scholarly journals Immunoglobulin G4 Sclerosing Cholangitis: An Unusual Cause of Obstructive Jaundice—Case Report and Literature Review

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Pragya Shrestha ◽  
Brian Le ◽  
Brent Wagner ◽  
William Pompella ◽  
Paras Karmacharya
Keyword(s):  
Sclerosing Cholangitis ◽  
Plasma Cells ◽  
Systemic Disease ◽  
Clinical Manifestations ◽  
Disease Process ◽  
High Serum ◽  
Diagnostic Challenge ◽  
Obliterative Phlebitis ◽  
Immunoglobulin G4 ◽  
Serum Igg4

IgG4-related sclerosing cholangitis (IgG4-SC) is one of the most common extra-pancreatic manifestation of IgG4-related disease (IgG4-RD) and is clinically distinct from primary sclerosing cholangitis (PSC). IgG4-RD is an increasingly recognized immune-mediated fibroinflammatory systemic disease, mostly affecting middle-aged and older male populations that can affect multiple organs. The presence of extra-biliary clinical manifestations of IgG4-RD, such as parotid and lacrimal swelling, lymphadenopathy, autoimmune pancreatitis, and retroperitoneal fibrosis, if present could provide important clues to diagnosis. High serum IgG4 levels, characteristic radiological (e.g., sausage-shaped pancreas or periaortitis) or biopsy findings (high percentage of IgG4+ plasma cells, lymphoplasmacytic infiltrate, storiform fibrosis, or obliterative phlebitis) in the setting of these features is diagnostic of this disease process. However, isolated IgG4-SC might be a diagnostic challenge, and the distinction is important as management of this disorder is vastly different from other causes of cholangitis such as PSC. Systemic corticosteroid therapy is the mainstay of therapy.

scholarly journals Immunoglobulin G4-related sclerosing cholangitis mimicking cholangiocarcinoma: a case report and literature review

2020 ◽  
Vol 48 (10) ◽  
pp. 030006052095921
Author(s):  
Cheng Xu ◽  
Yongmei Han
Keyword(s):  
Sclerosing Cholangitis ◽  
Plasma Cells ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
Immunoglobulin G4 ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
History Of ◽  
The Common

Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a novel clinical disease that is characterized by elevated serum IgG4 concentrations and tumefaction or tissue infiltrated by IgG4+ plasma cells. The clinical manifestations of IgG4-RD depend on the type of tissues affected. IgG4-related sclerosing cholangitis is a type of IgG4-RD. We report a patient who initially visited a local hospital with a 5-month history of jaundice. He was found to have a mass in the upper part of the common bile duct that mimicked cholangiocarcinoma. He underwent surgery in our hospital and was later diagnosed with IgG4-related sclerosing cholangitis. We administered prednisolone 40 mg once a day for treatment. Taking into account the possible side effects of moderate-dose hormone therapy, we also administered teprenone, potassium chloride, and calcium carbonate. The patient did not have any recurrence of symptoms or adverse drug reactions during follow-up.

scholarly journals IgG4-Related Sclerosing Cholangitis: Rarely Diagnosed, but not a Rare Disease

2021 ◽  
Vol 2021 ◽  
pp. 1-16
Author(s):  
Sylvia Drazilova ◽  
Eduard Veseliny ◽  
Patricia Denisa Lenartova ◽  
Dagmar Drazilova ◽  
Jakub Gazda ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Sclerosing Cholangitis ◽  
Plasma Cells ◽  
Histological Finding ◽  
Good Prognosis ◽  
High Serum ◽  
Obliterative Phlebitis ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Storiform Fibrosis

IgG4-related sclerosing cholangitis, a biliary manifestation of an IgG4-related disease, belongs to the spectrum of sclerosing cholangiopathies which result in biliary stenosis. It presents with signs of cholestasis and during differential diagnosis it should be distinguished from cholangiocarcinoma or from other forms of sclerosing cholangitis (primary and secondary sclerosing cholangitis). Despite increasing information and recently established diagnostic criteria, IgG4-related sclerosing cholangitis remains underdiagnosed in routine clinical practice. The diagnosis is based on a combination of the clinical picture, laboratory parameters, histological findings, and a cholangiogram. Increased serum IgG4 levels are nonspecific but are indeed a part of the diagnostic criteria proposed by the Japan Biliary Association and the HISORt criteria for IgG4-SC. High serum IgG4 retains clinical utility depending on the magnitude of elevation. Approximately 90% of patients have concomitant autoimmune pancreatitis, while 10% present with isolated biliary involvement only. About 26% of patients have other organ involvement, such as IgG4-related dacryoadenitis/sialadenitis, IgG4-related retroperitoneal fibrosis, or IgG4-related renal lesions. A full-blown histological finding characterized by IgG4-enriched lymphoplasmacytic infiltrates, obliterative phlebitis, and storiform fibrosis is difficult to capture in practice because of its subepithelial localization. However, the histological yield is increased by immunohistochemistry, with evidence of IgG4-positive plasma cells. Based on a cholangiogram, IgG-4 related sclerosing cholangitis is classified into four subtypes according to the localization of stenoses. The first-line treatment is corticosteroids. The aim of the initial treatment is to induce clinical and laboratory remission and cholangiogram normalization. Even though 30% of patients have a recurrent course, in the literature data, there is no consensus on chronic immunosuppressive maintenance therapy. The disease has a good prognosis when diagnosed early.

scholarly journals Multiple Artery Aneurysms: Unusual Presentation of IgG4 Vasculopathy

2021 ◽  
Vol 11 ◽  
pp. 17
Author(s):  
Anurag Yadav ◽  
Geetha Godasu ◽  
Tarvinder Bir Singh Buxi ◽  
Swapnil Sheth
Keyword(s):  
Plasma Cells ◽  
Systemic Disease ◽  
Artery Aneurysm ◽  
High Serum ◽  
Immunoglobulin G4 ◽  
Atypical Chest Pain ◽  
Igg4 Related Disease ◽  
Target Organs ◽  
Serum Igg4 ◽  
Giant Coronary Artery Aneurysm

Immunoglobulin G4 (IgG4)-related disease is a chronic systemic disease. It is characterized by inflammatory fibrosis and high serum IgG4 levels. IgG4-positive plasma cells infiltrate target organs in this disease. It may involve the pancreas, biliary tract, lacrimal glands, salivary glands, orbits, thyroid, kidneys, lymph nodes, or retroperitoneum. It may present as vasculitis with involvement of large to medium sized vessels such as the aorta, the common iliac, carotid, and coronary arteries. We present a case of 55-year-old male patient who presented with shortness of breath on exertion and atypical chest pain. On CT angiography, a giant coronary artery aneurysm involving the left anterior descending artery, multiple visceral and intercostal artery aneurysms, and nodular paravertebral soft-tissue thickening secondary to IgG4 vasculopathy.

scholarly journals Advances in IgG4-related Hepatobiliary Disease

2020 ◽  
Vol 25 (2) ◽  
pp. 100-106
Author(s):  
Sung-Hoon Moon
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Quantitative Polymerase Chain Reaction ◽  
High Serum ◽  
Hepatobiliary Disease ◽  
Glucocorticoid Therapy ◽  
Glucocorticoid Treatment ◽  
Immunoglobulin G4 ◽  
Serum Igg4 ◽  
Polymerase Chain

The emergence of the new disease entity of steroid responsive immunoglobulin G4 (IgG4)-related hepatobiliary disease has generated considerable interest among hepatobiliary society. IgG4-related hepatobiliary disease refers to mainly IgG4-related sclerosing cholangitis (IgG4-SC), and includes a small number of IgG4-related hepatopathy. As IgG4-SC responds well to glucocorticoid therapy, IgG4-SC should be differentiated from cholangiocarcinoma and primary sclerosing cholangitis. Timely diagnosis of IgG4-SC can lead clinicians to prescribe adequate glucocorticoid treatment that can reverse bile duct strictures and cholestatic liver function. Differentiation of IgG4-SC from primary sclerosing cholangitis is sometimes challenging because serum IgG4 and tissue IgG4 have demonstrated low positive predictive value in this setting. Recent research suggested that blood IgG4/IgG RNA ratio by quantitative polymerase chain reaction can be used for differentiation. Although most patients with IgG4-SC/autoimmune pancreatitis respond to glucocorticoid therapy, they frequently experience relapse of disease. The suggested relapse factors included very high serum IgG4, diffuse enlargement of the pancreas, proximal IgG4-SC, and multi-organ involvement. This review discusses the recent advances in the pathogenesis, diagnosis, management, disease relapse, and monitoring disease activity of IgG4-SC.

scholarly journals Fevers, Sweats, Weight Loss, Oh My! When IgG4 Related Disease Comes Knocking. . .

2021 ◽  
Vol 14 ◽  
pp. 117954762110463
Author(s):  
Francis Essien ◽  
Graey Wolfley ◽  
Joshua Tate ◽  
Matthew Carroll
Keyword(s):  
Weight Loss ◽  
Plasma Cells ◽  
Systemic Disease ◽  
Elevated Serum ◽  
Acute Onset ◽  
Immunoglobulin G4 ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Rare Manifestation ◽  
Organ Manifestations

Immunoglobulin G4-related systemic disease (IgG4-RSD) is a fibro-inflammatory immune condition characterized by IgG4 positive plasma cells, fibrosis, and frequently elevated serum IgG4 level. Akin to sarcoidosis, IgG4-RSD is a systemic disease with diverse organ manifestations linked by common histopathologic features. IgG4-RSD typically presents sub acutely without significant constitutional symptoms or fever. Hepatic Inflammatory pseudotumor (HIP) is a rare manifestation of IgG4-RSD composed of dense lymphoplasmacytic infiltrate and extensive fibrosis. We present an older Asian male who presented with acute onset of fever and weight loss found to have IgG4-RSD complicated by a HIP and concomitant MSSA abscess.

scholarly journals The Cellular and Molecular Bases of Allergy, Inflammation and Tissue Fibrosis in Patients with IgG4-related Disease

2020 ◽  
Vol 21 (14) ◽  
pp. 5082
Author(s):  
Song-Chou Hsieh ◽  
Chieh-Yu Shen ◽  
Hsien-Tzung Liao ◽  
Ming-Han Chen ◽  
Cheng-Han Wu ◽  
...  
Keyword(s):  
Plasma Cells ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
Malignant Neoplasms ◽  
Th2 Response ◽  
Obliterative Phlebitis ◽  
Inflammatory Reactions ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4

IgG4-related disease (IgG4-RD) is a spectrum of complex fibroinflammatory disorder with protean manifestations mimicking malignant neoplasms, infectious or non-infectious inflammatory process. The histopathologic features of IgG4-RD include lymphoplasmacytic infiltration, storiform fibrosis and obliterative phlebitis together with increased in situ infiltration of IgG4 bearing-plasma cells which account for more than 40% of all IgG-producing B cells. IgG4-RD can also be diagnosed based on an elevated serum IgG4 level of more than 110 mg/dL (normal < 86.5 mg/mL in adult) in conjunction with protean clinical manifestations in various organs such as pancreato–hepatobiliary inflammation with/without salivary/lacrimal gland enlargement. In the present review, we briefly discuss the role of genetic predisposition, environmental factors and candidate autoantibodies in the pathogenesis of IgG4-RD. Then, we discuss in detail the immunological paradox of IgG4 antibody, the mechanism of modified Th2 response for IgG4 rather than IgE antibody production and the controversial issues in the allergic reactions of IgG4-RD. Finally, we extensively review the implications of different immune-related cells, cytokines/chemokines/growth factors and Toll-like as well as NOD-like receptors in the pathogenesis of tissue fibro-inflammatory reactions. Our proposals for the future investigations and prospective therapeutic strategies for IgG4-RD are shown in the last part.

scholarly journals Immunoglobulin G4-Related Kidney Disease with Membranous Proliferative Glomerulonephritis Presenting with a Renal Pelvic Mass

2021 ◽  
Vol 96 (1) ◽  
pp. 48-52
Author(s):  
Jong Tae Cho ◽  
Eun-Kyoung Lee ◽  
Jai Hyang Go ◽  
Yong-Moon Lee ◽  
Hwa Young Lee ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Plasma Cells ◽  
Clinical Manifestations ◽  
Pelvic Mass ◽  
Multiple Organ ◽  
Proliferative Glomerulonephritis ◽  
Inflammatory Disorder ◽  
Obliterative Phlebitis ◽  
Immunoglobulin G4 ◽  
Radiologic Findings

Immunoglobulin G4 (IgG4)-related kidney disease is a chronic immune-mediated fibro-inflammatory disorder characterized by multiple organ infiltration with IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis or tumefactive lesions. Previous studies have explored IgG4-related kidney disease, increasing our understanding of its clinical manifestations, and pathological and radiologic findings. However, IgG4-related kidney disease can be misdiagnosed since it mimics malignancies. We report a case of a 77-year-old Korean man diagnosed with IgG4-related kidney disease with membranous proliferative glomerulonephritis, presenting with a renal pelvic mass suspected of being malignant.

scholarly journals Cross-sectional pictorial review of IgG4-related disease

2019 ◽  
Vol 92 (1103) ◽  
pp. 20190448 ◽  
Author(s):  
Darya Kurowecki ◽  
Michael N. Patlas ◽  
Ehsan A. Haider ◽  
Abdullah Alabousi
Keyword(s):  
Plasma Cells ◽  
Multidetector Ct ◽  
Current Knowledge ◽  
Systemic Disease ◽  
Autoimmune Disorder ◽  
Immunoglobulin G4 ◽  
Cross Sectional ◽  
Related Disease ◽  
Pictorial Review ◽  
Serum Igg4

Immunoglobulin G4-related disease (IgG4-RD) is an autoimmune disorder characterized by the infiltration of one or more organs with IgG4-positive plasma cells resulting in inflammatory lesions and fibrosis. Although the pancreas is the most commonly affected organ, involvement of extrapancreatic organs is an increasingly recognized manifestation of the disease. Patients may be asymptomatic and serum IgG4 concentrations may be elevated or normal. Treatment consists of glucocorticosteroid treatment, with excellent response. A definitive diagnosis requires histopathology with imaging playing a key role in avoiding treatment delays. This pictorial review will focus on the most current knowledge regarding IgG4-RD including its common and less common manifestations and the roles of multidetector CT, MRI and ultrasound in the diagnosis and management of suspected IgG4-RD. Knowledge of the varied imaging findings of this multi systemic disease is essential for radiologists to avoid misdiagnosis and assist with timely and effective treatment.

scholarly journals Immunoglobulin G4-related periodontitis: case report and review of the literature

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Jinmei Zhang ◽  
Lei Zhao ◽  
Jieyu Zhou ◽  
Wei Dong ◽  
Yafei Wu
Keyword(s):  
Case Report ◽  
Rare Case ◽  
Plasma Cells ◽  
Alveolar Bone ◽  
Systemic Disease ◽  
Unknown Etiology ◽  
High Power Field ◽  
Obliterative Phlebitis ◽  
Immunoglobulin G4 ◽  
Gingival Enlargement

Abstract Background Immunoglobulin G4-related disease (IgG4-RD) is a chronic inflammatory systemic disease of unknown etiology that can affect one or multiple organs. The disease can mimic many infectious and inflammatory diseases, mainly causing organ enlargement or hyperplasia. Its diagnosis primarily relies on clinical, serologic, and histological features (lymphoplasmacytic infiltrates, storiform fibrosis, and obliterative phlebitis of IgG4 + plasma cells). Here, we report a rare case of IgG4-related periodontitis, and review the relevant literatures. Case presentation A 38-year-old Chinese man visited the Department of Periodontics with gingival enlargement, loose teeth, and tooth loss. The patient had very poor oral hygiene and a large amount of calculus. Gingivae were edematous with deep periodontal pockets and attachment loss. Panoramic radiography showed alveolar bone loss. Serologic examination showed that IgG was 23.70 g/L and IgG4 concentration was 2.800 g/L. There was significant lymphoplasmacytic infiltration, a storiform pattern of fibrosis, and mitotic figures with hematoxylin and eosin staining; immunohistochemical staining showed 10 scattered IgG4-positive plasma cells in a high-power field. The patient was diagnosed as IgG4-related periodontitis. He received a course of corticosteroids with periodontal therapy, and the enlargement was significantly improved without recurrence. Conclusion IgG4-RD in the oral and maxillofacial region mainly involves salivary glands, but this rare case was characterized by enlarged gingivae. The differential diagnosis of IgG4-RD should be based on the clinical features and serologic (IgG4) and histopathological examinations. Corticosteroid therapy is effective for most IgG4-RD patients. Taken together, we hope this case report and the literature review can help dentists to improve their understanding of the IgG4-RD.

scholarly journals Immunoglobulin G4-related disease presenting with peripheral neuropathy: a case report

BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Jeong Bin Bong ◽  
Dong Kun Lee ◽  
Min A Lee ◽  
Byoung Wook Hwang ◽  
Hyun Goo Kang
Keyword(s):  
Nervous System ◽  
Peripheral Neuropathy ◽  
Peripheral Nerve ◽  
Plasma Cells ◽  
High Serum ◽  
High Dose ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Immune Mediated ◽  
Serum Igg4

Abstract Background Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibro-inflammatory condition characterized by high serum IgG4 concentrations and tissue infiltration by IgG4-positive plasma cells. Reports have demonstrated that IgG4-RD affects various organs, including the pancreas, kidney, lung, thyroid, and lacrimal and salivary glands. In the nervous system, hypertrophic pachymeningitis and hypophysitis are mainly related to IgG4-RD; however, the peripheral neuropathy involvement is unusual. Case presentation We report on a 69-year-old woman with multiple mononeuropathy, weight loss and kidney mass in the setting of IgG4-RD. Biopsies of the kidney mass showed lymphoplasmacytic sclerosing inflammation with numerous IgG4-positive plasma cells. IgG4 and IgG4/IgG ratios in the blood were elevated. The patient was treated with high dose methylprednisolone with improvement in her neuropathy. Conclusions IgG4-RD is a relatively recently reported systemic fibrous inflammatory disease caused by the infiltration of IgG4-positive plasma cells in various organs. In the nervous system, symptomatic peripheral nerve invasion is very rare. However, as demonstrated in our case, IgG4-RD may present with primarily peripheral nerve disease.

Sign in / Sign up

Export Citation Format

Share Document