Pursuing living donor liver transplantation improves outcomes of patients with autoimmune liver diseases: An intention-to-treat analysis.

Living donor liver transplantation (LDLT) offers the opportunity to decrease waitlist time and mortality for patients with autoimmune liver disease (AILD), autoimmune hepatitis, primary biliary cholangitis, and primary sclerosing cholangitis. We compared the survival of patients with a potential living donor (pLDLT) on the waitlist versus no potential living donor (pDDLT) on an intention-to-treat basis. Our retrospective cohort study investigated adults with AILD listed for a liver transplant in our program between 2000 and 2021. The pLDLT group comprised recipients with a potential living donor. Otherwise, they were included in the pDDLT group. Intention-to-treat survival was assessed from the time of listing. Of the 533 patients included, 244 (43.8%) had a potential living donor. Waitlist dropout was higher for the pDDLT groups among all AILDs (pDDLT 85 [29.4%] vs. pLDLT 9 [3.7%], p < 0.001). The 1-, 3-, and 5-year intention-to-treat survival rates were higher for pLDLT versus pDDLT among all AILDs (95.7% vs. 78.1%, 89.0% vs. 70.1%, and 87.1% vs. 65.5%, p < 0.001). After adjusting for covariates, pLDLT was associated with a 38% reduction in the risk of death among the AILD cohort (HR: 0.62, 95% CI: 0.42-0.93 [ p <0.05]), and 60% among the primary sclerosing cholangitis cohort (HR: 0.40, 95% CI: 0.22-0.74 [ p <0.05]). There were no differences in the 1-, 3-, and 5-year post-transplant survival between LDLT and DDLT (AILD: 95.6% vs. 92.1%, 89.9% vs. 89.4%, and 89.1% vs. 87.1%, p =0.41). This was consistent after adjusting for covariates (HR: 0.97, 95% CI: 0.56-1.68 [ p >0.9]). Our study suggests that having a potential living donor could decrease the risk of death in patients with primary sclerosing cholangitis on the waitlist. Importantly, the post-transplant outcomes in this population are similar between the LDLT and DDLT groups.

Breast Implant Illness as a Clinical Entity: A Systematic Review of the Literature.

BACKGROUND: Breast implant illness (BII) has become a contentious subject in recent years. Although some studies have reported associations between breast implants and autoimmune diseases, others have failed to establish a definitive link. OBJECTIVES: The objective of this study was to provide a comprehensive, up-to-date evaluation of the literature surrounding BII, with an emphasis on identifying patient-related factors that may be associated with BII. METHODS: A systematic review was performed following PRISMA guidelines by searching the PubMed (MEDLINE), Embase, and Cochrane databases for relevant studies published in the last 20 years. RESULTS: Thirty-one studies were included, which covered 39,505 implant patients with a mean [standard deviation] age of 44.2 [9.30] years. Fifteen studies reported implant explantation status, with 72.4% patients choosing to remove their implants. Among these, 9 studies reported symptom improvement in 83.5% patients. Fifty-three percent of patients undergoing explantation had total capsulectomy. Twenty-eight studies documented total numbers of patients experiencing symptoms related to BII, with 31.3% patients reporting such symptoms. Among these, 16 studies of 4109 BII patients distinguished whether the reason for implantation was cosmetic augmentation or reconstruction. When specified, more patients experiencing BII-related symptoms received implants for "cosmetic" vs "reconstructive" reasons (cosmetic, 3864/4109 [94.0%] vs reconstruction, 245/4109 [5.96%]; P < .001). CONCLUSIONS: This review provides an overview of the current state of knowledge regarding BII. The study highlights a potential relationship between BII and indication for implants (cosmetic vs reconstructive) among other variables, offering valuable insight on factors associated with BII and directions for future research.

Clinicopathological study of surgically treated non-neoplastic diseases of the pancreas with special reference to autoimmune pancreatitis.

PURPOSE: After the popularization of serum immunoglobulin G4 (IgG4) measurement and endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) in our institute, surgical resection for non-neoplastic diseases of the pancreas became less common. Although the incidence of such false-positive cases was clarified in the 10-year period after the introduction of these measures (2009-2018), these data were not compared with the 30 years before 2009 (1979-2008). This study was performed to determine the percentage of autoimmune pancreatitis (AIP) that was included during the latter period and how the numbers of false-positive cases differed between the two periods. METHODS: From 1979 to 2008, 51 patients had clinical suspicion of pancreatic carcinoma (false-positive disease). Among these 51 patients, 32 non-alcoholic patients who had tumor-forming chronic pancreatitis (TFCP) were clinically, histologically, and immunohistochemically compared with 11 patients who had TFCP during the latter 10-year period. RESULTS: Retrospective IgG4 immunostaining of false-positive TFCP revealed 14 (35.0%) cases of AIP in the former 30 years versus 5 (45.5%) in the latter 10 years. There were 40 (5.9%) cases of TFCP among 675 patients in the former 30 years and 11 (0.9%) among 1289 patients in the latter 10 years. CONCLUSIONS: When the TFCP ratio of pancreatic resections and the AIP ratio of false-positive TFCPs were compared between the two periods, the TFCP ratio was 5.9% versus 0.9% and the AIP ratio was 35.0% versus 45.5%, respectively. It can thus be speculated that IgG4 measurement and EUS-FNA are absolutely imperative for the diagnosis of TFCP.

Surgery in Autoimmune Pancreatitis.

INTRODUCTION: Autoimmune pancreatitis (AIP) is a disease that may mimic malignant pancreatic lesions both in terms of symptomatology and imaging appearance. The aim of the present study is to analyze experiences of surgery in patients with AIP in one of the largest European cohorts. PATIENTS AND METHODS: We performed a single-center retrospective study of patients diagnosed with AIP at the Department of Abdominal Diseases at Karolinska University Hospital in Stockholm, Sweden, between January 2001 and October 2020. RESULTS: There were 159 patients diagnosed with AIP, and among them, 35 (22.0%) patients had surgery: 20 (57.1%) males and 15 (42.9%) females; median age at surgery was 59 years (range 37-81). Median follow-up period after surgery was 50 months (range 1-235). AIP type 1 was diagnosed in 28 (80%) patients and AIP type 2 in 7 (20%) patients. Malignant and premalignant lesions were diagnosed in 8 (22.9%) patients for whom AIP was not the primary differential diagnosis, but in all cases, it was described as a simultaneous finding and recorded in retrospective analysis in histological reports of surgical specimens. CONCLUSIONS: Diagnosis of AIP is not always straightforward, and in some cases, it is not easy to differentiate it from the malignancy. Surgery is generally not indicated for AIP but might be considered in patients when suspicion of malignant/premalignant lesions cannot be excluded after complete diagnostic workup.

The mechanisms of cellular crosstalk between mesenchymal stem cells and natural killer cells: Therapeutic implications.

Mesenchymal stem cells (MSCs) are mesenchymal precursors of various origins, with well-known immunomodulatory effects. Natural killer (NK) cells, the major cells of the innate immune system, are critical for the antitumor and antiviral defenses; however, in certain cases, they may be the main culprits in the pathogenesis of some NK-related conditions such as autoimmunities and hematological malignancies. On the other hand, these cells seem to be the major responders in beneficial phenomena like graft versus leukemia. Substantial data suggest that MSCs can variably affect NK cells and can be affected by these cells. Accordingly, acquiring a profound understanding of the crosstalk between MSCs and NK cells and the involved mechanisms seems to be a necessity to develop therapeutic approaches based on such interactions. Therefore, in this study, we made a thorough review of the existing literature on the interactions between MSCs and NK cells with a focus on the underlying mechanisms. The current knowledge herein suggests that MSCs possess a great potential to be used as tools for therapeutic targeting of NK cells in disease context and that preconditioning of MSCs, as well as their genetic manipulation before administration, may provide a wider variety of options in terms of eliciting more specific and desirable therapeutic outcomes. Nevertheless, our knowledge regarding the effects of MSCs on NK cells is still in its infancy, and further studies with well-defined conditions are warranted herein.

Role of tonsillectomy and adenoidectomy in parental satisfaction of treatments for PANDAS.

OBJECTIVE: To examine caregiver satisfaction with treatments for pediatric autoimmune neuropsychiatric disorder associated with streptococcus (PANDAS) and how symptom frequency changes over time. METHODS: A list was created for PANDAS subjects seen at the Georgetown Pediatric Otolaryngology clinic from 2015 to 2018. Questionnaires were distributed to caregivers able to be contacted; 62% responded (n = 60). Subjects were placed in groups based on treatments reported: tonsillectomy and adenoidectomy (T&A, n = 28), T&A and intravenous immunoglobulin (IVIG, n = 22), or nonsurgical treatment(s) (n = 10). Caregivers reported frequencies for each of 10 associated symptoms from time of treatment to 12 months and also expressed their satisfaction with treatment. RESULTS: Patients were treated with antibiotics (n = 60, 100%), T&A (83.3%), IVIG (40%), Rituximab (15%), steroids (20%), and/or plasma exchange (10%). Caregivers for 66% (n = 33) of surgical patients identified T&A as the most effective treatment, and 80% would choose the operation again. No difference in median caregiver satisfaction level was found among the groups (n = 0.196). There was no significant difference in frequency for any of the symptoms (all p > 0.05) except choreiform movement (p = 0.0296). CONCLUSION: Caregivers reported a decreasing frequency of symptoms over time regardless of treatment and had no difference in satisfaction. T&A was the most preferred treatment and the most impactful on symptoms for surgical patients. Given the challenges of immunologic therapies, T&A in combination with antibiotics should be considered as an early intervention for PANDAS.

Comparison of whole-blood tacrolimus concentrations measured by different immunoassay systems.

INTRODUCTION: Different measured values for tacrolimus were obtained with different automated immunoassays. We aimed to examine the differences in the blood tacrolimus concentrations measured by the major immunoassay systems commercially available in Japan. METHODS: Whole-blood samples from 118 patients were assayed by 3 commercial assays: chemiluminescent enzyme immunoassay (CLIA), affinity column-mediated immunoassay (ACMIA), and enzyme-multiplied immunoassay technique (EMIT). Liquid chromatography-tandem mass spectrometry (LC-MS/MS) was used for reference. KEY FINDINGS: The correlation coefficient of immunoassay vs LC-MS/MS was excellent for ACMIA (.83) and CLIA (.81) and good for EMIT (.71). The mean error was negative for ACMIA and positive for CLIA and EMIT. The mean absolute error and root-mean-square error were almost the same for ACMIA and CLIA and lower than those for EMIT. CONCLUSIONS: The ACMIA and CLIA yield considerably better results than the EMIT for monitoring blood tacrolimus concentrations.

Fcγ receptor expression on splenic macrophages in adult immune thrombocytopenia.

Splenic macrophages play a key role in immune thrombocytopenia (ITP) pathogenesis by clearing opsonized platelets. Fcγ receptors (FcγR) participate in this phenomenon, but their expression on splenic macrophages and their modulation by treatment have scarcely been studied in human ITP. We aimed to compare the phenotype and function of splenic macrophages between six controls and 24 ITP patients and between ITP patients according to the treatments they received prior to splenectomy. CD86, human leucocyte antigen D-related (HLA-DR) and FcγR expression were measured by flow cytometry on splenic macrophages. The major FcγR polymorphisms were determined and splenic macrophage function was assessed by a phagocytosis assay. The expression of the activation markers CD86 and HLA-DR was higher on splenic macrophages during ITP compared to controls. While the expression of FcγR was not different between ITP and controls, the phagocytic function of splenic macrophages was reduced in ITP patients treated with intravenous immunoglobulin (IVIg) within the 2 weeks prior to splenectomy. The FCGR3A (158V/F) polymorphism, known to increase the affinity of FcγRIII to IgG, was over-represented in ITP patients. Thus, these are the first results arguing for the fact that the therapeutic use of IVIg during human chronic ITP does not modulate FcγR expression on splenic macrophages but decreases their phagocytic capabilities.

[A Case of an IgG4-Related Inflammatory Pseudotumor of the Liver Showing Enlargement That Was Difficult to Differentiate from Hepatic Cancer].

A 60-year-old man was admitted for a liver mass(S3), which rapidly increased in size during intraductal papillary mucinous neoplasm(IPMN)follow-up. Although EOB-MRIwas performed, the mass could not be accurately diagnosed as hepatic cancer. Thus, we performed a lateral segmentectomy. In the resected specimen, a solid tumor mass was clearly bound in segment 3 of the liver. Since histopathology revealed no malignant cells and many IgG4-positive cells, we confirmed the diagnosis as IgG4-related inflammatory pseudotumor of the liver. IgG4-related diseases occur in various organs in the body, and they are known to associate with autoimmune pancreatitis and sclerosing cholangitis, but an IgG4-related inflammatory pseudotumor of the liver is a rare disease. It is often difficult to distinguish from hepatic cancer and surgical resection is performed.

First-Degree Living-Related Donor Liver Transplantation in Autoimmune Liver Diseases.

Liver transplantation (LT) is the treatment of choice for end-stage autoimmune liver diseases. However, the underlying disease may recur in the graft in some 20% of cases. The aim of this study is to determine whether LT using living donor grafts from first-degree relatives results in higher rates of recurrence than grafts from more distant/unrelated donors. Two hundred sixty-three patients, who underwent a first LT in the Toronto liver transplant program between January 2000 and March 2015 for autoimmune liver diseases, and had at least 6 months of post-LT follow-up, were included in this study. Of these, 72 (27%) received a graft from a first-degree living-related donor, 56 (21%) from a distant/unrelated living donor, and 135 (51%) from a deceased donor for primary sclerosing cholangitis (PSC) (n = 138, 52%), primary biliary cholangitis (PBC) (n = 69, 26%), autoimmune hepatitis (AIH) (n = 44, 17%), and overlap syndromes (n = 12, 5%). Recurrence occurred in 52 (20%) patients. Recurrence rates for each autoimmune liver disease were not significantly different after first-degree living-related, living-unrelated, or deceased-donor LT. Similarly, time to recurrence, recurrence-related graft failure, graft survival, and patient survival were not significantly different between groups. In conclusion, first-degree living-related donor LT for PSC, PBC, or AIH is not associated with an increased risk of disease recurrence.

Successful treatment of murine autoimmune cholangitis by parabiosis: Implications for hematopoietic therapy.

There is a significant unmet need in the treatment of primary biliary cirrhosis (PBC) despite significant data on the effector pathways that lead to biliary duct damage. We focused attention on a murine model of PBC, the dominant negative transforming growth factor ß receptor II (Tg) mice. To further define the pathways that lead to biliary pathology in these mice, we developed Tg mice deleted of CD4 cells (CD4(-/-)Tg). Interestingly, these mice developed more severe cholangitis than control Tg mice. These mice, which lack CD4 cells, manifested increased levels of IFN-γ produced by effector CD8 cells. It appears that increased cholangitis is due to the absence of CD4 Treg cells. Based on these data, we parabiosed CD4(-/-)Tg mice with established disease at 8-9 weeks of age with C57BL/6 control mice. Such parabiotic "twins" had a significant reduction in autoimmune cholangitis, even though they had established pathology at the time of surgery. We prepared mixed bone marrow chimera mice constructed from CD4(-/-)Tg and CD8(-/-) mice and not only was cholangitis improved, but a decrease in terminally differentiated CD8(+) T effector cells in the presence of wild type CD4 cells was noted. In conclusion, "correcting" the CD4 T cell subset, even in the presence of pathogenic CD8 T cells, is effective in treating autoimmune cholangitis.

Resetting the immune system with immunoablation and autologous haematopoietic stem cell transplantation in autoimmune diseases.

Over the past 20 years, immunoablation followed by transplantation of autologous haematopoietic stem cells (ASCT) has emerged as a promising treatment option for patients with severe forms of autoimmune diseases (ADs) that insufficiently respond to standard immunosuppressive or novel biologic treatment. Meanwhile, mechanistic studies have provided the proof-of-concept that the long-term, treatment-free remissions achieved by ASCT are associated with the eradication of the autoreactive immunologic memory and a fundamental reconfiguration of the immune system. The latter comprises regeneration of naive B cells and a stable thymic reactivation with re-emergence of thymic-derived naive T cells, including Foxp3+ regulatory T cells, with new antigen receptors, i.e. immune reset. In this article, we discuss mechanistic studies that investigated how such immune renewal after ASCT may rewire a faulty immune system in ADs into a self-tolerant state, to induce long-term remissions.

Cardiac Mass, Aortic Intramural Hematoma, and IgG4-related Disease: A Case Report.

As a designated entity within medicine, immunoglobulin G4 (IgG4)-related disease is relatively new. It is immune-mediated origin, characterized by a tendency for formation of tumefactive lesions, the infiltration of IgG4-positive plasma cells, and frequent but not invariable elevations of IgG4 levels in the serum. IgG4-related cardiac mass accompanying aortic intramural hematoma is an extremely rare clinical presentation. Herein we present the case of a patient who was admitted to our department complaining of severe chest pain. Computed tomographic angiography examination revealed a cardiac mass accompanying an aortic intramural hematoma. He underwent a surgical resection of the cardiac mass and a replacement of the ascending aorta with Hemashield Platinum graft and made an uneventful recovery. A diagnosis of an IgG4-related disease was made based on laboratory results and pathological examination. Corticosteroids were administered postoperatively. This case shows that the heart itself can also be a potential site for IgG4-related disease.

Immunoglobulin G4-related disease presenting as bilateral ovarian masses and mimicking advanced ovarian cancer.

Immunoglobulin G4-related disease (IgG4-RD) is characterized by extensive infiltration of IgG4(+) plasma cells and fibrosis in various organs. However, the involvement of the ovary in IgG4-RD has never been reported. A 59-year-old woman presented with urinary retention. Magnetic resonance imaging and computed tomography revealed a huge multinodular pelvic mass and common iliac/para-aortic lymph node swelling. A laparotomy was performed under the suspicion of advanced ovarian cancer, and the pelvic mass was identified as ovary in origin. Histopathology of the excised tumor revealed massive lymphoplasmacytic infiltration (>90% were IgG4(+) plasma cells), storiform fibrosis, and obliterative phlebitis; thus leading to a diagnosis of IgG4-RD. We conclude that IgG4-RD can present as a bilateral ovarian mass along with lymphadenopathy, therefore mimicking ovarian cancer.

Immunoglobulin G4-related disease of the orbital cavity, cervical lymph nodes and greater auricular nerve: case report.

IgG4-related disease (IgG4-RD) is a novel clinicopathological entity characterised by elevated tissue levels of IgG4-positive plasma cells. It can present in almost every organ systems. We present a case of a 48year-old man with recurrent intra-orbital and cervical lymph node swelling and found to have greater auricular nerve involvement intraoperatively during open surgical biopsy. Histopathological evaluation of biopsied specimens from these lesions yielded IgG4-positive plasma cell infiltration on immunohistochemistry. Key pathological features such as prominent lymphoplasmacytic population, storiform fibrosis and obliterative phlebitis were also seen. A diagnosis of IgG4-RD was made. Oral prednisone therapy ameliorated the symptoms and patient remained in remission at followup. Literature review indicated that IgG4-RD is a rare condition that seldom occurs concurrently in the orbital cavity, cervical lymph nodes and involving the greater auricular nerve. The condition may often masquerade as malignancy or infection due to formation of tumefactive lesions but tend to respond favourably to glucocorticoid or immunosuppressants. The differential diagnosis of unusual mass lesions in these locations should include IgG4-RD. The otolaryngologist, as well as other health professionals, should be familiar with this novel disease to ensure timely diagnosis and treatment.

[Tonsillectomy for PANDAS?]. / Tonsillektomie bei PANDAS?

BACKGROUND: Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) is a disease attributed to children with obsessive compulsive or tic disorders associated with streptococcal infections. Because otolaryngologists evaluate a large number of pediatric patients with recurrent streptococcal infections, tonsillectomy (TE) is a common option of therapy. This study was undertaken to evaluate the efficacy of tonsillectomy in patients presenting with a verified PANDAS. MATERIAL AND METHODS: A PubMed research was performed using search terms "tonsillectomy" and "PANDAS", "OCD", "compulsive", "pediatric autoimmune", "Chorea" and "Tic" limited by publication date January 1, 1995 to July 31, 2015. Reviews without patients were not included in the review. RESULTS: Nine papers matched our search terms, including 6 case reports with 8 patients and 3 case series. Most case reports were in favor for TE, but this was by far not supported by the findings in the case series. The follow-up ranged form 2-36 months (case reports) and 24-36 months (case series). CONCLUSIONS: establishing the diagnosis of PANDAS is complicated by underlying co-morbidities in the field of neurology-psychiatry and the lack of a reliable biomarker. The positive outcome after TE as reported in case studies may be influenced by the postoperative medication and is not supported by the results of large-scale studies. In the light of the considerable postoperative morbidity and unavoidable mortality rate it appears wise to indicate TE only in clinical studies with standardized inclusion criteria.

[Endoscopic Endonasal Transethmoidal Biopsy for IgG4-Related Intraorbital Pseudotumor:A Case Report].

BACKGROUND: We experienced a case of intraorbital pseudotumor associated with IgG4-related disease, for which we successfully performed an endoscopic endonasal transethmoidal biopsy for the intraorbital pseudotumor as well as endoscopic sinus surgery for a refractory pansinusitis at the same time. CASE REPORT: A 59-year-old man was referred to our hospital because of an intraorbital mass lesion. MRI showed 2 mass lesions:a large intraconal lesion encasing the left optic nerve on the orbital apex, and a small extraconal lesion medial to the left medial rectus muscle extending into the anterior ethmoid canal. In addition, CT showed severe pansinusitis. A blood test showed a marked elevation of IgG4. IgG4-related pseudotumor was suspected, but IgG4-related MALT lymphoma was an alternative diagnosis, and a biopsy of the mass lesion was required. We successfully performed both, an endoscopic endonasal transethmoidal biopsy for the mass lesion and endoscopic sinus surgery for the refractory pansinusitis at the same time. The pathological diagnosis was an IgG4-related pseudotumor. CONCLUSION: Endoscopic endonasal transethmoidal biopsy is an effective and minimally invasive method for making a definitive diagnosis of IgG4-related intraorbital pseudotumor. Using this method, refractory pansinusitis frequently associated with this disease can be treated. For successful treatment, interdisciplinary decision making and collaborative team surgery are crucial.

Recipient factors associated with having a potential living donor for liver transplantation.

Because of a persistent discrepancy between the demand for liver transplantation (LT) and the supply of deceased donor organs, there is an interest in increasing living donation rates at centers trained in this method of transplantation. We examined a large socioeconomically heterogeneous cohort of patients listed for LT to identify recipient factors associated with living donation. We retrospectively reviewed 491 consecutive patients who were listed for LT at our center over a 24-month period. Demographic, medical, and socioeconomic data were extracted from electronic records and compared between those who had a potential living donor (LD) volunteer for assessment and those who did not; 245 patients (50%) had at least 1 potential LD volunteer for assessment. Multivariate logistic regression analysis identified that patients with a LD were more likely to have Child-Pugh C disease (odds ratio [OR], 2.44; P = 0.02), and less likely to be older (OR, 0.96; P = 0.002), single (OR, 0.34; P = 0.006), divorced (OR, 0.53; P = 0.03), immigrants (OR, 0.38; P = 0.049), or from the lowest income quintile (OR, 0.44; P = 0.02). In conclusion, this analysis has identified several factors associated with access to living donation. More research is warranted to define and overcome barriers to living donor liver transplantation through targeted interventions in underrepresented populations.

Unnecessary Procedures and Surgery in Autoimmune Pancreatitis.

BACKGROUND/AIMS: To identify the number and potential causes of unnecessary diagnostic procedures in a cohort of patients with autoimmune pancreatitis (AIP). METHODS: All AIP cases at our centre between April 2006 and April 2013 were collected and followed up. Diagnosis was established by the International Consensus Diagnostic Criteria (ICDC). Demographic, clinical, radiological, serological data, the number of diagnostic procedures and the reason for referral were recorded. Possible risk factors for a delayed diagnosis of AIP were analysed. RESULTS: A total of 29 patients (median age 60 years; 22 males and 7 females) were diagnosed with AIP using ICDC. Twenty-five patients were diagnosed with definite, 2 with possible type 1 AIP and 2 with type 2 AIP. In 29 patients, 50 ERCPs and 18 EUS were carried out; based on ICDC recommendations, a total of 20 ERCPs and 4 EUS were unnecessary diagnostic procedures. Eight patients (23.0%) were referred for unnecessary surgery. Jaundice was shown to be a significant risk factor for unnecessary endoscopic investigations (OR 11.00, 95% CI 1.14-106.43, p = 0.04). CONCLUSION: Diagnosis of AIP still remains a challenge. Patients with jaundice are at particular risk of being subjected to unnecessary endoscopic procedures. Use of ICDC would help avoid unnecessary examinations or even major surgeries at times.

[An IgG4-related pancreatitis mimicking an adenocarcinoma: A case report]. / Une pancréatite auto-immune à IgG4 simulant un adénocarcinome : à propos d'un cas.

Type 1 auto-immune pancreatitis (type 1 AIP) is the pancreatic manifestation of IgG4-related systemic disease (IgG4-RD). This disease has recently been individualized and is characterized by elevated serum IgG4 levels and extrapancreatic lesions with common histologic characteristic: dense infiltration of lymphocytes, IgG4-positive plasma cells and storiforme fibrosis. Obliterative phlebitis is frequently detected. The pancreas is frequently involved in this disease. As approach to the pancreas for histological examination is generally difficult, AIP is diagnosed using a combination of clinical, serological, morphological and histopathological features. In pseudotumoral cases, AIP can be misdiagnosed as pancreatic cancer. Since AIP responds dramatically to steroid therapy, accurate diagnosis of AIP can avoid unnecessary laparotomy or pancreatic resection. We report here a case of a patient who underwent surgery for presumed pancreatic cancer. The final diagnosis was type 1 AIP.