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igG4 related vasculitis

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
Shawna Mann, Michael A Seidman, Sean J Barbour, Adeera Levin, Mollie Carruthers, Luke Y C Chen
PURPOSE OF THE REVIEW: Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder affecting nearly all organs, including the kidney. Tubulointerstitial nephritis (IgG4-TIN) is the most common form of IgG4-related kidney disease (IgG4-RKD) and is the focus of this concise review. OBJECTIVE: The study aims to describe when IgG4-TIN should be suspected and to summarize the diagnosis, treatment, and natural history of the disease. SOURCES OF INFORMATION: Ovid MEDLINE, Google Scholar, and PubMed were searched for full-text English language articles up to January 2016...
2016: Canadian Journal of Kidney Health and Disease
Cory A Perugino, Zachary S Wallace, Nandini Meyersohn, George Oliveira, James R Stone, John H Stone
OBJECTIVES: IgG4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that can affect multiple organs and lead to tumefactive, tissue-destructive lesions. Reports have described inflammatory aortitis and periaortitis, the latter in the setting of retroperitoneal fibrosis (RPF), but have not distinguished adequately between these 2 manifestations. The frequency, radiologic features, and response of vascular complications to B cell depletion remain poorly defined...
July 2016: Medicine (Baltimore)
M Engelbarts, A Schad, C Matthias
This article presents the case of an IgG4-related disease in a patient with clinical signs of a malignant tumor of the oral cavity. After excluding the suspicion of a malignant lesion, vasculitis and various infectious diseases were ruled out. Finally, due to further immunohistochemical studies, IgG4-related disease was diagnosed.
July 14, 2016: HNO
Aleksandra Szczawinska-Poplonyk, Irena Wojsyk-Banaszak, Katarzyna Jonczyk-Potoczna, Anna Breborowicz
BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is a multiorgan fibroinflammatory condition with lymphoplasmacytic infiltrates containing abundant IgG4-positive plasma cells. The immunopathogenesis of the disease and the potential role of triggering autoantigens or infectious factors have not been clearly defined. Immunoglobulin G4-related lung disease is a new and emerging condition in pediatric patients and to date, there have been only two reports regarding pulmonary manifestation of IgG4-RD in children recently published...
2016: Italian Journal of Pediatrics
Mari Kamiya, Peter Y Shane, Makoto Soejima, Shuji Tohda, Nobuyuki Miyasaka, Hitoshi Kohsaka
A 68-year-old man was admitted because of weakness of the left leg, dysesthesiae of the extremities and bilateral lower extremity purpura. A neurological examination showed mononeuritis multiplex with laboratory evidence of hypocomplementemia, cryoglobulinemia and leukocytoclastic vasculitis in the biopsy of a skin specimen. The patient also exhibited bilateral submandibular gland swelling, elevated serum IgG4 levels and infiltration of a large number of IgG4-positive plasma cells in the submandibular glands...
2016: Internal Medicine
Sima Kavand, Julia S Lehman, Lawrence E Gibson
OBJECTIVES: To elucidate whether granuloma faciale (GF) and erythema elevatum diutinum (EED), two inflammatory skin dermatoses, meet the consensus histopathologic diagnostic criteria for immunoglobulin G4-related disease (IgG4-RD). METHODS: With institutional review board approval, we assessed the clinical, microscopic, and immunophenotypic features of skin specimens of patients with GF and EED. We compared these findings with previously published consensus diagnostic criteria for IgG4-RD...
March 2016: American Journal of Clinical Pathology
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
Hiroto Minamino, Hidefumi Inaba, Hiroyuki Ariyasu, Hiroto Furuta, Masahiro Nishi, Takashi Yoshimasu, Akinori Nishikawa, Masanori Nakanishi, Shigeki Tsuchihashi, Fumiyoshi Kojima, Shin-Ichi Murata, Gen Inoue, Takashi Akamizu
UNLABELLED: A 73-year-old man with Hashimoto's thyroiditis (HT) suffered from purpura on the lower legs. He was diagnosed with IgG4-related disease (IgG4-RD) with serum IgG4 elevation and dacryo-sialadenitis confirmed histologically. Serum Th2 and Treg cytokines, interleukin 7 (IL7), IL8 and Th2 chemokine levels were elevated, while skewed Th1 balance was seen in fluorescence-activated cell sorting (FACS). Therefore, preferential Th1 balance in HT appeared to be followed by IgG4-RD characterized with Th2 and Treg polarization...
2016: Endocrinology, Diabetes & Metabolism Case Reports
Zheng Zhao, Yanyan Wang, Zhiwei Guan, Jingyu Jin, Feng Huang, Jian Zhu
OBJECTIVES: The purpose of this work is to evaluate the usefulness of 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) imaging in management of IgG4-related disease (IgG4-RD) by retrospective analysis of PET/CT results in IgG4-RD patients. METHODS: Twenty-six patients diagnosed with IgG4-RD according to Japanese diagnostic criteria who underwent 18F-FDG-PET/CT scans in the PLA General Hospital from January 2010 to May 2015 were enrolled in the study...
January 2016: Clinical and Experimental Rheumatology
Keiko Ohno, Yoko Matsuda, Tomio Arai, Takahiko Sugihara, Shoko Iga, Yurika Kimura
BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have different clinical and pathological features. However, differentiation between these 2 disorders is sometimes difficult. OBJECTIVE: To report a case involving a patient with characteristics of both IgG4-RD and AAV. METHODS: Case report with literature review. RESULTS: We report a case of myeloperoxidase-ANCA-positive otitis media and rhinosinusitis with pathological features of IgG4-RD in a 73-year-old man...
June 2016: Annals of Otology, Rhinology, and Laryngology
Josep M Aragonès, Montserrat Arias-Rivero, Joan M García-Barrionuevo, Gianni Lucchetti
INTRODUCTION: Idiopathic hypertrophic pachymeningitis is a fibroinflammatory immune-mediated disease of the dura mater. Its diagnosis requires the preclusion of infectious, tumoral and other inflammatory diseases. In recent years new entities have been reported that can present with hypertrophic pachymeningitis, such as IgG4-associated disease and MPO-ANCA+ pachymeningitis, as a form of vasculitis limited to the central nervous system. CASE REPORT: We describe the case of a 64 years-old male with headaches and cervicalgia, predominantly at night, and clinical signs and symptoms of spinal cord compression...
November 16, 2015: Revista de Neurologia
Ken Ohyama, Haruki Koike, Mie Takahashi, Yuichi Kawagashira, Masahiro Iijima, Hirohisa Watanabe, Gen Sobue
OBJECTIVE: To evaluate the pathologic significance of immunoglobulin G4 (IgG4) in patients with inflammatory peripheral neuropathy. METHODS: We clinicopathologically examined 149 consecutive patients with peripheral neuropathy who had clusters of inflammatory cells with or without vasculitis in sural nerve biopsy specimens and in whom we were able to assess the serum IgG4 levels. RESULTS: Elevation of serum IgG4 levels and infiltration of IgG4-positive plasma cells, which are currently defined as the diagnostic criteria for IgG4-related disease, were found in 35 and 29 patients, respectively...
October 20, 2015: Neurology
Mariko Takao, Toshihisa Hamada, Tatsuya Kaji, Kazuko Ikeda-Mizuno, Chinatsu Takehara-Yasuhara, Kouichi Ichimura, Hiroyuki Yanai, Tadashi Yshino, Keiji Iwatsuki
BACKGROUND: Hypocomplementemic urticarial vasculitis (HUV) has been defined as a syndrome associated with urticarial lesions caused by leukocytoclastic vasculitis. It has been observed in patients with systemic lupus erythematosus and related conditions. Immunoglobulin G4 (IgG4)-related disease is a lymphoproliferative disorder characterized by sclerosing pancreatitis, retroperitoneal fibrosis, sclerotic cholangitis, acute interstitial nephritis, and Mikulicz's disease, and associated with elevated levels of IgG4 and hypocomplementemia...
April 2016: International Journal of Dermatology
Ophélie Bélissant, Mohamed Guernou, Philippe Rouvier, Caroline Compain, Gérald Bonardel
A 17-year-old adolescent girl was admitted with chronic arthralgia, Raynaud phenomenon, pericarditis, and evidences of chronic diffuse inflammation. F-FDG PET/CT scan was performed to search systemic vasculitis and showed diffuse moderate uptake in the kidneys. We suggested the existence of a nephritis, but the ultrasonography result was normal, and no treatment was introduced. Another F-FDG PET/CT scan was performed 7 months later to explore abdominal pain. It showed again diffuse intense uptake in both kidneys...
October 2015: Clinical Nuclear Medicine
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
Andreas Kronbichler, Nadezda Gut, Jochen Zwerina, Hannes Neuwirt, Michael Rudnicki, Gert Mayer
No abstract text is available yet for this article.
October 2015: 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
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