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COVID-19 vaccination is essential in cancer patients. However, there is limited evidence of the prognosis of these patients, especially for those taking immune checkpoint inhibitors (ICIs). We present a patient on pembrolizumab for advanced endometrioid adenocarcinoma experiencing continuous diarrhea and subsequent episodes of fever with pain in multiple joints following a second dose of the BNT162b2 mRNA COVID-19 vaccine. An ICI-induced immune-related adverse effect (irAE) was the main diagnosis; cytokine release syndrome and rheumatoid arthritis were also considered. Notably, the novel irAE occurred after the 19th pembrolizumab trial, highlighting the potential effect of changes in systemic immunogenicity after BNT162b2 vaccination. Ultimately, the patient was treated with steroid, which alleviated her symptoms. Here, we report a rare adverse effect after COVID-19 vaccination in an endometrioid carcinoma patient on ICI therapy. This report shows that there is a need to consider and investigate vaccine-related adverse events.

Background This study investigated the predictive potential of the albumin-to-alkaline phosphatase ratio (AAPR) for cerebrovascular accident (CVA) occurrence in patients with antineutrophil cytoplasmic antibody–associated vasculitis. Methods This study included 239 AAV patients and reviewed their medical records retrospectively. AAPR was calculated using the following formula: AAPR = serum albumin (g/dL)/serum alkaline phosphatase (IU/L). CVA was defined only as cerebral infarction after AAV diagnosis in this study. In patients with CVA and those without CVA, the follow-up duration based on CVA was defined as the period from AAV diagnosis to CVA occurrence and to the last visit day, respectively. Results The median age of 239 AAV patients (130 microscopic polyangiitis, 64 granulomatosis with polyangiitis, and 45 eosinophilic granulomatosis with polyangiitis) was 59.0 years and 32.6% were men. The median serum albumin and alkaline phosphatase levels, and AAPR were 3.7 g/dL, 70.5 IU/L and 0.051, respectively. Nineteen patients had CVA during the median follow-up duration of 34.8 months. Using the receiver operator characteristic curve analysis, the optimal cut-off of AAPR for CVA occurrence was obtained as ≤ 0.035. AAV patients with AAPR ≤ 0.035 showed a significantly higher risk of CVA occurrence after AAV diagnosis than those with AAPR >0.035 (relative risk 5.000, p < 0.001). In the multivariable Cox analysis, only AAPR ≤ 0.035 was independently associated with CVA occurrence among AAV patients (hazard ratio 3.195, 95% confidence interval 1.014, 10.062). Conclusion This study demonstrated the predictive potential of AAPR calculated at AAV diagnosis for CVA occurrence after AAV diagnosis among AAV patients.

Objectives This study investigated whether the nutritional risk index (NRI) score at diagnosis might be useful for anticipating poor prognosis, in particular, all-cause mortality and end-stage renal disease (ESRD) in patients with antineutrophil cytoplasmic antibody–associated vasculitis (AAV). Methods The medical records of 242 immunosuppressive drug-naive patients with AAV were retrospectively reviewed. Data at diagnosis and poor prognosis and medications during follow-up were assessed. The NRI score was calculated by 1.519 × serum albumin (g/L) + 41.7 × present (kg)/ideal body weight (kg). Results The median age at diagnosis of patients with AAV (131 microscopic polyangiitis, 62 granulomatosis with polyangiitis, and 49 eosinophilic granulomatosis with polyangiitis) was 60 years (85 male). During follow-up, twenty-nine patients (12.0%) died after a period of 35.9 months, and 42 patients (17.4%) had ESRD for a period of 30.0 months. Using the receiver operator characteristic curve, the cutoffs of the NRI scores for all-cause mortality and ESRD were calculated as NRI ≤ 101.95 (sensitivity, 46.5%; specificity, 89.7%) and NRI ≤ 99.85 (sensitivity, 57.0%; specificity, 83.3%). In the multivariable Cox hazard model analyses, age (hazard ratio [HR], 1.035), five-factor score (HR, 1.623), and the NRI score ≤ 101.95 (HR, 4.262) were independent predictors of all-cause mortality, whereas, five-factor score (HR, 1.516), hypertension (HR, 1.906), and the NRI score ≤ 99.85 (HR, 3.623) were independent predictors of ESRD occurrence during follow-up in patients with AAV. Conclusions The NRI score at diagnosis may be a useful index to anticipate all-cause mortality and ESRD occurrence during follow-up in patients with AAV.

Background/Aim: This study investigated a correlation between ex vivo interferon-gamma (IFN)-gamma production using IFN-gamma releasing assay (IGRA) and antineutrophil cytoplasmic antibody-associated vasculitis (AAV) activity assessed by Birmingham vasculitis activity score (BVAS) in AAV patients. Patients and Methods: A total of 113 patients with AAV were consecutively selected from the AAV cohort and their medical records were reviewed. IGRA was performed at AAV diagnosis and before the initiation of glucocorticoids or immunosuppressive drugs for AAV treatment. Results: The median age was 61.8 years and 41.6% of the patients were men. Eighteen, 28, and 67 patients had EGPA, GPA, and MPA, respectively. Eighteen, 84, and 11 patients had positive, negative, and indeterminate results, and the median Nil, tuberculosis antigen, mitogen, and ex vivo IFN-gamma production were 0.1, 0.1, 10.0, and 9.8 IU/ml, respectively. There was no significant correlation between ex vivo IFN-gamma production and BVAS in AAV patients. When AAV patients were divided into two groups according to BVAS of 18, the higher tertile of BVAS, there was no significant difference in ex vivo IFN-gamma production between AAV patients with BVAS ≥18 and those without. In addition, there were no statistically significant correlations between ex vivo IFN-gamma production and BVAS in AAV patients with either indeterminate and negative IGRA results or only negative IGRA results. Conclusion: There is no correlation between ex vivo IFN-gamma production and BVAS. This study provided information on the reliability of the IGRA results for latent tuberculosis screening in AAV patients regardless of the activity of AAV.

Objective This study investigated whether histopathological classification and histologic lesion scores could significantly and independently predict the progression to end-stage kidney disease (ESKD) in Korean patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis-glomerulonephritis (AAV-GN). Methods This study included 113 patients with AAV-GN confirmed by kidney biopsy. The glomerular, tubulointerstitial, and vascular lesions were systematically assessed using a scoring system. The scoring system was adopted from the Banff scoring system but also the Oxford study and the revision of the ISN/RPS. For comparison, the scores were classified into two groups; the low, and the high, and the difference was investigated between ESKD and non-ESKD groups using Cox proportional analysis. Results At diagnosis, the median age was 59.0 years and 33.6% were male. Of 113 patients, 44.2% showed ESKD progression during follow-up. There were significant differences in several kidney-, inflammation-, and AAV-pathogenesis-related variables between AAV-GN patients with ESKD and those without. The sclerotic class exhibited the worst renal prognosis among the four histopathological classes. Among histopathological features, high interstitial fibrosis, tubular atrophy and global glomerulitis scores were significantly associated with ESKD progression. Whereas multivariable Cox analysis revealed only a high global glomerulitis score which means global endocapillary hypercellularity in a larger number of glomeruli is an independent predictor of ESKD progression. Moreover, among clinical and histopathological features, a high global glomerulitis score could also predict ESKD progression in addition to serum blood urea nitrogen and creatinine. Conclusion This study demonstrated the worst renal prognosis for the sclerotic class and first discovered that a high global glomerulitis score was an independent predictor of ESKD in patients with AAV-GN.

Objectives To explore the course of lung function and RA disease activity and predictive factors for deteriorating lung function in patients with RA-interstitial lung disease (ILD). Methods The Korean Rheumatoid Arthritis–Interstitial Lung Disease cohort is a multicentre, prospective observational cohort. Patients with RA-ILD were enrolled and followed up annually for 3 years for RA disease activity and ILD status assessment. Group-based modelling was used to cluster a similar predicted percentage of forced vital capacity (FVC%) patterns into trajectories. Results This study included 140 patients who underwent at least two pulmonary function tests. Four distinctive trajectories for predicted FVC% were ‘improving’ [n = 11 (7.9%)], ‘stable’ [n = 68 (38.4%)], ‘slowly declining’ [n = 54 (48.6%)] and ‘rapidly declining’ [n = 7 (5.0%)]. Most (77.7%) patients maintained or improved to low RA disease activity. The lung function trajectory was not comparable to the RA disease activity trajectory. Age ≥70 years [relative risk (RR) 10.8 (95% CI 1.30, 89.71)] and early RA diagnosed within the preceding 2 years [RR 10.1 (95% CI 1.22, 84.2)] were associated with increased risk for rapidly declining predicted FVC%. The risk for deterioration or mortality increased in patients with a simultaneous diagnosis of RA and ILD within 24 weeks [RR 9.18 (95% CI 2.05, 41.0)] and the extent of lung involvement [RR 3.28 (95% CI 1.12, 9.60)]. Conclusion Most patients with RA-ILD experienced stable or slowly declining lung function. In 5% of patients, predicted FVC% deteriorated rapidly, especially in older adults with early RA. The lung function trajectory was not comparable to the RA disease activity trajectory.

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.

Objective To examine the association between MTX, LEF and tacrolimus use and the progression of RA-associated interstitial lung disease (ILD). Methods The Korean RA-ILD cohort prospectively enrolled patients with RA-associated ILD at multiple centres from 2015 to 2018 and followed up with them for 3 years. ILD progression was defined by any of the followings: a decrease of ≥10% in forced vital capacity, a decrease of ≥15% in the diffusing capacity of the lung for carbon monoxide, or death from respiratory failure. Results Of 143 patients, 64 patients experienced ILD progression during a median follow-up period of 33 months. The use of MTX [adjusted hazard ratio (aHR), 1.06; 95% CI, 0.59, 1.89], LEF (aHR, 1.75; 95% CI, 0.88, 3.46) and tacrolimus (aHR, 0.94; 95% CI, 0.52, 1.72) did not increase the risk of ILD progression. However, the association between LEF use and the risk of ILD progression was significant in subgroups with poor lung function (aHR, 8.42; 95% CI, 2.61, 27.15). Older age, male sex, a shorter RA duration, higher RA disease activity and extensive disease at baseline were independently associated with ILD progression. Conclusion None of the three treatments increased the risk of RA-associated ILD progression, except for LEF, which increased the risk of ILD progression in patients with severe ILD. The appropriate use of conventional synthetic disease-modifying antirheumatic drugs considering RA disease activity and ILD severity would be important for the management of RA-associated ILD.

This study investigated the frequency of cancer-associated vasculitis, the types of associated cancers and vasculitides, and the temporal relationship in Korean patients who were diagnosed with both cancers and vasculitides.This study performed a digital search of the clinical data repository using selected diagnostic terms of vasculitides among patients diagnosed with cancers from May 2001 to May 2021. The time gap between the time of diagnosis of 'cancers' and that of 'vasculitides' was limited to 3 years. The types of cancers and vasculitides were reviewed.The mean age of 73 patients with both cancers and vasculitides with a time gap of fewer than 3 years was 53.0 years and 42.5% were men. Of the 215,897 patients with cancers, 73 patients were also diagnosed with vasculitides (0.034%). The most common type of cancer was thyroid cancer (28.8%), followed by lymphoma (13.7%), whereas the most frequent type of vasculitis associated with cancer was Behcet disease (52.1%), followed by granulomatosis with polyangiitis (12.3%). The median time gap between cancer and vasculitis was - 17.0 days. Among vasculitides, Behcet disease was closely associated with various cancers compared to other types. Twenty-one patients exhibited both cancers and vasculitides between 0 and 90 days after the diagnosis of the corresponding cancer.The frequency of cancer-associated vasculitis was 0.034% in Korean patients. The types of cancers and vasculitides in cancer-associated vasculitis and the distributions of sex and age may be dependent on ethnic and geographic differences. Key Points • The frequency of cancer-associated vasculitis was 0.034% in Korean patients. • The most common cancer and vasculitis in cancer-associated vasculitis were thyroid cancer and Behcet disease. • The types of cancers and vasculitides in cancer-associated vasculitis seemed to be dependent on ethnic and geographic differences.

Background: This study investigated whether the empirical dietary inflammatory index (eDII) score is associated with the inflammatory burden as well as the depressive status in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV).Methods: Eighty-four patients with AAV participated in this study. Birmingham vasculitis activity score (BVAS) and short-form 36-item Health Survey mental component summary (SF-36 MCS) were considered as indices assessing the inflammatory burden and depressive status, respectively. The eDII includes 16 food components and consists of three groups: -9 to -2, the low eDII group; -1 to +1, the moderate eDII group; and +2 to +10, the high eDII group. Furthermore, the lower eDII group includes both the low and moderate eDII groups.Results: The median age was 64.5 years (36 men). The eDII scores inversely correlated with SF-36 MCS (r = -0.298, P = 0.006) but not with BVAS. SF-36 MCS significantly differ between the lower and higher eDII groups (69.7 vs. 56.7, P = 0.016), but not among the low, moderate, and high eDII groups. Additionally, when patients with AAV were divided into two groups according to the upper limit of the lowest tertile of SF-36 MCS of 55.31, patients in the higher eDII group exhibited a significantly higher risk for the lowest tertile of SF-36 MCS than those in the lower eDII group (RR 3.000). Conclusion: We demonstrated for the first time that the eDII could predict the depressive status by estimating SF-36 MCS without utilising K-CESD-R ≥ 16 in patients with AAV.

The pan-immune-inflammation value (PIIV), a novel, validated predictor of the prognosis of several diseases, has been recently introduced. We investigated whether PIIV at diagnosis could predict all-cause mortality during follow-up in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).Medical records of 219 immunosuppressive drug-naïve patients with AAV were reviewed. PIIV was calculated as follows: neutrophil count (x 1000/m3) x monocyte count (x 1000/m3) x platelet count (x 1000/mm3) / lymphocyte count (x 1000/m3). Additionally, conventional risk factors of mortality, AAV-specific indices, and acute-phase reactants at diagnosis were evaluated.The median age at diagnosis was 59.0 years and 32.9% of the patients were male. During follow-up, 24 patients (11.0%) died due to all causes. When the cut-off of PIIV at diagnosis for all-cause mortality was set at 1011.3, sensitivity and specificity of 52.0% and 71.2%, were attained (p=0.041). When AAV patients were divided into two groups according to the calculated cut-off, those with PIIV ≥1011.3 at diagnosis had a significantly lower cumulative survival rate than those without (p=0.009). In the multivariable Cox hazards model analysis, male gender (HR 2.307), FFS (HR 1.728) and PIIV ≥1011.3 (HR 2.689) were identified as significant and independent risk factors of all-cause mortality.PIIV at diagnosis exceeding the optimal cut-off for death could predict all-cause mortality during follow-up in AAV patients comparable to male gender and FFS at diagnosis.

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.

A systemic inflammation response index (SIRI) has been recently introduced as a tool for the assessment of the prognosis of several critical medical conditions. In this study, we investigated whether SIRI at diagnosis could estimate the cross-sectional disease activity and predict poor prognosis during follow-up in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We reviewed the medical records of 224 immunosuppressive drug-naive AAV patients and obtained clinical and laboratory data both at diagnosis and during follow-up. SIRI was calculated using the following equation: SIRI = peripheral blood neutrophil count × monocyte count/lymphocyte count. The median age of AAV patients at diagnosis was 59.0 years and 33% were male. In the univariable linear regression analysis, SIRI value at diagnosis was not significantly correlated with the cross-sectional Birmingham vasculitis activity score (BVAS) (r = 0.125, P = 0.062). When the SIRI cut-off value at diagnosis was set at 2847.9 mm−3 using the receiver operator characteristic curve, the sensitivity was 56.0% and the specificity was 68.3% for all-cause mortality [area 0.618, 95% confidence interval (CI) 0.502, 0.734]. AAV patients with SIRI ≥ 2847.9 mm−3 had a significantly higher risk for all-cause mortality than those with SIRI

The association between vitamin D levels and disease activity has been established in patients with several autoimmune rheumatic diseases. We aimed to examine the association between vitamin D and disease activity of antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Fifty-four AAV patients and 50 age- and sex-matched healthy controls without vitamin D supplements were included. Clinical and laboratory data were evaluated during the assessment of vitamin D levels. Two different forms of vitamin D in the sera—25(OH)D, which is the sum of 25(OH)D2 and 25(OH)D3, and 25(OH)D3, which only includes 25(OH)D in its D3 form—were measured, and the relationship between vitamin D and the obtained data was assessed. Variations in vitamin D levels relative to the season were also evaluated. Patients with AAV demonstrated considerably lower 25(OH)D serum levels than healthy controls (16.0 vs. 20.4 ng/mL, p = 0.016), and the proportion of individuals with vitamin D deficiency was higher in patients with AAV than in healthy controls (68.5% vs. 48.0%, p = 0.035). Both serum 25(OH)D and 25(OH)D3 were positively associated with the 36-item Short-form Health Survey (SF-36) physical component summary and SF-36 mental component summary (MCS) scores. A negative correlation was observed between 25(OH)D and 25(OH)D3 serum levels and Birmingham vasculitis activity score (BVAS), C‑reactive protein (CRP), and white blood cell count. Linear regression analysis indicated haemoglobin and 25(OH)D levels to be independently associated with BVAS and CRP and 25(OH)D levels with SF-36 MCS score. No seasonal variations were observed in vitamin D levels. The results from this study suggest that vitamin D levels could provide clinically useful information in AAV.

This study compared the frequency and severity of depressive disorders in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV) before and during the severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2) pandemic using the Korean version of the Center for Epidemiologic Studies Depression Scale-Revised (K-CESD-R) and the Korean version of the Profile of Mood States (K-POMS) depression, and further determined predictors of current depressive disorders in the patients during the pandemic.Of the 61 patients with AAV who participated before the pandemic, 8 patients were transferred to other hospitals, 3 patients died, and 2 patients refused to participate in this study. Finally, 48 patients participated in this study. Depression disorders were defined as K‑CESD-R ≥ 16.When comparing the patterns of mental health between patients with AAV before and during the pandemic, no change in K‑CESD‑R or K‑POMS subscale scores was observed. Among AAV-related indices, regardless of the pandemic, the short-form 36-item Health Survey (SF-36) mental component score (MCS) and physical component score (PCS) were significantly correlated with K‑CESD‑R and could predict current depressive disorders. When the cut-off of Birmingham vasculitis activity score (BVAS) for depressive disorders was obtained by the receiver operator characteristic curve, it significantly predicted current depressive disorders in patients with AAV during the pandemic, unlike those before the pandemic.We verified that SF-36 MCS and PCS could predict current depressive disorders, regardless of the pandemic, and furthermore, we demonstrated for the first time that BVAS was a predictor of current depressive disorders in patients with AAV during the pandemic unlike those before the pandemic.HINTERGRUND: In der vorliegenden Studie wurden die Häufigkeit und Schwere depressiver Störungen vor und während der durch das „severe acute respiratory syndrome coronavirus type 2“ (SARS-CoV-2) verursachten Pandemie bei Patienten verglichen, die an einer mit antineutrophilen zytoplasmatischen Antikörpern assoziierten Vaskulitis (AAV) erkrankt sind. Dazu wurden die koreanische Version der Center for Epidemiologic Studies Depression Scale-Revised (K-CESD-R) und die koreanische Version des Profile of Mood States (K-POMS) für Depression verwendet; außerdem wurden weitere Prädiktoren für aktuell bestehende depressive Störungen bei den Patienten während der Pandemie ermittelt.Von den 61 Patienten mit AAV, die vor der Pandemie teilnahmen, wurden 8 Patienten an andere Krankenhäuser überwiesen, 3 Patienten starben, und 2 verweigerten die Teilnahme an der Studie. Letztlich nahmen 48 Patienten an der Studie teil. Depressive Störungen wurden definiert als ein Wert für K‑CESD-R ≥ 16.Beim Vergleich der Muster für psychische Gesundheit von Patienten mit AAV vor und während der Pandemie wurde keine Veränderung hinsichtlich der Subskalenwerte für K‑CESD‑R oder K‑POMS festgestellt. Unter den AAV-bezogenen Indizes waren – unabhängig von der Pandemie – der Score für die psychische Komponente (MCS) und die physische Komponente (PCS) des Short-Form 36-Item Health Survey (SF-36) in signifikanter Weise mit der K‑CESD‑R korreliert, sie eigneten sich zur Vorhersage bestehender depressiver Störungen. Bei Ermittlung des Grenzwerts des Birmingham Vasculitis Activity Score (BVAS) für depressive Störungen anhand der „receiver operator characteristic curve“ erwies sich dieser als signifikanter Prädiktor für aktuell bestehende depressive Störungen bei Patienten mit AAV während der Pandemie – im Gegensatz zu vor der Pandemie.Es wurde erneut nachgewiesen, dass sich mit dem SF-36-MCS und -PCS aktuell bestehende depressive Störungen vorhersagen ließen – unabhängig von der Pandemie –, und darüber hinaus wurde hier erstmals gezeigt, dass der BVAS ein Prädiktor aktuell bestehender depressiver Störungen bei Patienten mit AAV während der Pandemie im Gegensatz zu vor der Pandemie war.

This study investigated the clinical impact of antineutrophil cytoplasmic antibody (ANCA) positivity on the occurrence of interstitial lung disease (ILD) in patients with probable and definite polymyositis (PM)/dermatomyositis (DM) who met both the Bohan and Peter and the 2017 European League Against Rheumatism/American College of Rheumatology criteria.The medical records of 75 PM/DM patients were retrospectively reviewed. ANCA and anti-Jo 1 positivity at diagnosis were obtained, and pulmonary function test and chest high-resolution computed tomography results at ILD occurrence were collected. The follow-up duration based on ILD was defined as the period from the time of PM/DM diagnosis to the occurrence of ILD in PM/DM patients with ILD and to the last visit for those without ILD.The median age was 50.0 years and 21.3% were male. ANCA and anti-Jo 1 were detected in 12 (16.0%) and 26 patients (34.7%), respectively. ILD occurred in 32 patients, 24 of whom had ILD at the time of PM/DM diagnosis. Anti-Jo 1 was detected more often in PM/DM patients without ANCA than those with (39.7% vs. 8.3%). ILD occurred more frequently in PM/DM patients with ANCA than those without ANCA (75.0% vs. 36.5%). However, the occurrence of ILD was not affected by anti-Jo 1 positivity. Furthermore, ANCA-positive PM/DM patients exhibited a significantly lower cumulative ILD-free survival rate than ANCA-negative PM/DM patients (P=0.009).ANCA positivity at the time of PM/DM diagnosis might be an important risk factor for ILD in PM/DM patients.

This study aimed to investigated whether triglyceride-glucose-body mass index (TyG-BMI) and a new index using TyG-BMI (NITGB) could predict all-cause mortality in nonobese patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). The medical records of 78 nonobese AAV patients (BMI

Background: Glycated albumin (GA) is known to reflect the current inflammatory burden in non-diabetes mellitus (DM) patients. In this study, we investigated whether GA at diagnosis could reflect the cross-sectional activity and predict poor outcomes during follow-up in non-DM patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).Methods: The medical records of 118 immunosuppressive drug-naïve AAV patients were retrospectively reviewed, and 76 patients who had both GA and glycated haemoglobin (HbA1c) results but not DM were included in this study. Demographic, clinical, and laboratory data at diagnosis were assessed. Results: The median age of AAV patients was 61 years, and 31 patients were male. GA was positively correlated with five-factor score (r = 0.282), Birmingham vasculitis activity score (BVAS) assigned to renal manifestation (r = 0.315), and blood urea nitrogen (r = 0.382), whereas negatively correlated with haemoglobin (r = -0.345). AAV patients with end-stage renal disease (ESRD) exhibited significantly higher GA than those without ESRD (15.8% vs. 13.6%). When the cut-off of GA at diagnosis for ESRD was set at GA ≥ 14.25%, AAV patients with GA ≥ 14.25% had a significantly higher risk for ESRD development than those without (relative risk 12.040). In addition, AAV patients with GA ≥ 14.25% exhibited significantly lower cumulative ESRD-free survival rates than those without (P = 0.020).Conclusion: In conclusion, GA at diagnosis can reflect the cross-sectional BVAS assigned to renal manifestation of AAV and predict ESRD development during follow-up better than HbA1c or GA/HbA1c in AAV patients.

This study applied the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology (the 2022 ACR/EULAR) criteria for microscopic polyangiitis (MPA) to patients with previously diagnosed MPA as per the 2007 European Medicines Agency algorithm (the 2007 EMA algorithm) and the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides (the 2012 CHCC definitions) The concordance rate between the new and old criteria was investigated.This study included 117 patients with MPA, and the new criteria were applied to these patients. MPA could be classified when the total score is ≥5.The median age was 64.0 years. The concordance rate between the new and old criteria reached 96.6%. Four patients with previously diagnosed MPA were unclassified. Of these, three patients without myeloperoxidase (MPO)-antineutrophil cytoplasmic antibody (ANCA) (or perinuclear [P]-ANCA) were not reclassified as having MPA according to the new criteria, despite histopathological findings that were suggestive of MPA based on both the 2007 EMA algorithm and the 2012 CHCC definitions. Conversely, three of four patients with both MPO-ANCA (or P-ANCA) and proteinase 3 (PR3)- ANCA (or cytoplasmic [C]-ANCA) were reclassified as having both MPA and granulomatosis with polyangiitis (GPA) simultaneously according to the 2022 ACR/EULAR criteria for MPA and GPA.In the new criteria, excessively high score was assigned to MPO-ANCA (or P-ANCA) and MPA-specific histopathological findings were not considered. Hence, the 2007 EMA algorithm and the 2012 CHCC definitions can be applied as additional criteria to complex cases.

This study investigated whether the metabolic syndrome (MetS) severity (MSSS) at diagnosis could predict poor outcomes during follow-up in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients with MetS. The equation for the MSSS at diagnosis used in this study was developed and validated in Korean adults aged 20–59 years. The medical records of 261 patients with AAV were retrospectively reviewed, and finally, 36 AAV patients with MetS aged 20–59 years fulfilling the inclusion criteria were included in this study. All-cause mortality, relapse, end-stage kidney disease (ESKD), cerebrovascular accident, and cardiovascular disease were assessed as the poor outcomes of AAV. Their median age was 51.2 years and 36.1% were male. The MSSS was significantly correlated with age and serum albumin but not AAV-specific indices. Among the five poor outcomes, only ESKD showed a relatively significant area under the curve (area 0.696) in receiver operating characteristic curve analysis. In the multivariable Cox hazards model analysis, both serum creatinine (HR 3.033) and MSSS (HR = 2.221) were significantly associated with ESKD occurrence. When the cut-off of the MSSS for ESKD was set at 1.72, ESKD occurred more frequently in patients with MSSS ≥ 1.72 than in those with MSSS < 1.72 (75.0% versus 14.3%, p = 0.002). Furthermore, patients with MSSS ≥ 1.72 exhibited a significantly lower cumulative ESKD-free survival rate than those with MSSS < 1.72 (p = 0.001). MSSS at the time of AAV diagnosis independently predicted the occurrence of ESKD during follow-up in patients with AAV and MetS.

This study investigated the effect of the number of metabolic syndrome (MetS) components on all-cause mortality in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV) with MetS.The medical records of 93 AAV patients with MetS were retrospectively reviewed. MetS was diagnosed when three or more the following MetS components for Asians were met: (i) increased waist circumference; ii) high blood pressure; (iii) hypertriglyceridaemia; (iv) low level of high-density lipoprotein (HDL)-cholesterol; and (v) impaired fasting glucose (IFG) or type 2 diabetes mellitus (T2DM). All-cause mortality was defined as death owing to any aetiology.The median age was 61.4 years and 33 patients were men. Among 93 AAV patients with MetS, as the number of MetS components increased, the cumulative patient survival rate significantly decreased (p = 0.024). Compared to surviving AAV patients with MetS, deceased AAV patients with MetS were older, had higher Birmingham vasculitis activity score (BVAS) and Five-factor score (FFS), a lower frequency of IFG or T2DM, and a higher number of MetS components. In the multivariable Cox analysis, AAV patients with MetS who had all five MetS components were approximately 62 times more susceptible to all-cause mortality than those who had only three components. In terms of IFG or T2DM, patients with only IFG exhibited a significantly lower cumulative patients' survival rate than those without.The presence of many MetS components at the initial diagnosis of AAV was an independent and significant predictor of all-cause mortality in AAV patients with MetS.

Background This study investigated whether serum progranulin could act as a predictive marker for high disease activity of antineutrophil cytoplasmic antibody (ANCA)‐associated vasculitis (AAV). Methods Fifty‐eight AAV patients were included in this study. Clinical and laboratory data were obtained at blood collection. The Short‐Form 36‐Item Health Survey Physical and Mental Component Summaries (SF‐36 PCS and SF‐36 MCS), Birmingham Vasculitis activity score (BVAS), Five‐Factor Score (FFS), and Vasculitis Damage Index (VDI) were assessed as AAV‐specific indices. Whole blood was collected and serum samples were isolated and stored at −80°C. Serum progranulin concentration was quantified by ELISA kits. Results The median age of patients was 63.0 years (19 men). The median BVAS was 11.0, and the median serum progranulin level was 49.0 ng/ml. Serum progranulin was significantly correlated with BVAS, FFS, erythrocyte sedimentation rate, C‐reactive protein level, SF‐36 PCS, haemoglobin, and serum albumin. Severe AAV was arbitrarily defined as the highest tertile of BVAS (BVAS ≥16). When the cut‐offs of serum progranulin were set as 55.16 ng/ml and 43.01 ng/ml for severe AAV, AAV patients with serum progranulin ≥55.16 and 43.01 ng/ml had significantly higher risks of severe AAV than those without (relative risk (RR) 4.167 and 4.524, respectively). Conclusions Progranulin might play an anti‐inflammatory role in AAV pathogenesis and serum progranulin could be used as a predictive marker for high activity of AAV., Serum progranulin was significantly correlated with BVAS (r = 0.342) as well as acute‐phase reactants such as ESR (r = 0.379) in AAV patients. AAV, antineutrophil cytoplasmic antibody‐associated vasculitis; BVAS, Birmingham vasculitis activity score; CRP, C‐reactive protein; ESR, erythrocyte sedimentation rate.

We provided a predictable method that measures the risk of progression to end-stage renal disease (ESRD) in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) by using a few routine serum markers of kidney functions at diagnosis. In a retrospective cohort study, the medical records of 254 AAV patients were reviewed. We derived a novel equation for the prediction of the progression to ESRD using variables with a P-value < 0.1 in the multivariable Cox hazard model analysis. We assigned a weight to each variable according to the slopes like a coefficient of a linear equation. The median age of the AAV patients was 59 years and 42 AAV patients progressed to ESRD. In the multivariable Cox analysis using variables with significance in the univariable analysis, MPO-ANCA (or P-ANCA) positivity, blood urea nitrogen, serum creatinine, and serum albumin tended to be associated with the progression to ESRD (P-value < 0.1). We develop an Equation for predicting ESRD in AAV (EPEA) using those variables with the slope of each one. When the cut-off of EPEA was set as -0.094, AAV patients with EPEA ≥ -0.094 had a significantly higher risk of progression to ESRD than those with EPEA < -0.094 (RR, 39.622). AAV patients with EPEA ≥ -0.094 exhibited a significantly lower ESRD-free survival rate than those with EPEA < -0.094. We provided a method to obtain EPEA and demonstrated its predictive potential for ESRD in immunosuppressive drug-naive AAV patients. Key points • A novel equation for the prediction of the progression to ESRD was developed using variables with a P-value < 0.1 in the multivariable Cox hazard model analysis. A weight was assigned to each variable according to the slopes like a coefficient of a linear equation. • An optimal cut-off of EPEA for progression to ESRD was obtained using the receiver operator characteristic (ROC) curve analysis. • AAV patients with EPEA more than the cut-off had a significantly higher risk of progression to ESRD than those without (RR, 39.622). • AAV patients with EPEA more than the cut-off exhibited a significantly lower ESRD-free survival rate than those without.