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 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 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 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 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 The Utility of Serum IgG4 Concentrations as a Biomarker

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Shigeyuki Kawa ◽  
Tetsuya Ito ◽  
Takayuki Watanabe ◽  
Masahiro Maruyama ◽  
Hideaki Hamano ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
Plasma Cells ◽  
High Serum ◽  
Wall Thickening ◽  
Systemic Involvement ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Long Term Follow Up ◽  
Characteristic Features

IgG4-related disease is a new disease entity involving IgG4 in its clinical presentation and having 6 characteristic features: (1) systemic involvement; (2) solitary or multiple lesions showing diffuse or localized swelling, masses, nodules, and/or wall thickening on imaging; (3) high serum IgG4 concentration >135 mg/dL; (4) abundant infiltration of lymphoplasmacytes and IgG4-bearing plasma cells; (5) a positive response to corticosteroid therapy; and (6) complications of other IgG4-related diseases. To date, most IgG4-related diseases have been recognized as extrapancreatic lesions of autoimmune pancreatitis. This paper will discuss the utility of IgG4 as a biomarker of IgG4-related diseases, including in the diagnosis of autoimmune pancreatitis and its differentiation from pancreatic cancer, in the prediction of relapse, in the long-term follow-up of patients with autoimmune pancreatitis and normal or elevated IgG4 concentrations, and in patients with autoimmune pancreatitis and extrapancreatic lesions, as well as the role of IgG4 in the pathogenesis of IgG4-related disease.

scholarly journals IgG4 Related Disease of The Larynx Mimicking Malignancy: A Case Report and Review of the Literature

2021 ◽  
Author(s):  
Sevda Akyol ◽  
Ozlem Saraydaroglu ◽  
Omer Afsin Ozmen
Keyword(s):  
Plasma Cells ◽  
Clinical Laboratory ◽  
Review Of The Literature ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Storiform Fibrosis ◽  
Lymphoplasmacytic Infiltration ◽  
Igg4 Level

Abstract Objectives: Immunoglobulin G4–related disease is characterized by increased serum IgG4 level, enlargement in the relevant organs and histopathologically intense storiform fibrosis, lymphoplasmacytic infiltration rich in IgG4 positive plasma cells, and obliterative phlebitis.Methods and Results: In this report, a patient who underwent a laryngeal biopsy with a pre-diagnosis of malignancy, but had findings consistent with immunoglobulin G4–related disease in the biopsy sample, is described.Conclusion: Immunoglobulin G4–related disease can be seen in very rare localizations. It should be kept in mind in differential diagnosis when tissues especially containing inflammation rich in plasma cells are encountered. Clinical, laboratory and pathological correlation is extremely important in the diagnosis of an IgG4-related disease.

IgG4-related disease: an analysis of the clinicopathological spectrum: UK centre experience

2021 ◽  
pp. jclinpath-2021-207748
Author(s):  
Wajira Dassanayaka ◽  
Kanchana Sanjeewani Liyanaarachchi ◽  
Aftab Ala ◽  
Izhar N Bagwan
Keyword(s):  
Plasma Cells ◽  
High Serum ◽  
High Power Field ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Storiform Fibrosis ◽  
Common Clinical Presentation ◽  
Characteristic Features

AimTo retrospectively evaluate the characteristic clinicopathological spectrum in patients with suspicion of IgG4-related disease (IgG4RD).MethodsWinpath histology database from January 2011 to April 2018 identified all suspected IgG4RD cases wherein IgG4 immunohistochemistry was performed. The histology slides were reviewed to categorise cases into Boston criteria groups—highly suggestive of IgG4RD, probable IgG4RD and insufficient evidence. Information regarding clinical data, treatment received, follow-up and serum IgG4 levels was obtained from medical records and AllScripts Patient Administration System (APAS) clinical database.ResultsThe study included 204 patients and the most common sites of biopsy/resection were pancreas and duodenum. The most common clinical presentation was fibroinflammatory lesion or mass/lump. On histology, 54/204 (26.47%) cases showed typical storiform fibrosis, 65/204 (32.64%) had >10 IgG4+ plasma cells per high power field and only one case showed thrombophlebitis (0.49%). There were 14/204 (6.78%) cases categorised as highly suggestive of IgG4RD; 8 of these showed high serum IgG4 levels and were managed clinically as true IgG4RD.ConclusionHistological diagnosis of IgG4RD remains challenging, as not all characteristic features are always present especially in small biopsies. Due to the novelty of its experience, fear of over diagnosis in the context of malignancy and features overlapping with diseases of similar clinical scenario, diagnosis of IgG4RD has become more puzzling. Further multicentre clinical trials/studies are advisable.

scholarly journals IgG4-Related Tubulointerstitial Nephritis Associated with Membranous Nephropathy in Two Patients: Remission after Administering a Combination of Steroid and Mizoribine

2014 ◽  
Vol 2014 ◽  
pp. 1-6 ◽  
Author(s):  
Kana N. Miyata ◽  
Hiromi Kihira ◽  
Manabu Haneda ◽  
Yasuhide Nishio
Keyword(s):  
Renal Biopsy ◽  
Membranous Nephropathy ◽  
Plasma Cells ◽  
Tubulointerstitial Nephritis ◽  
High Serum ◽  
Immunoglobulin G4 ◽  
Japanese Men ◽  
Renal Lesions ◽  
Igg4 Related Disease ◽  
Prednisolone Treatment

We report two cases of Japanese men who presented with proteinuria, eosinophilia, hypocomplementemia, and high serum immunoglobulin G4 (IgG4) concentration and were diagnosed with membranous nephropathy associated with IgG4-related tubulointerstitial nephritis on renal biopsy. The typical renal lesions of IgG4-related disease are tubulointerstitial nephritis, which improves remarkably with steroid therapy, and occasional glomerular changes. In our two cases, renal biopsy revealed IgG4-positive immune complex deposits in glomeruli in a pattern of membranous nephropathy and concurrent tubulointerstitial nephritis with IgG4 plasma cells. In both cases, proteinuria persisted with initial prednisolone treatment and was resolved only after the addition of mizoribine. We report the first two cases in which the combination of prednisolone and mizoribine was effective for treating membranous nephropathy associated with IgG4-related tubulointerstitial nephritis.

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.

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