Background/Aims: This study evaluated the clinical utility of serum periostin measured at diagnosis in reflecting activity at diagnosis and predicting all-cause mortality during follow-up in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Methods: This study included 76 patients with AAV whose serum periostin was measured from sera collected and stored at diagnosis. The correlation of either serum periostin or the Birmingham Vasculitis Activity Score (BVAS) with other variables was evaluated. Cumulative survival rates were compared using Kaplan–Meier survival analysis. The variables at diagnosis were compared between deceased and surviving patients. Hazard ratios were obtained by Cox proportional hazard analysis. Results: The median age of the 76 patients was 64.0 years and 60.5% were female. The median BVAS and serum periostin were 5.0 and 10.9 ng/mL, respectively. Five of the 76 patients (6.6%) died. Serum periostin was independently correlated with cross-sectional BVAS, the Vasculitis Damage Index (VDI), white blood cell count, and serum albumin. Patients with serum periostin ≥ 15.9 ng/mL at diagnosis had a significantly lower cumulative survival rate than those without. In addition to high VDI, dyslipidaemia frequency, and C-reactive protein, deceased patients showed higher serum periostin than surviving patients. In multivariable Cox analysis, however, only dyslipidaemia rather than serum periostin was identified as an independent predictor of all-cause mortality. Conclusions This study is the first to demonstrate that serum periostin at diagnosis could independently reflect cross-sectional BVAS and further partially contribute to all-cause mortality prediction in patients with AAV.

Background/Aims: This study evaluated the clinical utility of serum periostin measured at diagnosis in reflecting activity at diagnosis and predicting all-cause mortality during follow-up in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Methods: This study included 76 patients with AAV whose serum periostin was measured from sera collected and stored at diagnosis. The correlation of either serum periostin or the Birmingham Vasculitis Activity Score (BVAS) with other variables was evaluated. Cumulative survival rates were compared using Kaplan–Meier survival analysis. The variables at diagnosis were compared between deceased and surviving patients. Hazard ratios were obtained by Cox proportional hazard analysis. Results: The median age of the 76 patients was 64.0 years and 60.5% were female. The median BVAS and serum periostin were 5.0 and 10.9 ng/mL, respectively. Five of the 76 patients (6.6%) died. Serum periostin was independently correlated with cross-sectional BVAS, the Vasculitis Damage Index (VDI), white blood cell count, and serum albumin. Patients with serum periostin ≥ 15.9 ng/mL at diagnosis had a significantly lower cumulative survival rate than those without. In addition to high VDI, dyslipidaemia frequency, and C-reactive protein, deceased patients showed higher serum periostin than surviving patients. In multivariable Cox analysis, however, only dyslipidaemia rather than serum periostin was identified as an independent predictor of all-cause mortality. Conclusions This study is the first to demonstrate that serum periostin at diagnosis could independently reflect cross-sectional BVAS and further partially contribute to all-cause mortality prediction in patients with AAV.

Background/Aims: This study evaluated the clinical utility of serum periostin measured at diagnosis in reflecting activity at diagnosis and predicting all-cause mortality during follow-up in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Methods: This study included 76 patients with AAV whose serum periostin was measured from sera collected and stored at diagnosis. The correlation of either serum periostin or the Birmingham Vasculitis Activity Score (BVAS) with other variables was evaluated. Cumulative survival rates were compared using Kaplan–Meier survival analysis. The variables at diagnosis were compared between deceased and surviving patients. Hazard ratios were obtained by Cox proportional hazard analysis. Results: The median age of the 76 patients was 64.0 years and 60.5% were female. The median BVAS and serum periostin were 5.0 and 10.9 ng/mL, respectively. Five of the 76 patients (6.6%) died. Serum periostin was independently correlated with cross-sectional BVAS, the Vasculitis Damage Index (VDI), white blood cell count, and serum albumin. Patients with serum periostin ≥ 15.9 ng/mL at diagnosis had a significantly lower cumulative survival rate than those without. In addition to high VDI, dyslipidaemia frequency, and C-reactive protein, deceased patients showed higher serum periostin than surviving patients. In multivariable Cox analysis, however, only dyslipidaemia rather than serum periostin was identified as an independent predictor of all-cause mortality. Conclusions This study is the first to demonstrate that serum periostin at diagnosis could independently reflect cross-sectional BVAS and further partially contribute to all-cause mortality prediction in patients with AAV.

Background/Aims: This study evaluated the clinical utility of serum periostin measured at diagnosis in reflecting activity at diagnosis and predicting all-cause mortality during follow-up in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Methods: This study included 76 patients with AAV whose serum periostin was measured from sera collected and stored at diagnosis. The correlation of either serum periostin or the Birmingham Vasculitis Activity Score (BVAS) with other variables was evaluated. Cumulative survival rates were compared using Kaplan–Meier survival analysis. The variables at diagnosis were compared between deceased and surviving patients. Hazard ratios were obtained by Cox proportional hazard analysis. Results: The median age of the 76 patients was 64.0 years and 60.5% were female. The median BVAS and serum periostin were 5.0 and 10.9 ng/mL, respectively. Five of the 76 patients (6.6%) died. Serum periostin was independently correlated with cross-sectional BVAS, the Vasculitis Damage Index (VDI), white blood cell count, and serum albumin. Patients with serum periostin ≥ 15.9 ng/mL at diagnosis had a significantly lower cumulative survival rate than those without. In addition to high VDI, dyslipidaemia frequency, and C-reactive protein, deceased patients showed higher serum periostin than surviving patients. In multivariable Cox analysis, however, only dyslipidaemia rather than serum periostin was identified as an independent predictor of all-cause mortality. Conclusions This study is the first to demonstrate that serum periostin at diagnosis could independently reflect cross-sectional BVAS and further partially contribute to all-cause mortality prediction in patients with AAV.

Background/Aims: This study evaluated the clinical utility of serum periostin measured at diagnosis in reflecting activity at diagnosis and predicting all-cause mortality during follow-up in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Methods: This study included 76 patients with AAV whose serum periostin was measured from sera collected and stored at diagnosis. The correlation of either serum periostin or the Birmingham Vasculitis Activity Score (BVAS) with other variables was evaluated. Cumulative survival rates were compared using Kaplan–Meier survival analysis. The variables at diagnosis were compared between deceased and surviving patients. Hazard ratios were obtained by Cox proportional hazard analysis. Results: The median age of the 76 patients was 64.0 years and 60.5% were female. The median BVAS and serum periostin were 5.0 and 10.9 ng/mL, respectively. Five of the 76 patients (6.6%) died. Serum periostin was independently correlated with cross-sectional BVAS, the Vasculitis Damage Index (VDI), white blood cell count, and serum albumin. Patients with serum periostin ≥ 15.9 ng/mL at diagnosis had a significantly lower cumulative survival rate than those without. In addition to high VDI, dyslipidaemia frequency, and C-reactive protein, deceased patients showed higher serum periostin than surviving patients. In multivariable Cox analysis, however, only dyslipidaemia rather than serum periostin was identified as an independent predictor of all-cause mortality. Conclusions This study is the first to demonstrate that serum periostin at diagnosis could independently reflect cross-sectional BVAS and further partially contribute to all-cause mortality prediction in patients with AAV.

Lectin-like oxidized low-density lipoprotein (LDL) receptor 1 (LOX1) binds to oxidized LDL, which is associated with inflammation in various vascular disorders. Here, we aimed to investigate the potential of soluble LOX1 (sLOX1) as an indicator of antineutrophil cytoplasmic antibody-associated vasculitis (AAV) activity. Serum levels of sLOX1 in frozen samples from patients with AAV enrolled in a prospective observational cohort study at the Severance Hospital were measured using enzyme-linked immunosorbent assay. Clinical and laboratory data were collected on the date when the blood sampling was performed. The association between sLOX1 and clinical and laboratory data was assessed using Pearson’s correlation analysis. The median age of the recruited 79 patients was 62.0 years, and 27 (34.2%) patients were men. The median Birmingham vasculitis activity score (BVAS), five-factor score, vasculitis damage index, and sLOX1 level were 6, 1, 3, and 911.9 pg/mL, respectively. Correlation analysis based on BVAS revealed that sLOX1 and total cholesterol were significantly inversely correlated with BVAS (r=-0.224, p=0.047 and r= -0.424, p<0.001, respectively). No significant correlations were observed between continuous variables and sLOX1 except for BVAS, although total cholesterol tended to correlate with sLOX1 (r=0.190, p=0.093). Additionally, sLOX1 was not influenced by sex, hypertension, diabetes mellitus, or the presence of pulmonary, cardiovascular, and renal involvement of AAV. In summary, sLOX1 was inversely correlated with BVAS in AAV patients, which is different from other vascular diseases or inflammatory diseases.

. Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is an autoimmune disease (AID) characterised by necrotising intravascular inflammation. Growing evidence suggests that immune system triggers altered lipid metabolism in AIDs. We investigated whether changes in lipid profile correlate with severity of disease in AAV. Methods. Seven lipid profiles were evaluated utilizing frozen serum samples from 67 patients registered in the Severance Hospital ANCA-associated VasculitidEs cohort by a chemistry autoanalyzer. The Birmingham Vasculitis Activity Score (BVAS) version 3 was used to measure patient’s assessment of global disease activity. The relationship between the BVAS with continuous variables was calculated by Pearson’s correlation analysis. Results. Thirty-five (52.2%), 19 (28.4%), and 13 (19.4%) patients were diagnosed with microscopic polyangiitis, granulomatosis with polyangiitis, and eosinophilic granulomatosis with polyangiitis, respectively. Patients’ mean age was 60.0 years, and 22 (32.8%) were male. Among the lipid profiles investigated, total cholesterol, high-density lipoprotein, and low-density lipoprotein, and apolipoprotein A1 and B were significantly associated with BVAS; apolipoprotein A1 showed the highest correlation with BVAS (r=−0.521, p＜0.001), remaining consistent even in patients with new-onset disease (r=−0.430, p=0.012). Apolipoprotein A1 had the highest association with the renal manifestation score among the clinical scores comprising BVAS (r=−0.457, p＜0.001). Conclusion. Decreased lipid levels, especially apolipoprotein A1, are relevant to increased AAV disease activity, and differ according to organ involvement. Measuring lipid profiles could have clinical implications regarding the assessment of global disease activity and organ involvement patterns.

Purpose: We investigated whether serum clusterin levels could reflect the current antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV)-specific indices. Materials and Methods: Fifty-seven patients with AAV and 40 healthy controls were included in this study. AAV-specific indices included the Short-Form 36-Item Health Survey Physical and Mental Component Summaries (SF-36 PCS and MCS) scores, Birmingham vasculitis activity score (BVAS), five-factor score (FFS), and vasculitis damage index. Clinical and laboratory data and AAV-specific indices were obtained at blood collection. The highest tertile of BVAS (≥16) was defined as high activity of AAV. Results: The median age of AAV patients was 64.0 years and 19 patients were male. SF-36 PCS score (r=0.328), SF-36 MCS score (r=0.289), BVAS (r=-0.404), erythrocyte sedimentation rate (r=-0.336), and C-reactive protein levels (r=-0.421) were significantly correlated with serum clusterin levels. In the multivariable linear regression analysis using AAV-specific indices and serum clusterin levels, both FFS (β=0.412) and serum clusterin levels (β=-0.250) were significantly associated with BVAS. When the optimal serum clusterin cut-off level for high activity of AAV was identified as 130.45 μg/mL, patients with serum clusterin level ≤130.45 μg/mL had a significantly higher risk for high activity of AAV than did those without (relative risk 7.194). Patients with AAV exhibited significantly lower serum clusterin levels than did healthy controls (168.2 μg/mL vs. 230.5 μg/mL). Conclusion Serum clusterin levels could reflect the current disease activity in patients with AAV.

Purpose: We investigated whether serum clusterin levels could reflect the current antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV)-specific indices. Materials and Methods: Fifty-seven patients with AAV and 40 healthy controls were included in this study. AAV-specific indices included the Short-Form 36-Item Health Survey Physical and Mental Component Summaries (SF-36 PCS and MCS) scores, Birmingham vasculitis activity score (BVAS), five-factor score (FFS), and vasculitis damage index. Clinical and laboratory data and AAV-specific indices were obtained at blood collection. The highest tertile of BVAS (≥16) was defined as high activity of AAV. Results: The median age of AAV patients was 64.0 years and 19 patients were male. SF-36 PCS score (r=0.328), SF-36 MCS score (r=0.289), BVAS (r=-0.404), erythrocyte sedimentation rate (r=-0.336), and C-reactive protein levels (r=-0.421) were significantly correlated with serum clusterin levels. In the multivariable linear regression analysis using AAV-specific indices and serum clusterin levels, both FFS (β=0.412) and serum clusterin levels (β=-0.250) were significantly associated with BVAS. When the optimal serum clusterin cut-off level for high activity of AAV was identified as 130.45 μg/mL, patients with serum clusterin level ≤130.45 μg/mL had a significantly higher risk for high activity of AAV than did those without (relative risk 7.194). Patients with AAV exhibited significantly lower serum clusterin levels than did healthy controls (168.2 μg/mL vs. 230.5 μg/mL). Conclusion Serum clusterin levels could reflect the current disease activity in patients with AAV.

Purpose: The present study investigated and compared the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) activity-predicting ability of the serum concentrations of the four interleukin (IL)-12 family cytokines including IL-23, IL-27, IL-35, and IL-39 in patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA). Materials and Methods: The present study included 70 patients with MPA and GPA. Clinical and laboratory data, particularly Birmingham Vasculitis Activity Score (BVAS), at the time of blood collection were obtained. The serum concentrations of IL-23, IL-27, IL-35, and IL-37 were measured using sera stored at -80°C. Patients were divided into two groups: the upper half of BVAS (BVAS ≥12) and the lower half of BVAS (BVAS <12). Results: The serum concentrations of IL-23 and IL-27 reflected AAV activity. Patients with the upper half of BVAS exhibited significantly higher serum concentrations of IL-23 and IL-27 than those without. Patients with the serum concentrations of IL-23 ≥132.1 pg/mL or IL-27 ≥684.7 pg/mL exhibited higher frequency and risk for the upper half of BVAS than those without [relative risks (RR) 5.143 and RR 4.091, respectively]. The serum concentrations of IL-27 were associated with age ≥65 years and proteinase 3-ANCA (or C-ANCA) negativity, whereas, those of IL-23 were associated with MPA. However, the serum concentrations of IL-35 and IL-39 were not useful in predicting AAV activity in this study. Conclusion The present study is the first to demonstrate that among the various members of IL-12 family cytokines, the serum concentrations of IL-23 and IL-27 possess AAV activity-predicting ability.