Autoimmune cholangitis mimicking a klatskin tumor: a case report

<p>Abstract</p> <p>Introduction</p> <p>Autoimmune cholangitis remains an elusive manifestation of immunoglobulin G4-associated systemic disease most commonly encountered in patients with autoimmune pancreatitis. No strict diagnostic criteria have been described to date and diagnosis mainly relies on a combination of clinical and histopathologic findings. It is hence even more challenging to diagnose autoimmune cholangitis in patients with late or atypical presentations, such as without concomitant pancreatic involvement. Early diagnosis of this rare disorder can significantly improve outcomes considering high rates of surgical intervention, as well as high relapse rates in the absence of steroid treatment. To the best of our knowledge the literature is quite sparse on cases with atypical presentations of autoimmune cholangitis.</p> <p>Case presentation</p> <p>We report a case of a previously healthy 65-year-old man of Middle-Eastern origin, with a history of pancreatic insufficiency of unknown etiology, evaluated for elevated liver function tests found incidentally on a routine physical examination. Imaging studies revealed an atrophic pancreas and biliary duct dilatation consistent with obstruction. Subsequent endoscopic retrograde cholangiopancreatography showed a bile duct narrowing pattern suggestive of cholangiocarcinoma, but brushings failed to reveal malignant cells. Our patient proceeded to undergo surgical resection. Histological examination of the resected mass revealed lymphoplasmacytic infiltrate with no malignant features. Our patient returned three months later with persistently high liver function tests and no evidence of biliary obstruction on imaging. A presumptive diagnosis of autoimmune cholangitis was made and our patient's symptoms resolved after a short course of an oral steroid regimen. Post factum staining of the resection specimen revealed an immunoglobulin G4 antibody positive immune cell infiltrate, consistent with the proposed diagnosis.</p> <p>Conclusion</p> <p>Our case thus highlights the importance of clinician awareness of the autoimmune spectrum of biliary pathologies when confronted with atypical clinical presentations, the paucity of diagnostic measures and the benefit from long-term steroid and/or immunosuppressive treatment.</p

Successful treatment of pediatric IgG4 related systemic disease with mycophenolate mofetil: case report and a review of the pediatric autoimmune pancreatitis literature

Autoimmune pancreatitis is frequently associated with elevated serum and tissue IgG4 levels in the adult population, but there are few reports of pediatric autoimmune pancreatitis, and even fewer reports of IgG4 related systemic disease in a pediatric population. The standard of care treatment in adults is systemic corticosteroids with resolution of symptoms in most cases; however, multiple courses of corticosteroids are occasionally required and some patients require long term corticosteroids. In these instances, steroid sparing disease modify treatments are in demand. We describe a 13-year-old girl with IgG4 related systemic disease who presented with chronic recurrent autoimmune pancreatitis resulting in surgical intervention for obstructive hyperbilirubinemia and chronic corticosteroid treatment. In addition, she developed fibrosing medianstinitis as part of her IgG4 related systemic disease. She was eventually successfully treated with mycophenolate mofetil allowing for discontinuation of corticosteroids. This is the first reported use of mycophenolate mofetil for IgG4 related pancreatitis. Although autoimmune pancreatitis as part of IgG4 related systemic disease is rarely reported in pediatrics, autoimmune pancreatitis is also characterized as idiopathic fibrosing pancreatitis. All pediatric autoimmune pancreatitis cases reported in the world medical literature were identified via a PUBMED search and are reviewed herein. Twelve reports of pediatric autoimmune pancreatitis were identified, most of which were treated with corticosteroids or surgical approaches. Most case reports failed to report IgG4 levels, so it remains unclear how commonly IgG4 related autoimmune pancreatitis occurs during childhood. Increased evaluation of IgG4 levels in patients with autoimmune pancreatitis may shed further light on the association of IgG4 with pancreatitis and the underlying pathophysiology

Diagnostic Dilemma in a Patient with Jaundice: How to Differentiate between Autoimmune Pancreatitis, Primary Sclerosing Cholangitis and Pancreas Carcinoma

A 68-year-old male patient was referred to our institution in May 2011 for a suspected tumor in the pancreatic head with consecutive jaundice. Using magnetic resonance imaging, further differentiation between chronic inflammation and a malignant process was not possible with certainty. Apart from cholestasis, laboratory studies showed increased values for CA 19-9 to 532 U/ml (normal <37 U/ml) and hypergammaglobulinemia (immunoglobulin G, IgG) of 19.3% (normal 8.0–15.8%) with an elevation of the IgG4 subtype to 2,350 mg/l (normal 52–1,250 mg/l). Endoscopic retrograde cholangiopancreatography revealed a prominent stenosis of the distal ductus hepaticus communis caused by pancreatic head swelling and also a bihilar stenosis of the main hepatic bile ducts. Cytology demonstrated inflammatory cells without evidence of malignancy. Under suspicion of autoimmune pancreatitis with IgG4-associated cholangitis, immunosuppressive therapy with steroids and azathioprine was started. Follow-up endoscopic retrograde cholangiopancreatography after 3 months displayed regressive development of the diverse stenoses. Jaundice had disappeared and blood values had returned to normal ranges. Moreover, no tumor of the pancreatic head was present in the magnetic resonance control images. Due to clinical and radiological similarities but a consecutive completely different prognosis and therapy, it is of fundamental importance to differentiate between pancreatic cancer and autoimmune pancreatitis. Especially, determination of serum IgG4 levels and associated bile duct lesions induced by inflammation should clarify the diagnosis of autoimmune pancreatitis and legitimate immunosuppressive therapy

An unusual case of autoimmune pancreatitis presenting as pancreatic mass and obstructive jaundice: a case report and review of the literature

<p>Abstract</p> <p>Background</p> <p>Autoimmune pancreatitis is a rare chronic inflammatory pancreatic disease that is increasingly being diagnosed worldwide. As a result of overlap in clinical and radiological features, it is often misdiagnosed as pancreatic cancer. We report the case of a patient with autoimmune pancreatitis that was initially misdiagnosed as pancreatic cancer.</p> <p>Case presentation</p> <p>A 31-year-old Caucasian man presented to our hospital with epigastric pain, jaundice and weight loss. His CA 19-9 level was elevated, and computed tomography and endoscopic ultrasound revealed a pancreatic head mass abutting the portal vein. Endoscopic retrograde cholangiopancreaticography showed narrowing of the biliary duct and poor visualization of the pancreatic duct. Fine-needle aspiration biopsy revealed atypical ductal epithelial cells, which raised clinical suspicion of adenocarcinoma. Because of the patient's unusual age for the onset of pancreatic cancer and the acuity of his symptoms, he was referred to a tertiary care center for further evaluation. His immunoglobulin G4 antibody level was 365 mg/dL, and repeat computed tomography showed features typical of autoimmune pancreatitis. The patient's symptoms resolved with corticosteroid therapy.</p> <p>Conclusion</p> <p>Autoimmune pancreatitis is a rare disease with an excellent response to corticosteroid therapy. Its unique histological appearance and response to corticosteroid therapy can reduce unnecessary surgical procedures. A thorough evaluation by a multidisciplinary team is important in rendering the diagnosis of autoimmune pancreatitis.</p

The risk factors and related hospitalizations for cases with positive and negative COVID-19 tests: A case-control study

Background: The aim of this study was to evaluate the risk factors for hospitalizations of cases with positive and negative COVID-19 tests. Methods: In this case-control study, the case and control groups consisted of 292 COVID-19 patients and 296 non-COVID-19 patients. Patients who referred to a reference laboratory in Tehran (Iran) in March 2020 were selected and interviewed. The patients were contacted by telephone and data were recorded through a questionnaire. Results: The sample of this study consisted of 588 patients (349 59% females, 239 41% males) with a mean age of 42 ± 15. The results of this study showed that comorbidities like diabetes (OR = 7.42), hypertension (OR = 4.85), asthma and respiratory diseases (OR = 5.64) in addition to symptoms including fever (OR = 6.67), chills (OR = 11.2), anorexia (OR = 11.3), dyspnea (OR = 4.8), weakness and lethargy (OR = 5.7) were the most predictive variables for hospitalization of non-COVID-19 cases. Furthermore, demographical variables like male gender (OR = 3.71), high age (>50; OR = 3.12), BMI (>25; OR = 2.37), travel (OR = 2.79), comorbidities including diabetes (OR = 5.26), hypertension (OR = 3.7) and underlying immunosuppressant patients receiving corticosteroid therapy (OR = 3.62) in addition to symptoms like anorexia OR = 2.55 and dyspnea (OR = 6.99) tend to increase the risk of hospital admission in COVID-19 patients, suggesting their predictive values for hospitalization of COVID-19 patients. Conclusion: Our results indicated that different factors tend to increase the odds of hospital admission in patients with positive and negative COVID-19 tests, suggesting their predictive values for hospitalization. © 202

Pancreatic Ductal Adenocarcinoma Associated with Autoimmune Pancreatitis

Autoimmune pancreatitis (AIP), in contrast to other benign chronic pancreatic diseases, can be cured with immunosuppressant drugs, thus the differentiation of AIP from pancreatic cancer is of particular interest in clinical practice. There is the possibility that some patients with AIP may develop pancreatic cancer, and this possibility contributes to increasing our difficulties in differentiating AIP from pancreatic cancer. We herein report the case of a 70-year-old man in whom pancreatic adenocarcinoma and AIP were detected simultaneously. We must carefully monitor AIP patients for the simultaneous presence of pancreatic cancer, even when a diagnosis of AIP is confirmed

IgG4-Related Sclerosing Disease, an Emerging Entity: A Review of a Multi-System Disease

Immunoglobulin G4-related systemic disease (IgG4-RSD) is a recently defined emerging entity characterized by a diffuse or mass forming inflammatory reaction rich in IgG4-positive plasma cells associated with fibrosclerosis and obliterative phlebitis. IgG4-RSD usually affects middle aged and elderly patients, with a male predominance. It is associated with an elevated serum titer of IgG4, which acts as a marker for this recently characterized entity. The prototype is IgG4-related sclerosing pancreatitis or autoimmune pancreatitis (AIP). Other common sites of involvement are the hepatobiliary tract, salivary gland, orbit, and lymph node, however practically any organ can be involved, including upper aerodigestive tract, lung, aorta, mediastinum, retroperitoneum, soft tissue, skin, central nervous system, breast, kidney, and prostate. Fever or constitutional symptoms usually do not comprise part of the clinical picture. Laboratory findings detected include raised serum globulin, IgG and IgG4. An association with autoantibody detection (such as antinuclear antibodies and rheumatoid factor) is seen in some cases. Steroid therapy comprises the mainstay of treatment. Disease progression with involvement of multiple organ-sites may be encountered in a subset of cases and may follow a relapsing-remitting course. The principal histopathologic findings in several extranodal sites include lymphoplasmacytic infiltration, lymphoid follicle formation, sclerosis and obliterative phlebitis, along with atrophy and destruction of tissues. Immunohistochemical staining shows increased IgG4+ cells in the involved tissues (>50 per high-power field, with IgG4/IgG ratio >40%). IgG4-RSD may potentially be rarely associated with the development of lymphoma and carcinoma. However, the nature and pathogenesis of IgG4-RSD are yet to be fully elucidated and provide immense scope for further studies

IgG4-related kidney diseases and conditions: Renal pelvic and ureteral diseases

In the literature on IgG4-related urinary tract diseases, reports of cases with involvement of the renal pelvis and ureters are increasing. IgG4-related renal pelvic and ureteral lesions accompany extra-renal organ involvement, including IgG4-related type 1 autoimmune pancreatitis, sialadenitis, and orbital disease, and are characterized by the common pathological features of IgG4-related disease (IgG4-RD), including substantial numbers of IgG4-positive plasma cells, storiform fibrosis, and stenosis in the affected organs. Similar to other mucosal organs affected in IgG4-RD, these inflammatory findings are observed within the fibroadipose tissue in the renal hilum and around the ureters. The urothelial epithelium covering the renal pelvis and ureter is preserved. Nodular lesions such as pseudotumors can also form and it is important to differentiate these from malignant tumors. At present, comprehensive diagnostic criteria that include pathological parameters have been proposed for IgG4-RD; however, obtaining diagnostic findings in small biopsy specimens is often challenging. Therefore, the diagnosis can only be rendered following careful consideration of the patient’s clinical, serologic, radiologic, and pathologic features, including the possibility of involvement in other organs. © Springer Japan 2016.[Book Chapter

Safety and efficacy of Favipiravir in moderate to severe SARS-CoV-2 pneumonia

Background: We examined the safety and efficacy of a treatment protocol containing Favipiravir for the treatment of SARS-CoV-2. Methods: We did a multicenter randomized open-labeled clinical trial on moderate to severe cases infections of SARS-CoV-2. Patients with typical ground glass appearance on chest computerized tomography scan (CT scan) and oxygen saturation (SpO2) of less than 93 were enrolled. They were randomly allocated into Favipiravir (1.6 gr loading, 1.8 gr daily) and Lopinavir/Ritonavir (800/200 mg daily) treatment regimens in addition to standard care. In-hospital mortality, ICU admission, intubation, time to clinical recovery, changes in daily SpO2 after 5 min discontinuation of supplemental oxygen, and length of hospital stay were quantified and compared in the two groups. Results: 380 patients were randomly allocated into Favipiravir (1 9 3) and Lopinavir/Ritonavir (1 8 7) groups in 13 centers. The number of deaths, intubations, and ICU admissions were not significantly different (26, 27, 31 and 21, 17, 25 respectively). Mean hospital stay was also not different (7.9 days SD = 6 in the Favipiravir and 8.1 SD = 6.5 days in Lopinavir/Ritonavir groups) (p = 0.61). Time to clinical recovery in the Favipiravir group was similar to Lopinavir/Ritonavir group (HR = 0.94, 95% CI 0.75 � 1.17) and likewise the changes in the daily SpO2 after discontinuation of supplemental oxygen (p = 0.46) Conclusion: Adding Favipiravir to the treatment protocol did not reduce the number of ICU admissions or intubations or In-hospital mortality compared to Lopinavir/Ritonavir regimen. It also did not shorten time to clinical recovery and length of hospital stay. © 2021 Elsevier B.V