Plasma exchanges for the treatment of severe systemic necrotizing vasculitides in clinical daily practice: Data from the French Vasculitis Study Group

Luna, Gonzalo De; Chauveau, Dominique; Aniort, Julien; Carron, Pierre-Louis; Gobert, P.; Karras, Alexandre; Marchand-Adam, S.; Maurier, F.; Hatron, Pierre‐Yves; Mania, Alexandre; Guenno, G. Le; Bally, Stéphane; Bienvenu, Boris; Cardineau, Eric; Goulenok, Tiphaine; Jourde-Chiche, Noémie; Samson, Maxime; Huart, Antoine; Pourrat, Jacques; Tiple, A.; Aumaître, O.; Puéchal, Xavier; Heshmati, Farhad; Jeunne, Claire Le; Mouthon, Luc; Guillevin, Loı̈c; Terrier, Benjamin

doi:10.1016/j.jaut.2015.08.003

Cited by 35 publications

(24 citation statements)

References 34 publications

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“…In Japan, Hirayama et al reported that the 1‐year survival rate was 56.2% and the 5‐year survival rate was 41.5% in 175 DAH patients with rapidly progressive glomerulonephritis, where 50 of the 175 patients received PLEX therapy . Several studies reported the effectiveness of PLEX in DAH patients with AAV , and clinical guidelines have recommended PLEX for this population . Although the 1‐year survival in our study was only 26%, one likely reason is that very few patients (20%) received PLEX therapy.…”

Section: Discussionmentioning

confidence: 52%

Plasma Exchange Therapy to Reduce Mortality in Japanese Patients With Diffuse Alveolar Hemorrhage and Microscopic Polyangiitis

et al. 2019

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Diffuse alveolar hemorrhage (DAH) is well known as a serious complication of microscopic polyangiitis (MPA). We examined the effectiveness of plasma exchange (PLEX) therapy to reduce mortality in Japanese DAH patients with MPA. This retrospective, double‐center, observational cohort study included 20 DAH patients with MPA who were admitted to Juntendo University Hospital or Juntendo Koto Geriatric Medical Center between April 1998 and March 2018. The primary outcome was non–disease‐specific mortality. The 1‐year survival rate of patients with PLEX therapy (N = 4) was higher than that of patients with conventional therapy (N = 16, 75% and 13%, respectively, P = 0.037). Higher values of the 1996 Five‐Factor Score (FFS) and 2009 FFS were associated with increased mortality, with hazard ratios of 2.29 (P = 0.040) and 2.41 (P = 0.043), respectively, by Cox univariate analysis. We investigated PLEX therapy for reducing mortality in DAH patients with MPA, and the 1996 FFS and 2009 FFS were both independent prognostic factors.

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Section: Discussionmentioning

confidence: 52%

Plasma Exchange Therapy to Reduce Mortality in Japanese Patients With Diffuse Alveolar Hemorrhage and Microscopic Polyangiitis

et al. 2019

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“…Some clinical studies have demonstrated that plasma exchange, which removes these factors, improves renal dysfunction in patients with AAV and shortens the duration of hospitalization. 122,123 A clinical trial of plasma exchange and glucocorticoid treatment in patients with AAV (the PEXIVAS study, NCT00987389, https://clinicaltrials.gov/ct2/show/NCT00987389) has been completed and analysis of the results is now ongoing.…”

Section: [H2] Targeting Humoral Factorsmentioning

confidence: 99%

Pathogenesis and therapeutic interventions for ANCA-associated vasculitis

et al. 2018

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Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a vasculitis that affects systemic small vessels, accompanied by the presence of ANCAs in the serum. This disease entity includes microscopic polyangiitis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and drug-induced AAV. Similar to other autoimmune diseases, AAV develops in patients with a predisposing genetic background who have been exposed to causative environmental factors. The mechanism by which ANCAs cause vasculitis involves ANCA-mediated excessive activation of neutrophils that subsequently release inflammatory cytokines, reactive oxygen species and lytic enzymes. In addition, this excessive activation of neutrophils by ANCAs induces formation of neutrophil extracellular traps (NETs). Although NETs are essential elements in the innate immunity, excessive NET formation is harmful to small vessels. Moreover, NETs are involved not only in ANCA-mediated vascular injury but also in the production of ANCAs themselves. Therefore, a vicious cycle of NET formation and ANCA production is considered to be involved in the pathogenesis of AAV.

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“…Patients with a FFS ≥ 1 should receive intermittent intravenous cyclophosphamide in addition to corticosteroids [26,27]. In the steroid-refractory and recurrent PAN rituximab and infliximab or plasma exchanges has been used occasionally [28][29][30]. As our patient had FFS 2 (proteinuria and elevated serum creatinine), after nephrectomy, which was indicated given the combination of renal artery aneurism and stenosis with peri-vasal hematoma, kidney shrinking and absence of kidney function, he was started on the combination of prednisone and cyclophosphamide.…”

Section: Discussionmentioning

confidence: 99%

Polyarteriitis Nodosa with Real Arteries and Vein Involvement and Uroligical Manifestations: Case Report

Zakharova¹,

Shubin²,

Makarova³

et al. 2017

UNOAJ

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Plasma exchanges for the treatment of severe systemic necrotizing vasculitides in clinical daily practice: Data from the French Vasculitis Study Group

Cited by 35 publications

References 34 publications

Plasma Exchange Therapy to Reduce Mortality in Japanese Patients With Diffuse Alveolar Hemorrhage and Microscopic Polyangiitis

Plasma Exchange Therapy to Reduce Mortality in Japanese Patients With Diffuse Alveolar Hemorrhage and Microscopic Polyangiitis

Pathogenesis and therapeutic interventions for ANCA-associated vasculitis

Polyarteriitis Nodosa with Real Arteries and Vein Involvement and Uroligical Manifestations: Case Report

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