Comparison of the 2022 ACR/EULAR Classification Criteria for Antineutrophil Cytoplasmic Antibody-Associated Vasculitis with Previous Criteria

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) comprises group of small vessel vasculitides, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). In 2022, the American College of Rheumatology (ACR) and the European Alliance of Associations for Rheumatology (EULAR) jointly proposed new classification criteria for AAV (the 2022 ACR/EULAR criteria). In this review, we briefly summarize the 2022 ACR/EULAR criteria for GPA, MPA, and EGPA, and introduce our clinical experience with applying them to patients who were previously diagnosed with AAV based on three criteria: firstly, the classification criteria for GPA and EGPA proposed by the ACR in 1990; secondly, the algorithm for the classification of AAV and polyarteritis nodosa proposed by the European Medicines Agency algorithm in 2007 (the 2007 EMA algorithm); and thirdly, the revised International Chapel Hill Consensus Conference nomenclature of vasculitides in 2012 (the 2012 CHCC definitions). We found that concordance rate was highest in patients with MPA (96.6%), followed by those with EGPA (86.3%) and GPA (73.8%). In addition, compared to previous criteria, we noted several issues of the undervalued or overvalued items in the 2022 ACR/EULAR criteria for classifying AAV and provided several suggestions. To increase the diagnostic accuracy and reduce the discordance rate among the new and previous criteria for AAV, we suggest that the previous criteria should be considered together with the 2022 ACR/EULAR criteria when applying the classification criteria for AAV to patients suspected of AAV.

Implication of Serious Infections in Patients With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis for the First Cycle of Rituximab: A Pilot Study in a Single Korean Center

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.

Modified Body Mass Index at Diagnosis is a Useful Predictor of Mortality in Patients With Antineutrophil Cytoplasmic Antibody-associated Vasculitis

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.

Association Between Idiopathic Cutaneous Leukocytoclastic Angiitis and ANCA-negative Microscopic Polyangiitis

Objective@#This study retrospectively reviewed the process of classifying antineutrophil cytoplasmic antibody (ANCA)-negative granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) in a cohort of patients with ANCA-associated vasculitis (AAV), and investigated the association between recurrent idiopathic cutaneous leukocytoclastic angiitis and ANCA-negative MPA. @*Methods@#The medical records of 242 patients with AAV were retrospectively reviewed. Of 49 patients with ANCA-negative AAV, 24 patients with ANCA-negative eosinophilic GPA (EGPA) were excluded, because ANCA positivity or negativity is not critical in classifying EGPA. Ultimately, 25 patients with ANCA-negative GPA and MPA were analysed in this study. The classification of GPA and MPA were based on the 2007 European Medicines Agency algorithm for AAV. @*Results@#The median age of patients with ANCA-negative GPA and MPA was 54.0 years and 24% were male. Of the 25 patients without ANCA, 8 patients were classified as GPA and 17 as MPA. Eight patients with ANCA-negative GPA were easily confirmed as definitive GPA. Fourteen of the 17 patients ANCA-negative MPA were classified as MPA based on histological features suggestive of AAV without granuloma formation and the absence of surrogate markers for GPA. Meanwhile, three of the patients that were ANCA-negative exhibited only recurrent idiopathic cutaneous leukocytoclastic angiitis without other major organs affected and thus were classified as possible MPA. Within one year, they were classified as definitive MPA based on ANCA positivity and/or renal histology. @*Conclusion@#Recurrent idiopathic cutaneous leukocytoclastic angiitis may be associated with ANCA-negative MPA in patients who exhibit cutaneous necrotising vasculitis.

Serum Clusterin Level Could Reflect the Current Activity of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

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.

The Efficacy of Mycophenolate Mofetil in Remission Maintenance Therapy for Microscopic Polyangiitis and Granulomatosis with Polyangiitis

Purpose@#The present study compared the efficacy of mycophenolate mofetil (MMF) with that of azathioprine (AZA) in Korean patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA). @*Materials and Methods@#The medical records of 69 patients with MPA and GPA who received cyclophosphamide and subsequently received AZA or MMF for remission maintenance therapy were reviewed. All-cause mortality, relapse, end-stage renal disease (ESRD), cerebrovascular accident, and cardiovascular disease were evaluated as poor outcomes. Having a lower Birmingham Vasculitis Activity Score (BVAS) was defined as the lowest tertile of BVAS (BVAS ≤11 in this study). @*Results@#In comparative analysis of the occurrence of poor outcomes among patients taking AZA only, MMF only, and MMF after AZA, patients taking MMF only exhibited a significantly lower cumulative ESRD-free survival rate than patients taking AZA only (p=0.028). In terms of ESRD occurrence between the groups based on BVAS at diagnosis, among patients with MPA and GPA with higher BVAS at diagnosis, patients taking MMF only exhibited a significantly lower cumulative ESRD-free survival rate than those taking AZA only (p=0.047). Among patients with MPA and GPA with the lowest tertile of BVAS at diagnosis, cumulative ESRD-free survival rates did not differ. @*Conclusion@#With regard to ESRD occurrence, the efficacy of MMF in remission maintenance therapy was less effective than AZA in patients with MPA and GPA. However, among patients with lower BVAS, there was no difference in the occurrence of poor outcomes between patients taking MMF and those taking AZA.

Male Sex Is a Significant Predictor of All-cause Mortality in Patients with Antineutrophil Cytoplasmic Antibodyassociated Vasculitis

Background@#We investigated and compared the initial clinical features at diagnosis and the poor outcomes during follow-up in Korean patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) based on sex. @*Methods@#The medical records of 223 immunosuppressive drug-naïve patients with AAV were reviewed. Age, body mass index (BMI), smoking history, AAV subtypes, ANCA positivity, clinical manifestations, Birmingham vasculitis activity score (BVAS), five-factor score (FFS), erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) at diagnosis were collected. All-cause mortality, end-stage renal disease (ESRD), cerebrovascular accident (CVA) and cardiovascular disease (CVD) were assessed as the poor outcomes of AAV during follow-up. @*Results@#The median age was 59.0 years and 74 of 223 AAV patients (33.2%) were men.Among variables at diagnosis, male patients exhibited higher BMI than female. However, there were no differences in other demographic data, AAV subtypes, ANCA positivity, BVAS, FFS, ESR and CRP between the two groups. Male patients received cyclophosphamide more frequently, but there were no significant differences in the frequencies of the poor outcomes of AAV between the two groups. Male patients exhibited a significantly lower cumulative patients' survival rate than female patients during the follow-up period based on all-cause mortality (P = 0.037). In the multivariable analysis, both male sex (hazard ratio [HR], 2.378) and FFS (HR, 1.693) at diagnosis were significantly and independently associated with allcause mortality during follow-up. @*Conclusion@#Male sex is a significant and independent predictor of all-cause mortality in AAV patients.

Serum Clusterin Level Could Reflect the Current Activity of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

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.

The Efficacy of Mycophenolate Mofetil in Remission Maintenance Therapy for Microscopic Polyangiitis and Granulomatosis with Polyangiitis

Purpose@#The present study compared the efficacy of mycophenolate mofetil (MMF) with that of azathioprine (AZA) in Korean patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA). @*Materials and Methods@#The medical records of 69 patients with MPA and GPA who received cyclophosphamide and subsequently received AZA or MMF for remission maintenance therapy were reviewed. All-cause mortality, relapse, end-stage renal disease (ESRD), cerebrovascular accident, and cardiovascular disease were evaluated as poor outcomes. Having a lower Birmingham Vasculitis Activity Score (BVAS) was defined as the lowest tertile of BVAS (BVAS ≤11 in this study). @*Results@#In comparative analysis of the occurrence of poor outcomes among patients taking AZA only, MMF only, and MMF after AZA, patients taking MMF only exhibited a significantly lower cumulative ESRD-free survival rate than patients taking AZA only (p=0.028). In terms of ESRD occurrence between the groups based on BVAS at diagnosis, among patients with MPA and GPA with higher BVAS at diagnosis, patients taking MMF only exhibited a significantly lower cumulative ESRD-free survival rate than those taking AZA only (p=0.047). Among patients with MPA and GPA with the lowest tertile of BVAS at diagnosis, cumulative ESRD-free survival rates did not differ. @*Conclusion@#With regard to ESRD occurrence, the efficacy of MMF in remission maintenance therapy was less effective than AZA in patients with MPA and GPA. However, among patients with lower BVAS, there was no difference in the occurrence of poor outcomes between patients taking MMF and those taking AZA.

Male Sex Is a Significant Predictor of All-cause Mortality in Patients with Antineutrophil Cytoplasmic Antibodyassociated Vasculitis

Background@#We investigated and compared the initial clinical features at diagnosis and the poor outcomes during follow-up in Korean patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) based on sex. @*Methods@#The medical records of 223 immunosuppressive drug-naïve patients with AAV were reviewed. Age, body mass index (BMI), smoking history, AAV subtypes, ANCA positivity, clinical manifestations, Birmingham vasculitis activity score (BVAS), five-factor score (FFS), erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) at diagnosis were collected. All-cause mortality, end-stage renal disease (ESRD), cerebrovascular accident (CVA) and cardiovascular disease (CVD) were assessed as the poor outcomes of AAV during follow-up. @*Results@#The median age was 59.0 years and 74 of 223 AAV patients (33.2%) were men.Among variables at diagnosis, male patients exhibited higher BMI than female. However, there were no differences in other demographic data, AAV subtypes, ANCA positivity, BVAS, FFS, ESR and CRP between the two groups. Male patients received cyclophosphamide more frequently, but there were no significant differences in the frequencies of the poor outcomes of AAV between the two groups. Male patients exhibited a significantly lower cumulative patients' survival rate than female patients during the follow-up period based on all-cause mortality (P = 0.037). In the multivariable analysis, both male sex (hazard ratio [HR], 2.378) and FFS (HR, 1.693) at diagnosis were significantly and independently associated with allcause mortality during follow-up. @*Conclusion@#Male sex is a significant and independent predictor of all-cause mortality in AAV patients.

Total Haemolytic Complement Activity at Diagnosis as an Indicator of the Baseline Activity of Antineutrophil Cytoplasmic Antibody-associated 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.

Association between follistatin-related protein 1 and the functional status of patients with anti-neutrophil cytoplasmic antibody-associated vasculitis / 中华医学杂志(英文版)

BACKGROUND@#Follistatin-like 1 (FSTL1) plays both pro-inflammatory and anti-inflammatory roles in the inflammatory processes. We investigated whether serum FSTL1 could predict the current anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV)-specific indices.@*METHODS@#We randomly selected 74 patients with AAV from a prospective and observational cohort of Korean patients with AAV. Clinical and laboratory data and AAV-specific indices were recorded. FSTL1 concentration was determined using the stored sera. The lowest tertile of the short-form 36-item health survey (SF-36) was defined as the current low SF-36. The cutoffs of serum FSTL1 for the current low SF-36 physical component summary (PCS) and SF-36 mental component summary (MCS) were extrapolated by the receiver operator characteristic curve.@*RESULTS@#The median age was 62.5 years (55.4% were women). Serum FSTL1 was significantly correlated with SF-36 PCS (r =  - 0.374), SF-36 MCS (r = -0.377), and C-reactive protein (CRP) (r = 0.307), but not with Birmingham vasculitis activity score (BVAS). In the multivariable linear regression analyses, BVAS, CRP, and serum FSTL1 were independently associated with the current SF-36 PCS (β = -0.255, β = -0.430, and β = -0.266, respectively) and the current SF-36 MCS (β = -0.234, β =-0.229, and β = -0.296, respectively). Patients with serum FSTL1 ≥779.8 pg/mL and those with serum FSTL1 ≥841.6 pg/mL exhibited a significantly higher risk of having the current low SF-36 PCS and SF-36 MCS than those without (relative risk 7.583 and 6.200, respectively).@*CONCLUSION@#Serum FSTL1 could predict the current functional status in AAV patients.

Pregnancy Morbidities in Korean Patients with Takayasu Arteritis: A Monocentric Pilot Study

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.

Subsequent Thrombotic Outcomes in Patients with Ischemic Stroke with Antiphospholipid Antibody Positivity

PURPOSE: International consensus criteria for antiphospholipid syndrome (APS) require persistently positive antiphospholipid antibodies (aPL) and medium or high titers in association with clinical manifestations. However, the clinical relevance of persistence and titers of aPL in patients with stroke has not been identified. We aimed to investigate the risk of subsequent thrombotic events in patients with ischemic stroke with aPL positivity in terms of aPL status. MATERIALS AND METHODS: We reviewed the medical records of 99 patients with ischemic stroke with at least one or more aPL-positivity (i.e., positivity for aCL, anti-β2-glycoprotein-1, and/or lupus anticoagulants). The patients were divided into two groups: “definite APS” who fulfilled the laboratory criteria and “indefinite APS” who fell short of the criteria. We compared the risk of subsequent thrombotic events between the two groups. Cox proportional hazards model and Kaplan-Meier survival curves were used for the analyses. RESULTS: Of the 99 patients, 46 (46%) were classified as having definite APS and 53 (54%) as having indefinite APS. The mean follow-up was 51.6 months. Overall event numbers were 14 (30.4%) in definite APS and 16 (30.2%) in indefinite APS. Increased subsequent thrombotic events (hazard ratio 1.039; 95% confidence interval 0.449–2.404; p=0.930) and decreased time to thrombotic events (log-rank p=0.321) were not associated with aPL status. CONCLUSION: There was no increased risk of subsequent thrombotic events in ischemic stroke patients with definite APS, compared with those with indefinite APS.

The Presence of Anti-ribonucleoprotein at Diagnosis Is Associated with the Flare during the First Follow-up Year in Korean Patients with Systemic Lupus Erythematosus

OBJECTIVE: The aim of this study was to examine whether the presence of anti-ribonucleoprotein (anti-RNP) antibodies at diagnosis is associated with systemic lupus erythematosus (SLE) flares in newly diagnosed patients during the first year of follow-up. METHODS: The medical records of 71 newly diagnosed SLE patients without other concomitant autoimmune disease, serious infection, or malignancy were reviewed retrospectively. SLE flares were defined according to the SLE Disease Activity Index 2000. Patients were divided into 2 groups according to the presence or absence of anti-RNP, and variables were compared between the groups. RESULTS: During the first year of follow-up, SLE patients with anti-RNP at diagnosis more frequently presented with mucosal ulcers (p=0.003), rash (p=0.001), and arthritis (p=0.007), compared to those without anti-RNP. The SLE flare incidence was remarkably higher in patients with anti-RNP than in those without anti-RNP (62.5% vs. 23.1%, p=0.001). SLE patients with anti-RNP at diagnosis had a significantly higher risk of ever experiencing a SLE flare during the first year of follow-up, compared to those without anti-RNP (odds ratio=8.250). CONCLUSION: In conclusion, SLE patients with anti-RNP at diagnosis were more than 8-fold more likely to experience an SLE flare during the first year of follow-up.

Brain meningioma in a patient with systemic lupus erythematosus / 영남의대학술지

Brain meningioma, the most common benign brain tumor, has been reported to account for 13-26% of all intracranial tumors, with a crude incidence rate of 2.3 per 100,000 persons for all types of meningiomas. The prevalence of neuropsychiatric lupus erythematosus is 15-91% and its clinical manifestations are diverse: from mild cognitive dysfunction to serious neurological or psychiatric symptoms. Here, we report the first Korean patient with brain meningioma and systemic lupus erythematosus who had undergone surgical tumor resection.

Delta Neutrophil Index as an Early Marker for Differential Diagnosis of Adult-Onset Still's Disease and Sepsis

PURPOSE: To investigate clinical implications of delta neutrophil index (DNI) to discriminate adult onset Still's disease (AOSD) from sepsis. MATERIALS AND METHODS: We reviewed the medical records of 13 patients with AOSD and 33 gender and age-matched patients with sepsis. In all subjects, microbial tests were performed to exclude or confirm sepsis. All laboratory data were measured two or three times during the first 3 days and represented by their mean levels. DNI was measured automatically by ADVIA 2120 for the first 3 days. RESULTS: There were no significant differences in white blood cell counts, neutrophil proportion, erythrocyte sedimentation rate and C-reactive protein between two groups. AOSD patients had notably lower DNI than sepsis patients regardless of the presence of bacteremia or not. However, both DNI and ferritin were not significant independent factors for predicting sepsis in the multivariate logistic regression analysis. Meanwhile, the area under the receiver operating characteristic curve (AUROC) of DNI was slightly higher than that of ferritin. When we set DNI of 2.75% as the cut-off value for predicting sepsis, 11 (84.6%) of AOSD patients had a DNI value below 2.75% and 2 (15.4%) of them had a DNI over 2.75% (relative risk for sepsis 176). CONCLUSION: We suggest that DNI may be a useful marker for differential diagnosis of AOSD from sepsis in the early phase as supplementary to ferritin.

Dermatomyositis Associated with Gallbladder Cancer

Dermatomyositis, an idiopathic inflammatory myopathy with characteristic skin manifestations, is associated with several types of cancer. Only three cases of gallbladder cancer with dermatomyositis have been reported worldwide, and none has been reported in Korea. We present a case of a 71-year-old male with proven dermatomyositis associated with gallbladder cancer to emphasize the necessity of work-up for malignancies with low incidence in patients with inflammatory myopathies.

Typical 18-FDG-PET/CT Findings of Polymyalgia Rheumatica: A Case Report

Polymyalgia rheumatica (PMR) is an inflammatory rheumatic condition characterized by generalized pain and morning stiffness in the shoulders, hip girdle, and neck. Since the pathogenesis of PMR is still uncertain, the diagnosis of PMR depends on clinical features. There have been several studies regarding radiological tools for the diagnosis of PMR. Recent studies using 18-FDG-PET showed bursitis, synovitis, uptake in the spinous process and asymptomatic large-vessel vasculitis in PMR patients. However, there was no report on the efficacy of 18-FDG-PET for diagnosis of PMR in Korea. Here, we are first reporting a case of a Korean patient with PMR, who had radiological findings including bursitis, synovitis, uptake in the spinous process and asymptomatic large-vessel vasculitis on 18-FDG-PET/CT.

Ventricular Tachyarrhythmias in a Patient with Andersen-Tawil Syndrome

Andersen-Tawil syndrome (ATS), a rare autosomal dominant disorder, is characterized by periodic paralysis, dysmorphic features and cardiac arrhythmias. This syndrome is caused by mutations of KCNJ2 gene, which encodes inward rectifying potassium channel. Here, we report an 18-year-old girl who was presented with life-threatening cardiac arrhythmia and acute respiratory distress. She was diagnosed with ATS, based on dysmorphic features, ventricular arrhythmia, and periodic paralysis. This is the first case to be reported in Korea who experienced a fatal cardiac arrest and respiratory failure caused by ATS.