Author(s):  
Itaru Naitoh ◽  
Takahiro Nakazawa ◽  
Kazuki Hayashi ◽  
Katsuyuki Miyabe ◽  
Shuya Shimizu ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Clinical Evaluation ◽  
Autoimmune Pancreatitis ◽  
International Consensus ◽  
Type 1 Autoimmune Pancreatitis

scholarly journals Kidney involvement in patients with type 1 autoimmune pancreatitis

Pancreatology ◽  
2019 ◽  
Vol 19 ◽  
pp. S133
Author(s):  
Miroslav Vujasinovic ◽  
Raffaella Maria Pozzi Mucelli ◽  
Roberto Valente ◽  
Caroline S. Verbeke ◽  
Stephan L. Haas ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
Kidney Involvement ◽  
Type 1 Autoimmune Pancreatitis

scholarly journals Identification of serum IFN-α and IL-33 as novel biomarkers for type 1 autoimmune pancreatitis and IgG4-related disease

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Kosuke Minaga ◽  
Tomohiro Watanabe ◽  
Akane Hara ◽  
Ken Kamata ◽  
Shunsuke Omoto ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
Characteristic Curve ◽  
Elevated Serum ◽  
Normal Serum ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Number Of Patients ◽  
Serum Igg4 ◽  
Type 1 Autoimmune Pancreatitis

Abstract IgG4-related disease (IgG4-RD) is a multi-organ autoimmune disease characterized by elevated serum IgG4 concentration. Although serum IgG4 concentration is widely used as a biomarker for IgG4-RD and type 1 autoimmune pancreatitis (AIP), a pancreatic manifestation of IgG4-RD, a significant number of patients have normal serum IgG4 levels, even in the active phase of the disease. Recently, we reported that the development of experimental AIP and human type 1 AIP is associated with increased expression of IFN-α and IL-33 in the pancreas. In this study, we assessed the utility of serum IFN-α and IL-33 levels as biomarkers for type 1 AIP and IgG4-RD. Serum IFN-α and IL-33 concentrations in patients who met the diagnostic criteria for definite type 1 AIP and/or IgG4-RD were significantly higher than in those with chronic pancreatitis or in healthy controls. Strong correlations between serum IFN-α, IL-33, and IgG4 concentrations were observed. Diagnostic performance of serum IFN-α and IL-33 concentrations as markers of type 1 AIP and/or IgG4-RD was comparable to that of serum IgG4 concentration, as calculated by the receiver operating characteristic curve analysis. Induction of remission by prednisolone treatment markedly decreased the serum concentration of these cytokines. We conclude that serum IFN-α and IL-33 concentrations can be useful as biomarkers for type 1 AIP and IgG4-RD.

scholarly journals Utility of endoscopic ultrasound-guided fine-needle biopsy in the diagnosis of type 1 autoimmune pancreatitis

2020 ◽  
Vol 08 (12) ◽  
pp. E1855-E1861
Author(s):  
Kofi W. Oppong ◽  
Pardeep Maheshwari ◽  
Manu K. Nayar ◽  
Antony Darne ◽  
Daniel Parkinson ◽  
...  
Keyword(s):  
Endoscopic Ultrasound ◽  
Autoimmune Pancreatitis ◽  
Needle Biopsy ◽  
Final Diagnosis ◽  
Fine Needle Biopsy ◽  
Pathological Feature ◽  
International Consensus ◽  
Fine Needle ◽  
Type 1 Autoimmune Pancreatitis

Abstract Background and study aims Endoscopic ultrasound (EUS)-guided fine-needle aspiration (FNA) performs poorly in the histological diagnosis of type 1 autoimmune pancreatitis (AIP). The aim of this study was to assess the performance of fine-needle biopsy (FNB) comparing reverse bevel (RB) and fork-tip (FT) needles. Patients and methods A retrospective study of prospectively maintained databases was performed. Patients with a final diagnosis of type 1 AIP who underwent EUS-FNB during diagnostic workup were included. Pathology reports were reviewed and classified as per international consensus diagnostic criteria (ICDC). The Primary outcome was EUS-FNB sensitivity in diagnosing type 1 AIP. Results Between March 2011 and December 2018, 24 patients with a final diagnosis of type 1 AIP underwent FNB. Six patients underwent biopsy with the RB needle and 18 with the FT needle. Mean age (± SD) 62.2 (± 11.4), 17 (70.8 %) male. No RB samples were diagnostic compared to 14 (78 %) FT; P = 0.001; of which 13 (72 %) were level 1. In eight (44 %) of FT cases a diagnosis was not possible without histology. Initial biopsy was diagnostic in five (62.5 %) of these cases. Including repeat biopsy, seven (87 %) had a diagnosis made by FT needle. Obliterative phlebitis (44 %) was the least frequently identified pathological feature and immunoglobulin (IgG)4 + plasma cells > 10 per high power field (78 %) the most common. Conclusion The FT needle demonstrated good performance for diagnosing type 1 AIP. The results support the preferential use of this core biopsy needle for EUS pancreatic tissue sampling.

scholarly journals Type 1 autoimmune pancreatitis

2020 ◽  
Vol 53 (1) ◽  
pp. 81-87 ◽  
Author(s):  
Gustavo Mesquita De Oliveira ◽  
Lia de Freitas Araújo Alves ◽  
Paloma Lucena Cabral ◽  
Ana Luiza Viana Pequeno ◽  
Clóvis Rêgo Coelho ◽  
...  
Keyword(s):  
Pancreatic Adenocarcinoma ◽  
Liver Abscess ◽  
Autoimmune Pancreatitis ◽  
Epigastric Pain ◽  
Systemic Disease ◽  
Disease Case ◽  
Significant Clinical Improvement ◽  
Type 1 Autoimmune Pancreatitis

Type 1 autoimmune pancreatitis is a cause of chronic pancreatitis related to the systemic disease known as IgG4-related Sclerosing Disease. Case report: We report the case of a 64-year-old male patient who presented recurrent epigastric pain radiating to the back, associated with jaundice, xerostomia, nausea, and vomiting, since 2014, diagnosed two years later with an unresectable pancreatic adenocarcinoma. The diagnosis was questioned after a few follow-up months without clinical deterioration when it was suggested the possibility of type 1 autoimmune pancreatitis in its pseudotumoral form. The patient was then treated with glucocorticoids, obtaining significant clinical improvement. After two years of follow-up, he returned asymptomatic with images suggestive of sclerosing cholangitis and a large liver abscess. Importance of the issue: The present case denotes the difficulty found in this diagnosis due to clinical and radiological resemblances with pancreatic adenocarcinoma. Besides that, it presents a seldom described disease complication, the liver abscess.

scholarly journals Regulatory T Cells in Type 1 Autoimmune Pancreatitis

2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
Kazushige Uchida ◽  
Takeo Kusuda ◽  
Masanori Koyabu ◽  
Hideaki Miyoshi ◽  
Norimasa Fukata ◽  
...  
Keyword(s):  
T Cells ◽  
Regulatory T Cells ◽  
Autoimmune Pancreatitis ◽  
Plasma Cells ◽  
Costimulatory Molecule ◽  
International Consensus ◽  
Serum Igg4 ◽  
Storiform Fibrosis ◽  
Type 1 Autoimmune Pancreatitis

Autoimmune pancreatitis (AIP) is a newly recognized pancreatic disorder. Recently, International Consensus Diagnostic Criteria for AIP (ICDC) was published. In this ICDC, AIP was classified into Type 1 and Type 2. Patients with Type 1 AIP have several immunologic and histologic abnormalities specific to the disease, including increased levels of serum IgG4 and storiform fibrosis with infiltration of lymphocytes and IgG4-positive plasmacytes in the involved organs. Among the involved organs showing extrapancreatic lesions, the bile duct is the most common, exhibiting sclerosing cholangitis (IgG4-SC). However, the role of IgG4 is unclear. Recently, it has been reported that regulatory T cells (Tregs) are involved in both the development of various autoimmune diseases and the shift of B cells toward IgG4, producing plasmacytes. Our study showed that Tregs were increased in the pancreas with Type 1 AIP and IgG4-SC compared with control. In the patients with Type 1 AIP and IgG4-SC, the numbers of infiltrated Tregs were significantly positively correlated with IgG4-positive plasma cells. In Type 1 AIP, inducible costimulatory molecule (ICOS)+and IL-10+Tregs significantly increased compared with control groups. Our data suggest that increased quantities of ICOS+Tregs may influence IgG4 production via IL-10 in Type 1 AIP.

Performance of international consensus diagnostic criteria (ICDC) for Type 1 autoimmune pancreatitis (AIP)

Pancreatology ◽  
2013 ◽  
Vol 13 (2) ◽  
pp. e7
Author(s):  
R. Sah ◽  
P. Hart ◽  
M. Levy ◽  
N. Takahashi ◽  
T. Smyrk ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Autoimmune Pancreatitis ◽  
International Consensus ◽  
Type 1 Autoimmune Pancreatitis

scholarly journals A Follow-Up Study of a European IgG4-Related Disease Cohort Treated with Rituximab

2021 ◽  
Vol 10 (6) ◽  
pp. 1329
Author(s):  
Johanna Backhus ◽  
Christian Neumann ◽  
Lukas Perkhofer ◽  
Lucas A Schulte ◽  
Benjamin Mayer ◽  
...  
Keyword(s):  
Disease Activity ◽  
Clinical Activity ◽  
Inflammatory Disorder ◽  
International Consensus ◽  
Steroid Pulse ◽  
Related Disease ◽  
Igg4 Related Disease

Objectives: IgG4-related disease (IgG4-RD) is a chronic fibro-inflammatory disorder affecting virtually any organ. Type 1 autoimmune (type 1 AIP) is its pancreatic manifestation. To date, steroids are considered the first-line pancreatitis treatment. The CD20-binding antibody rituximab (RTX) appears a promising steroid-sparing therapy, although long-term data are lacking. We aimed to bridge this gap with a cohort of IgG4-RD patients treated with RTX and to assess the potential value of the Responder Index (RI) as a discriminatory score for disease activity. Methods: We retrospectively evaluated 46 patients from a tertiary referral centre who were diagnosed with IgG4-RD and/or type 1 AIP according to the International Consensus Diagnostic Criteria or Unifying-AIP criteria between June 2006 and August 2019. Results: Patients resembled previous cohorts in terms of characteristics, diagnosis, and therapeutic response. Thirteen of the 46 patients with IgG4-RD/type 1 AIP were treated with RTX pulse therapy due to relapse, adverse reactions to steroids, or high-risk constellations predicting a severe course of disease with multi-organ involvement. Median follow-up after diagnosis was 52 months for all subjects, and 71 months in IgG4-RD patients treated with RTX. While patients in the RTX group showed no significant response to an initial steroid pulse, clinical activity as measured by the RI significantly decreased in the short-term after RTX induction. Within 16 months, 61% of patients relapsed in the RTX group but responded well to re-induction. Clinical and laboratory parameters improved equally in response to RTX. Conclusion: RTX therapy in patients with IgG4-RD is an effective and safe treatment to induce treatment response and possible long-term remission. Repeated RTX administration after 6–9 months may be of value in reducing the risk of relapse. The RI appears to be a reasonable index to assess disease activity and to identify patients with IgG4-related disease who may benefit from B-cell-depleting therapy.

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