Are plasma cell-rich inflammatory conditions of the oral mucosa manifestations of IgG4-related disease?

2015 ◽  
Vol 68 (10) ◽  
pp. 802-807 ◽  
Author(s):  
Hannah Cottom ◽  
Alan J Mighell ◽  
Alec High ◽  
Adrian C Bateman
Keyword(s):  
Oral Mucosa ◽  
Plasma Cell ◽  
Plasma Cells ◽  
Mucosal Inflammation ◽  
High Power Field ◽  
Immunoglobulin G4 ◽  
Cell Ratio ◽  
Related Disease ◽  
Inflammatory Conditions ◽  
Medical Histories

AimThe aim of this study was to characterise plasma cell infiltrates, in terms of IgG4 positivity, in specific and non-specific plasma cell-rich chronic inflammatory conditions of the oral mucosa. Exploring the possibility that specific plasma cell-rich oral inflammatory conditions have association with or represent an oral manifestation of immunoglobulin G4-related disease (IgG4-RD).MethodsTen patients with plasma cell-rich chronic inflammatory conditions of the oral mucosa were identified (seven—plasma cell mucositis and three—non-specific diffuse oral mucosal inflammation with ulceration). For each patient, the clinical record and H&E-stained sections were reviewed. Immunohistochemistry for IgG and IgG4 antibodies was performed on sections from the corresponding paraffin block, permitting calculation of the mean number of IgG4+ plasma cells per high-power field (HPF) and the IgG4+/IgG+ plasma cell ratio.ResultsIn all the cases, only one histological hallmark of IgG4-RD—a dense lymphoplasmacytic infiltrate—was seen. Review of the medical histories did not reveal any features representing other manifestations of IgG4-RD. The number of IgG4+ plasma cells exceeded 100 per HPF in half of the cases. Only two cases had an IgG4+/IgG+ plasma cell ratio of >40%; both of which were in the non-specific oral inflammatory group.ConclusionsOur study suggests that plasma cell mucositis does not meet microscopic criteria for IgG4-RD. It importantly reinforces the opinion that IgG4+ plasma cells are major components of chronic inflammation in the oral cavity and the pertinence of correct contextual interpretation of histopathological features with clinical findings.

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.

IgG4-Related Appendiceal Disease: A First Case Report Fulfilling All Pathological Diagnostic Criteria and With Concomitant S100-Positive Dendritic/Schwann Cell Hyperplasia

2019 ◽  
Vol 27 (5) ◽  
pp. 541-546
Author(s):  
Dong Hyang Kwon ◽  
Bhaskar Kallakury ◽  
Pedro DeBrito ◽  
Norio Azumi
Keyword(s):  
Diagnostic Criteria ◽  
Plasma Cell ◽  
High Power Field ◽  
Surgical Morbidity ◽  
Cell Hyperplasia ◽  
Cell Ratio ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
First Case ◽  
Organ Systems

IgG4-related disease is a recent entity that has been described in a wide variety of organ systems. A 46-year-old female presented with acute appendicitis accompanied by a mass-forming lesion, raising a concern for neoplasm, and therefore, hemicolectomy was performed. The lesion revealed a dense lymphoplasmacytic infiltrate accompanied by storiform fibrosis and obliterative phlebitis. The IgG4/IgG plasma cell ratio was >50%, and the number of IgG4-positive plasma cells was >100/high-power field. In order to assess the IgG4/IgG plasma cell ratio, MUM1 was employed instead of IgG to successfully estimate the plasma cell concentration. There was also a concomitant hyperplasia of S100-positive cell, which could represent dendritic or Schwannian origin and possibly play a pathophysiologic role. The hyperplasia was significant by itself that it may mimic a mass-forming lesion. This newly described entity of the past decade deserves increased recognition due to clinical implication and surgical morbidity. This is the first case of IgG4-related disease in the appendix to our knowledge that fully satisfied all the pathological diagnostic criteria. We would like to also highlight our innovative approach of evaluating the IgG4/IgG plasma cell.

scholarly journals Numerous IgG4-positive plasma cells are ubiquitous in diverse localised non-specific chronic inflammatory conditions and need to be distinguished from IgG4-related systemic disorders

2011 ◽  
Vol 64 (3) ◽  
pp. 237-243 ◽  
Author(s):  
Johanna D Strehl ◽  
Arndt Hartmann ◽  
Abbas Agaimy
Keyword(s):  
Inflammatory Response ◽  
Oral Cavity ◽  
Plasma Cell ◽  
Plasma Cells ◽  
Systemic Disease ◽  
High Power Field ◽  
Histopathological Features ◽  
Inflammatory Conditions ◽  
Cell Counts ◽  
Gastrointestinal Lesions

BackgroundIgG4-related systemic fibrosclerosis is a recently defined disorder characterised by a diffuse or tumefactive inflammatory reaction rich in IgG4-positive plasma cells associated with sclerosis and obliterative phlebitis. Although characteristic histopathological features are essential for the diagnosis of these disorders, to date there exists no consensus regarding the cut-off values used to define a ‘significant IgG4-positive plasma cell count,’ and data regarding the distribution of IgG4-positive plasma cells under common (non-specific) inflammatory conditions are lacking.MethodsThe authors analysed 121 randomly selected histopathological specimens containing prominent lymphoplasmacytic infiltrates (11 obstructive sialadenitis, 27 inflammatory lesions of the oral cavity, 24 inflammatory gastrointestinal lesions, 15 rheumatoid synovitis, 15 non-specific synovitis, eight non-specific dermatitis and 21 primary carcinomas with a peritumoral inflammatory response). For comparison, seven cases of sclerosing sialadenitis (Küttner tumour) were examined.ResultsHigh counts of IgG4 plasma cells were found in sclerosing sialadenitis (mean 40/high-power field (hpf)), contrasting sharply with sialadenitis caused by sialolithiasis (mean 3/hpf). Greatly varied but generally high counts of IgG4-positive plasma cells were also seen in several of the other lesions, particularly in rheumatoid synovitis (mean 55/hpf), oral cavity lesions (mean 79/hpf) and carcinoma-associated inflammatory response (mean 24/hpf). The mean IgG4/IgG ratios for all lesions varied between 0 and 0.4.ConclusionsThe results demonstrate the ubiquitous occurrence of variably high numbers of IgG4-positive plasma cells under diverse non-specific inflammatory conditions, indicating that high IgG4-positive plasma cell counts and high IgG4/IgG ratios per se do not reliably distinguish IgG4-associated systemic disease from non-specific conditions, and that the IgG4 counts must be cautiously interpreted in the context of appropriate clinical and histopathological features.

scholarly journals IgG4-Related Disease With Testicular Involvement: A Case Report and Review of Literature

2021 ◽  
Vol 12 ◽  
Author(s):  
Gang Wang ◽  
Ning Zhuo ◽  
Xiaowen Luo ◽  
Feng Tian ◽  
Zhenhua Wen ◽  
...  
Keyword(s):  
Plasma Cells ◽  
Young Male ◽  
Reproductive Organ ◽  
High Power Field ◽  
Serological Tests ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Multiple Organs ◽  
Igg4 Related Disease ◽  
The Right

Immunoglobulin G4-related disease (IgG4-RD) is an autoimmune inflammatory disease characterized by infiltration of IgG4+ plasma cells that can simulate a tumor manifesting as a tumor-like mass. This disease involves the pancreas, biliary tract, kidneys, salivary glands, lymph nodes, aorta, and retroperitoneum amongst other organs. However, testicular involvement is a rare entity in this disease. The treatment of testicular involvement in IgG4-RD is currently controversial. We present the case of a 65-year-old man with swelling and pain in his right scrotum three months ago. On examination, a mobile mass of approximately 2 cm in diameter was found in the right scrotum. Serological tests showed elevated levels of IgG4 and negative for tumor markers. Enhanced computed tomography of the scrotum showed a nodular hyperdense shadow with a diameter of approximately 23 mm on the right epididymis. Pathological biopsy of the right epididymis showed infiltration of plasma cells, lymphocytes, and a few neutrophils. IgG4+ plasma cells stained positive, with an IgG4/IgG ratio of more than 40% and more than 30 IgG4+ plasma cells per high-power field. A diagnosis of IgG4-RD involving the testicles was made. Prednisone 30 mg/d was given for three weeks. No scrotum swelling or pain was observed at the follow-up after six months. IgG4-related disease should be considered whenever a mass-like lesion with typical histomorphologic features involving multiple organs/anatomical sites is encountered. The testicles are an important male reproductive organ, especially for young male patients with fertility requirements. For patients with IgG4-RD testicular involvement, surgical or medical treatment requires further study.

scholarly journals Pleuritis associated with immunoglobulin G4-related disease under normal thoracoscopic findings: a case report

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Hiroki Shimada ◽  
Yuto Kato ◽  
Miyuki Okuda ◽  
Koji Fukuda ◽  
Nobuya Tanaka ◽  
...  
Keyword(s):  
Pleural Effusion ◽  
Plasma Cells ◽  
Diagnostic Yield ◽  
Class Iii ◽  
High Power Field ◽  
Pleural Biopsy ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Left Pleural Effusion

Abstract Background Immunoglobulin G4 (IgG4)-related disease is a chronic inflammatory disease that was recognized in 2011. Pleuritis associated with IgG4-related disease is rare and can be difficult to diagnose. Although there have been previous reports on pleuritis associated with IgG4-related disease by thoracoscopic findings, this is the first to observe pleuritis with IgG4-related disease from normal pleural thoracoscopic findings. Case presentation A 70-year-old Japanese female treated for breast cancer 33 years ago was referred to our hospital complaining of dyspnea on exertion. Chest computed tomography (CT) revealed left pleural effusion that was exudative and predominant with lymphocytes, elevated adenosine deaminase (ADA) and Class III cytology (malignancy suspected). Subsequently, thoracoscopic pleural biopsy was performed for definitive diagnosis. Although pleural macroscopic findings appeared normal, we performed pleural biopsy at random sites. This patient was negative for mycobacterium tuberculosis, and neither granulomas nor malignant cells were found in the collected specimens. An infiltration of inflammatory cells, mainly plasma cells and lymphocytes, was observed. Immunostaining revealed the number of IgG4-positive plasma cells was 102/high power field (HPF), and the percentage of IgG4 positive/immunoglobulin G (IgG)-positive cells was 41.4%. Since IgG4 serum levels were high and IgG4-related submandibular sialadenitis was also observed, a definitive diagnose of pleuritis associated with IgG4-related disease was confirmed. Conclusions We diagnosed pleuritis associated with IgG4-related disease by thoracoscopic pleural biopsy samples taken from a visually normal pleura. Although exudative pleural effusion with high ADA and lymphocyte predominance is a characteristic of tuberculous pleuritis, other diseases might be present. Since thoracoscopy can increase the diagnostic yield, pleural biopsy should be considered even if thoracoscopic pleural findings are deemed normal.

scholarly journals Plasma Cell Granuloma of the Thyroid: Review of an Uncommon Entity

2018 ◽  
Vol 142 (8) ◽  
pp. 998-1005 ◽  
Author(s):  
Pallavi A. Patil ◽  
Ronald A. DeLellis
Keyword(s):  
Plasma Cell ◽  
Inflammatory Myofibroblastic Tumor ◽  
Plasma Cells ◽  
Specific Treatment ◽  
Surgical Excision ◽  
Hashimoto Thyroiditis ◽  
Plasma Cell Granuloma ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Inflammatory Myofibroblastic Tumors

Plasma cell granuloma (PCG) is characterized by proliferation of polyclonal plasma cells with associated fibrosis and is often considered part of the heterogeneous group of inflammatory myofibroblastic tumors (IMTs). The thyroid is rarely affected by PCG. A patient having PCG associated with Hashimoto thyroiditis (HT) prompted our literature search that revealed 18 cases of PCG, 55% (n = 10) of which occurred together with HT. The etiopathogenesis of PCG is unknown and there is no specific treatment except surgical excision for compressive symptoms. This entity has an excellent prognosis with no evidence of recurrence or metastasis. Lesions of the thyroid with infiltrating plasma cells include HT, fibrous variant of HT, plasmacytoma, plasma cell myeloma, Riedel thyroiditis, IgG4 (immunoglobulin G4)-related disease, IMT, and PCG. Inflammatory myofibroblastic tumor has ALK gene rearrangements and is considered a neoplasm as opposed to PCG, which is a reactive polyclonal plasma cell proliferation. We believe IMT and PCG are distinct entities and consensus definitions are required for avoiding confusion in the literature.

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.

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