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Anca vasculitidis

Eoin F McKinney, Lisa C Willcocks, Verena Broecker, Kenneth G C Smith
The small-vessel vasculitides are a group of disorders characterised by variable patterns of small blood vessel inflammation producing a markedly heterogeneous clinical phenotype. While any vessel in any organ may be involved, distinct but often overlapping sets of clinical features have allowed the description of three subtypes associated with the presence of circulating anti-neutrophil cytoplasmic antibodies (ANCA), namely granulomatosis with polyangiitis (GPA, formerly known as Wegener's Granulomatosis), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (eGPA, formerly known as Churg-Strauss syndrome)...
July 2014: Seminars in Immunopathology
Luis Felipe Flores-Suárez
The primary vasculitidies are complex diseases with varied clinical manifestations, which may be common to those present in multiple diseases. The antineutrophil cytoplasm autoantibodies (ANCA) led to a revolution in the diagnosis and research of these diseases, being the first and so far, the only biomarkers for three of these diseases, which affect small caliber vessels. From their description, much progress has been made, but there are still gray or misunderstood areas regarding their best use in the clinic...
November 2012: Reumatología Clinica
Beatriz Zazueta Montiel, Luis Felipe Flores Suárez
The primary systemic vasculitidies (PSV) comprise an heterogeneous group of complex clinical entities with a common substrate: inflammation and necrosis of blood vessels. The inflammatory process involves vessels of any caliber. The location and the different size of the affected vessels, the severity of vascular damage and the different histopathological patterns which may predominate are the basic characteristics that define the different vasculitic syndromes and enable individualization. The description and progress in the knowledge of the primary vasculitidies have evolved considerably in recent decades, allowing for a better resolution of the complex problems placed by these patients...
December 2011: Reumatología Clinica
Ralph Kettritz
Renal involvement in autoimmunity has many facets. Glomerular, tubular and vascular structures are targeted and damaged as a consequence of autoimmune processes. Most dramatic and life-threatening causes are observed with diseases that result in rapidly progressive glomerulonephritis (GN), frequently accompanied by involvement of additional non-renal organs. Typical diseases with these characteristics are anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitidis, anti-glomerular basement membrane (anti-GBM) GN and proliferative lupus nephritis...
2008: Scandinavian Journal of Clinical and Laboratory Investigation. Supplementum
Loïc Guillevin, Christian Pagnoux
Systemic vasculitides are characterized by different histological aspects: fibrinoid necrosis of the arterial wall, giant cell arteritis, non-necrotizing arteritides without granuloma or giant cell infiltration. Each histological form is associated with a spectrum of diseases with variable clinical expression: giant cell angeitides, such as Takayasu's arteritis and giant cell arteritis, necrotizing angeitides, such as polyarteritis nodosa, Kawasaki disease, Wegener's granulomatosis, Henoch-Schönlein purpura or Churg-Strauss syndrome...
March 15, 2008: La Revue du Praticien
C J Day, P Hewins, C O Savage
In recent years there have been substantial developments in the understanding of the pathogenesis of ANCA-associated vasculitidies. Animal models have now been developed that finally prove a direct pathogenic role for ANCA, a subject fiercely debated since their original identification. We are also closer to understanding how ANCA exert their effects to cause disease. Progress has been made in elucidating how ANCA activate neutrophils, from how they bind antigen and where that antigen is located, to how antigen binding is translated into intracellular activity...
November 2003: Clinical and Experimental Rheumatology
A A Kaplan
Many primary renal diseases are associated with either antibody deposition within the glomerulus or an antibody associated autoimmunity, as may be seen with certain vasculitidies. Examples of these diseases include Goodpasture's syndrome, cryoglobulinemia, antineutrophil cytoplasmic antibody positive syndromes, and other forms of rapidly progressive glomerulonephritis. Immunoglobulins also may be nephrotoxic to the tubules such as is the case with myeloma related light chains. Given the rapid removal of immunoglobulins by therapeutic plasma exchange, this modality has been considered an appealing management option in the treatment of these renal diseases...
April 2001: Therapeutic Apheresis
Y M van der Geld, P C Limburg, C G Kallenberg
Proteinase 3 (PR3) is one of four serine protease homologues in the azurophilic granules of neutrophils and granules of monocytes. It is of importance that anti-neutrophil cytoplasmic antibodies (ANCA) in patients with Wegener's granulomatosis (WG) are mainly directed against PR3 only. Furthermore, PR3 is overexpressed in a variety of acute and chronic myeloid leukemia cells. Cytotoxic T lymphocytes specific for a PR3-derived peptide have been shown to specifically lyse leukemia cells that overexpress PR3. This review will focus on PR3 and the characteristics of PR3 that might implicate this particular antigen in the pathogenesis of WG and as target for immunotherapy in myeloid leukemias...
February 2001: Journal of Leukocyte Biology
P Gionchetti, M Vecchi, F Rizzello, M Ferretti, C Calabresi, A Venturi, M B Bianchi, C Brignola, R A Sinico, R De Franchis, M Miglioli, M Campieri
BACKGROUND: Antineutrophil cytoplasmic antibodies (ANCAs) from patients with vasculitidis can induce neutrophils to release oxygen radicals in vitro. ANCAs with a perinuclear pattern of immunofluorescence are found in most patients with ulcerative colitis, but several findings are against ANCAs having a pathogenetic role in this disease. AIMS: To evaluate the influence of ANCAs associated with ulcerative colitis on the respiratory burst activity of neutrophils. PATIENTS: Serum samples were obtained from 14 patients with ulcerative colitis, seven of whom showed positivity for p-ANCAs, three patients with vasculitidis, two with positivity for p-ANCAs, and one for c-ANCAs, and seven healthy volunteers...
January 1997: Gut
J L Niles
Two important types of antineutrophil cytoplasmic antibodies (ANCA) have been identified: anti-proteinase 3 and anti-myeloperoxidase antibodies. In the appropriate clinical setting, the presence of either is virtually diagnostic of the subset of vasculitis that includes Wegener's granulomatosis, microscopic polyangiitis (microscopic polyarteritis), the Churg-Strauss syndrome, idiopathic pauci-immune necrotizing and crescentic glomerulonephritis, and related and overlapping forms of these vasculitidies. The finding of ANCA throughout this group identifies these syndromes as belonging to a single category or spectrum of disease...
1996: Annual Review of Medicine
P Billing, S Tahir, B Calfin, G Gagne, L Cobb, S Targan, A Vidrich
Antineutrophil cytoplasmic antibodies (ANCAs) identified in the serum of 50 to 80% of ulcerative colitis (UC) patients yield a perinuclear staining pattern (pANCA) with alcohol-fixed neutrophils. The ANCAs of UC are distinguishable from those described for Wegener's granulomatosis and other vasculitidies. These various non-UC ANCAs recognize neutrophil granule constituents, but the antigenic moiety specific for the UC pANCA remains unknown. Although the perinuclear nature of some ANCA reactions is an artifact of the alcohol fixation of neutrophils, which causes cytoplasmic granules to redistribute around the nucleus, the UC pANCA reaction has been found not to be similarly affected...
October 1995: American Journal of Pathology
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