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 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 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 Recognizing Immunoglobulin G4-Related Overlap Syndromes in Patients with Pancreatic and Hepatobiliary Diseases

2008 ◽  
Vol 22 (10) ◽  
pp. 840-846 ◽  
Author(s):  
Aldo J Montano-Loza ◽  
Eoin Lalor ◽  
Andrew L Mason
Keyword(s):  
Plasma Cells ◽  
Systemic Disease ◽  
Correct Diagnosis ◽  
Elevated Serum ◽  
Immunoglobulin G4 ◽  
Overlap Syndromes ◽  
Igg4 Related Disease ◽  
Hepatobiliary Diseases ◽  
Made In

The first description of autoimmune pancreatitis and elevated serum immunoglobulin-G4 (IgG4) in 2001 heralded further reports of several related autoimmune diseases with raised IgG4 levels. It is now recognized that a spectrum of overlap syndromes associated with increased IgG4 and biopsy evidence of IgG4-producing plasma cells, which has now been convincingly linked with cholangitis, autoimmune hepatitis, Sjögren’s syndrome, nephritis and retroperitoneal fibrosis. Collectively, this disease cluster is referred to as IgG4-related systemic disease. The importance of making the correct diagnosis is underscored by the management of individuals with IgG4-related systemic disease. In the first instance, patients generally have a dramatic response to immunosuppressive therapy, whereas patients with other forms of cholangitis and pancreatitis do not. Also, surgical management of pancreatic malignancy can be avoided once the correct diagnosis of IgG4-related disease has been made. In the present review, an overview of the current information regarding the role of IgG4 and IgG4-positive cells affecting the biliary system, pancreas and liver is provided.

scholarly journals Immunoglobulin G4-Related Disease Mimicking Asthma

2013 ◽  
Vol 20 (2) ◽  
pp. 87-89 ◽  
Author(s):  
Hiroshi Sekiguchi ◽  
Ryohei Horie ◽  
Timothy R Aksamit ◽  
Eunhee S Yi ◽  
Jay H Ryu
Keyword(s):  
Airway Inflammation ◽  
Submandibular Gland ◽  
Autoimmune Pancreatitis ◽  
Plasma Cells ◽  
Systemic Disease ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Organ Manifestations

Immunoglobulin (Ig) G4-related disease (also known as ‘IgG4-related sclerosing disease’, ‘IgG4-related systemic disease’ or ‘hyper-IgG4-disease’) is a recently recognized systemic fibroinflammatory disease associated with IgG4-positive plasma cells in tissue lesions. IgG4-related disease was initially described as autoimmune pancreatitis, but it is now known to affect virtually any organ. The authors describe a patient presenting with multi-organ manifestations, including airway inflammation mimicking asthma, pulmonary parenchymal infiltrates, intrathoracic lymphadenopathy, submandibular gland swelling and a kidney mass.

scholarly journals Immunoglobulin G4-related disease - diagnostic dilemma and importance of clinical judgement: a case report

2020 ◽  
Vol 8 (11) ◽  
pp. 4144
Author(s):  
Jagadeesh Chandrasekaran ◽  
Phani Krishna Machiraju
Keyword(s):  
Early Recognition ◽  
Elevated Serum ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Inflammatory Disorder ◽  
Imaging Features ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
The Right

Immunoglobulin G4 (IgG4)-related disease is a multi-organ, immune-mediated, fibro-inflammatory disorder characterized by tumefactive masses in the affected organs. Incidence and prevalence of IgG4-related disease (RD) are not clearly known and have slight male preponderance. It often involves multiple organs at the time of presentation or over the course of disease mimicking malignancy, Sjogren's syndrome, antineutrophil cytoplasmic antibodies associated vasculitis, infections. A thorough workup is needed to rule out these mimickers. A 33-year-old gentleman presented to us with history of progressive swelling in the right peri-orbital region for four years. On evaluation, abdominal imaging was notable for the sausage-shaped pancreas and hypoenchancing nodules in bilateral kidneys. Histological examination of right lacrimal gland revealed lymphoplasmacytic infiltrate and storiform fibrosis. Serum IgG4 levels were normal, and immunostaining was negative. A diagnosis of IgG4-RD was suggested because of multi-organ involvement, classical radiological and histopathological features. Awareness about IgG4-RD, an under-recognized entity is essential, as it is treatable, and early recognition may help in a favourable outcome. Appropriate use of clinicopathological, serological and imaging features in the right clinical context may help in accurate diagnosis. Elevated serum IgG4 levels and biopsy are not mandatory for the diagnosis.

scholarly journals Urologic manifestations of IgG4-related disease

2020 ◽  
Vol 80 (5) ◽  
pp. 1-10
Author(s):  
Benjamin Enrique Montaño Roca ◽  
Davide Vanacore ◽  
Gustavo Gallegos Sánchez ◽  
César Eduardo Rosales Velázquez ◽  
Guillermo Enrique Ruvalcaba Oceguera ◽  
...  
Keyword(s):  
Plasma Cells ◽  
Elevated Serum ◽  
Aged Patients ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Lymphoplasmacytic Infiltrate ◽  
Urinary Tract Symptoms ◽  
Serum Igg4 ◽  
Inflammatory Pseudotumors ◽  
Clinically Significant

IgG4-related disease (IgG4-RD) is a clinical entity characterized by elevated serum IgG4 and tumor-like inflammation, with tissue infiltration by IgG4 and plasma cells. IgG4-RD is rare, but clinically significant, and its urologic manifestations have been reported in the literature. The present review covers a broad spectrum, describing the pathologies related to the area of urology. In 2003, Terumi Kamisawa was the first to recognize IgG4-RD, characterized by multiorgan lesions in patients with autoimmune pancreatitis and classified as an inflammatory and fibrotic entity with a dense lymphoplasmacytic infiltrate, positive for  IgG4.(1–3) It presents in middle-aged patients, between 59-68 years of age, with no clear distribution by sex, (4–6) and has different clinical presentations. The main urologic manifestations are inflammatory pseudotumors and lower urinary tract symptoms. The present article offers a clear, general overview of the disease, encompassing its pathophysiology, diagnosis, and treatment, from the perspective of urology. 

scholarly journals An Overlapping Case of IgG4-Related Disease and Systemic Lupus Erythematosus

2019 ◽  
Vol 7 ◽  
pp. 232470961986229 ◽  
Author(s):  
Srikanth Naramala ◽  
Sharmi Biswas ◽  
Sreedhar Adapa ◽  
Vijay Gayam ◽  
Venu Madhav Konala ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Systemic Disease ◽  
Lymph Node Biopsy ◽  
Systemic Lupus ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Double Stranded Dna ◽  
Serum Igg4

We are reporting a case of a 63-year-old Chinese female who presented to the rheumatology clinic with positive antinuclear antibody and unintentional weight loss along with lymphadenopathy. Further workup revealed eosinophilia, elevated anti–double stranded DNA, serum protein, and serum IgG4 (immunoglobulin G4). The patient was diagnosed with systemic lupus erythematosus. Due to the raised IgG4 level along with eosinophilia and diffuse lymphadenopathy, IgG4-related systemic disease was suspected. It was confirmed with IgG4 staining on lymph node biopsy. Our case is presenting the fact that systemic lupus erythematosus and IgG4-related disease can be present in the same patient with multiple overlapping features making accurate diagnosis challenging.

scholarly journals IgG4-Related Lymphadenopathy

2012 ◽  
Vol 2012 ◽  
pp. 1-8 ◽  
Author(s):  
Yasuharu Sato ◽  
Tadashi Yoshino
Keyword(s):  
Lymph Node ◽  
Plasma Cells ◽  
Elevated Serum ◽  
Serum Levels ◽  
Lymph Node Involvement ◽  
Lymph Node Biopsy ◽  
Immunoglobulin G4 ◽  
Reactive Protein ◽  
Serum Igg4 ◽  
Node Involvement

Lymphadenopathy is frequently observed in patients with immunoglobulin G4-related disease (IgG4-RD) and sometimes appears as the first manifestation of the disease. The diagnosis of IgG4-related lymphadenopathy is complicated owing to a great histological diversity, with at least 5 histological subtypes. Indeed, lymph node biopsy may be performed under the suspicion that the lymphadenopathy is a malignant lymphoma or other lymphoproliferative disorder. The diagnosis of IgG4-RD is characterized by both elevated serum IgG4 (>135?mg/dL) and histopathological features, including a dense lymphoplasmacytic infiltrate rich in IgG4+plasma cells (IgG4+/IgG+plasma cell ratio >40%). However, patients with hyper-interleukin (IL-) 6 syndromes such as multicentric Castleman’s disease, rheumatoid arthritis, and other immune-mediated conditions frequently show lymph node involvement and often fulfill the diagnostic criteria for IgG4-RD. Owing to these factors, IgG4-RD cannot be differentiated from hyper-IL-6 syndromes on the basis of histological findings alone. Laboratory analyses are crucial to differentiate between the 2 diseases. Hyper-IL-6 syndromes are characterized by elevated serum levels of IgG, IgA, IgM, and C-reactive protein (CRP); thrombocytosis; anemia; hypoalbuminemia; hypocholesterolemia. In contrast, IgG4-RD does not share any of these characteristics. Therefore, the diagnosis of IgG4-RD requires not only pathological findings but also clinical and laboratory analyses.

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 IgG4-Related Disease: A Multispecialty Condition

2014 ◽  
Vol 2014 ◽  
pp. 1-3
Author(s):  
Iuri Usêda Santana ◽  
Emanuela Pimenta da Fonseca ◽  
Mittermayer Barreto Santiago
Keyword(s):  
Plasma Cells ◽  
Elevated Serum ◽  
Diffuse Abdominal Pain ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Lymphoplasmacytic Infiltrate ◽  
Serum Igg4 ◽  
Polyclonal Hypergammaglobulinemia ◽  
History Of ◽  
Systemic Condition

IgG4-related disease (IgG4-RD) is a recently recognized group of conditions, characterized by tumor-like swelling of involved organs, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, variable degrees of fibrosis, and elevated serum IgG4 concentrations. Currently IgG4-RD is recognized as a systemic condition that can affect several organs and tissues. Herein we report the case of a 34-year-old male patient who was admitted to our hospital with diffuse abdominal pain, weight loss, and painful stiffness in his neck. He had a history of tumoral mass of the left maxillary region, right palpebral ptosis with protrusion of the eyeball, and chronic dry cough for about 6 years. Laboratory tests revealed polyclonal hypergammaglobulinemia and increased serum IgG4 levels. Immunohistochemical staining of the maxillary biopsy was compatible with IgG4-RD. He had an excellent response to corticosteroid therapy. This case highlights that IgG4-RD should be included in the differential diagnosis with multisystem diseases.

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