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 IgG4-related disease in the head and neck region: report of two cases and review of the literature

2016 ◽  
Vol 4 ◽  
pp. 370-375 ◽  
Author(s):  
Michał Gontarz ◽  
Grażyna Wyszyńska-Pawelec ◽  
Jan Zapała ◽  
Krystyna Gałązka ◽  
Romana Tomaszewska ◽  
...  
Keyword(s):  
Head And Neck ◽  
Neck Region ◽  
Review Of The Literature ◽  
Head And Neck Region ◽  
Related Disease ◽  
Igg4 Related Disease

IgG4-related Disease in the Head and Neck Region

2013 ◽  
Vol 106 (8) ◽  
pp. 671-682
Author(s):  
Tetsuo Himi ◽  
Kenichi Takano ◽  
Nobuhiko Seki ◽  
Ayumi Moriyama ◽  
Motohisa Yamamoto ◽  
...  
Keyword(s):  
Head And Neck ◽  
Neck Region ◽  
Head And Neck Region ◽  
Related Disease ◽  
Igg4 Related Disease

scholarly journals IgG4-related skin diseases: A brief review

2020 ◽  
Vol 2 ◽  
pp. 94-98
Author(s):  
T. P. Afra ◽  
Muhammed Razmi T ◽  
N. A. Bishurul Hafi
Keyword(s):  
Plasma Cells ◽  
Skin Diseases ◽  
Early Recognition ◽  
Neck Region ◽  
Obliterative Phlebitis ◽  
Immunoglobulin G4 ◽  
Head And Neck Region ◽  
Systemic Involvement ◽  
Related Disease ◽  
Storiform Pattern

Immunoglobulin G4-related disease (IgG4-RD) is an increasingly recognized syndrome which shares similar pathologic, serologic, and clinical features in the affected organs. Subacute development of a mass or diffuse enlargement of the organ is a common presenting feature. It is more commonly seen in middle-aged or older men. Lymphadenopathy is common in them. Tissue infiltration with lymphoplasmacytic collection, predominantly of IgG4-positive plasma cells, accompanied by “storiform” pattern of fibrosis, obliterative phlebitis, and increased tissue eosinophils are the hallmark histologic findings. Rapid response to systemic steroids is characteristic. If present, the typical cutaneous findings such as papulonodules or plaques in the head-and-neck region may serve as an initial clue to the underlying systemic involvement in IgG4-RD. Hence, dermatologists need to be aware of this entity for early recognition of underlying organ involvement and thus the prompt management.

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 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 Extramedullary Plasmacytoma of Head and Neck Region: Report of Six Cases with Different Localizations

2013 ◽  
Vol 5 (3) ◽  
pp. 157-163
Author(s):  
Kerem Öztürk ◽  
Mustafa Şahin ◽  
Raşit Midilli ◽  
Gülce Gürsan ◽  
Nazan Özsan ◽  
...  
Keyword(s):  
Head And Neck ◽  
Soft Tissues ◽  
Plasma Cells ◽  
Neck Region ◽  
Extramedullary Plasmacytoma ◽  
Mastoid Cavity ◽  
Upper Respiratory Tract ◽  
Head And Neck Region ◽  
Systemic Dissemination ◽  
Upper Respiratory

ABSTRACT Extramedullary plasmacytoma (EMP) is a rarely seen neoplasm of plasma cells which may be locally destructive by involving submucosal soft tissues of upper respiratory tract but without systemic dissemination. It comprises less than 1% of head and neck malignities. Clinical presentation depends on tumor's effect and its involvement site. Diagnosis is made with histopathologic examination of tissue biopsy. Responses to its treatment with local radiotherapy and surgery are similar. In this article; it is intended to update our knowledge regarding EMP and remind this rarely encountered disease by presenting various cases detected in nasopharynx, tonsil, buccal mucosa, larynx, mastoid cavity and sphenoid sinus, with literature line. How to cite this article Öztürk K, Şahin M, Midilli R, Gürsan G, Özsan N, Savaş R. Extramedullary Plasmacytoma of Head and Neck Region: Report of Six Cases with Different Localizations. Int J Otorhinolaryngol Clin 2013;5(3):157-163.

scholarly journals Increased IgG4-Positive Plasma Cells in Granulomatosis with Polyangiitis: A Diagnostic Pitfall of IgG4-Related Disease

2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
Sing Yun Chang ◽  
Karina Keogh ◽  
Jean E. Lewis ◽  
Jay H. Ryu ◽  
Eunhee S. Yi
Keyword(s):  
Differential Diagnosis ◽  
Oral Cavity ◽  
Head And Neck ◽  
Granulomatosis With Polyangiitis ◽  
Plasma Cells ◽  
Iliac Fossa ◽  
Related Disease ◽  
Diagnostic Pitfall ◽  
Igg4 Related Disease ◽  
Pathologic Assessment

Granulomatosis with polyangiitis (Wegener’s) (GPA) may mimic IgG4-related disease (IgG4-RD) on histologic examination of some biopsies, especially those from head and neck sites. IgG4 immunostain is often performed in this context for differential diagnosis with IgG4-RD. However, the prevalence of IgG4+ cells in GPA has not been explored. We examined the IgG4+ cells in 26 cases confirmed as GPA by a thorough clinical and pathologic assessment. Twenty-six biopsies consisted of 14 sinonasal/oral cavity/nasopharynx, 7 orbit/periorbital, 3 lung/pleura, 1 iliac fossa/kidney, and 1 dura specimens. Eight of 26 (31%) biopsies revealed increased IgG4+ cells (>30/HPF and >40% in IgG4+/IgG+ ratio). The IgG4+ cells and IgG4+/IgG+ ratio ranged 37–137/hpf and 44–83%, respectively. Eight biopsies with increased IgG4+ cells were from sinonasal(n=4)or orbital/periorbital(n=4)sites. In conclusion, increased IgG4+ cells are not uncommonly seen in sinonasal or orbital/periorbital biopsies of GPA, which could pose as a diagnostic pitfall.

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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