Association of Low Serum Complement C3 with Reduced Patient and Renal Survival in Antimyeloperoxidase-Associated Small-Vessel Vasculitis

2014 ◽  
Vol 126 (1) ◽  
pp. 67-74 ◽  
Author(s):  
Yair Molad ◽  
Ana Tovar ◽  
Shachaf Ofer-Shiber
PLoS ONE ◽  
2016 ◽  
Vol 11 (7) ◽  
pp. e0158871 ◽  
Author(s):  
Jean-François Augusto ◽  
Virginie Langs ◽  
Julien Demiselle ◽  
Christian Lavigne ◽  
Benoit Brilland ◽  
...  

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1224.1-1224
Author(s):  
H. Sakai ◽  
H. Yamashita ◽  
S. Nakajima ◽  
Y. Takahashi ◽  
H. Kaneko

Background:The alternative pathway of complement activation has recently been recognized as a key pathogenic event in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Some previous studies have reported that low serum complement C3 level in AAV patients is associated with more severe renal disease, worse renal prognosis, or higher mortality. However, the correlation between low serum C3 level and AAV relapse remains unclear.Objectives:To analyze the clinical characteristics and outcomes of AAV patients with low serum C3 levels at the time of diagnosis.Methods:We conducted a retrospective observational cohort study including 83 consecutive patients diagnosed with AAV in our hospital from January 1999 to December 2020. Serum C3 levels were measured at diagnosis. AAV included microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (EGPA); patients with ANCA-negative AAV were excluded. Patients were divided into low- and high-C3 groups (C3 < 100 and ≥ 100 mg/dL, respectively). We compared the clinical characteristics, and relapse-free and overall survival rates, of the two groups, and identified predictors of AAV relapse.Results:Of the 83 patients (MPA, n = 61; GPA, n = 18; EGPA, n = 4), 20 (24%) were in the low-C3 group. We found no significant group difference in sex, body mass index, disease type, ANCA subtype, Birmingham Vasculitis Activity Score (BVAS), or treatment. The low-C3 group patients were older (p=0.01), and had a higher Five Factor Score (FFS) (p=0.01) and a lower remission rate (p=0.02), than the high-C3 group. The generalized Wilcoxon test revealed that the relapse-free survival time was significantly shorter in the low-C3 group (29 months; 95% confidence interval [CI]: 15–49) than in the high-C3 group (82 months; 95% CI: 61–NA; p=0.01) (Figure 1A). The overall survival was also shorter in the low-C3 group (83 months; 95% CI: 8-121) than in the high-C3 group (112 months; 95% CI: 77-NA; p=0.03) (Figure 1B). In the Cox proportional hazards model, a low C3 level (< 100 mg/dL) (hazard ratio [HR], 3.01; 95% CI: 1.29–7.04], p=0.01) and GPA (HR, 3.04; 95% CI: 1.32–7.01; p=0.01) were independent predictors of AAV relapse.Figure 1.Kaplan-Meier estimates of the relapse-free (A) and overall (B) survival rates of AAV patients by baseline serum C3 levels. Eight patients who did not show remission were excluded in the relapse-free survival analysis. Black line: high-C3 group (≥ 100 mg/dL); red line: low-C3 group (< 100 mg/dL).Conclusion:AAV patients with low C3 levels at diagnosis were at higher risk of relapse. Larger prospective studies are required to confirm these findings.Disclosure of Interests:None declared


2018 ◽  
Vol 13 (3) ◽  
pp. 215-221 ◽  
Author(s):  
Francesco Ursini ◽  
Salvatore D`Angelo ◽  
Emilio Russo ◽  
Giorgio Ammerata ◽  
Ludovico Abenavoli ◽  
...  

Pathogens ◽  
2021 ◽  
Vol 10 (1) ◽  
pp. 31
Author(s):  
Céline Betti ◽  
Pietro Camozzi ◽  
Viola Gennaro ◽  
Mario G. Bianchetti ◽  
Martin Scoglio ◽  
...  

Leukocytoclastic small-vessel vasculitis of the skin (with or without systemic involvement) is often preceded by infections such as common cold, tonsillopharyngitis, or otitis media. Our purpose was to document pediatric (≤18 years) cases preceded by a symptomatic disease caused by an atypical bacterial pathogen. We performed a literature search following the Preferred Reporting of Systematic Reviews and Meta-Analyses guidelines. We retained 19 reports including 22 cases (13 females and 9 males, 1.0 to 17, median 6.3 years of age) associated with a Mycoplasma pneumoniae infection. We did not find any case linked to Chlamydophila pneumoniae, Chlamydophila psittaci, Coxiella burnetii, Francisella tularensis, or Legionella pneumophila. Patients with a systemic vasculitis (N = 14) and with a skin-limited (N = 8) vasculitis did not significantly differ with respect to gender and age. The time to recovery was ≤12 weeks in all patients with this information. In conclusion, a cutaneous small-vessel vasculitis with or without systemic involvement may occur in childhood after an infection caused by the atypical bacterial pathogen Mycoplasma pneumoniae. The clinical picture and the course of cases preceded by recognized triggers and by this atypical pathogen are indistinguishable.


2003 ◽  
Vol 104 (s49) ◽  
pp. 50P-51P
Author(s):  
C.L. Smyth ◽  
J. Smith ◽  
H.T. Cook ◽  
D.O. Haskard ◽  
C.D. Pusey

Nephron ◽  
2002 ◽  
Vol 92 (3) ◽  
pp. 673-675 ◽  
Author(s):  
Harun Akar ◽  
Cigdem Ozbaslı-Levi ◽  
Taskın Senturk ◽  
Gurhan Kadıkoylu ◽  
Edi Levi ◽  
...  

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