scholarly journals Pleomorphic Pulmonary Manifestations of IgG4-Related Disease

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Srikanth Naramala ◽  
Sharmi Biswas ◽  
Sreedhar Adapa ◽  
Vijay Gayam ◽  
Romeo C. Castillo ◽  
...  
Keyword(s):  
Human Body ◽  
Plasma Cells ◽  
Clear Picture ◽  
Lung Involvement ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Pulmonary Manifestations ◽  
Sclerosing Pancreatitis

Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory disorder which has been first reported in 2001 by Hamano and colleagues in a patient with autoimmune sclerosing pancreatitis. Almost every organ in the human body can be affected by IgG4-RD from infiltration with IgG4-positive plasma cells. Involvement of lungs with IgG4 is reported previously, but still, there is no clear picture of the pathophysiology behind lung involvement. Here, we are presenting a patient who has IgG4-RD presenting as pseudotumor of the lungs.

scholarly journals Sudden Cardiac Death Due to Coronary Artery Involvement by IgG4-Related Disease: A Rare, Serious Complication of a Rare Disease

2014 ◽  
Vol 138 (6) ◽  
pp. 833-836 ◽  
Author(s):  
Nimesh R. Patel ◽  
Mary L. Anzalone ◽  
L. Maximilian Buja ◽  
M. Tarek Elghetany
Keyword(s):  
Sudden Cardiac Death ◽  
Coronary Arteries ◽  
Cardiac Death ◽  
Plasma Cells ◽  
Severe Stenosis ◽  
Immunoglobulin G4 ◽  
Emergency Center ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Systemic Disorder

Immunoglobulin G4–related disease (IgG4-RD) is a systemic disorder characterized by multiorgan fibrosis with IgG4-producing plasma cells, increased IgG4 serum concentration, and responsiveness to steroid therapy. Involvement of the pancreas, salivary glands, orbit, aorta, and other sites has been well documented in the literature; however, there have been limited reports of cases involving the coronary arteries. We report the case of a 53-year-old Hispanic man who was brought to the emergency center and diagnosed with sudden cardiac death. Autopsy was subsequently performed, revealing multiorgan involvement by IgG4-RD, including involvement of the coronary arteries. The inflammation and fibrosis, in combination with concomitant atherosclerotic disease, resulted in severe stenosis of the coronary arteries. Two of the coronary arteries were further occluded by thrombosis. These factors led to cardiac hypoperfusion, myocardial infarction and, ultimately, sudden cardiac death. Fatal involvement of the coronary arteries has not been previously reported, raising a new concern for a severe complication of IgG4-RD.

scholarly journals Eosinophilic Angiocentric Fibrosis: A Review and Update of Its Association With Immunoglobulin G4–Related Disease

2018 ◽  
Vol 142 (12) ◽  
pp. 1560-1563
Author(s):  
Janice Ahn ◽  
Melina Flanagan
Keyword(s):  
Head And Neck ◽  
Plasma Cells ◽  
Neck Region ◽  
Immunoglobulin G4 ◽  
Head And Neck Region ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Previous Trauma ◽  
Eosinophilic Angiocentric Fibrosis

Eosinophilic angiocentric fibrosis is a rare indolent lesion of the head and neck region that has characteristic histologic findings of onionskin fibrosis and prominent eosinophils. Its pathogenesis has been poorly understood and has been most commonly attributed to hypersensitivity or previous trauma. Recently, the lesion has been included in the spectrum of immunoglobulin G4 (IgG4)–related disease. However, few of the existing cases of eosinophilic angiocentric fibrosis have been evaluated for IgG4+ and IgG+ plasma cells. Therefore, we provide an update on the clinical and histologic features of eosinophilic angiocentric fibrosis to increase awareness of the entity and encourage its further characterization as an IgG4-related disease.

IgG4-related lung disease on the horizon

2018 ◽  
Vol 0 (0) ◽  
Author(s):  
Tin Lok Lai ◽  
Cheuk Wan Yim
Keyword(s):  
Lung Disease ◽  
Lung Biopsy ◽  
Lung Nodules ◽  
Lung Involvement ◽  
Immunoglobulin G4 ◽  
X Ray ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Chest X Ray ◽  
Diffuse Lung

Abstract Immunoglobulin G4 (IgG4) related lung disease is an emerging entity. We report a case of a 42-year-old man presented with fever and cough with minimal sputum. Chest X-ray revealed diffuse reticulonodular shadows. Extensive investigations were performed, including video-assisted thoracoscopic lung biopsy, which confirmed the diagnosis of IgG4-related disease (IgG4-RD) with lung involvement. This case report aims to illustrate that IgG4-related lung involvement can present as diffuse lung nodules and can affect different pulmonary structures. IgG4-RD should always be considered when a similar scenario is encountered.

scholarly journals Localized IgG4-related disease manifested on the tongue: a case report

2021 ◽  
Vol 126 (1) ◽  
Author(s):  
Helya Hashemi ◽  
Andreas Thor ◽  
Erik Hellbacher ◽  
Marie Carlson ◽  
Miklós Gulyás ◽  
...  
Keyword(s):  
Case Report ◽  
Oral Mucosa ◽  
Plasma Cells ◽  
Clinical Manifestations ◽  
Assessment System ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Multiple Organs ◽  
Igg4 Related Disease ◽  
Initial Symptoms

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that can affect multiple organs. IgG4-RD may show a variety of initial symptoms. In the oral mucosa, lesions present as inflammatory fibrosis with a large number of IgG4-positive plasma cells. Evaluating treatment is a well-known problem in IgG4-RD due to the absence of an established assessment system. There are difficulties in defining the severity of the disease, which is why treatment is primarily based on its clinical manifestations. We present a case report of localized IgG4-RD with ulcerative and proliferative manifestations on the tongue, which clinically mimicked oral squamous cell carcinoma. A tumor-like lesion on the tongue can indicate something else other than the malignant or reactive changes commonly found in the oral mucosa. Multiple differential diagnoses of these atypical oral lesions, including localized IgG4-RD, should be considered.

scholarly journals Abnormal Expression of CD20 on IgG4 Plasma Cells Associated With IgG4-Related Lymphadenopathy

2013 ◽  
Vol 137 (9) ◽  
pp. 1282-1285 ◽  
Author(s):  
Kate E. Grimm ◽  
Antony Bakke ◽  
Dennis P. O'Malley
Keyword(s):  
Flow Cytometry ◽  
Lymph Node ◽  
Clinical Efficacy ◽  
Plasma Cells ◽  
Lymph Node Biopsy ◽  
Histologic Diagnosis ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Node Biopsy

Context.—Immunoglobulin G4 (IgG4)–related disease is a recently described entity that presents as mass-forming lesions in soft tissue, exocrine glands, and in lymph nodes as IgG4-related lymphadenopathy. The underlying pathologic mechanism of IgG4-related disease is unclear; however, rituximab (an anti-CD20 monoclonal antibody) has been shown to have clinical efficacy. Objective.—To look for the presence or absence of CD20 on the IgG4-expressing plasma cells in IgG4-related lymphadenopathy. Design.—Twelve flow cytometry cases were identified through a retrospective review from the authors' institutions files. Cases were selected by the presence of a lymph node biopsy specimen with increased IgG4 plasma cells by immunohistochemistry and a histologic diagnosis compatible with IgG4-related lymphadenopathy. Results.—We report dim CD20 expression on plasma cells in all cases for which a plasma cell population was clearly identified by flow cytometry. These cases were from patients with lymph node biopsy specimens that met published criteria for IgG4-related lymphadenopathy. Conclusions.—This finding may be one potential explanation for the clinical efficacy of rituximab in IgG4-related disease.

scholarly journals P167 IgG4 related disease: response to immunosuppressive therapy: a single centre retrospective study in the United Kingdom

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Shirish Sangle ◽  
Neil T Morton ◽  
Alina Casian ◽  
Louise Nel ◽  
Jennifer Hanna ◽  
...  
Keyword(s):  
Observational Study ◽  
Immunosuppressive Therapy ◽  
Plasma Cells ◽  
Ct Scanning ◽  
Retrospective Observational Study ◽  
Inflammatory Disorder ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Markers Of Inflammation

Abstract Background/Aims  IgG4 related disease (IgG4-RD) is a rare immune-mediated condition, increasingly being recognised as a multi-organ disorder. It is a relatively new entity and the precise prevalence is not known. It is a chronic fibro-inflammatory condition with raised circulating levels of immunoglobulin G4 and accumulation of plasma cells and fibrosis in the affected tissue. We report a retrospective observational study of patients with IgG4 related disease (IgG4-RD). Methods  We conducted a retrospective observational study of patients seen at Guy’s and St Thomas’ NHS Foundation Trust Hospitals, London, UK. The data collected was analysed for clinical presentation, laboratory markers of inflammation, immunoglobulin subsets, autoantibody profiles, imaging and histopathology and compared to the 2019 ACR/EULAR criteria to determine a confirmed diagnosis of IgG4-RD. Data was also collected post standard-of-care treatment, including patient clinical outcomes and possible improvement. Results  The study included a multi-ethnic cohort of 83 patients with multi-organ involvement. Fifty-nine of the eighty-three patients were classified as confirmed IgG4-RD (71%). Seventy one patients had biopsies of which 49 of the 59 (91%) confirmed IgG4-RD patients had biopsies consistent with IgG4-RD. Fifty patients underwent PET-CT scanning of which uptake was seen in 76% of those with confirmed IgG4-RD (26/34). Immunoglobulin IgG subclass analysis showed significantly higher IgG4 levels in the confirmed IgG4-RD group as compared to possible IgG4. Treatments were with corticosteroids and immunosuppressants such as azathioprine, methotrexate, mycophenolate, cyclophosphamide and rituximab. P167 Table 1:IgG4 levels and inflammatory markers for pre and post treatment groupsPre-RxPost-Rxp-valueCRPmean29 mg/l9 mg/l0.0298*Range0-300 mg/l1-70 mg/lESRmean35 mm/hr17 mm/hr0.0002*Range2-131 mm/hr1-74 mm/hrSerum IgG4mean4.9 g/L2.83 g/L0.0001*Range0.1-24.2 g/L0.02-13.3 g/L Conclusion  IgG4-RD is a fibro-inflammatory disorder involving multiple organs. If not treated adequately may develop severe organ damage. It often responds to corticosteroids but may require other immunosuppressive therapy. Treatment with biologics such as B cell depletion therapy results are encouraging. Disclosure  S. Sangle: None. N.T. Morton: None. A. Casian: None. L. Nel: None. J. Hanna: None. A. Fernando: None. R. Bell: None. P. Taylor: None. J. Pattison: None. T. O'Brien: None. D. D'Cruz: None.

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 of the Gastrointestinal Tract: A 21st Century Chameleon

2015 ◽  
Vol 139 (6) ◽  
pp. 742-749 ◽  
Author(s):  
Vikram Deshpande
Keyword(s):  
Gastrointestinal Tract ◽  
Personal Experience ◽  
Plasma Cells ◽  
Diagnostic Algorithm ◽  
Obliterative Phlebitis ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Lymphoplasmacytic Infiltrate ◽  
Histologic Changes

Context Immunoglobulin G4 (IgG4)–related disease is a systemic fibroinflammatory disease capable of affecting virtually any organ. Although the pancreas and hepatobiliary system are commonly affected, involvement of the tubular gut is unusual. The pancreatic manifestations of this disease (autoimmune pancreatitis) often mimic pancreatic carcinoma, whereas the hepatobiliary manifestations are mistaken for cholangiocarcinoma or primary sclerosing cholangitis. The characteristic histologic features include a dense lymphoplasmacytic infiltrate, storiform-type fibrosis, and obliterative phlebitis. An increase in IgG4+ plasma cells and an IgG4 to IgG ratio of more than 40% are considered obligatory components of the diagnostic algorithm. Objective To review the challenges associated with the diagnosis of IgG4-related disease of the gastrointestinal tract. Data Sources A review of pertinent literature, along with the author's personal experience, based on institutional and consultation materials. Conclusion The complete spectrum of histologic changes is seldom captured in a biopsy specimen, and thus, the histopathology findings are best interpreted within the overall clinical context. Increased IgG4+ plasma cells are identified in a variety of benign and malignant diseases of the gastrointestinal tract.

IgG4-related Diseases Including Mikulicz’s Disease and Sclerosing Pancreatitis: Diagnostic Insights

2010 ◽  
Vol 37 (7) ◽  
pp. 1380-1385 ◽  
Author(s):  
YASUFUMI MASAKI ◽  
SUSUMU SUGAI ◽  
HISANORI UMEHARA
Keyword(s):  
Differential Diagnosis ◽  
Plasma Cells ◽  
Elevated Serum ◽  
Treatment Strategies ◽  
Mikulicz’S Disease ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Sclerosing Pancreatitis ◽  
Systemic Condition

Since the first report of serum IgG4 elevation in sclerosing pancreatitis in 2001, various systemic disorders have been reported to elevate IgG4, and many names have been proposed from the perspective of the systemic condition. Despite similarities in the organs damaged in IgG4-related Mikulicz’s disease and Sjögren’s syndrome, there are marked clinical and pathological differences between the 2 entities. The majority of cases diagnosed with autoimmune pancreatitis in Japan are IgG4-related sclerosing pancreatitis, and it should be recognized that this is distinct from the Western type. Diagnosis of IgG4-related disease is defined by both elevated serum IgG4 (> 1.35 g/l) and histopathological features, including lymphocyte and IgG4+ plasma cell infiltration (IgG4+ plasma cells/IgG+ plasma cells > 50% on a highly magnified slide checked at 5 points). Differential diagnosis from other distinct disorders is necessary: these include sarcoidosis, Castleman’s disease, Wegener’s granulomatosis, lymphoma, cancer, and other existing conditions. The Japanese IgG4 research group has begun multicenter prospective studies to improve diagnostic criteria and treatment strategies.

scholarly journals IgG4-related lung disease on the horizon

2018 ◽  
Vol 18 (1) ◽  
pp. 14-17
Author(s):  
Tin Lok Lai ◽  
Cheuk Wan Yim
Keyword(s):  
Lung Disease ◽  
Lung Biopsy ◽  
Lung Nodules ◽  
Lung Involvement ◽  
Immunoglobulin G4 ◽  
X Ray ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Chest X Ray ◽  
Diffuse Lung

Abstract Immunoglobulin G4 (IgG4) related lung disease is an emerging entity. We report a case of a 42-year-old man presented with fever and cough with minimal sputum. Chest X-ray revealed diffuse reticulonodular shadows. Extensive investigations were performed, including video-assisted thoracoscopic lung biopsy, which confirmed the diagnosis of IgG4-related disease (IgG4-RD) with lung involvement. This case report aims to illustrate that IgG4-related lung involvement can present as diffuse lung nodules and can affect different pulmonary structures. IgG4-RD should always be considered when a similar scenario is encountered.

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