Diagnostic Criteria For Autoimmune Pancreatitis Research Articles

IgG4-related uveitis. A French cohort and literature review

IntroductionIgG4-related disease (IgG4-RD) is a fibro-inflammatory disorder that can affect almost any organ. IgG4-related ophthalmic disease is a protean condition involving the orbit and ocular adnexa. Although a few cases of uveitis have been reported, the exact pattern of IgG4-related intraocular manifestations remains unclear. Here, we report on a nationwide French multicenter cohort of patients with IgG4-RD and uveitis and conducted a literature review. MethodsPatients with uveitis and a concomitant definite diagnosis of IgG4-RD (Revised Comprehensive Diagnostic criteria, American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-RD, International Consensus Diagnostic Criteria for auto-immune pancreatitis, or diagnostic criteria for IgG4-related hypophysitis), were screened from our national IgG4-RD and systemic fibrosis database. Concomitantly, we conducted a PubMed literature review and selected cases of definite IgG4-RD with uveitis. ResultsWe reported on 16 patients (8 from our database and 8 from the literature) and a total of 30 episodes of uveitis. Uveitis cases represented 3 % of total IgG4-RD patients in the national database on IgG4-RD and systemic fibrosis. Uveitis was inaugural in IgG4-RD in 4/16 cases (25 %) (appearing before any other IgG4-related symptom, at a median of 9 months), occurred concurrently to other IgG4-related symptoms in 9/16 cases (56 %) (at a median of 15 months before IgG4-RD diagnosis), and appeared during follow up in 3/16 patients (19 %) (at a median of 57 months after first IgG4-related symptoms). When uveitis occurred during follow up, it was associated with IgG4-RD manifestations in other organs in 6/9 patients (67 %). Uveitis was bilateral in 8/16 cases (50 %) and granulomatous in 5/10 cases (50 %). It was anterior in 8/13 (62 %), intermediate in 3/13 (23 %), and global (panuveitis) in 4/13 patients (31 %). Median serum IgG4 at diagnosis was 3.2 g/L. Median follow up time was of 6 years, during which 8/16 patients (50 %) experienced at least one relapse of uveitis. Treatment data was available for 29/30 uveitis flares. Steroids were used in 28/29 episodes of uveitis (97 %), leading to remission of uveitis in 16/28 cases (57 %). Methotrexate and rituximab (in combination with systemic steroids) were administered as second- or third-line therapy in 6/29 (21 %) and 5/29 (17 %) episodes of uveitis, respectively, and led to remission of uveitis in 4/6 cases (67 %) and 4/5 cases (80 %), respectively. One third of uveitides required at least two different lines of treatment for remission induction (mainly a combination of both systemic steroids and methotrexate or rituximab). Discussion and conclusionsUveitis may be one of the initial symptoms of IgG4-RD, and IgG4-RD should be considered in the diagnostic workup of uveitis. Its early onset in IgG4-RD may help with early diagnosis and treatment of the disease. Steroid monotherapy may be sufficient to treat IgG4-related uveitis, yet relapses were frequent (50 %) and ultimately a third of patients required at least two lines of treatment. Hence, steroid-sparing agents can be considered at early stages of the disease, particularly for patients with a high risk of relapse or steroid-related complications.

Main Diagnostic Criteria Usually Does Not Work for Autoimmune Pancreatitis Wrongly Presuming Malignancy

Background. Autoimmune pancreatitis (AIP) usually responds dramatically to steroid therapy. Occasionally, however, misdiagnosed patients have undergone pancreaticoduodenectomy. This study is aimed at providing useful information to improve the accuracy of diagnosis before surgery and thus avoid unnecessary resections in patients with AIP. Methods. From January 2015 to February 2020, a series of patients were enrolled, having undergone pancreaticoduodenectomy for presumed malignancy. AIP diagnoses were confirmed by postoperative pathology. The demographic and clinical data of the AIP patients were evaluated. The main diagnostic criteria (HISORt, Asian, and ICDC) for AIP were applied to assess whether and how unnecessary surgery could have been avoided. Results. A total of 124 cases of pancreaticoduodenectomy were performed for presumed malignancy. Six patients were diagnosed with benign disease and five with AIP. The prevalences of benign disease and AIP were 4.8% and 4%, respectively. Four patients were female and 1 male, with a mean age of 60.0 years old. Jaundice, pain, and weight loss were observed in 100%, 20%, and 40% of AIP patients, respectively. The radiologic features of the AIP patients were a diffusely enlarged gland (40.0%), a focally enlarged gland (40.0%), pancreatic ductal dilatation (60.0%), upstream parenchymal atrophy (20.0%), bile duct thickening (66.0%), and bile duct stricture (40.0%). Based on the diagnostic criteria for AIP, surgery could have been avoided in two cases. Conclusions. IgG4 measurement and integrated use of major diagnostic criteria should be emphasized in every patient eligible for pancreaticoduodenectomies.

Diagnostic rate and issues regarding the Japanese clinical diagnostic criteria for autoimmune pancreatitis 2018

近年，わが国の自己免疫性膵炎（AIP）臨床診断基準の改訂版（JPS2018）が発表された．本研究の目的は，旧診断基準（JPS2011）および国際基準（ICDC）との比較からJPS2018が持つ1型AIPの診断率や課題を明らかにすることである．1型AIP（準確診/probable AIP以上）の診断率は，JPS2018では91％，JPS2011では83.4％，ICDCでは87.6％であり，JPS2018の診断率は他の診断基準に比べ高かった．JPS2018において診断率が改善した主な理由は，MRCP所見と超音波内視鏡下穿刺吸引（EUS-FNA）での癌の否定所見が採用されたことであった．切除例はいずれの診断基準でも術前の病理診断がなければAIPと診断できない症例であった．さらなるAIPの診断率向上にはEUS-FNA検体での組織診断が必要と思われる．

Pancreatitis autoinmune. Serie de casos y actualización bibliográfica

The concept and diagnostic criteria for autoimmune pancreatitis have evolved since the first case reports published in the 1960s. The advances in knowledge during the past two decades have resulted in the recognition of several pancreatic conditions that show, on histological evaluation, variable combinations of inflammation and fibrosis. In fact, autoimmune pancreatitis comprises two distinct diseases: type 1 and type 2. Type 2 autoimmune pancreatitis is a particular pancreatic disease about which there is limited data; it differs from the ‘classic’ IgG4-related type 1 autoimmune pancreatitis in terms of epidemiology, pathological characteristics and natural evolution. There are patients with complex pancreatic conditions where an autoimmune component and good response to steroids are demonstrated, but they do not meet the suggested criteria for the diagnosis of type 1 or type 2 autoimmune pancreatitis. Although the term steroid-responsive pancreatitis may be interpreted as a synonym to type 1 and 2 autoimmune pancreatitis, these are not the only pancreatic conditions that improve with this type of treatment. Here, we present three clinical cases and a literature review related to the diagnosis and treatment of autoimmune pancreatitis and other less frequent pancreatic disorders that present a good response to steroid therapy.

Diagnostic imaging guide for autoimmune pancreatitis.

The International Consensus Diagnosis Criteria for autoimmune pancreatitis (AIP) has been published internationally for the diagnosis of AIP. However, since the revisions in 2006 and 2011, the Clinical Diagnostic Criteria for Autoimmune Pancreatitis 2018 have been published. The criteria were revised based the Clinical Diagnostic Criteria 2011, and included descriptions of characteristic imaging findings such as (1) pancreatic enlargement and (2) distinctive narrowing of the main pancreatic duct. In addition, pancreatic duct images obtained by magnetic resonance cholangiopancreatography as well as conventional endoscopic retrograde pancreatography were newly adopted. The guideline explains some characteristic imaging findings, but does not contain descriptions of the imaging methods, such as detailed imaging parameters and optimal timings of dynamic contrast-enhanced computed tomography/magnetic resonance imaging. It is a matter of concern that imaging methods can vary from hospital to hospital. Although other characteristic findings have been reported, these findings were not described in the guideline. The present paper describes the imaging methods for obtaining optimal images and the characteristic imaging findings with the aim of standardizing image quality and improving diagnostic accuracy when radiologists diagnose AIP in actual clinical settings.

Diagnosis of Autoimmune Pancreatitis: The Evolution of Diagnostic Criteria for a Rare Disease

Diagnosis of Autoimmune Pancreatitis: The Evolution of Diagnostic Criteria for a Rare Disease

The role of endoscopic ultrasonography in diagnosis and treatment of autoimmune pancreatitis

Background:Although endoscopic ultrasonography (EUS) is useful for diagnosis of pancreatic diseases, EUS findings are not employed in the clinical diagnostic criteria for autoimmune pancreatitis (AIP).Objectives:We assessed EUS findings in AIP and retrospectively analyzed them before and after steroid therapy.Methods:Twenty-one patients with AIP who received steroid therapy underwent EUS. Following seven EUS findings for AIP (a) hyperechoic foci, (b) strands, (c) lobularity, (d) reduced echogenicity, (e) capsule-like rim, (f) focal mass forming, and (g) contrast imaging pattern (poor vascular pattern) by contrast agent were assessed before and after steroid therapy. The total numbers of positive EUS findings before and after the steroid therapy were compared.Results:EUS findings before steroid therapy a, b, c, d, e, f, and g were observed in 100%, 91%, 86%, 91%, 86%, 52%, and 48%, respectively. EUS findings after steroid therapy a, b, c, d, e, f, and g were observed in 91%, 57%, 24%, 67%, 29%, 14%, and 10%, respectively. Strands, lobularity, capsule-like rim, focal mass forming, and contrast imaging pattern decreased significantly after steroid therapy (P < 0.05). The total number of positive EUS findings after the steroid therapy (5.1 ± 0.29) was significantly lower than their numbers before treatment (2.81 ± 0.13) (P < 0.001).Conclusion:Strands, lobularity, capsule-like rim, focal mass forming, and poor vascular pattern may reflect the disease activity of AIP. EUS is useful for the evaluation of response to steroid therapy.

Resonancia magnética de la pancreatitis autoinmune: una revisión de la literatura

Autoimmune pancreatitis (AIP) is a distinct form of chronic pancreatitis caused by an autoimmune pathogenetic mechanism. It affects males (60%) slightly more frequently, and generally occurs between 40 to 60 years, although the possible range is much wider (14-80 years). Histologically AIP is characterised by the presence of a dense inflammatory infiltrate, with variable extension. It can be focal or diffuse throughout the whole pancreas. Clinical presentation in most cases is non-specific, with patients presenting with mild abdominal pain, rarely, acute pancreatitis symptoms, weight loss, and jaundice. Several studies reported multiple organ involvement due to the autoimmune process, suggesting a systemic nature of the disease.In 2011 the International Consensus Diagnostic Criteria for autoimmune pancreatitis (ICDC) defined the guidelines, and magnetic resonance imaging (MRI) and MR-cholangiopancreatography (MRCP) became the reference diagnostic technique in the diagnosis of the disease.This paper will review the imaging characteristics, the differential diagnosis, and the imaging features after treatment and follow up. Furthermore, our experience in this uncommon and challenging disease is reviewed

Current Concepts and Diagnosis of IgG4-Related Pancreatitis (Type 1 AIP).

Although now considered to be a member of the systemic entity of immunoglobulin G4- (IgG4-) related disease, IgG4-related pancreatitis is generally referred to as type 1 autoimmune pancreatitis (AIP). Type 1 AIP was established based on a pathological background of lymphoplasmacytic sclerosing pancreatitis, high serum IgG4 concentration, and abundant IgG4-bearing plasma cell infiltration. The characteristic clinical features of type 1 AIP, such as elderly male preponderance, obstructive jaundice, and mass-forming lesions in the pancreas, often mimic those of pancreatic cancer. However, because AIP responds favorably to corticosteroid treatment, careful differentiation from pancreatic cancer is required. An AIP diagnosis is currently based on the 2011 International Consensus Diagnostic Criteria for AIP, which are based on high sensitivity, selectivity, and accuracy. Over the long term, AIP can progress to a chronic condition, with pancreatic stone formation and atrophy resembling that of chronic pancreatitis. Although AIP has been linked to the complication of malignancies, it remains controversial whether an association exists between the disease and tumor formation.

Chronic Pancreatitis: A Review.

This is to incorporate the recent trends in chronic pancreatitis. Extensive literature search was done from Pubmed and Ovid SP. Full text articles and abstracts related to chronic pancreatitis were reviewed and the article was prepared. Chronic pancreatitis is evolving fast on its etiology and treatment areas. The main etiological factors are pointing towards genetic, alcohol, and smoking. Autoimmune has also been added as a cause. Endoscopic ultra sound (EUS) becoming the standard diagnostic procedure. Surgery is becoming the treatment of choice for pain and mechanical complications and not the endotherapy in the long-term follow-up. The numbers of surgeries are getting narrowed down. The two etiological groups of chronic pancreatitis namely alcohol + smoking and genetic getting clearer, where the later group present at an early age. Endoscopic ultrasound and imaging with secretin is diagnostic of chronic pancreatitis before the structural changes. Endotherapy is found to be inferior to surgery in long-term pain relief. Diagnostic criteria for autoimmune pancreatitis are established. Pancreaticogenic diabetes (Type3c) and its problem associated with fat malabsorption are being understood.

A case of obstructive jaundice due to autoimmune pancreatitis and a review of the literature

A 33-year-old man presented with jaundice, mild pancreatitis, and some weight loss. Laboratory tests showed cholestatic icterus. Imaging revealed distal bile duct obstruction and a mass in the head of the pancreas suspicious for cancer. Besides the enlargement of the head, the whole pancreatic parenchyma was altered. Therefore, autoimmune pancreatitis was considered. Endoscopic ultrasound biopsy found pancreatic tissue with lymphoplasmacytic inflammation and fibrosis. IgG4 serum concentration was within normal range. Due to the benign histology, a therapeutic attempt with prednisolone was initiated. The radiologic changes improved within 2 weeks, which supported the diagnosis of autoimmune pancreatitis. Bile duct obstruction and radiologic abnormalities of the pancreas completely resolved after a 4-month course of steroid treatment. Within 10 months, no relapse occurred. In context with this case, the recent literature was reviewed and the international consensus diagnostic criteria for autoimmune pancreatitis are summarized.

IgG4 Related disease – a retrospective descriptive study highlighting Canadian experiences in diagnosis and management

BackgroundAppreciating the utility of published diagnostic criteria for autoimmune pancreatitis, when compared to the characteristics of patients clinically managed as having disease, informs and refines ongoing clinical practice.MethodsComparative retrospective descriptive evaluation of patients with autoimmune pancreatitis including dedicated radiology review.Results66 subjects with radiographic OR clinical features of autoimmune pancreatitis were initially identifiable (Male: n = 50), with 55 confirmed for evaluation. The most common presentation included pain (67%), weight loss (65%), and jaundice (62%). Diffuse enlargement of the pancreas was evident in 38%, whilst multifocal, focal, or atrophic changes were seen in 7%, 33% and 9% respectively. 13% had no pancreatic parenchymal involvement. Peripheral rim enhancement was seen in 23 patients (42%). Where discernible, disease was a) Sclerosing pancreatitis and cholangitis, n = 21; b) Sclerosing cholangitis, n = 9; c) Sclerosing pancreatitis, n = 4; d) Sclerosing pancreatitis and cholangitis with pancreatic pseudotumour, n = 7; e) Sclerosing cholangitis with hepatic pseudotumour, n = 3; f) Sclerosing pancreatitis with pancreatic pseudotumour, n = 1. 56% of the patients had systemic manifestations and the median serum IgG4 at diagnosis was 5.12 g/L. The Korean criteria identified most patients (82%) compared to HISORt (55%) or the Japan Pancreas Society (56%). The majority (HISORt 60%; Japan Pancreas Society 55%; Korean 58%) met diagnostic criterion by radiological findings and elevated serum IgG4. Treatment and response did not differ when stratified by diagnostic criteria.ConclusionOur descriptive and retrospective dataset confirms that in non-expert practice settings, autoimmune pancreatitis scoring systems with a focus on radiology and serology capture most patients who are clinically felt to have disease.

Clinical validation of the international consensus diagnostic criteria for autoimmune pancreatitis

Clinical validation of the international consensus diagnostic criteria for autoimmune pancreatitis

A proposal of a diagnostic algorithm with validation of International Consensus Diagnostic Criteria for autoimmune pancreatitis in a Japanese cohort

BackgroundAmong many diagnostic criteria for autoimmune pancreatitis (AIP), the International Consensus Diagnostic Criteria (ICDC) first enabled us to diagnose and compare type 1 and type 2 AIP, which permitted tailoring individual diagnostic algorithms depending on local expertise. We compared them and validated ICDC with special reference to levels 1 and 2, and proposed a diagnostic algorithm for AIP in Japan. MethodsThe diagnostic sensitivity of 5 major criteria (ICDC, Korean, Japanese-2011, Asian, and HISORt criteria) was compared, using 61 patients with AIP. Fifty six patients with pancreatic cancer served as a control. Pancreas imaging on computed tomography (CT) and endoscopic retrograde pancreatography (ERP) were independently evaluated by 3 pancreatologists (5, 10, and 20 years of career experience) and each diagnostic criterion of ICDC was validated with special reference to levels 1 and 2. ResultsThe sensitivities of 5 major criteria were 95.1% (ICDC), 90.2% (Korean), 86.9% (Japanese), 83.6% (Asian), and 83.6% (HISORt) with 100% of specificity in each. In the evaluation of pancreas imaging, diagnostic sensitivities of combination with CT and ERP in segmental/focal type AIP were significantly higher than single imaging (26% in CT (P < 0.01) or 35% in ERP (P < 0.05) vs 63% in CT + ERP), but not significantly different in the diffuse type. ConclusionsOf the 5 criteria, ICDC is the most sensitive and useful for diagnosing AIP. We have proposed a diagnostic algorithm with CT for the diffuse type of AIP, and combination with CT + ERP followed by EUS-FNA for the segmental/focal type.

International Consensus Diagnostic Criteria for Autoimmune Pancreatitis and Its Japanese Amendment Have Improved Diagnostic Ability over Existing Criteria

Objectives. The recent International Consensus Diagnostic Criteria (ICDC) for autoimmune pancreatitis (AIP) and its Japanese amendment developed by the Japanese Pancreas Society (JPS 2011) may have overcome the drawbacks of earlier criteria and achieved a higher diagnostic ability for AIP. The aim of the present study is to evaluate this possibility and identify the underlying causes of this change. Methods. We compared the diagnostic abilities of the ICDC and JPS 2011 with those of the Japanese diagnostic criteria 2006 (JPS 2006), Korean diagnostic criteria (Korean), Asian diagnostic criteria (Asian), and HISORt diagnostic criteria in 110 patients with AIP and 31 patients with malignant pancreatic cancer. Results. The ICDC achieved the highest diagnostic ability in terms of accuracy (95.0%), followed by JPS 2011 (92.9%), Korean (92.2%), HISORt (88.7%), Asian (87.2%), and JPS 2006 (85.1%). Nearly all criteria systems exhibited a high specificity of 100%, indicating that the enhanced diagnostic ability of the ICDC and JPS 2011 likely stemmed from increased sensitivity brought about by inclusion of diagnostic items requiring no endoscopic retrograde pancreatography. The diagnostic ability of JPS 2011 was nearly equivalent to that of the ICDC. Conclusions. The ICDC and JPS 2011 have improved diagnostic ability as compared with earlier criteria sets because of an increase in sensitivity.

Capability and Limitations of Recent Diagnostic Criteria for Autoimmune Pancreatitis

Because a diagnostic serological marker is unavailable, autoimmune pancreatitis (AIP) is diagnosed based on unique features. The diagnostic capabilities and potential limitations of four sets of diagnostic criteria for AIP (Japanese diagnostic criteria 2006 and 2011, Asian diagnostic criteria, and international consensus diagnostic criteria (ICDC)) were compared among 85 patients who were diagnosed AIP according to at least one of the four sets. AIP was diagnosed in 87%, 95%, 95%, and 95% of the patients according to the Japanese 2006, Asian, ICDC, and Japanese 2011 criteria, respectively. The ICDC can diagnose types 1 and 2 AIP independently and show high sensitivity for diagnosis of AIP. However, as the ICDC are rather complex, diagnostic criteria for AIP should perhaps be revised and tailored to each country based on the ICDC.

Clinical Update on Autoimmune Pancreatitis

Autoimmune pancreatitis (AIP) is a unique type of chronic pancreatitis in which pathogenesis involves autoimmune mechanism. During the past decade, several different diagnostic criteria for AIP have been reported from various countries. A set of international consensus diagnostic criteria and algorithm for AIP has recently been proposed by a consensus of expert opinion. The goal of the international consensus diagnostic criteria and algorithm for AIP is to develop criteria that can be applied worldwide, taking into consideration the marked differences in clinical practice patterns between countries. They also aim at diagnosing AIP nonsurgically and avoiding misdiagnosis of pancreatobiliary malignancies as AIP. This review discusses the recent advances in the diagnosis and management of AIP based on the international consensus diagnostic criteria. We also suggest modified algorithm for diagnosing AIP for Korean practice. (Korean J Med 2013;84:789-797) Keywords: Autoimmune pancreatitis; Pancreatic cancer; IgG4-related systemic disease 중심 단어: 자가면역성 췌장염; 췌장암; ì „ì‹ IgG4 연관질환

PMO-109 IGG4 related autoimmune disease—experience from north east of England

IntroductionIgG4 related disease is now well recognised as a multisystem disease. This condition, originally discovered in Japan in 1995 is now being increasingly recognised in the Western World. Apart from...

Current status and problems in the diagnostic criteria for autoimmune pancreatitis

Current status and problems in the diagnostic criteria for autoimmune pancreatitis

Advances in clinical research on autoimmune pancreatitis

In recent years,research on autoimmune pancreatitis (AIP) has gained significant progress.AIP is a special type of chronic pancreatitis which is mediated by an autoimmune mechanism.It is characterized by the infiltration of lymphocytes and plasma cells leading to pancreatic fibrosis and dysfunction.AIP can affect the bile ducts,salivary glands,kidneys and other organs outside the pancreas.The common manifestations include obstructive jaundice,abdominal pain,and weight loss,which are similar to pancreatic cancer.Steroid therapy is of significant effect.There is no consensus nowadays on the diagnostic criteria of AIP across the world.There are many studies on the pathological subtypes,imaging,serological markers and biopsy techniques on AIP,and research focuses on the differential diagnosis from pancreatic cancer. Key words: Autoimmune pancreatitis; Diagnostic criteria; Differential diagnosis