Disease Associations with Anti-Neutrophil Cytoplasmic Antibodies

Pusey, Charles D.; Gaskin, G.

doi:10.1007/978-1-4757-9182-2_25

Cited by 8 publications

(5 citation statements)

References 27 publications

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“…53 ANCA are rare in PAN 11 and were found in only 10.7% of the sera of patients with HBV-related PAN (n = 28), a model of "classic PAN," and in 27.3% of PAN without HBV (n = 22), including pa tients with PAN and MPA. Pusey and Gaskin 12 have also found p-ANCA (perinuclear staining pattern) with MPO reactivity in a small number of patients with PAN. Hauschild et al 56 reported an ANCA fre quency of 19.4% in 36 patients with PAN (p-ANCA in 3, c-ANCA [cytoplasmic staining] in 4).…”

Section: Laboratory Tests and Angiographic Investigationsmentioning

confidence: 89%

Polyarteritis Nodosa, Microscopic Polyangiitis, and Churg-Strauss Syndrome

Lhote¹,

Guillevin²

1998

Semin Respir Crit Care Med

115

164

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Section: Laboratory Tests and Angiographic Investigationsmentioning

confidence: 89%

Polyarteritis Nodosa, Microscopic Polyangiitis, and Churg-Strauss Syndrome

Lhote¹,

Guillevin²

1998

Semin Respir Crit Care Med

115

164

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“…Previous studies have reported that 50% to 80% of MPA patients are positive for ANCA (8-10). In one study, ANCA were present in 74.5% of patients at the time of diagnosis; among them, 87% had a perinuclear staining pattern (pANCA) and 13% had a cytoplasmic pattern (cANCA) (3).…”

Section: Discussionmentioning

confidence: 99%

Clinical Features and Outcomes of Microscopic Polyangiitis in Korea

Lee

Kim

et al. 2009

J Korean Med Sci

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Microscopic polyangiitis (MPA) is a systemic vasculitis affecting small vessels. To determine the clinical features and outcomes of MPA in Korean patients, we retrospectively reviewed the medical records of patients diagnosed with MPA at a single medical center in Korea between 1989 and 2006. The 18 patients who met the Chapel Hill criteria for MPA had a mean (±SD) age at the time of diagnosis of 62.4±12.7 yr. Renal manifestations and general symptoms were the most common features of MPA, with lung involvement also very common. Antineutrophil cytoplasmic antibodies (ANCA) were present in 17 of the 18 patients (94%). Of 17 patients treated with steroids and cyclophosphamide, 11 (65%) had stable or improved course. One patient treated with steroids without cyclophosphamide showed disease progression. Ten of the 18 patients (56%) died at a median follow-up of 8 months. MPA in Korean patients was distinguished by a higher rate of lung involvement, especially alveolar hemorrhage, which was the leading cause of death in our patients. Korean patients were also older at MPA onset and were more likely positive for ANCA. Other overall clinical manifestations did not differ significantly.

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“…Two types of antineutrophil cytoplasmic antibody (ANCA) are seen in these disorders, perinuclear (P)-ANCA and cytoplasmic (C)-ANCA, as defined by their appearance on indirect immunofluorescence of normal human neutrophils [1]. P-ANCA are found in a wider spectrum of disorders than C-ANCA, and have a greater variety of target autoantigens [2]. When P-ANCA are present in MP, the target autoantigen is usually MPO [3], a neutrophil granule enzyme involved in host defence.…”

Section: Introductionmentioning

confidence: 99%

“…When P-ANCA are present in MP, the target autoantigen is usually MPO [3], a neutrophil granule enzyme involved in host defence. C-ANCA predominate in WG, although they are also found in MP [2]. The major target antigen of C-ANCA is PR3 [4,5], a neutrophil granule enzyme with bacteriocidal properties, which is identical to myeloblastin, a leucocyte growth factor [6].…”

Section: Introductionmentioning

confidence: 99%

T cell responses to myeloperoxidase (MPO) and proteinase 3 (PR3) in patients with systemic vasculitis

Griffith

Coulthart

Pusey

1996

Clinical and Experimental Immunology

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SUMMARYT cell-mediated immune responses are likely to be important in the pathogenesis of systemic vasculitis. However, identifying the T cells involved has proved difficult, and there are conflicting reports regarding T cell proliferation in response to different autoantigens. Perinuclear (P) and cytoplasmic (C) anti-neutrophil cytoplasmic antibodies (ANCA) are closely associated with systemic vasculitis, and are generally specific for MPO or PR3, respectively. We studied the proliferative responses to MPO and PR3 of peripheral blood mononuclear cells from patients with P-ANCA or C-ANCA specific for these antigens by ELISA. These responses were compared with those of normal controls, and of disease controls with P-or C-ANCA not specific for MPO or PR3. The patient group as a whole showed significant T cell proliferation in response to the autoantigens compared with controls (P 0 : 005). Cells from nine of 13 P-ANCA-positive, anti-MPO-positive patients proliferated in response to MPO, compared with five of 16 controls (P 0 : 04). Cells from five of eight C-ANCA-positive, anti-PR3-positive patients proliferated in response to PR3, compared with two of 11 controls (P 0 : 05). These experiments demonstrate that patients with P-ANCA or C-ANCA possess T cells which respond to MPO or PR3, respectively. As in other autoimmune diseases, responses to both antigens were also seen in a proportion of healthy controls. Further analysis of these responses will be important in understanding the pathogenesis of systemic vasculitis and in designing specific immunotherapy.

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Disease Associations with Anti-Neutrophil Cytoplasmic Antibodies

Cited by 8 publications

References 27 publications

Polyarteritis Nodosa, Microscopic Polyangiitis, and Churg-Strauss Syndrome

Polyarteritis Nodosa, Microscopic Polyangiitis, and Churg-Strauss Syndrome

Clinical Features and Outcomes of Microscopic Polyangiitis in Korea

T cell responses to myeloperoxidase (MPO) and proteinase 3 (PR3) in patients with systemic vasculitis

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