scholarly journals Thoracic Paravertebral Mass as an Infrequent Manifestation of IgG4-Related Disease

2017 ◽  
Vol 2017 ◽  
pp. 1-4
Author(s):  
Melissa Matzumura Kuan ◽  
Bernard Rubin ◽  
Alireza Meysami
Keyword(s):  
Submandibular Gland ◽  
Plasma Cells ◽  
Inflammatory Cells ◽  
Disease Recurrence ◽  
Tissue Mass ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Chronic Sclerosing Sialadenitis ◽  
Resistant Disease ◽  
Paravertebral Mass

Case. A 50-year-old African American male presented with abdominal pain and significant weight loss. On physical examination, he had parotid and submandibular gland enlargement associated with right eye proptosis. Computed tomography showed a thoracic paravertebral soft tissue mass, enlarged lymph nodes, and ascending aortic aneurysm. Laboratory results were remarkable for elevated total IgG and IgG4 subclass. The submandibular gland pathology revealed chronic sclerosing sialadenitis, with a large subset of inflammatory cells positively staining for IgG4. The histology of the paravertebral mass demonstrated fibrosclerosis with increased lymphocytic infiltrate, associated with increased IgG4 plasma cells. He was diagnosed with immunoglobulin G4-related disease (IgG4-RD). Steroid therapy initially yielded improvement; however, after steroids were stopped, there was disease recurrence. Prednisone was restarted, and the plan was to start him on rituximab. Interestingly, the patient’s brother also had IgG4-RD. Conclusion. IgG4-RD can present as a paravertebral mass which is usually responsive to steroids; however, recurrent and resistant disease can be seen for which steroid-sparing agents such as rituximab should be considered. In addition, to the best of our knowledge, this is the first reported case of IgG4-RD in two family members presenting as a paravertebral mass, highlighting an exciting area for more research in the future.

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.

Immunoglobulin G4 related chronic sclerosing sialadenitis

2015 ◽  
Vol 129 (3) ◽  
pp. 226-231 ◽  
Author(s):  
E L Culver ◽  
A Hunt ◽  
E Crewe ◽  
K A Shah ◽  
P Martinez-Devesa
Keyword(s):  
Literature Search ◽  
Systemic Disease ◽  
Clinical Suspicion ◽  
Cochrane Library ◽  
Disease Entity ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Chronic Sclerosing Sialadenitis ◽  
Search Terms ◽  
Salivary Gland Disease

AbstractBackground:ENT surgeons may be the first specialists to encounter and diagnose patients with salivary gland disease. A new entity involving the salivary glands has recently been described of which ENT surgeons need to be aware: immunoglobulin G4 related chronic sclerosing sialadenitis.Method:A literature search of Medline, Embase and Cochrane Library databases was performed, using the search terms ‘IgG4’, ‘hyperIgG4 syndrome’ and ‘IgG4 related chronic sclerosing sialadenitis’.Results:Knowledge concerning immunoglobulin G4 related chronic sclerosing sialadenitis is rapidly increasing. This new entity is part of a fibro-inflammatory corticosteroid-responsive systemic disease (immunoglobulin G4 related disease) and has been described in almost every organ. Biopsy of the submandibular gland can be diagnostic. However, the diagnosis can easily be overlooked if: clinical suspicion is not high, one is unaware of the classical morphology and/or immunoglobulin G4 staining is not performed. This paper presents a summary of the current understanding of the disease and its management.Conclusion:ENT surgeons should be aware of this new disease entity. Patients with systemic disease should be managed under a multidisciplinary team, with input from clinicians who have an interest in such diseases (such as gastroenterologists and rheumatologists), and input from histopathologists and radiologists.

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.

scholarly journals Manifestations of immunoglobulin G4 related disease in otolaryngology: case reports and review of the literature

2014 ◽  
Vol 128 (S2) ◽  
pp. S10-S15 ◽  
Author(s):  
W K Wong ◽  
R Campbell ◽  
R Douglas
Keyword(s):  
B Lymphocyte ◽  
Plasma Cells ◽  
Case Reports ◽  
Correct Diagnosis ◽  
Infratemporal Fossa ◽  
The Body ◽  
Infraorbital Nerve ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Storiform Fibrosis

AbstractBackground:Immunoglobulin G4 related disease is an inflammatory condition characterised by the presence of fibrotic lesions infiltrated by immunoglobulin G4 positive plasma cells. It can arise from almost any region of the body and it is being increasingly recognised in the head and neck. Regardless of the site of involvement, the histopathological resemblance is remarkable. Dense lymphoplasmacytic infiltration, overabundance of immunoglobulin G4 bearing plasma cells and presence of storiform fibrosis are typical findings.Case reports:This paper presents two cases of immunoglobulin G4 related disease in which there was involvement of the orbit, the infraorbital nerve and the infratemporal fossa. Diagnosis was established in both cases by biopsying radiologically abnormal tissue in the infratemporal fossa.Conclusion:An awareness of this condition is required to establish the diagnosis and initiate appropriate therapy. Glucocorticoids are the mainstay of initial treatment. The effectiveness of B-lymphocyte depletion with rituximab has also been reported. Correct diagnosis may spare patients from unnecessarily radical surgery.

scholarly journals Unique angiographic findings in a patient with myocardial ischemia and immunoglobulin G4-related disease

2017 ◽  
Vol 5 ◽  
pp. 2050313X1772801
Author(s):  
Yusuke Kanzaki ◽  
Takashi Miura ◽  
Naoto Hashizume ◽  
Tatsuya Saigusa ◽  
Soichiro Ebisawa ◽  
...  
Keyword(s):  
Coronary Artery Disease ◽  
Coronary Artery ◽  
Coronary Angiography ◽  
Intravascular Ultrasound ◽  
Plasma Cells ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Multiple Organs ◽  
History Of ◽  
Artery Disease

Immunoglobulin G4-related disease characteristically involves multiple organs including the heart and coronary arteries. Immunoglobulin G4-related coronary artery disease is thought to be due to periarteritis and histopathologically is characterized by marked thickening of the adventitia and periarterial fat with infiltration of immunoglobulin G4-positive plasma cells. Although comprehensive diagnostic criteria require a biopsy for a definite or probable diagnosis of immunoglobulin G4-related disease, obtaining a coronary artery biopsy is difficult and risky. However, imaging findings including coronary angiography and intravascular ultrasound might be useful tools to establish a diagnosis of immunoglobulin G4-related coronary artery disease. We report a case of a 63-year-old man with a history of immunoglobulin G4-related disease who presented with exertional chest pain. We found unique angiographic and intravascular ultrasound features of immunoglobulin G4-related coronary artery disease that distinguished it from those of arteriosclerotic coronary artery disease and suggest that coronary angiography and intravascular ultrasound might be useful tools in the diagnosis of immunoglobulin G4-related coronary artery disease.

scholarly journals Chronic sclerosing sialadenitis (Küttner's tumor): an uncommon cause of submandibular swelling

2021 ◽  
Vol 7 (3) ◽  
pp. 561
Author(s):  
Ranjana Giri ◽  
Subhransu Kumar Hota ◽  
Devika Chauhan ◽  
Hardik Kabra ◽  
Prita Pradhan ◽  
...  
Keyword(s):  
Submandibular Gland ◽  
Gold Standard ◽  
Benign Condition ◽  
Related Disease ◽  
Chronic Sclerosing Sialadenitis ◽  
Igg4 Related Disease ◽  
Küttner’S Tumor

<p class="Noindentpara">Chronic sclerosing sialadenitis (CSS) or Küttner’s tumour, a rare benign condition affecting submandibular gland, is now believed to be a localised type of IgG4-related disease. It presents as an asymptomatic swelling. Histopathology is the gold standard for diagnosis. Here we discuss the same in a 55-year-old male presenting as a painless submandibular swelling.</p>

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.

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