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igG4 related vasculitis

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https://www.readbyqxmd.com/read/29139157/seronegative-granulomatosis-with-polyangiitis-presenting-with-multiple-cranial-nerve-palsies
#1
Eunhye Lee, Jynkyun Park, Seung Hong Choi, Sung-Hye Park
Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), is a rare systemic disease characterized by necrotizing granulomas and vasculitis that usually presents in the respiratory tract and renal system. Here, we report a case of anti-neutrophil cytoplasmic antibody (ANCA)-negative GPA with diagnostic difficulties. Neurologic involvement in GPA is not uncommon, with up to 50% of patients manifesting with neurologic defects; however, it is usually a late event. This patient was a 50-year-old man whose first manifestation was multiple neurologic defects without respiratory or renal symptoms...
November 14, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29125145/the-2017-doyne-lecture-the-orbit-as-a-window-to-systemic-disease
#2
REVIEW
A A McNab
A very large number of disorders affect the orbit, and many of these occur in the setting of systemic disease. This lecture covers selected aspects of orbital diseases with systemic associations in which the author has a particular clinical or research interest. Spontaneous orbital haemorrhage often occurs in the presence of bleeding diatheses. Thrombosis of orbital veins and ischaemic necrosis of orbital and ocular adnexal tissues occur with thrombophilic disorders, vasculitis, and certain bacterial and fungal infections...
November 10, 2017: Eye
https://www.readbyqxmd.com/read/28987046/-igg4-related-disease-a-case-report
#3
Justyna Milczarek-Banach, Kinga Brodzińska, Anna Jankowska, Urszula Ambroziak, Benedykt Szczepankiewicz, Jolanta Nałęcz-Janik, Piotr Miśkiewicz
Immunoglobulin G4-related disease (IgG4-RD) is a comparatively new condition that may involve more than one organ. The lack of characteristic, pathognomonic clinical symptoms may delay the diagnosis of this disease. The diagnosis is based upon clinical manifestation, elevated serum levels of IgG4 and histopathologic examination with immunohistochemical staining to reveal infiltration of IgG4-positive plasma cells. The first line treatment is oral glucocorticoids. A CASE REPORT: 38-year-old woman with Hashimoto disease, chronic sinusitis and chronic hepatitis of unknown etiology was admitted to the Department of Endocrinology because of moderate eyelids swelling accompanied by redness for 3 years...
September 29, 2017: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/28928271/the-value-of-18f-fdg-pet-ct-in-identifying-the-cause-of-fever-of-unknown-origin-fuo-and-inflammation-of-unknown-origin-iuo-data-from-a-prospective-study
#4
Verena Schönau, Kristin Vogel, Matthias Englbrecht, Jochen Wacker, Daniela Schmidt, Bernhard Manger, Torsten Kuwert, Georg Schett
BACKGROUND: Fever of unknown origin (FUO) and inflammation of unknown origin (IUO) are diagnostically challenging conditions. Diagnosis of underlying disease may be improved by 18F-fluorodesoxyglucose positron emission tomography (18F-FDG-PET). METHODS: Prospective study to test diagnostic utility of 18F-FDG-PET/CT in a large cohort of patients with FUO or IUO and to define parameters that increase the likelihood of diagnostic 18F-FDG-PET/CT. Patients with FUO or IUO received 18F-FDG-PET/CT scanning in addition to standard diagnostic work-up...
September 19, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28811201/the-unmet-need-in-rheumatology-reports-from-the-targeted-therapies-meeting-2017
#5
Kevin L Winthrop, Vibeke Strand, Désirée van der Heijde, Philip Mease, Mary Crow, Michael Weinblatt, Joan Bathon, Gerd R Burmester, Maxime Dougados, Johnathan Kay, Xavier Mariette, Ronald Van Vollenhoven, Joachim Sieper, Fritz Melchers, Ferdinand C Breedfeld, Joachim Kalden, Josef S Smolen, Daniel E Furst
The 19th annual international Targeted Therapies meeting brought together over 100 leading basic scientists and clinical researchers from around the world in the field of immunology, molecular biology and rheumatology and other specialties. During the meeting, breakout sessions were held consisting of 5 disease-specific groups with 20-40 experts assigned to each group based on clinical or scientific expertise. Specific groups included: rheumatoid arthritis, psoriatic arthritis, axial spondyloarthritis, systemic lupus erythematous, connective tissue diseases (e...
August 12, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28780079/antineutrophil-cytoplasmic-antibody-associated-vasculitides-and-igg4-related-disease-a-new-overlap-syndrome
#6
REVIEW
François-Xavier Danlos, Giovanni Maria Rossi, Daniel Blockmans, Giacomo Emmi, Andreas Kronbichler, Stéphane Durupt, Claire Maynard, Luminita Luca, Cyril Garrouste, Bertrand Lioger, Rachel Mourot-Cottet, Robin Dhote, Jean-Benoit Arlet, Thomas Hanslik, Philippe Rouvier, Mikael Ebbo, Xavier Puéchal, Dominique Nochy, Agnès Carlotti, Luc Mouthon, Loïc Guillevin, Augusto Vaglio, Benjamin Terrier
OBJECTIVE: Atypical manifestations have been described in patients with ANCA-associated vasculitides (AAV), such as pachymeningitis, orbital mass or chronic periaortitis. Because these manifestations have been associated to the spectrum of IgG4-related disease (IgG4-RD), we hypothesized that both diseases could overlap. METHODS: We conducted a European retrospective multicenter observational study including patients fulfilling ACR and Chapel Hill criteria for AAV and IgG4-RD Comprehensive Diagnostic Criteria...
October 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28503707/the-value-of-ultrasound-in-diagnosing-extracranial-large-vessel-vasculitis-compared-to-fdg-pet-ct-a-retrospective-study
#7
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...
May 15, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28500877/clinically-isolated-aortitis-pitfalls-progress-and-possibilities
#8
REVIEW
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
https://www.readbyqxmd.com/read/28374231/neurological-manifestations-of-igg4-related-disease
#9
REVIEW
Bernardo Baptista, Alina Casian, Harsha Gunawardena, David D'Cruz, Claire M Rice
IgG4-related disease (IgG4-RD) is a multisystem inflammatory disorder. Early recognition of IgG4-RD is important to avoid permanent organ dysfunction and disability. Neurological involvement by IgG4-RD is relatively uncommon, but well recognised-hypertrophic pachymeningitis and hypophysitis are the most frequent manifestations. Although the nervous system may be involved in isolation, this more frequently occurs in conjunction with involvement of other systems. Elevated circulating levels of IgG4 are suggestive of the condition, but these are not pathognomonic and exclusion of other inflammatory disorders including vasculitis is required...
April 2017: Current Treatment Options in Neurology
https://www.readbyqxmd.com/read/28297814/-igg4-related-lung-disease-analysis-of-8-cases-and-literature-review
#10
REVIEW
G J Han, H Hu, D Mao, X Bai, D Y She, S F Zhao, Z L Wen, J Gao
Objective: To improve the understanding and treatment of IgG4-related lung disease (IgG4-RLD). Methods: The clinical characteristics, serum IgG4 levels, pathological features, chest CT, therapy and prognosis of 8 patients with IgG4-RLD were retrospectively analyzed. These patients were admitted to the People's Liberation Army General Hospital and the pathological diagnosis was made between December 2005 and March 2016. Relevant literatures were reviewed. Results: The 8 patients with IgG4-RLD included 4 men and 4 women, with an average age of (59±4) years (range, 37-74)...
March 12, 2017: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/28207938/comparison-of-clinical-and-pathological-features-of-lung-lesions-of-systemic-igg4-related-disease-and-idiopathic-multicentric-castleman-s-disease
#11
Yasuhiro Terasaki, Soichiro Ikushima, Shoko Matsui, Akira Hebisawa, Yasunori Ichimura, Shinyu Izumi, Masuo Ujita, Machiko Arita, Keisuke Tomii, Yuko Komase, Isoko Owan, Tetsuji Kawamura, Yasuo Matsuzawa, Miho Murakami, Hiroshi Ishimoto, Hiroshi Kimura, Masashi Bando, Norihiro Nishimoto, Yoshinori Kawabata, Yuh Fukuda, Takashi Ogura
AIMS: The lung lesion [immunoglobulin (Ig)G4-L] of IgG4-related disease (IgG4-RD) is a condition that occurs together with IgG4-RD and often mimics the lung lesion [idiopathic multicentric Castleman's disease (iMCD-L)] of idiopathic multicentric Castleman's disease (iMCD). Because no clinical and pathological studies had previously compared features of these diseases, we undertook this comparison with clinical and histological data. METHODS AND RESULTS: Nine patients had IgG4-L (high levels of serum IgG4 and of IgG4(+) cells in lung specimens; typical extrapulmonary manifestations)...
June 2017: Histopathology
https://www.readbyqxmd.com/read/27896847/a-case-of-leucocytoclastic-vasculitis-as-a-complication-of-igg4-related-skin-disease
#12
S Nakagawa, Y Nakamura, S Yasui, O Yokosuka, H Matsue
No abstract text is available yet for this article.
November 29, 2016: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/27787337/approach-to-cutaneous-vasculitides-with-special-emphasis-on-small-vessel-vasculitis-histopathology-and-direct-immunofluorescence
#13
REVIEW
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
https://www.readbyqxmd.com/read/27553003/idiopathic-mediastinal-fibrosis-a-systemic-immune-mediated-disorder-a-case-series-and-a-review-of-the-literature
#14
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
https://www.readbyqxmd.com/read/27429760/recognizing-igg4-related-tubulointerstitial-nephritis
#15
REVIEW
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
https://www.readbyqxmd.com/read/27428181/large-vessel-involvement-by-igg4-related-disease
#16
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)
https://www.readbyqxmd.com/read/27418350/-unusual-dysphagia-in-igg4-related-disease
#17
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.
August 2017: HNO
https://www.readbyqxmd.com/read/27277432/pulmonary-manifestation-of-immunoglobulin-g4-related-disease-in-a-7-year-old-immunodeficient-boy-with-epstein-barr-virus-infection-a-case-report
#18
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...
June 8, 2016: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/27181547/igg4-related-sialoadenitis-with-a-skin-lesion-and-multiple-mononeuropathies-suggesting-coexistent-cryoglobulinemic-vasculitis
#19
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
https://www.readbyqxmd.com/read/27124923/granuloma-faciale-and-erythema-elevatum-diutinum-in-relation-to-immunoglobulin-g4-related-disease-an-appraisal-of-32-cases
#20
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
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