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IgG4 related arteritis

Xiaoting Lv, Feng Gao, Qicai Liu, Sheng Zhang, Zhihua Huang, Yongping Zhu, Haiyang Zong, Quwen Li, Sanyan Li
IgG4-related interstitial lung disease (IgG4-RILD), which is characterized by increased IgG4 levels, IgG4+ plasma cell infiltration and irregular whorled fibrosis, is a recently described lung disorder that belongs to the group of systemic fibroinflammatory IgG4-related diseases (IgG4-RD). The aim of the present study was to improve the current knowledge regarding the specific clinical and histopathological characteristics of IgG4-RILD and to investigate its underlying immune mechanism in vivo . A total of 7 patients newly diagnosed with IgG4-RILD were enrolled in the present study (4 men and 3 women; mean age, 57 years; range, 29-71 years)...
February 2018: Experimental and Therapeutic Medicine
Sosei Kuma, Tsubasa Takeshima, Takefumi Ohga, Tadahiro Nozoe, Katsuo Sueishi
A 68-year-old man was admitted because of a pulsatile mass and pain in the left temporal region, and computed tomography demonstrated the superficial temporal artery aneurysm. He underwent aneurysmectomy, and pathologic investigation revealed marked thickness of the adventitia with substantial plasmacyte infiltration. On immunoglobulin G4 (IgG4) immunohistochemistry, IgG4-positive lymphocytes were scattered in the adventitia, and biochemical tests revealed elevation of IgG4 (200 mg/dL). The case satisfied the criteria for both giant cell arteritis and IgG4-related disease (IgG4-RD)...
December 2017: Journal of Vascular Surgery Cases and Innovative Techniques
Ryo Rokutanda, Yosuke Nishihata, Masato Okada
No abstract text is available yet for this article.
October 2017: Journal of Rheumatology
Mikael Barbu, Ulf Lindström, Claes Nordborg, Andreas Martinsson, Christian Dworeck, Anders Jeppsson
We present the case of a 55-year-old woman admitted for a coronary artery bypass operation because of three-vessel coronary artery disease based on angiographic findings and clinical symptoms. Unexpected intraoperative findings with diffuse tissue thickening of the ascending aorta and coronary arteries indicated an alternate pathogenesis rather than coronary artery atherosclerosis. Histopathologic findings and clinical evaluation could confirm IgG4-related disease (IgG4-RD). IgG4-RD is a newly recognized fibroinflammatory condition that can present in a variety of organs and is characterized by common histopathologic features...
June 2017: Annals of Thoracic Surgery
Christian Löffler, Johannes Hoffend, Urs Benck, Bernhard K Krämer, Raoul Bergner
Large-vessel vasculitis (LVV) is a group of diseases mainly comprised of giant-cell arteritis (GCA), Takayasu arteritis, and a series of rare diseases like Behçet's disease, IgG4-related disease, infectious aortitis, and other unfrequent entities. Besides clinical and laboratory features, Doppler sonography (DS) can assist in establishing the diagnosis. Its diagnostic sensitivity has been evaluated in various studies, most of them, however, in temporal arteritis (TA) respectively in LVV with involvement of the temporal artery...
September 2017: Clinical Rheumatology
Ilkay Cinar, He Wang, James R Stone
Non-infectious aortitis may be caused by several distinct systemic rheumatologic diseases. In some patients, aortitis is identified either pathologically or radiologically in the absence of clinical evidence of a systemic vasculitis. By consensus nomenclature, such cases are referred to as clinically isolated aortitis (CIA). Some systemic disorders may initially present as CIA including giant cell arteritis (GCA), IgG4-related disease, infectious aortitis, and granulomatosis with polyangiitis. CIA most commonly occurs in women of European descent over the age of 50 and, thus, mirrors the gender, age, and geographic distribution of GCA...
July 2017: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
Sophie Mavrogeni, George Markousis-Mavrogenis, Genovefa Kolovou
Immunoglobulin 4-related disease (IgG4-related disease) is a systemic inflammatory disease that presents with increases of serum IgG4. It may affect various systems, including the cardiovascular (CV) system. Assessment of serum IgG4 levels and involved organ biopsy are necessary for diagnosis. IgG4-related disease is characterized by fibrosclerosis, lymphocytic infiltration and presence of IgG4-positive plasma cells. The disease usually responds to treatment with corticosteroids and/or immunosuppressive medication...
January 2017: European Journal of Radiology
Mara Burkel, Fabian Arndt, Jan-Henrik Schirmer, Frank Moosig, Julia U Holle
OBJECTIVES: To date, no specific serum marker for giant cell arteritis and polymyalgia rheumatica has been established in routine practice. Therefore, the aim of this study was to examine whether immunoglobulin G4 serum concentrations could be a potential biomarker for the differentiation of both diseases. METHODS: Serum immunoglobulin G4 (IgG4) concentrations were measured in patients with giant cell arteritis (n=41) and polymyalgia rheumatica (n=27) by an in-house enzyme-linked immunosorbent assay...
March 2017: Clinical and Experimental Rheumatology
Cuyan Demirkesen
PURPOSE OF REVIEW: The present review will focus on recent publications in cutaneous vasculitides. RECENT FINDINGS: Some histopathological and clinical features, such as papillary dermal edema, perivascular C3 deposition, clinically evident edema, and lesions above the waist, may point out renal or gastrointestinal involvement in Henoch-Schönlein purpura (HSP). HSP associated with familial Mediterranean fever differs from typical isolated HSP by showing no deposits of IgA, much younger age, and location of the lesions on the face or the trunk...
January 2017: Current Opinion in Rheumatology
Giovanni M Rossi, Giacomo Emmi, Domenico Corradi, Maria L Urban, Federica Maritati, Federica Landini, Paola Galli, Alessandra Palmisano, Augusto Vaglio
Mediastinal fibrosis is a rare disease characterised by fibrous proliferation in the mediastinum. It can be idiopathic or secondary to several conditions such as infections and malignancies. Anecdotal reports have described idiopathic mediastinal fibrosis (IMF) in association with other fibro-inflammatory or autoimmune diseases. We report nine new IMF cases recently seen at our Fibro-Inflammatory Disease Clinic and reviewed the IMF cases reported in the English language literature throughout 2006-2016. The purposes of our literature search were to assess the frequency of the association between IMF and other immune-mediated disorders and to analyse which disorders most often coexist with IMF...
June 2017: Clinical Reviews in Allergy & Immunology
Guillermo Delgado-García, Sergio Sánchez-Salazar, Erick Rendón-Ramírez, Mario Castro-Medina, Bárbara Sáenz-Ibarra, Álvaro Barboza-Quintana, María Azalea Loredo-Alanis, David Hernández-Barajas, Dionicio Galarza-Delgado
Coronary involvement in IgG4-related disease (IgG4-RD) has been scarcely reported, and myocardial ischemia as its presenting feature is even rarer. Here, we describe an additional case with novel and relevant observations. The patient was a previously healthy, middle-aged woman who presented to the clinic with new-onset typical angina. One tumefactive lesion encasing the left anterior descending artery was found during her workup. The most common underlying malignancies with secondary cardiac involvement were rationally ruled out...
November 2016: Clinical Rheumatology
Archana Srinivasan, Teri T Kleinberg, Ann P Murchison, Jurij R Bilyk
PURPOSE: To review the important laboratory serological investigations routinely performed for the diagnosis of autoimmune and inflammatory diseases of the orbit and ocular adnexa. METHODS: Review of the literature as well as personal clinical experience of the authors. Due to the extensive nature of the topic, the review has been split into 2 parts. Part I covers thyroid eye disease, IgG4-related disease, and myasthenia gravis. In part II of the review, sarcoidosis, vasculitis, Sjögren syndrome, and giant cell arteritis will be discussed...
January 2017: Ophthalmic Plastic and Reconstructive Surgery
Archana Srinivasan, Teri T Kleinberg, Ann P Murchison, Jurij R Bilyk
PURPOSE: To review the important laboratory serological investigations routinely performed for the diagnosis of autoimmune and inflammatory diseases of the orbit and ocular adnexa. METHODS: Review of the literature as well as personal clinical experience of the authors. RESULTS: Periocular inflammation is a blanket term which refers to a wide variety of conditions affecting the orbit and the ocular adnexa. Although the etiology of each of these conditions is different, their similarity lies in their clinical presentation and in the fact that each of them can be vision-threatening and even life-threatening if left untreated...
September 2016: Ophthalmic Plastic and Reconstructive Surgery
Ludwig Caspary
Inflammatory aortic diseases may occur with and without dilatation and are complicated by obstruction, rupture and dissection. Infections originate from periaortic foci or septicaemia and tend to result in the rapid development of aneurysms. Large vessel vasculitis due to Takayasu arteritis in younger and giant cell arteritis (GCA) in older patients is located in all layers of the aortic wall and prevails in the thoracic section. GCA patients are prone to developing aortic complications in the late course of disease...
January 2016: VASA. Zeitschrift Für Gefässkrankheiten
Aisheng Dong, Yang Wang, Changjing Zuo
IgG4-related peripulmonary arteritis is rare. A 60-year-old woman presented with fatigue for more than 2 years. Transthoracic echocardiography revealed a lesion measuring 2.1 × 1.5 cm in the pulmonary artery bifurcation. CT pulmonary angiography showed marked stenosis of the right and left pulmonary arteries. FDG PET/CT showed increased FDG uptake of the thickened walls in the bilateral pulmonary arteries with SUVmax of 5.8. The patient underwent partial resection of the pulmonary artery lesion. IgG4-related peripulmonary arteritis was confirmed by pathology...
October 2016: Clinical Nuclear Medicine
C Rousselin, F Pontana, P Puech, M Lambert
Aortitis are mainly described in inflammatory disorders such as Takayasu arteritis, giant cell arteritis or Behçet's disease. Aortitis is sometimes qualified as idiopathic. However, differential diagnoses must be searched since they need specific interventions. Infectious aortitis should be ruled out first as its rapid evolution and short-term poor prognosis makes it a therapeutic emergency. Furthermore, rarer differential diagnoses should be known as they require specific care that might sometimes differ from the treatment of inflammatory aortitis, such as retroperitoneal fibrosis mostly idiopathic but also secondary to neoplasia or malignant hemopathies...
April 2016: La Revue de Médecine Interne
Abhishek R Keraliya, David J Murphy, Ayaz Aghayev, Michael L Steigner
No abstract text is available yet for this article.
March 2016: Circulation. Cardiovascular Imaging
Jian Yang, Zhixing Fan, Jun Yang, Chaojun Yang
No abstract text is available yet for this article.
November 1, 2016: International Journal of Cardiology
Masaru Kojima, Yoshimasa Nakazato, Kaoru Hirabayashi, Nobuhide Masawa, Naoya Nakamura
We report 2 rare cases of IgG4-related plasma cell granuloma of the maxillary sinus. Histologically, both lesions were characterized by severe lymphoplasmacytic infiltration. In 1 case, fibrous sclerosis and obliterative arteritis were observed. Immunohistochemical study demonstrated that IgG4-positive cells comprised more than 40% of the IgG-positive plasma cells. Moreover, the serum IgG4 level was elevated in both cases. A good responsiveness to steroid therapy has been seen in IgG4-related disease. From a therapeutic perspective, it is important to recognize IgG4-related plasma cell granuloma...
September 2015: Ear, Nose, & Throat Journal
Dinesh Pradhan, Niharika Pattnaik, Russell Silowash, Sambit Kumar Mohanty
IgG4-related disease (IgG4-RD) is a recently recognized systemic autoimmune disorder characterized by high levels of serum IgG4 and dense infiltration of IgG4-positive plasma cells in multiple organs. The condition was first described as a disease of the pancreas, and has since been recognized in various organ systems including the kidneys. IgG4 related kidney disease (IgG4-RKD) signifies any form of renal involvement by IgG4-RD. The most common renal involvement by IgG4-RD is tubulointerstitial nephritis. Glomerular disease, in particular membranous glomerulonephritis, may also be seen...
October 2015: Pathology, Research and Practice
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