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IgG4 related arteritis

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...
August 23, 2016: 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...
May 4, 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...
April 25, 2016: 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
Yonglan He, Xuechao Du, Ning Ding, Zhuo Li, Weixun Zhou, Bo Chen, Zhengyu Jin, Hao Sun, Liang Zhu, Huadan Xue
PURPOSE: To discuss the diverse imaging features of pancreatic and extra-pancreatic lesions of IgG4-related disease (IgG4-RD) on multi-detector CT (MDCT). METHODS: This retrospective study was approved by our institutional research ethics board, and informed consent was waived. From 2008 to 2013, 159 patients including 120 men (mean age 57 years; range 27-79 years) and 39 women (mean age 51 years; range 25-83 years) who met the comprehensive diagnostic criteria for IgG4-RD (2011) were enrolled...
October 2015: Abdominal Imaging
Kaname Ohyama, Miyako Baba, Mami Tamai, Motohisa Yamamoto, Kunihiro Ichinose, Naoya Kishikawa, Hiroki Takahashi, Atsushi Kawakami, Naotaka Kuroda
OBJECTIVES: IgG4-related disease (IgG4-RD) is characterized by various serological abnormalities. Some patients with IgG4-RD present with hypergammaglobulinemia, hypocomplementemia, and autoantibodies recognizing rheumatoid factors and nuclear factors. However, whether IgG4-RD is an autoimmune disease remains unclear. METHODS: Here, we used immune complexome analysis to comprehensively identify constituent antigens of circulating immune complexes isolated from 10 patients with IgG4-related dacryoadenitis and/or sialadenitis (IgG4-RDS) which is one condition associated with IgG4-RD...
2016: Modern Rheumatology
Mengtao Li, Xinping Tian, Wen Zhang, Xiaomei Leng, Xiaofeng Zeng
This review introduces the history of development, organizational structure, funding resources, data collection, and quality control of the Chinese Rheumatism Data Center (CRDC) and summarizes the collection of data. In 2009, Peking Union Medical College Hospital (PUMCH), together with several rheumatism centers, established the Chinese Systemic Lupus Erythematosus (SLE) Treatment and Research Group (CSTAR) to collect data on Chinese patients for the study of SLE disease characteristics. In 2011, CSTAR was extended with the formation of the CRDC at PUMCH with direction from the National Health and Family Planning Commission of the PRC...
August 2015: Clinical Rheumatology
Ivana Vodopivec, Ann-Marie Lobo, Sashank Prasad
Neuroimmunologic and systemic rheumatic diseases are frequently accompanied by inflammation of the eye, ocular adnexa, and orbital tissues. An understanding of the diverse forms of ophthalmic pathology in these conditions aids the clinician in making appropriate preventative, diagnostic, therapeutic, and prognostic decisions. In this review, the authors address ocular inflammation in neurorheumatic disease in three sections: first, they highlight current perspectives on immune mechanisms in the development of these disorders; next, they provide a framework for the recognition and evaluation of ophthalmologic inflammatory entities; finally, they discuss in detail several inflammatory conditions that affect the nervous system and the eye, emphasizing the features that should alert neurologists to initiate ophthalmologic evaluation...
September 2014: Seminars in Neurology
Joseph J Maleszewski
Inflammatory diseases of the aorta comprise a spectrum of disease with diverse clinical and histopathologic presentations. Broadly, they may be dichotomized into infectious and noninfectious varieties. Although uncommon, infectious forms, caused by bacteria, fungi, or mycobacteria, may result from hematogenous seeding of the aorta or direct spread from a contiguous infectious source. The noninfectious forms include a number of entities, the most common of which is atherosclerosis, a disease that primarily affects the aortic intima but has important secondary effects on the media and adventitia that may result in aneurysm formation...
February 2015: Journal of Thoracic and Cardiovascular Surgery
Nimesh R Patel, Mary L Anzalone, L Maximilian Buja, M Tarek Elghetany
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...
June 2014: Archives of Pathology & Laboratory Medicine
Lauren Xu, Jonathon Heath, Allen Burke
There are few single-institution clinicopathological series of aortitis. In this study, all ascending aneurysms were prospectively evaluated pathologically with ≥6 aortic sections over a 6-year period.Of 300 ascending aortic resections, there were 21 cases of aortitis (7%), in 11 women and 10 men (mean 67, range 41-88 years). There were 19 patients with aneurysms, and two patients with sclerosing periaortitis, clinically suspected to have intramural haematoma. Of the 19 patients with aneurysms (11 women), two had prior temporal arteritis, one ankylosing spondylitis, one IgA nephropathy, one undifferentiated autoimmune disease, one Lyme disease, and one fibromyalgia...
June 2014: Pathology
Bon San Koo, Young Wha Koh, SeokChan Hong, You Jae Kim, Yong-Gil Kim, Chang-Keun Lee, Bin Yoo
AIMS: To identify the frequency of immunoglobulin G4 (IgG4)-related aortitis in patients who undergo aorta surgery and are diagnosed by pathology as having chronic aortic inflammation and to compare IgG4-related aortitis with other non-infectious aortitises in terms of clinical characteristics. METHODS: The aorta specimen pathological reports of 1418 patients who underwent aortic aneurysm or dissection surgery were reviewed. In total, 41 had chronic aortic inflammation without atherosclerosis, cancer or infection...
May 2014: International Journal of Rheumatic Diseases
Miyu Tajima, Yukio Hiroi, Yutaka Takazawa, Hironori Muraoka, Hiroshi Iwata, Hiroshi Yamashita, Yasunobu Hirata, Ryozo Nagai
Immunoglobulin G4 (IgG4)-related disorders in various organs have recently been described, but multiple systemic aneurysms have not yet been reported. Here, we present a 68-year-old Japanese man with multiple systemic aneurysms and tumor-forming pericoronary arteritis who was undergoing low-dose corticosteroid therapy. Elevated serum IgG4 (2390 mg/dL) and IgG4-positive plasmacyte infiltration in the salivary glands led to a diagnosis of IgG4-related disease. High-dose corticosteroid therapy was initiated, whereupon the inflammatory lesions shrank...
January 2014: Human Pathology
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