International Consensus Diagnostic Criteria Research Articles

Autoimmune pancreatitis type 2.

Type 2 autoimmune pancreatitis (AIP) is a rare inflammatory disease of the pancreas. Very few data have been published on this particular subtype, which differs from the 'classical' IgG4-related type 1 AIP in terms of pathological features, epidemiology and risk of relapse. The aim of the current review is to summarize the available literature, suggesting a diagnostic and therapeutic approach to this disease. Based on the International Consensus Diagnostic Criteria, to achieve a 'definitive' diagnosis of type 2 AIP, histology is required. If a definitive histological diagnosis is lacking (not-performed or inconclusive), concomitant presence of inflammatory bowel disease (IBD) and effective response to steroids are needed for a 'probable' diagnosis of type 2 AIP. Type 2 AIP is a selective pancreatic disease, without association to other organ involvement. The lack of validated serological markers makes the diagnosis challenging in clinical practice, particularly in focal forms. A careful evaluation of the clinical profile (especially of a concomitant IBD), associated with an accurate imaging, might help in clinical practice to suspect type 2 AIP. Response to steroids is crucial to achieve diagnosis in patients without a diagnostic histology.

Efficacy and limitations of the histological diagnosis of type 1 autoimmune pancreatitis with endoscopic ultrasound-guided fine needle biopsy with large tissue amounts

ObjectivesWe examined the efficacy and limitations of acquiring large specimens by endoscopic ultrasound-guided fine needle biopsy (EUS-FNB) for diagnosing type 1 autoimmune pancreatitis (AIP). MethodsPatients from 12 institutions with non-neoplastic diseases or pancreatic ductal adenocarcinoma (PDAC) with large EUS-FNB specimens were investigated. Slides stained with hematoxylin-eosin, elastic, IgG4, and IgG stains were evaluated. The IgG4- and IgG-positive cell numbers were counted in three foci. The diagnoses were based on the Japan Pancreas Society 2011 (JPS 2011) criteria and the International Consensus Diagnostic Criteria (ICDC). ResultsWe analyzed 85 non-neoplastic (definite type 1 AIP in 73/85 based on the ICDC) cases and 64 PDAC cases. IgG4-positive cells were numerous (>10 in 85.9%), and the IgG4/IgG ratios were high (>40% in 81.2%). Plasma cell crushing by an artifact caused unsuccessful immunostaining, notably in smaller samples. Tissue lengths were an important factor for the presence of storiform fibrosis and obliterative phlebitis, but storiform fibrosis was equivocal even in large tissues. A definite or possible histological diagnosis was achieved in 45.9% (39/85) and 41.2% (35/85), respectively, and contributed to the definite final diagnosis of type 1 AIP in 33.3% (ICDC) and 55.6% (JPS 2011) in cases with segmental/focal lesions. In the PDAC group, >10 IgG4-positive cells was rare (2/58), but elastic stains revealed fibrous venous occlusions in 10.3% (6/58). ConclusionsEUS-FNB with large tissue amounts was useful for diagnosing type 1 AIP, notably by facilitating successful IgG4 immunostaining, but definite diagnosis may not be achieved even in cases with large specimens.

The 2020 International Alliance for the Control of Scabies Consensus Criteria for the Diagnosis of Scabies.

SummaryBackgroundScabies is a common parasitic skin condition that causes considerable morbidity globally. Clinical and epidemiological research for scabies has been limited by a lack of standardization of diagnostic methods.ObjectivesTo develop consensus criteria for the diagnosis of common scabies that could be implemented in a variety of settings.MethodsConsensus diagnostic criteria were developed through a Delphi study with international experts. Detailed recommendations were collected from the expert panel to define the criteria features and guide their implementation. These comments were then combined with a comprehensive review of the available literature and the opinion of an expanded group of international experts to develop detailed, evidence‐based definitions and diagnostic methods.ResultsThe 2020 International Alliance for the Control of Scabies (IACS) Consensus Criteria for the Diagnosis of Scabies include three levels of diagnostic certainty and eight subcategories. Confirmed scabies (level A) requires direct visualization of the mite or its products. Clinical scabies (level B) and suspected scabies (level C) rely on clinical assessment of signs and symptoms. Evidence‐based, consensus methods for microscopy, visualization and clinical symptoms and signs were developed, along with a media library.ConclusionsThe 2020 IACS Criteria represent a pragmatic yet robust set of diagnostic features and methods. The criteria may be implemented in a range of research, public health and clinical settings by selecting the appropriate diagnostic levels and subcategories. These criteria may provide greater consistency and standardization for scabies diagnosis. Validation studies, development of training materials and development of survey methods are now required. What is already known about this topic? The diagnosis of scabies is limited by the lack of accurate, objective tests. Microscopy of skin scrapings can confirm the diagnosis, but it is insensitive, invasive and often impractical.Diagnosis usually relies on clinical assessment, although visualization using dermoscopy is becoming increasingly common.These diagnostic methods have not been standardized, hampering the interpretation of findings from clinical research and epidemiological surveys, and the development of scabies control strategies. What does this study add? International consensus diagnostic criteria for common scabies were developed through a Delphi study with global experts.The 2020 International Alliance for the Control of Scabies (IACS) Criteria categorize diagnosis at three levels of diagnostic certainty (confirmed, clinical and suspected scabies) and eight subcategories, and can be adapted to a range of research and public health settings.Detailed definitions and figures are included to aid training and implementation. The 2020 IACS Criteria may facilitate the standardization of scabies diagnosis.

Can the wet suction technique change the efficacy of endoscopic ultrasound-guided fine-needle aspiration for diagnosing autoimmune pancreatitis type 1? A prospective single-arm study.

Other than surgery, endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) is the only procedure for histologically diagnosing autoimmune pancreatitis (AIP). However, adequate specimens are difficult to obtain. Recently, more adequate specimens were reported to be obtained with EUS-FNA with a wet suction technique (WEST) than with conventional EUS-FNA. To histologically diagnose AIP by EUS-FNA with a WEST. Eleven patients with possible type 1 AIP between February 2016 and August 2018 underwent EUS-FNA with a WEST (WEST group), with four punctures by 19 or 22 G needles. As a historical control, 23 type 1 AIP patients who underwent no fewer than four punctures with 19 or 22 G needles were enrolled (DRY group). Patient characteristics and histological findings were compared between the two groups. Three histopathological factors according to the International Consensus Diagnostic Criteria were significantly greater in the WEST group than the DRY group [lymphoplasmacytic infiltrate without granulocytic infiltration: 9 (81.8%) vs 6 (26.1%), P = 0.003, storiform fibrosis: 5 (45.5%) vs 1 (4.3%), P = 0.008, abundant (> 10 cells/HPF) IgG4-positive cells: 7 (63.6%) vs 5 (21.7%), P = 0.026]. Level 1 or level 2 histopathological findings were observed more often in the WEST group than in the DRY group [8 (72.7%) vs 3 (13.0%), P = 0.001]. EUS-FNA with a WEST was more successful than standard EUS-FNA in histologically diagnosing AIP.

Maladie de Castleman multicentrique idiopathique : comment faire le diagnostic ?

Castleman’s disease (CD) is a non clonal lymphoproliferative disease, rare and sometimes life-threatening. It can be an unicentric disease involving only one lymph node area or multicentric, presenting with polyadenopathies. Multicentric CD (MCD) should be divided into three major subtypes : HHV-8 associated MCD, POEMS associated MCD and idiopathic MCD (iMCD). An international evidence-based consensus diagnostic criteria has been set up and iMCD is now considered only after excluding a wide range of malignant, autoimmune, or infectious disorders. It is therefore an exclusion diagnosis. iMCD is based on both a combination of histopathologic elementary lesions and clinicolaboratory criteria. It is therefore not possible to diagnose iMCD only on histopathology without clinical and biological data. This review will underline the important features for the histopathologist.

Comparison of a 22-gauge Franseen-tip needle with a 20-gauge forward-bevel needle for the diagnosis of type 1 autoimmune pancreatitis: a prospective, randomized, controlled, multicenter study (COMPAS study)

Histologic diagnosis of autoimmune pancreatitis (AIP) using EUS-guided FNA (EUS-FNA) is difficult. To address this issue, new fine-needle biopsy (FNB) needles were recently developed. Here, we prospectively evaluated 2 newly designed EUS-FNB needles for histologic evaluation in patients with type 1 AIP. This was a prospective, randomized, multicenter trial comparing biopsy specimens obtained with a 22-gauge Franseen needle or a 20-gauge forward-bevel needle in patients with suspected type 1 AIP. AIP was diagnosed according to international consensus diagnostic criteria. The primary endpoint was the sensitivity of EUS-FNB needles, and secondary endpoints were the amount of specimen obtained, histology of the pancreas based on evaluation of lymphoplasmacytic sclerosing pancreatitis (LPSP), and contribution of histologic findings to the diagnosis of AIP. One hundred ten patients were randomly assigned to the Franseen group (22-gauge Franseen needle) or the forward-bevel group (20-gauge forward-bevel needle). EUS-FNB sampling was successful in all patients. Nine patients were excluded because of diagnoses other than AIP. Compared with the forward-bevel needle, the Franseen needle obtained a significantly greater number of high-power fields. Of 101 patients, 39 patients (78%) in the Franseen group and 23 patients (45%) in the Forward-bevel group were diagnosed with level 1 or 2 LPSP (P= .001). Thirty-six patients could not be diagnosed with type 1 AIP without EUS-FNB specimen results. The 22-gauge Franseen needle should be routinely used for histologic diagnosis of type 1 AIP. (Clinical trial registration number: UMIN 000027668.).

Pancreatic tumor in type 1 autoimmune pancreatitis: a diagnostic challenge

BackgroundThe co-occurrence of type 1 autoimmune pancreatitis (AIP) and pancreatic tumor (PaT) has been previously reported. Pure AIP cases have favorable prognosis and are primarily treated with steroids, while AIP cases with PaT are associated with poor prognosis where the primary management is pancreatic resection. However, it’s a challenge to timely identify the concurrent PaT in AIP because of their similar clinical and radiological manifestations.MethodsWe retrospectively reviewed the data in two medical centers from January 2010 to April 2019. The inclusion criteria were as follows: 1) completion of abdominal CT imaging before invasive procedures to the pancreas, 2) a final diagnosis of type 1 AIP using the 2011 international consensus diagnostic criteria, 3) follow-up duration of at least one month unless AIP and PaT were identified simultaneously. The presence of PaT in AIP was made based on histopathological confirmation, and the absence of PaT in AIP was defined as no pathological or radiological evidence of concurrent PaT. Clinical and radiological characteristics including gender, age, surveillance period, serum IgG4 and Ca-199 levels, biopsy, extrapancreatic involvement, CT and MR (if performed) imaging characteristics were compared between AIP with and without PaT. The Fisher’s exact test was used for qualitative variables, and nonparametric Mann-Whitney test for quantitative variables. A p value ≤0.05 was considered statistically significant.ResultsA total of 74 patients with type 1 AIP were included, of which 5 (6.7%) had the concurrent PaT. The subtypes were pancreatic ductal adenocarcinoma (3/5), solitary extramedullary plasmacytoma in the pancreas (1/5) and cholangiocarcinoma in the pancreatic segment (1/5), respectively. Gender (p = 0.044), the pattern of pancreatic enlargement (p = 0.003), heterogeneity (p = 0.015), low-density (p = 0.004) on CT and rim enhancement on MRI (p = 0.050) differed significantly between AIP with and without PaT. None of the low-density characteristics on CT or other assessed MRI characteristics could significantly differentiate the two groups (p>0.05).ConclusionsFemale, focal pancreatic enlargement, pancreatic heterogeneity, low-density on CT and rim enhancement on MRI are suggestive of the concurrent PaT in type 1 AIP. The characteristics of low-density on CT or other MRI characteristics did not provide further diagnostic values.

Electroconvulsive Therapy for Neuroleptic Malignant Syndrome: A Case Series.

Neuroleptic malignant syndrome (NMS) is an uncommon condition associated with significant morbidity and mortality. Data on treatment interventions are limited. In this case series, we sought to describe all NMS cases requiring ECT from a large academic institution over a nearly 2-decade period. We retrospectively identified all patients with NMS who were treated with ECT over a 17-year period. Patients were included in the study based on chart review using the International Consensus Diagnostic Criteria for NMS. Data were collected related to clinical findings, treatment course, and response to ECT. We identified 15 patients meeting the inclusion criteria. Most patients had neurocognitive or schizophrenia spectrum disorders and developed NMS after exposure to multiple antipsychotic drugs. All patients received bitemporal ECT after failed pharmacotherapy for NMS. Electroconvulsive therapy was well tolerated and resulted in a remission rate of 73.3% (n = 11). Patients showed early initial response to ECT (mean of 4.2 treatments), but an average of 17.7 treatments was necessary to minimize recurrence of catatonic signs. One patient died after interruption of the index course of ECT because of severe infection, and another was discharged to hospice care after limited response. These cases highlight the lethality of NMS and its complications despite aggressive treatment measures. Bitemporal ECT was well tolerated and effective in treating NMS refractory to pharmacotherapy. We suggest that ECT be considered early in cases of NMS that are refractory to pharmacological interventions, especially if the underlying condition is also responsive to ECT.

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

Background/AimsType 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.MethodsType 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.ResultsAmong 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.ConclusionsIn 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.

Clinical time course of pediatric acute disseminated encephalomyelitis

The detailed clinical time course in acute disseminated encephalomyelitis (ADEM) from initial symptoms, through exacerbation, to remission has not been widely reported. Hence, this study aimed to investigate the clinical time course of pediatric ADEM. This was a multicenter retrospective study based on registry data from medical chart reviews. The study included children who met the international consensus diagnostic criteria for ADEM. The patients comprised 18 boys and 6 girls, with a mean age of 5.5 ± 3.3 years at onset. From onset, the time until peak neurological symptoms, time until initial improvement, and time until full recovery was 3.1 ± 3.7 days, 6.0 ± 4.5 days, and 26 ± 34 days, respectively. Twenty-three (96%) patients were treated with high-dose methylprednisolone (mPSL) with a mean duration of 4.1 ± 4.0 days from onset. The condition of 15 patients (65%) improved within 3 days of high-dose mPSL initiation, whereas, that of four patients began to improve after >5 days of high-dose mPSL initiation. Only one patient (4%) did not achieve full recovery despite treatment with high-dose mPSL, intravenous immunoglobulin, and plasma exchange. This study presents the detailed clinical time course in pediatric ADEM in Japan. Progression of neurologic deficits typically lasts a few days, with initial improvement in 1 week leading to full recovery within 1 month.

Kidney Involvement in Patients with Type 1 Autoimmune Pancreatitis.

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, Paraclinical and Imaging Characterization of a Population of Colombian Patients with Neuromyelitis Optica Spectrum Disorder at the Hospital Universitario San Ignacio, Bogotá, Colombia

INTRODUCTION:Neuromyelitis Optica (NMO) is an inflammatory syndrome of the central nervous system, different from multiple sclerosis, that is associated with aquaporin-4 IgG antibodies (AQP4-IgG). The new nomenclature defines a unified term of Neuromyelitis Optica Spectrum Disorder (NMOSD), seropositive or seronegative according to the AQP4-IgG positivity. OBJECTIVES:Demographic, clinical, imaging and cerebrospinal fluid (CSF) cytochemistry characterization of patients diagnosed with NMOSD at the Hospital Universitario San Ignacio (HUSI), Bogotá, Colombia, during 2006-2017. METHODS:A descriptive observational longitudinal study of patients diagnosed with NMO according to the International consensus diagnostic criteria for neuromyelitis optica spectrum disorders 2015 evaluated in HUSI during 2006-2017. An analysis of quantitative variables was performed with mean, standard deviation, median and interquartile range (IQR), and of qualitative variables with absolute numbers and percentages. A Wilcoxon sign-rank sum test was performed for paired data to evaluate the correlation between visual acuity (VA) and EDSS disability scale at admission and discharge after treatment. RESULTS:Data was collected for 37 patients. The mean age was 42 years-old. The form of presentation was optic neuritis (ON) in 81.1% of the cases. In patients who presented as ON, it was typical in 21.6%, atypical in 43.2% and bilateral in 18.9% of them. An average of 4.6 plasmapheresis (PPH) were performed; at discharge 45.9% presented a visual acuity (VA) lower than 20/800. The mean Expanded Disability Status Scale (EDSS) on admission was 2.8 (SD 1,4) and 2.2 (SD: 1,4). at discharge. CONCLUSION:Colombian NMOSD patients have shown an increasingly frequent phenotype variability Including a higher proportion of patients with bilateral optic neuritis, smaller number of patients with oligoclonal bands pattern II and with typical lesions of multiple sclerosis (MS) in MRI with seropositive NMO and a greater number of cases debuting with partial segment partial myelitis.

Kabuki syndrome: international consensus diagnostic criteria

BackgroundKabuki syndrome (KS) is a clinically recognisable syndrome in which 70% of patients have a pathogenic variant in KMT2D or KDM6A. Understanding the function of these genes opens the door...

The role of EUS-guided fine needle aspiration in autoimmune pancreatitis: a single center prospective study

Objectives: Histopathological examination is pivotal in diagnosing autoimmune pancreatitis (AIP). The usefulness of EUS-guided fine needle aspiration (EUS-FNA) in diagnosing AIP remains controversial worldwide. The authors conducted this study to evaluate the efficacy of EUS-FNA for AIP diagnosis using a 22-gauge needle.Methods: Between January 2013 and May 2017, 37 patients had imaging studies suggestive of AIP at Tongji Hospital, and 27 patients of them were enrolled in this study. Tissue specimens acquired through EUS-FNA were analyzed for periductal lymphoplasmacytic infiltrate (LPI), storiform fibrosis (SF), obliterative phlebitis (OP) and immunoglobulin G4 (IgG4)-positive plasma cell counts. Clinical Trials.gov no: TJ-C20121220.Results: LPI and SF were present in 18 (66.67%) and 18 (66.67%) of 27 patients, respectively. Abundant IgG4-positive plasmacyte infiltration >10/high-power field (HPF) was detected in 8 of 27 patients (29.63%). OP and the characteristic findings of idiopathic duct-centric chronic pancreatitis (IDCP) and granulocytic epithelial lesion (GEL) were not detected in this study. According to the International Consensus Diagnostic Criteria (ICDC) for AIP, 5 and 12 of 27 patients were assessed as having level 1 and level 2 histological findings, respectively, suggesting that 17 of 27 patients (62.96%) had lymphoplasmacytic sclerosing pancreatitis (LPSP) based on the ICDC.Conclusions: In 92.6% of patients, pancreatic tissues with >5 HPFs were obtained by EUS-FNA using a 22-G needle. In 63% of patients, histology was evaluated to be ≥ level 2 according to the ICDC. The study indicates that EUS-FNA with a 22-G needle is valuable in the histopathological diagnosis of AIP.

Serum and histological IgG4-negative type 1 autoimmune pancreatitis

A 66-year-old man who was on oral medication for type 2 diabetes experienced a rapid decline in glycemic control (increase in glycosylated hemoglobin level from 7.7 to 10.2% over 3months). Abdominal ultrasonography revealed a 20-mm hypoechoic mass in the pancreatic tail. Serum tumor marker carbohydrate antigen 19-9 and DUPAN2 levels were within the respective normal ranges; serum IgG4 level was also normal at 21.8mg/dL. Abdominal contrast computed tomography revealed a 26-mm tumor in the pancreatic tail. Magnetic resonance cholangiopancreatography revealed disruption of the main pancreatic duct and dilation of the caudal pancreatic duct. Endoscopic ultrasonography revealed a near-round-shaped hypoechoic mass with interspersed hyperechoic areas. Endoscopic ultrasonography-guided fine needle aspiration was performed using a 22-G needle, but no malignant findings were observed. There were no signs of sialadenitis, retroperitoneal fibrosis, nephropathy, or other conditions associated with IgG4-related diseases. Distal pancreatectomy was performed; a 23-mm white mass was resected from the pancreatic tail. A histopathological examination showed advanced inflammatory cell infiltration mainly involving lymphocytes/plasma cells along with storiform fibrosis and obliterative phlebitis. No more than five IgG4-positive cells were observed per high-power field. These were level 1 pathological findings, and a definitive diagnosis of type 1 autoimmune pancreatitis (AIP) was made according to the International Consensus Diagnostic Criteria. Type 1 AIP associated with normal serum IgG4 levels and absence of IgG4-positive cells on histological examination is a rare clinical entity, which is very difficult to distinguish from pancreatic cancer. Here we report such a case and present a review of the relevant literature.

Type 2 autoimmune pancreatitis (or IDCP): an australian retrospective epidemiological analysis and review of the current ICDC criteria

Introduction: Type 2 autoimmune pancreatitis (or idiopathic duct-centric chronic pancreatitis) is a rare, benign disease with inherent challenges in diagnosis. The objective of this study was to analyse the clinical epidemiology of this disease and to assess validity of diagnostic criteria. Method: Five patients, retrospectively collected over 5-years, that met the ‘probable’ or ‘definitive’ current International Consensus Diagnostic Criteria (ICDC), for type 2 autoimmune pancreatitis were included. Results: Two patients, both female aged 16 and 19, met the definitive criteria. Three patients, two male and one female, aged 26-61, met the probable criteria. Four patients had pre-existing inflammatory bowel disease. Four patients had focal changes or demonstrable mass on endoscopic ultrasound (EUS) or cross-sectional imaging (CT or MRCP). Of this group, one patient underwent surgery (laparoscopic distal pancreatectomy and splenectomy) for a malignant-appearing lesion in the pancreatic body with specimen histology confirming type 2 AIP. A total of three patients began and responded well to steroid and/or other immunosuppressant therapy. None of the patients have experienced relapse in their disease post-medical or surgical management at 6-24 months follow-up. Conclusions: The epidemiology of type 2 autoimmune pancreatitis is distinctly different to the more sinister pathologies that it may mimic. Clinicians must be aware of this fact in their approach to the serum IgG4-negative patient with recurrent pancreatitis, particularly in the young, female demographic and in the presence of co-existing IBD. There is value in diagnostic rubrics in distinguishing this disease.

Summary of the updated international consensus diagnostic criteria for eosinophilic esophagitis: AGREE conference

Summary of the updated international consensus diagnostic criteria for eosinophilic esophagitis: AGREE conference

Short transverse myelitis in Chinese patients with neuromyelitis optica spectrum disorders

BackgroundShort transverse myelitis (STM) is considered uncommon in neuromyelitis optica spectrum disorders (NMOSD). Poor recognition of STM occurring in NMOSD may lead to increased delay in diagnosis and appropriate treatment. ObjectivesThe aim of this study was to assess the frequency and characteristics of STM in Chinese patients with NMOSD. MethodsWe enrolled 91 patients with NMOSD based on the 2015 International Consensus Diagnostic Criteria for NMOSD. The patients were divided into STM group and longitudinally extensive transverse myelitis (LETM) group according to the length of initial spinal cord lesions at the initial myelitis manifestation of NMOSD. ResultsInitial STM was observed in 18 patients (18/91, 19.8%). The STM episode was the first manifestation of NMOSD in 9 patients (50%) and preceded by optic neuritis in 3 patients (16.7%), area postrema syndrome in 5(27.8%) and brainstem syndrome in 1(5.6%). Compared to the NMOSD patients with an initial LETM, patients with STM suffered less motor and bowel or bladder disability, had minor EDSS at clinical onset, but suffered earlier relapse (P＜.05). Thirteen patients had single short spinal lesion (13/18, 72.2%) and 5 patients had two short lesions. Of the 23 STM lesions, 4 lesions spanned 2.5 vertebral segments, 12 showed a length of continuous 2 vertebral segments, 7 were confined to single vertebral segment. The lesions on axial imaging involved the central grey matter in 61.1% (11/18) patients with STM and in 95.9%（70/73）patients with LETM (P＜.05). Both the patients with STM（50%）and LETM (34.2%) had brain lesions. ConclusionsInitial STM does not exclude consideration of NMOSD diagnosis.

Relapse rate and predictors of relapse in a large single center cohort of type 1 autoimmune pancreatitis: long-term follow-up results after steroid therapy with short-duration maintenance treatment.

Type 1 autoimmune pancreatitis (AIP), as a pancreatic manifestation of IgG4-related disease, shows a favorable prognosis in the short term. However, disease relapse is common in long-term follow-up, despite a successful initial treatment response. This study aimed to identify the predictors of relapse and long-term outcomes in patients with type 1 AIP. Patients with more than 2years of follow-up who met the International Consensus Diagnostic Criteria for type 1 AIP were included. Patients who had undergone pancreatic operations associated with AIP or who lacked sufficient clinical data were excluded. All 138 patients achieved clinical remission with initial steroid therapy, and 66 (47.8%) experienced relapse during a median 60 (range 24-197) months follow-up. Among the relapsed patients, about 74% (49/66) relapsed within 3years. About 60% (82/138) had other organ involvement (OOI), most commonly in the proximal bile duct (26.8%). At first diagnosis, OOI, and especially OOI of the proximal bile duct, was a significant independent predictor of relapse (hazard ratio 2.65; 95% confidence interval 1.44-4.89; p=0.002), according to multivariate analysis. During the follow-up period, 16 (11.6%) patients experienced endocrine/exocrine dysfunction and 32 (23.2%) patients developed de novo pancreatic calcifications/stones. No pancreatic cancer occurred in any patients. Type 1 AIP has common relapses, and patients with OOI, especially OOI of the proximal bile duct, appear to be at increased risk for relapse. Long-term sequelae, including pancreatic insufficiency and pancreatic calcifications/stones, are common in patients with relapse. To reduce the relapse, longer maintenance treatment may be needed especially for patients at high risk for relapse.

IgG4-Unrelated Type 1 Auto Immune Pancreatitis Manifesting with Pancreatic Head Mass Mimicking Cancer

Auto immune pancreatitis (AIP) is an uncommon form of chronic pancreatitis that has been divided into type 1 and type 2 which have distinct histopathology and clinical features. Type 1 AIP seems to be the pancreatic manifestation of an IgG4-related systemic disease, characterized by elevated IgG4 serum levels, infiltration of IgG4-positive plasma cells and extrapancreatic lesions. When manifesting as a focal disease in the pancreas, it can be challenging to differentiate it from pancreatic cancer (PC). Because AIP is typically responsive to steroid therapy without the need for resection, differentiation between these two diseases is critical. We report the case of a 56-year-old man with initial suspect of PC, and final diagnosis of type 1 AIP according to the International Consensus Diagnostic Criteria (ICDC) without elevated levels of serum IgG4 or need for a histology sample. We take a review of literature in order to improve the diagnostic accuracy of AIP and describe clinical and imaging features to differentiate it from PC and avoid unnecessary surgery due to misdiagnosis.