IgG4 sclerosing disease

Chronic sclerosing sialadenitis (CSS), currently included in the group of immunoglobulin G4 (IgG4)-related diseases, is an under-recognized inflammatory lesion that afflicts mostly the submandibular gland of 40-60 years adults. To our knowledge, only one case of CSS located in the submandibular gland has been reported in childhood to date. We present a case of CSS in a 5-year-old male child. He presented with bilateral submandibular swellings that clinically resembled discrete lumps, suspected to be tumors...

The etiology of primary sclerosing cholangitis (PSC) remains unclear, which explains in part the lack of a causal treatment. The differential diagnostic distinction from the even rarer immunoglobulin 4 (IgG4)-associated cholangitis (IAC) is becoming increasingly more successful. Advances in the understanding of different clinical courses, improvements in noninvasive diagnostics through modern magnetic resonance imaging (MRI) and the introduction of liver elastography have led to the development of improved prognostic models...

BACKGROUND: Autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) are two common clinical autoimmune liver diseases, and some patients have both diseases; this feature is called AIH-PBC overlap syndrome. Autoimmune thyroid disease (AITD) is the most frequently overlapping extrahepatic autoimmune disease. Immunoglobulin (IgG) 4-related disease is an autoimmune disease recognized in recent years, characterized by elevated serum IgG4 levels and infiltration of IgG4-positive plasma cells in tissues...

IgG4-related disease is an uncommon multisystem inflammatory disease characterized by tissue infiltration by IgG4 plasma cells, extensive fibrosis, and sclerosing inflammation. Diffuse orbital involvement extending to the orbital apex and cavernous sinus has been reported, but a solitary well-circumscribed lesion at the apex has not been previously reported. Herein, we report a unique case of IgG4-related orbital disease presenting as a solitary well-circumscribed orbital apex lesion causing subacute visual decline in a 45-year-old male...

IgG4-related diseases (IgG4-RD) are a group of chronic progressive autoimmune diseases of unknown etiology that are increasingly recognized as an important pathophysiological basis for a variety of systemic diseases. It is thought to involve almost any organ of the body, but the involvement of the central nervous system is relatively rare. We report the case of a 56-year-old male patient admitted to the hospital d recurrent dizziness and nausea for more than 3 months. The preoperative imaging was misdiagnosed as a meningioma, with this lesion demonstrated localized inhomogeneous thickening of the meninges in the left parietal region on T2-weighted and T2 fat suppression sequences with localized nodular changes...

IgG4-related sclerosing cholangitis (IgG4-SC) is a unique, steroid-responsive, fibroinflammatory condition that is more commonly found in older men and is strongly associated with autoimmune pancreatitis (AIP). It may pose a diagnostic challenge at times due to closely mimicking hepatobiliary diseases such as primary sclerosing cholangitis (PSC), secondary sclerosing cholangitis (SSC), and cholangiocarcinoma. IgG4-SC has an excellent prognosis when promptly diagnosed and treated with steroids. Literature search strategy: The authors searched PubMed and Google Scholar, for articles with the search terms "autoimmune cholangiopathy" and "IgG4 cholangiopathy...

Granulomatous lobular mastitis (GLM) is a rare inflammatory breast disease with unknown etiology, characterized by non-caseous granulomatous inflammation of the lobules, which infiltrate lymphocytes, neutrophils, plasma cells, monocytes, and eosinophils may accompany. GLM is often misdiagnosed as breast cancer due to the lack of specificity in clinical and imaging examinations, and therefore histopathology is the main basis for confirming the diagnosis. This review provides an overview of the pathological features of granulomatous lobular mastitis and cystic neutrophil granulomatous mastitis (CNGM, a pathologic subtype of GLM)...

Immunoglobulin G-4 related disease (IgG4-RD) is an immune-mediated, inflammatory disease that often involves multiple organ systems. IgG4-RD can be classified according to the organs involved. Type 1 IgG4-RD is related to acute pancreatitis and sclerosing cholangitis. Disease manifestation is also seen in the retroperitoneal region, pelvic organs, and orbital space. Here we describe a rare case of IgG4-RD causing isolated acute kidney injury.

With the growing recognition of IgG4-related hepatobiliary disease, establishing a definitive diagnosis relies mainly on a combination of clinical findings, serological markers, and imaging modalities. However, the role of histopathological evaluation remains indispensable, particularly in cases necessitating differential diagnosis or malignancy exclusion. While diagnosing IgG4-related hepatobiliary disease through surgical resection specimens is often straightforward, pathologists encounter substantial challenges when evaluating biopsies...

Primary sclerosing cholangitis (PSC) is a progressive cholestatic liver disease characterized by intrahepatic and extrahepatic bile duct strictures leading to cirrhosis. A subtype with elevated serum immunoglobulin (Ig) G4 levels has been recently identified. Elevated IgG4 titers can be present in 9%-15% of patients with PSC. Currently, liver transplantation is the only effective treatment of PSC, although multiple medical therapies are under evaluation. We report a case of a young adult with PSC and elevated IgG4 levels who had marked serum aminotransferase elevation; the patient had an incomplete response to steroids but achieved complete biochemical remission after initiation of oral vancomycin...

OBJECTIVES: This study aimed to investigate the histological characteristics and treatment efficacy of non-immunoglobulin G4-related fibrosing mediastinitis and discuss differential diagnoses for this rare entity. METHODS: We present a case study of non-immunoglobulin G4-related fibrosing mediastinitis diagnosed on core biopsy and treated with steroids. A total of four 18-gauge core needle biopsy specimens were obtained for surgical pathology. Analysis of the patient's medical history, radiological characteristics of fibrosing mediastinitis, histological features, immunohistochemistry results, the differential diagnosis and treatment efficacy of different types of fibrosing mediastinitis was performed...

A 63-year-old male presented to our oncological hospital with a one-year evolving abdominal pain, with an abdominal mass feeling. Contrast-enhanced computed tomography displayed two soft tissue masses, one at the mesentery root and the second around the pancreatic tail; at the same time the patient presented with hyperlipasemia. Endoscopic biopsy for the pancreatic mass and surgical biopsy of the mesenteric one were performed in order to narrow diagnosis. No neoplastic cells but only dense fibro-inflammatory changes with immunoglobulin G4 (IgG4)-positive plasma cell inclusions were observed for both biopsies...

INTRODUCTION: Primary sclerosing cholangitis (PSC) is a chronic, cholestatic liver disease that is characterized by an inflammatory and fibrotic process affecting bile ducts which eventually develops into liver cirrhosis and liver failure. The aim of this study was to investigate serum IgG subclass distribution in patients with PSC and its possible association with PSC outcomes. PATIENTS AND METHODS: We performed a retrospective analysis of 181 patients who had been diagnosed with PSC between January 1970 and December 2015 and followed at our outpatient clinic...

An asymptomatic 77-year-old man with intrahepatic bile duct dilation was referred to our hospital. Cholangiography revealed alternations between strictures and dilated segments from the right and left hepatic ducts to the lower bile ducts, with findings of a pruned tree, beaded, shaggy appearance, and diverticulum-like outpouching. Histopathology revealed abundant immunoglobulin G4 (IgG4)-positive plasma cells (> 10 per high-power field) with an IgG4/IgG-positive cell ratio of 40-50%. After 2 weeks of steroid therapy, the cholangiography markedly improved...

BACKGROUND: The risk and prognosis of pancreatobiliary cancer and in patients with autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis (IgG4-SC) remain unclear. Therefore, we retrospectively investigated the risk of pancreatobiliary cancer and prognosis in patients with AIP and IgG4-SC. METHODS: Patients with AIP and IgG4-SC at seven centers between 1998 and 2022 were investigated. The following data were evaluated: (1) the number of cancers diagnosed and standardized incidence ratio (SIR) for pancreatobiliary and other cancers during the observational period and (2) prognosis after diagnosis of AIP and IgG4-SC using standardized mortality ratio (SMR)...

Idiopathic mediastinal fibrosis, also called sclerosing or fibrosing mediastinitis, is a very rare and aggressive fibroinflammatory process characterized by fibrous tissue proliferation in the mediastinal region. Herein, we present a rare case of idiopathic mediastinal fibrosis presenting with esophageal obstruction, most likely associated with immunoglobulin G (IgG4)-related disease, affecting the posterior mediastinum with intrapulmonary infiltration. Computed tomography revealed a narrowed lumen and thickened wall of the distal esophagus surrounded by a necrotic mass with infiltration into the nearby structures, suggesting a locally advanced malignant process...

A man in his 70s presented to the emergency department with painless obstructive jaundice. Initial blood test results show a predominantly cholestatic picture with elevated tumour markers, and imaging findings are concerning for a pancreatic head neoplasm or cholangiocarcinoma with involvement of the entire common bile duct. The patient underwent staging laparoscopy and biopsies including peritoneal washing, but did not identify any features of malignancy. Immunoglobulin G and immunoglobulin G4 testing were subsequently tested and shown to be elevated...

Primary sclerosing cholangitis is a heterogenous immune-mediated disorder characterized by chronic inflammation and stricturing of the bile ducts. Though the driving pathophysiologic mechanisms remain elusive, there are several observed clinical phenotypes of the disease. The distribution of bile duct involvement, presence of concomitant inflammatory bowel disease, significant infiltration of IgG4-positive plasma cells, and overlapping features with other autoimmune disease has significant implications for prognosis and treatment...

Cholangiopathies are defined as focal or extensive damage of the bile ducts. According to the pathogenetic mechanism, it may be immune-mediated or due to genetic, infectious, toxic, vascular, and obstructive causes. Their chronic evolution is characterized by inflammation, obstruction of bile flow, cholangiocyte proliferation, and progression toward fibrosis and cirrhosis. Immune-mediated cholangiopathies comprise primary sclerosing cholangitis (PSC), autoimmune cholangitis and IgG4-associated cholangitis in adults and biliary atresia (BA), neonatal sclerosing cholangitis (NSC) in children...

BACKGROUND/AIM: IgG4-related disease (IgG4RD) is a rare autoimmune proinflammatory condition that mimics other cancers and has unique pathological findings. The effects of radiotherapy in patients with IgG4RD remain unknown. CASE REPORT: A male patient in his seventies who received radiotherapy (68 Gy/39 fr) for bladder cancer 5 months prior, presented to our hospital with fatigue and swelling in both legs. The patient had a history of IgG4-related sclerosing cholangitis, a subtype of IgG4RD...