OBJECTIVE: We investigated the association of platelet distribution width (PDW) and mean platelet volume (MPV) with disease activity indices of ankylosing spondylitis (AS) in patients whose laboratory results or medical conditions would not affect PDW and MPV levels. METHODS: We analysed demographic and laboratory data of 88 patients with AS. On the same day as the laboratory tests were done, we assessed AS disease activity using the Bath Ankylosing Spondylitis Disease Activity Index, Bath Ankylosing Spondylitis Functional Index, Bath Ankylosing Spondylitis Patients Global Score and Ankylosing Spondylitis Disease Activity Score (ASDAS), including erythrocyte sedimentation rate (ESR) (ASDAS-ESR) and C-reactive protein (CRP) (ASDAS-CRP). The association was analyzed by linear regression. RESULTS: The median age of 88 patients was 38.0 years and the median length of observation was 5.5 years. The median platelet count was 266,500.0/µL, the median PDW was 10.7 fL and the median MPV 9.6 fL. The median ESR was 19.0 mm/hr and CRP was 2.5 mg/L. Among acute reactants, only CRP was negatively correlated with MPV, but not PDW (r=−0.218, p<0.041). However, both PDW and MPV were not significantly correlated with any disease activity index of AS. On multivariate linear regression analysis, only the length of observation was significantly correlated with MPV (β=0.224, p<0.044). CONCLUSION: PDW and MPV were not potent surrogate markers to reflect AS activity, with potential confounding strictly controlled, to affect MPV and PDW levels.
Baths ; Biomarkers ; Blood Platelets* ; Blood Sedimentation ; C-Reactive Protein ; Humans ; Linear Models ; Mean Platelet Volume* ; Platelet Count ; Spondylitis, Ankylosing*

Background/Aims: We compared the clinical and laboratory data between elderly and non-elderly patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) at diagnosis; further, we investigated the predictors at diagnosis for all-cause mortality and end-stage renal disease (ESRD) occurrence during follow-up in Korean elderly patients with AAV. Methods: We reviewed the medical records of 191 AAV patients regarding clinical manifestations and laboratory results at diagnosis and during follow-up. The follow-up duration was defined as the period from diagnosis to death for deceased patients or to the time of dialysis for ESRD patients, or to the last visit. Elderly (n = 67) and non-elderly (n = 124) patients were grouped based on an age threshold of 65 years. Results: At diagnosis, elderly patients exhibited higher median Birmingham Vasculitis Activity Score (BVAS) and higher frequencies of ANCA positivity and pulmonary manifestations than non-elderly patients. Furthermore, elderly patients exhibited increased median white blood cell count, blood urea nitrogen (BUN), alkaline phosphatase, erythrocyte sedimentation rate, and C-reactive protein and decreased median hemoglobin. However, there were no significant differences in all-cause mortality and ESRD occurrence between elderly and non-elderly patients. Meanwhile, elderly patients exhibited lower cumulative patients’ and ESRD-free survival rates than non-elderly patients. In the multivariable Cox hazards model, BUN, creatinine and serum albumin at diagnosis were independent predictors for ESRD occurrence, whereas there were no independent predictors at diagnosis for all-cause mortality. Conclusions Elderly AAV patients exhibited substantially higher rates of all-cause mortality and ESRD occurrence during follow-up compared than non-elderly AAV patients.

Objective: We investigated whether modified body mass index (mBMI) at diagnosis could predict all-cause mortality during follow-up in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Methods: The medical records of 203 AAV patients with BMI ≥18.5 kg/m 2 were reviewed. mBMI was calculated using an equation: mBMI=BMI (kg/m 2 )×serum albumin (g/L). All-cause mortality was considered as a poor outcome, and the follow-up duration based on all-cause mortality was defined as the period from AAV diagnosis to death for deceased patients, and the period from AAV diagnosis to the last visit for surviving patients. Results: The median age was 59.0 years (35.5% were male). The median BMI and mBMI were 22.8 kg/m2 and 813.2 kg · g/m2 · L.Twenty-five patients (12.3%) died. mBMI was well correlated with age, BVAS, FFS, erythrocyte sedimentation rate and C-reactive protein at diagnosis. Deceased patients exhibited significantly lower mBMI at diagnosis compared to surviving patients. AAV patients mBMI ≤570.1 kg g/m2 · L showed a significantly higher frequency of all-cause mortality (38.5% vs. 8.5%), and furthermore, exhibited a significantly higher risk for all-cause mortality than those with mBMI >570.1 kg · g/m2 · L (RR 6.750). mBMI ≤570.1 kg · g/m2 · L showed a significantly lower cumulative patients’ survival rate than those with mBMI >570.1 kg · g/m2 · L. In the multivariable Cox hazards model analysis, either serum albumin or mBMI was significantly associated with all-cause mortality in AAV patients. Conclusion In conclusion, mBMI ≤570.1 kg · g/m2 · L at diagnosis may be a useful predictor of all-cause mortality during followup additionally to serum albumin in AAV patients.

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.

Purpose: There has been no extensive study to compare the efficacy between rituximab originator (Mabthera®) and its biosimilar (Truxima®) for microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA). Here, we investigated the clinical effects of rituximab on poor outcomes of MPA and GPA in Korean patients, and compared those between Mabthera® and Truxima®. Materials and Methods: We retrospectively reviewed the medical records of a total of 139 patients, including 97 MPA patients and 42 GPA patients. At diagnosis, antineutrophil cytoplasmic antibody positivity and comorbidities were assessed. During follow-up, all-cause mortality, relapse, end-stage renal disease, cerebrovascular accident and acute coronary syndrome were evaluated as poor outcomes. In this study, rituximab was used as either Mabthera® or Truxima®. Results: The median age at diagnosis was 60.1 years and 46 patients were men (97 MPA and 42 GPA patients). Among poor outcomes, patients receiving rituximab exhibited a significantly lower cumulative relapse-free survival rate compared to those not receiving rituximab (p=0.002). Nevertheless, rituximab use did not make any difference in other poor outcomes of MPA and GPA except for relapse, which might be a rebuttal to the fact that rituximab use after relapse eventually led to better prognosis. There were no significant differences in variables at diagnosis and during follow-up between patients receiving Mabthera® and those receiving Truxima®. Patients receiving Truxima® exhibited a similar pattern of the cumulative survival rates of each poor outcome to those receiving Mabthera®. Conclusion Truxima® prevents poor outcomes of MPA and GPA as effectively as does Mabthera®.

We investigated pregnancy morbidities in Korean patients with Takayasu arteritis (TA) in a single tertiary hospital as a pilot study.We retrospectively reviewed the medical records of 12 pregnancies in seven patients with TA. All patients were diagnosed with TA based on the 1990 American College of Rheumatology classification criteria. The medical records of patients were well-documented, allowing review of clinical data including pregnancy morbidities. The angiographic and Ishikawa classifications at diagnosis and TA activity at delivery were assessed. Of the 12 pregnancies, two pregnancies ended in spontaneous abortion (16.7%), and one pregnancy (8.3%) had therapeutic abortion at 9 weeks due to maternal morbidity. Among the remaining nine pregnancies, only one child was delivered via normal spontaneous vaginal delivery, and the remaining eight were delivered by Caesarean section. Two out of nine (22.2%) neonates were born with low birth weight, and one of them was born at 30 weeks of gestation. The most common maternal complication was hypertension affecting 7/12 (58.3%) pregnancies. Preeclampsia occurred in one pregnancy, and gestational diabetes mellitus (GDM) occurred in two pregnancies. At delivery, disease activity of TA was stable in all pregnancies, and glucocorticoids were administered in nine pregnancies. Live birth rate of pregnant Korean patients with TA was 75%. Future studies are needed to reduce pregnancy-related complications.

OBJECTIVE: This study examined whether glycated hemoglobin (HbA1c) and glycated albumin (GA) are well correlated with the Ankylosing Spondylitis Disease Activity Score (ASDAS)-erythrocyte sedimentation rate (ESR), and ASDAS-C-reactive protein (CRP) in AS patients without medical conditions affecting the glycated protein levels. METHODS: The data of 76 patients with AS were analyzed. Univariate and multivariate analyses of the variables associated with ASDAS-ESR and ASDAS-CRP were performed using a linear regression test. The patients were divided into active and inactive AS groups based on an ASDAS-CRP of 2.1, and the variables between the two groups were compared. RESULTS: ASDAS-ESR did not correlated with either HbA1c or GA. ASDAS-CRP was positively correlated with HbA1c (r=0.315, p=0.006) and the white blood cell (r=0.288, p=0.012), and inversely correlated with hemoglobin (r=−0.241, p=0.036) and serum albumin (r=−0.262, p=0.022), but not GA. Multivariate analysis revealed HbA1c and white blood cell to be significantly correlated with ASDAS-CRP (β=0.234, p=0.033 and β=0.265, p=0.017). The mean HbA1c, not GA, of the active group was significantly higher than that of the inactive group (p=0.020). In addition, the optimal cut-off value of HbA1c was set to 5.6, and the patients with HbA1c ≥5.6 were found to have a 3.3 times higher risk of active AS than those without. CONCLUSION: HbA1c was significantly correlated with ASDAS-CRP, and could be a useful marker to reflect ASDAS-CRP in AS patients without medical conditions affecting the glycated protein levels.
Hemoglobin A, Glycosylated ; Humans ; Leukocytes ; Linear Models ; Multivariate Analysis ; Serum Albumin ; Spondylitis, Ankylosing*

PURPOSE: The controlling nutritional status (CONUT) score was developed to detect undernutrition in patients. Here, we investigated whether the CONUT score estimated at diagnosis could help predict poor outcomes [all-cause mortality, relapse, and end-stage renal disease (ESRD)] of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). MATERIALS AND METHODS: We retrospectively reviewed and collated data, including baseline characteristics, clinical manifestations (to calculate AAV-specific indices), and laboratory results, from 196 newly diagnosed AAV patients. Serum albumin, peripheral lymphocyte, and total cholesterol levels (at diagnosis) were used to calculate CONUT scores. RESULTS: In total, 111 patients had high CONUT scores (≥3), which showed higher frequency of myeloperoxidase-ANCA and ANCA positivity, and demonstrated higher AAV-specific indices. The optimal cut-offs of CONUT score (at diagnosis) for predicting all-cause mortality and ESRD were ≥3.5 and ≥2.5, respectively. Patients with CONUT scores higher than the cut-off at diagnosis exhibited lower cumulative and ESRD-free survival rates compared to those with lower scores than the cut-off. In multivariable analyses, diabetes mellitus [hazard ratio (HR): 4.394], five-factor score (HR: 3.051), and CONUT score ≥3.5 (HR: 4.307) at diagnosis were independent predictors of all-cause mortality, while only serum creatinine (HR: 1.714) was an independent predictor of ESRD occurrence. CONCLUSION: CONUT score at diagnosis is associated with all-cause mortality in AAV patients.
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; Antibodies, Antineutrophil Cytoplasmic ; Cholesterol ; Creatinine ; Cytoplasm ; Diabetes Mellitus ; Diagnosis ; Humans ; Kidney Failure, Chronic ; Lymphocytes ; Malnutrition ; Mortality ; Nutritional Status ; Recurrence ; Retrospective Studies ; Serum Albumin ; Survival Rate ; Vasculitis

Objective: The total haemolytic complement activity (CH50) assay evaluates the functioning of the complement system. Accumulating evidence indicates that the activation of the complement system plays a critical role in the pathogenesis of antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Therefore, this study aimed to investigate whether CH50 levels at diagnosis could reflect the baseline activity of AAV. Methods: This retrospective study included 101 immunosuppressive drug-naïve patients with AAV. At diagnosis, all patients underwent clinical assessments for disease activity, including measurement of the Birmingham Vasculitis Activity Score (BVAS) and Five Factor Score (FFS), and laboratory evaluations, such as tests for CH50, C3, and C4 levels. The association between CH50 levels and disease activity was determined. Results: The median BVAS and FFS at diagnosis were 12.0 and 1.0, respectively, whereas the median CH50 level was 60.4 U/mL. There was a negative correlation between the CH50 level and BVAS (r=−0.241; p=0.015). A CH50 cut-off value of 62.1 U/mL was used to classify the patients into two groups: patients with CH50 levels ＜62.1 U/mL (low-CH50 group) and those with CH50 levels ≥ 62.1 U/mL (high-CH50 group). The low-CH50 group had a higher proportion of patients with high disease activity, based on the BVAS, than the high-CH50 group (52.5% vs. 23.8%, p=0.004). Additionally, the low-CH50 group exhibited a lower relapse-free survival rate than the high-CH50 group; however, this difference was not statistically significant (p=0.082). Conclusion Low CH50 levels at diagnosis may reflect high baseline activity of AAV.

Background/Aims: We investigated the concordance rate of the classification of polymyositis (PM) and dermatomyositis (DM) between the Bohan and Peter criteria and the 2017 European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria for idiopathic inflammatory myopathies (IIMs) (the 2017 EULAR/ACR criteria) in Korean patients. Methods: We retrospectively reviewed the medical records of 137 patients with PM and DM. We finally included 72 PM patients and 49 DM patients who fulfilled the Bohan and Peter criteria for PM and DM and reclassified them by the 2017 EULAR/ ACR criteria. Results: Three patients (4.2%) with probable PM were newly reclassified as non-IIM due to a total score of 5.3 or smaller. Meanwhile, one patient with possible PM was newly reclassified as probable PM due to the presence of dysphagia. In addition, eight patients (16.3%) with possible DM with DM-specific typical skin rash were newly reclassified as amyopathic DM (ADM) due to the absence of proximal muscle weakness. The concordance rate of the classification between the Bohan and Peter criteria and the 2017 EULAR/ACR criteria was 95.8% for PM patients and 83.7% for DM patients. Conclusions The Bohan and Peter criteria were comparable to the 2017 EULAR/ ACR criteria for classifying PM and DM in Korean patients. Considering the convenience of the Bohan and Peter criteria in the real clinical settings, we suggest that the old criteria should be preferentially applied and then performing muscle biopsy should be considered in a patient suspected of PM without antihistidyl tRNA synthetase (anti-Jo-1). Moreover, we suggest that ADM could also clinically be classified by the old criteria.