ANCA-Positive Vasculitis

Kamesh, Lavanya; Harper, Lorraine; Savage, Caroline O. S.

doi:10.1097/01.asn.0000016442.33680.3e

Cited by 108 publications

(77 citation statements)

References 45 publications

(37 reference statements)

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“…Modern treatment protocols with high dose steroids, cyclophosphamide, rituximab and plasmapheresis have improved the prognosis of this disease, but all are also associated with significant morbidities. Also in adult populations, it is documented that induction therapy fails to induce remission in approximately 10 % of patients [3]. Moreover, after induction therapy and remission, many patients suffer from clinical or serologic relapse, and kidney function has been reported to be inversely associated with relapse rate [22].…”

Section: Discussionmentioning

confidence: 99%

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Clinical presentation and outcome of pediatric ANCA-associated glomerulonephritis

Kouri

Andreoli

2016

Pediatr Nephrol

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Section: Discussionmentioning

confidence: 99%

“…It is a disease classically seen in adults and is rare in the pediatric population. The peak age of onset is commonly between the fifth and seventh decade of life [3]. As a result, much of the data and treatment protocols are extrapolated from adult data and applied to pediatric patients.…”

Section: Introductionmentioning

confidence: 99%

Clinical presentation and outcome of pediatric ANCA-associated glomerulonephritis

Kouri

Andreoli

2016

Pediatr Nephrol

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“…21 TNF-␣ is considered to play a pivotal role in the pathogenesis of AASV. 30 Interestingly, in cultured cells, TNF-␣ reduces endothelial NO bioavailability, and in vivo TNF-␣ may also directly alter endothelial vasomotor function. 31 Therefore, we elected to use primary systemic vasculitis as a model of clinical inflammation.…”

Section: Booth Et Al Vasculitis and Endothelial Dysfunctionmentioning

confidence: 99%

Infliximab Improves Endothelial Dysfunction in Systemic Vasculitis

et al. 2004

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Background-Endothelial vasomotor dysfunction and markers of systemic inflammation are independent determinants of cardiovascular risk. However, the link between clinical inflammation and endothelial dysfunction is unclear. The aim of this study was to use anti-neutrophil cytoplasmic antibody-associated systemic vasculitis (AASV) as a model of systemic inflammation in which to test the hypothesis that inflammation is associated with endothelial dysfunction and can be reversed with anti-tumor necrosis factor-␣ (TNF-␣) therapy. Methods and Results-Fourteen patients with active AASV and 21 age-matched control subjects were studied. Endothelial function was assessed through the use of forearm plethysmography and related to clinical disease activity: Birmingham Vasculitis Activity Score (BVAS) and serum levels of C-reactive protein (CRP), interleukin-6 (IL-6), and TNF-␣. The effects of anti-TNF-␣ therapy (infliximab), either alone (nϭ6) or in combination with standard treatment (nϭ4), on endothelial function were subsequently determined. Patients had a mean BVAS of 11Ϯ1, and CRP and IL-6 were higher in the AASV group than in control subjects (34.8Ϯ10.5 versus 1.6Ϯ0.2 pg/mL, PϽ0.001; 9.0Ϯ0.7 versus 6.7Ϯ0.6 pg/mL, Pϭ0.02). Forearm blood flow response to acetylcholine (ACh) was reduced in the patients compared with control subjects (Pϭ0.002), but sodium nitroprusside (SNP) responses were not (Pϭ0.3). The response to ACh improved with infliximab treatment (Pϭ0.004) in particular, with infliximab alone (Pϭ0.03). Conclusions-AASV is associated with endothelial dysfunction. Anti-TNF-␣ therapy, alone or in combination with standard treatment, results in clinical remission, reduced inflammation, and improved endothelium-dependent vasomotor responses.

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“…The majority of patients with PNCGN, with or without associated systemic vasculitis, have circulating antineutrophil cytoplasmic antibodies (ANCAs), which have been directly implicated in the pathogenesis of this form of glomerular injury. In contrast to patients with MGN, those with PNCGN typically present with rapidly progressive glomerulonephritis (RPGN) and an active urine sediment with red blood cell casts (3,4). PNCGN is an aggressive disease with a 1-yr mortality rate of up to 80% in the absence of immunosuppressive therapy.…”

mentioning

confidence: 99%