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"Small vessel vasculitis"

Sergio Prieto-González, Georgina Espígol-Frigolé, Ana García-Martínez, Marco A Alba, Itziar Tavera-Bahillo, José Hernández-Rodríguez, Arturo Renú, Rosa Gilabert, Francisco Lomeña, Maria C Cid
Various imaging modalities, including color duplex ultrasonography, CT angiography, magnetic resonance angiography, and PET, are emerging as important aids to the diagnosis, staging, evaluation of disease activity and response to treatment in systemic vasculitis. Although large-vessel vasculitis is the main target of imaging, refinement and increasing accuracy of imaging modalities are also providing useful information in the evaluation of medium-vessel and small-vessel vasculitis.
November 2016: Rheumatic Diseases Clinics of North America
Jacqueline K Andersen, Ingvild Oma, Richard A Prayson, Ingjerd Lien Kvelstad, Sven Martin Almdahl, Morten Wang Fagerland, Ivana Hollan
BACKGROUND: The cause of premature cardiovascular disease (CVD) in inflammatory rheumatic diseases (IRDs) has not been fully elucidated. As inflammation may play a role, we wanted to compare the occurrence and extent of inflammatory cell infiltrates (ICIs), small vessel vasculitis, and the amount of adipose tissue and collagen in cardiac biopsies taken from patients with coronary artery disease with and without IRDs. METHODS: From among the Feiring Heart Biopsy Study subjects, we selected patients undergoing coronary artery bypass grafting from whom paraffin-embedded, formalin-fixed specimens from the right atrium were available...
October 12, 2016: Arthritis Research & Therapy
Mussa Mensa, Zita M Jessop, Nick Wilson-Jones, Iain S Whitaker
Leukocytoclastic vasculitis (LCV) is a rare small-vessel vasculitis characterised by neutrophilic inflammation of post-capillary venules. Incidence varies from 3 to 4.5 per 100 000 people per year. Patients typically present with painful, itchy purpura and erythema, although clinical manifestations can vary making diagnosis a challenge. We report the case of a 75-year-old man with a history of a previously completely excised and grafted squamous cell carcinoma (SCC) on the dorsum of his hand, who presented with an acutely swollen, erythematous and ulcerated lesion adjacent to the graft site...
September 29, 2016: International Wound Journal
Stavros Giaglis, Sinuhe Hahn, Paul Hasler
Neutrophil extracellular trap (NET) formation represents a form of cell death distinct from apoptosis or necrosis, by which invading pathogens are simultaneously entangled and potentially eliminated. Increased NET formation is observed in systemic lupus erythematosus (SLE), rheumatoid arthritis, antineutrophil cytoplasmic antibody-associated small vessel vasculitis, antiphospholipid antibody syndrome (APS), and psoriasis. NETs contribute to the pathogenesis of autoimmunity by exposing cryptic autoepitopes, which may facilitate the generation of autoantibodies, induce the production of interferons, and activate the complement cascade...
2016: Frontiers in Pediatrics
Mayuko Kawabe, Izumi Yamamoto, Yo Komatsuzaki, Takafumi Yamakawa, Haruki Katsumata, Ai Katsuma, Aki Mafune, Yasuyuki Nakada, Akimitsu Kobayashi, Yudo Tanno, Ichiro Ohkido, Nobuo Tsuboi, Keitaro Yokoyama, Shigeru Horita, Masayoshi Okumi, Hideki Ishida, Hiroyasu Yamamoto, Takashi Yokoo, Kazunari Tanabe
BACKGROUND: IgA vasculitis, a rare condition resulting in end-stage renal disease, is a small-vessel vasculitis that affects the kidney in 49-83 % of adults. The reported recurrence rate of IgA vasculitis in renal transplant recipients is 11.5-60 %, leading to graft loss in 0-50 % of these patients. However, limited data are available on recurrence and graft loss after renal transplantation. METHODS: We evaluated renal transplant recipients seen from 1987 to 2015 at the Jikei University School of Medicine and the Department of Urology, Tokyo Women's Medical University...
September 27, 2016: Clinical and Experimental Nephrology
E F Johnson, D A Wetter, J S Lehman, J L Hand, D M R Davis, M M Tollefson
BACKGROUND: Leukocytoclastic vasculitis (LCV) in children is a complex group of conditions. OBJECTIVES: This study presents the demographics, clinical features, direct immunofluorescence (DIF) results and suspected aetiologies of 56 biopsy-confirmed cases of leukocytoclastic vasculitis in children. METHODS: Retrospective review of 56 children seen at Mayo Clinic in Rochester, Minnesota, from 1993 to 2013 with clinical features and cutaneous biopsy consistent with LCV...
August 31, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
María C De Paoli, Dino Moretti, Carlos M Scolari Pasinato, Martín G Buncuga
The Henoch-Schönlein purpura (HSP) is a small vessel vasculitis with IgA immune complex deposition. The presentation in adults is rare and severe. Reported cases of HSP in patients infected with HIV are scarce. Neutrophil cytoplasmic antibodies (ANCA) are commonly found in other systemic vasculitis, but rarely in HSP and even more unusual the perinuclear pattern. Beside small vessel vasculitis, positivity of ANCA can be detected in a number of different pathological conditions in association with infectious processes, including HIV, or cocaine use, and especially the pattern of ANCA-p, associated with drugs, inflammatory bowel or autoimmune diseases...
2016: Medicina
Ralph Kettritz
Neutrophil serine proteases (NSPs) exercise tissue-degrading and microbial-killing effects. The spectrum of NSP-mediated functions grows continuously, not least because of methodological progress. Sensitive and specific FRET substrates were developed to study the proteolytic activity of each NSP member. Advanced biochemical methods are beginning to characterize common and specific NSP substrates. The resulting novel information indicates that NSPs contribute not only to genuine inflammatory neutrophil functions but also to autoimmunity, metabolic conditions, and cancer...
September 2016: Immunological Reviews
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
Sophia Lionaki, John N Boletis
BACKGROUND: Pauci-immune glomerulonephritis is the most common cause of aggressive glomerulonephritis and occurs as a renal-limited disease or as a component of systemic necrotizing small-vessel vasculitis. It is characterized by paucity of staining for immunoglobulins, by immunofluorescence along with fibrinoid necrosis and crescent formation by light microscopy, while the vast majority of patients have anti-neutrophil cytoplasmic antibodies (ANCA) in their circulation, which also participate in the pathogenesis of the disease...
March 2016: Kidney Diseases
Masataka Umeda, Jin Ikenaga, Tomohiro Koga, Toru Michitsuji, Toshimasa Shimizu, Shoichi Fukui, Ayako Nishino, Yoshikazu Nakasima, Sin-Ya Kawashiri, Naoki Iwamoto, Kunihiro Ichinose, Yasuko Hirai, Mami Tamai, Hideki Nakamura, Tomoki Origuchi, Atsushi Kawakami
A 78-year-old woman diagnosed with cyclic neutropenia 5 years previously had been treated with recombinant granulocyte-colony stimulating factor (G-CSF). She developed fever, tenderness and distension of temporal arteries after the treatment with G-CSF. Magnetic resonance imaging and ultrasonography revealed wall thickening of the temporal arteries. She was therefore diagnosed with giant cell arteritis (GCA). Small vessel vasculitis has been reported as a complication of G-CSF. However, the development of large vessel vasculitis after G-CSF treatment is quite rare...
2016: Internal Medicine
Sanat Phatak, Amita Aggarwal, Vikas Agarwal, Able Lawrence, Ramnath Misra
AIM: Anti-neutrophil cytoplasmic antibodies (ANCA) are associated with small vessel vasculitis now termed 'ANCA associated vasculitis' (AAV). ANCAs are reported in diverse diseases where they have no clinical utility. We carried out an audit in a clinical immunology laboratory and assessed if use of ordering practices could have improved utility of ANCA. METHODS: All samples received for ANCA testing during 2014 were tested by indirect immunofluorescence (IIF) and automated enzyme-linked immunosorbent assay (ELISA)...
July 26, 2016: International Journal of Rheumatic Diseases
Roberta Fenoglio, Carla Naretto, Bruno Basolo, Giacomo Quattrocchio, Michela Ferro, Paola Mesiano, Giulietta Beltrame, Dario Roccatello
Henoch-Schonlein purpura, also called IgA-vasculitis, is a systemic small vessels vasculitis with immunoglobulin A1-dominant immune deposits. The optimal treatment remains controversial. Because IgA-vasculitis is characterized by leukocyte infiltration of the blood vessel walls along with immunoglobulin A deposition, and because glucocorticosteroids inhibit inflammatory processes, early administration of glucocorticosteroids has been postulated to be effective, but this indication remains controversial. Immunosuppressive agents (azathioprine, cyclophosphamide, cyclosporine, mycophenolate) have been used in combination with glucocorticosteroids without definitive evidence of effectiveness...
July 23, 2016: Immunologic Research
Kévin Bouiller, Sylvain Audia, Hervé Devilliers, Evelyne Collet, Marie Hélène Aubriot, Vanessa Leguy-Seguin, Sabine Berthier, Philippe Bonniaud, Pascal Chavanet, Jean-François Besancenot, Pierre Vabres, Laurent Martin, Maxime Samson, Bernard Bonnotte
In this study, outcomes of patients with leukocytoclastic vasculitis (LCV) were analyzed focusing on clinical, histopathology and laboratory findings, relapses, and survival.Data from patients with cutaneous vasculitis diagnosed between January 1, 2000, and December 31, 2010, at Dijon University Hospital (France) were retrospectively reviewed. LCV was defined as perivascular neutrophilic infiltrate, endothelial cell nuclear swelling, extravasation of red blood cells, and/or fibrin deposition in vessels. Patients were classified according to the 2012 Chapel Hill Consensus Conference...
July 2016: Medicine (Baltimore)
Yoshihiro Kusunoki, Daigo Nakazawa, Haruki Shida, Fumihiko Hattanda, Arina Miyoshi, Sakiko Masuda, Saori Nishio, Utano Tomaru, Tatsuya Atsumi, Akihiro Ishizu
Myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA)-associated vasculitis is a systemic small-vessel vasculitis, wherein, MPO-ANCA plays a critical role in the pathogenesis. Neutrophil extracellular traps (NETs) released from activated neutrophils are composed of extracellular web-like DNA and antimicrobial proteins, including MPO. Diverse stimuli, such as phorbol myristate acetate (PMA) and ligands of toll-like receptors (TLR), induce NETs. Although TLR-mediated NET formation can occur with preservation of living neutrophilic functions (called vital NETosis), PMA-stimulated neutrophils undergo cell death with NET formation (called suicidal NETosis)...
2016: Frontiers in Immunology
Giulia Camilla Varnier, Neil Sebire, Georgi Christov, Despina Eleftheriou, Paul A Brogan
Granulomatosis with polyangiitis (GPA) is a rare but serious small vessel vasculitis with heterogeneous clinical presentation ranging from mainly localised disease with a chronic course, to a florid, acute small vessel vasculitic form characterised by severe pulmonary haemorrhage and/or rapidly progressive vasculitis or other severe systemic vasculitic manifestations. Cardiac involvement is, however, uncommon in the paediatric population. We report a case of a 16-year-old male who presented with peripheral gangrene and vegetation with unusual location on the supporting apparatus of the tricuspid valve, initially considered to have infective endocarditis but ultimately diagnosed with GPA...
September 2016: Clinical Rheumatology
Shiber Shachaf, Molad Yair
BACKGROUND: Cryoglobulinemia is an immune-complex-mediated small vessel vasculitis that classically involves the skin, kidneys and peripheral nerves. Antiphospholipid syndrome (APS) is an autoimmune hypercoagulable disorder which causes blood vessel thrombosis. It can present as a multi-organ microthrombotic disorder which is called catastrophic APS. OBJECTIVE: In this case series we aim to describe the diagnostic and management challenges that arise when these two severe disorders simultaneously present in the same patient...
January 2016: Revista Brasileira de Reumatologia
Eun Jeong Gong, Do Hoon Kim, Joo Hyun Chun, Ji Yong Ahn, Kwi-Sook Choi, Kee Wook Jung, Jeong Hoon Lee, Kee Don Choi, Ho June Song, Gin Hyug Lee, Hwoon-Yong Jung, Jin Ho Kim, In Hye Song, Yong-Gil Kim
BACKGROUND/AIMS: Gastrointestinal involvement in vasculitis may result in life-threatening complications. However, its variable clinical presentations and endoscopic features, and the rarity of the disease, often result in delayed diagnosis. METHODS: Clinical characteristics, endoscopic features, and histopathological findings were reviewed from medical records. RESULTS: Of 6,477 patients with vasculitis, 148 were diagnosed as primary vasculitis with upper gastrointestinal involvement...
July 15, 2016: Gut and Liver
Uni Wong, Harris Yfantis, Guofeng Xie
Urticarial vasculitis (UV) is a rare small vessel vasculitis. UV is often idiopathic but can also present in the context of autoimmune disorders such as systemic lupus erythematosus, drug reactions, infections, or a paraneoplastic syndrome. Extracutaneous complications include intestinal ischemic injuries, in UV patients with nonspecific gastrointestinal symptoms such as abdominal pain and nausea. Prompt recognition and treatment can minimize morbidity and mortality. This paper describes a case of urticarial vasculitis-associated intestinal ischemia...
2016: Case Reports in Gastrointestinal Medicine
Ritu Aggarwal, Anju Gupta, Jasmine Naru, Noureddine Berka, Neha Nanda, Deepti Suri, Surjit Singh
Etiology of Henoch-Schönlein purpura (HSP) a small vessel vasculitis remains elusive. Susceptibility may be conferred by major histocompatibility complex. There are limited reports on the association of human leucocyte antigens (HLA) and HSP. The aim was to investigate the association of HLA-DRB1 (HLA class II antigen) with HSP. Forty three patients, <14years, who fulfilled the diagnostic criteria of HSP, laid by 'European League Against Rheumatism' were enrolled. Fifty four age-matched, healthy controls were included...
July 2016: Human Immunology
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