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*

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: 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.

Purpose: We investigated whether antineutrophil cytoplasmic antibody (ANCA) positivity is associated with vascular manifestations at diagnosis of Behçet's disease (BD) and poor outcomes during follow-up. Materials and Methods: We retrospectively reviewed the medical records of 1060 patients with BD. Among them, 808 patients could be diagnosed with BD based on the revised version of the International Criteria for Behçet's Disease (ICBD) in 2014 (2014 ICBD criteria) and 588 patients could be diagnosed with BD based on the International Study Group (ISG) criteria proposed in 1990 (1990 ISG criteria). We examined the sites and patterns of vascular involvement in the BD patients at diagnosis and evaluated adverse outcomes during follow up, such as all-cause mortality, acute coronary syndrome, and deep vein thrombosis. Results: Among the 808 patients with BD based on the 2014 ICBD criteria, the rate of ANCA positivity at diagnosis was 2.2%. ANCA-positive BD patients exhibited a higher frequency of overall vascular manifestations (22.2% vs. 6.1%) and higher frequencies of vascular involvement in the upper extremities and visceral arteries than ANCA-negative BD patients (5.6% vs. 0.1% and 5.6% vs. 0.1%). Among the 588 BD patients based on the 1990 ISG criteria, similarly, ANCA-positive BD patients exhibited a higher frequency of vascular manifestations than ANCA-negative BD patients. ANCA positivity, however, did not seem to be associated with poor outcomes in BD patients during follow up. Conclusion ANCA positivity in BD patients was found to be associated with cross-sectional vascular involvement in the upper extremities and visceral arteries at diagnosis but was not predictive of poor outcomes during follow-up.

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: This study investigated the clinical implications of serious infections in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV) who received the first cycle of rituximab (RTX) during the first 6 months of follow-up. Methods: The medical records of 36 AAV patients treated with RTX were reviewed. A weekly dose of 375 mg/m 2 RTX was administered for 4 weeks to all patients along with glucocorticoids. Serious infections were defined as those requiring hospitalization. All-cause mortality during the first 6 months of follow-up was counted. The follow-up duration was defined as the period from the first RTX infusion to 6 months after the first RTX infusion. Results: The median age was 60.5 years, and 16 patients were male. Seven of 36 patients (19.4%) died and three AAV patients had five cases of serious infection such as enterocolitis, pulmonary aspergillosis, atypical pneumonia, cytomegalovirus pneumonia, and cellulitis. AAV patients with serious infections during the first 6 months of follow-up exhibited a significantly lower cumulative survival rate than those without serious infections (p<0.001). However, we found no independent predictor of serious infections using the Cox hazard model analysis. Conclusion Serious infection is an important predictor of all-cause mortality in Korean patients with AAV who received their first cycle of RTX but there were no significant variables to predict the occurrence of serious infections at the first RTX. Thus, in cases refractory to other induction therapies, RTX should be strongly considered, despite an increase in mortality rate.

Background/Aims: This study investigated the clinical implication of proteinase 3 (PR3)-antineutrophil cytoplasmic antibody (ANCA) in Korean patients with eosinophilic granulomatosis with polyangiitis (EGPA). Methods: Among the 242 patients with ANCA-associated vasculitis identified from the hospital database, 49 patients with EGPA were selected and analysed in this study. Demographic, clinical, and laboratory data at diagnosis were reviewed to compare the features of patients with PR3-ANCA and without, as well as the clinical outcomes of relapse and end-stage renal disease (ESRD) during the follow-up period. The outcomes of patients with PR3-ANCA and without were compared by using the Kaplan-Meier survival analysis. Results: The median age of the patients was 54 years, 17 (34.7%) were male, and six (12.2%) patients had PR3-ANCA at baseline. The most common items of the 1990 American College of Rheumatology criteria for EGPA were sinusitis (95.9%) and asthma (or asthmatic history) (93.9%). During the follow-up, none died, eight experienced relapse and two progressed to ESRD. EGPA patients with PR3-ANCA exhibited peripheral eosinophilia less frequently than those without (50.0% vs. 88.4%, p = 0.047). On the other hand, EGPA patients with PR3-ANCA experienced relapse more often compared to those without (50.0% vs. 11.6%, p = 0.047), and the cumulative relapse-free survival rate was lower compared to those without PR3-ANCA (p = 0.012). Conclusions EGPA patients possessing PR3-ANCA at disease diagnosis had distinct clinical feature and outcome compared to those without PR3-ANCA. These results should be taken into account in the management of patients with EGPA.