Clinical features and prognosis of ANCA-associated vasculitis patients who were double-seropositive for myeloperoxidase-ANCA and proteinase 3-ANCA.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients with dual positivity for proteinase 3-ANCA (PR3-ANCA) and myeloperoxidase-ANCA (MPO-ANCA) are uncommon. We aimed to investigate these idiopathic double-positive AAV patients' clinical features, histological characteristics, and prognosis. We reviewed all the electronic medical records of patients diagnosed with AAV to obtain clinical data and renal histological information from January 2010 to December 2020 in a large center in China. Patients were assigned to the MPO-AAV group or PR3-AAV group or idiopathic double-positive AAV group by ANCA specificity. We explored features of idiopathic double-positive AAV. Of the 340 patients who fulfilled the study inclusion criteria, 159 (46.76%) were female, with a mean age of 58.41 years at the time of AAV diagnosis. Similar to MPO-AAV, idiopathic double-positive AAV patients were older and had more severe anemia, lower Birmingham Vasculitis Activity Score (BVAS) and C-reactive protein (CRP) levels, less ear, nose, and throat (ENT) involvement, higher initial serum creatinine and a lower estimated glomerular filtration rate (eGFR) when compared with PR3-AAV (P < 0.05). The proportion of normal glomeruli of idiopathic double-positive AAV was the lowest among the three groups (P < 0.05). The idiopathic double-positive AAV patients had the worst remission rate (58.8%) among the three groups (P < 0.05). The relapse rate of double-positive AAV (40.0%) was comparable with PR3-AAV (44.8%) (P > 0.05). Although there was a trend toward a higher relapse rate of idiopathic double-positive AAV (40.0%) compared with MPO-AAV (23.5%), this did not reach statistical significance (P > 0.05). The proportion of patients who progressed to ESRD was 47.1% and 44.4% in the idiopathic double-positive AAV group and MPO-AAV group respectively, without statistical significance. Long-term patient survival also varied among the three groups (P < 0.05). Idiopathic double-positive AAV is a rare clinical entity with hybrid features of MPO-AAV and PR3-AAV. MPO-AAV is the "dominant" phenotype in idiopathic double-positive AAV.

Development of a radiomics nomogram to predict the treatment resistance of Chinese MPO-AAV patients with lung involvement: a two-center study.

Background: Previous studies from our group and other investigators have shown that lung involvement is one of the independent predictors for treatment resistance in patients with myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (MPO-AAV). However, it is unclear which image features of lung involvement can predict the therapeutic response in MPO-AAV patients, which is vital in decision-making for these patients. Our aim was to develop and validate a radiomics nomogram to predict treatment resistance of Chinese MPO-AAV patients based on low-dose multiple slices computed tomography (MSCT) of the involved lung with cohorts from two centers. Methods: A total of 151 MPO-AAV patients with lung involvement (MPO-AAV-LI) from two centers were enrolled. Two different models (Model 1: radiomics signature; Model 2: radiomics nomogram) were built based on the clinical and MSCT data to predict the treatment resistance of MPO-AAV with lung involvement in training and test cohorts. The performance of the models was assessed using the area under the curve (AUC). The better model was further validated. A nomogram was constructed and evaluated by DCA and calibration curves, which further tested in all enrolled data and compared with the other model. Results: Model 2 had a higher predicting ability than Model 1 both in training (AUC: 0.948 vs. 0.824; p = 0.039) and test cohorts (AUC: 0.913 vs. 0.898; p = 0.043). As a better model, Model 2 obtained an excellent predictive performance (AUC: 0.929; 95% CI: 0.827-1.000) in the validation cohort. The DCA curve demonstrated that Model 2 was clinically feasible. The calibration curves of Model 2 closely aligned with the true treatment resistance rate in the training (p = 0.28) and test sets (p = 0.70). In addition, the predictive performance of Model 2 (AUC: 0.929; 95% CI: 0.875-0.964) was superior to Model 1 (AUC: 0.862; 95% CI: 0.796-0.913) and serum creatinine (AUC: 0.867; 95% CI: 0.802-0.917) in all patients (all p< 0.05). Conclusion: The radiomics nomogram (Model 2) is a useful, non-invasive tool for predicting the treatment resistance of MPO-AAV patients with lung involvement, which might aid in individualizing treatment decisions.

Sex disparities in clinicopathological features and outcomes of patients with myeloperoxidase-ANCA-associated vasculitis: a retrospective study of 366 cases in a single Chinese center.

There are a few studies that reported sex disparities in clinical features, pathological features and outcomes among ANCA-associated vasculitis (AAV) patients, but studies focusing on sex-specific differences of myeloperoxidase (MPO)-AAV patients are scarce. Therefore, the purpose of this study was to analyze sex differences in clinicopathological features and outcomes of MPO-AAV. Patients diagnosed with MPO-AAV in Xiangya Hospital from January 2010 to June 2021 were included in the study and separated into female and male groups. The differences in clinical manifestations, laboratory parameters, pathological features and prognosis between the two groups were retrospectively analyzed. Three hundred and sixty-six patients were included and divided into female group (n = 176) and male group (n = 190). The age of the male group was 62.41 ± 10.49 years, significantly higher than that of the female group (58.69 ± 16.39, p = 0.011). Compared with the female group, the male group had a shorter duration of disease, higher levels of hemoglobin, eosinophil count, proteinuria, serum C4, and lower levels of serum globulin, serum IgG and serum IgM (p < 0.05). No significant differences in kidney pathological features were observed between the two groups. During a median follow-up of 37.6 months, there was no significant difference in renal survival and patient survival between the two groups, but male patients had a worse composite outcome of renal and patient survival compared with the female patients (p = 0.044). This study found that male patients with MPO-AAV had a higher age of onset, shorter duration of disease, higher levels of hemoglobin, eosinophil count, proteinuria, serum C4, and lower levels of serum globulin, serum IgG and serum IgM. Male patients fared worse than female patients in terms of the composite outcome of renal and patient survival.

The effect of discontinuation of maintenance therapy in Chinese patients with MPO-ANCA associated vasculitis who were in long-term stable remission.

There is a consensus that maintenance therapy should be used to prevent relapse of myeloperoxidase-anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (MPO-AAV), but there is a debate about the optimal duration of maintenance therapy. Therefore, the purpose of this study was to determine whether discontinuation of maintenance therapy in MPO-AAV patients who were in long-term stable remission affects relapse, renal survival and patient survival. Seventy-nine patients with MPO-AAV diagnosed at Xiangya hospital from June 2010 to June 2019 who were in stable remission for at least 18 months following maintenance therapy were included. Patient records were retrospectively reviewed, and based on whether patients discontinued maintenance therapy 18 months after commencing maintenance therapy, patients were assigned into either the withdrawal group (n = 26) or maintenance group (n = 53). The endpoint was the percentage of relapse, relapse-free survival, renal survival and patient survival during follow-up. Ten relapses (38.5%) occurred in the withdrawal group (n = 26) and 8 relapses (15.1%) occurred in the maintenance group (n = 53) (p = 0.020). Compared to the withdrawal group, the maintenance group had similar relapse-free survival (log-rank test p = 0.099). But maintenance group had a better renal survival (p = 0.035), with no difference in patient survival or adverse events. This study suggests that discontinuing maintenance therapy at 18 months following induction of sustained remission leads to a significant increase in the percentage of relapse, and decreases renal survival in patients with MPO-AAV, but does not decrease relapse-free survival or patient survival.

Association of podocyte injury with clinical features and prognosis in patients with mesangial proliferative lupus nephritis.

OBJECTIVES: The aim of this study is to explore the association of podocyte injury with clinical features and outcomes in mesangial proliferative (Class II) lupus nephritis (LN). METHODS: We conducted a retrospective and clinicopathologic analysis with 576 LN patients with renal biopsy and screened 58 patients with Class II LN. Then, the 58 cases were divided into 4 groups based on the degree of podocyte damage and immune complex (IC) deposits on light microscope (histological and immunofluorescence) and electron microscope: Podocyte Injury Group, IC deposits Group, Podocyte Injury and IC Group, and Less-lesion Group. Clinical and pathologic information was collected from the patients' medical records at the time of the kidney biopsy and at follow-up. The data of demography, clinical parameters, therapy, remission, and relapse rates were analyzed and compared across groups. RESULTS: A significant difference was observed in the ages of patients among four Class II LN groups. The onset age of patients with FPE ≥ 50% was significant later. The frequency of thrombocytopenia was statistically different among the four groups and the patients with FPE ≥ 50% had lower frequency of thrombocytopenia. Patients with FPE ≥ 50% had lower serum albumin, eGFR, and elevated proteinuria and serum lipids. In this study, most patients received glucocorticoids in combination with immunosuppressants. Among the 4 groups, the use of ACEI/ARBs was highest in the podocyte injury group. There was a statistical difference in the renal relapse rates among the 4 Class II LN groups. Moreover, the recurrence rate was higher in the FPE ≥ 50% group. CONCLUSION: Our data identified Class II LN patients with podocyte injury (FPE ≥ 50%) present prominent renal damage and higher rate of renal relapse, suggesting more aggressive treatment and close follow-up for these patients.

Clinical features and prognosis of MPO-ANCA and anti-GBM double-seropositive patients.

Background: Several lines of evidence implicate that there are distinct differences between patients with myeloperoxidase (MPO)-antineutrophil cytoplasmic antibody (ANCA) and anti-glomerular basement membrane (GBM) antibody double-seropositive patients (DPPs) and single-positive patients. Hence, we conducted a retrospective study from a single center in China to analyze the clinical and pathological features, and prognosis of DPPs. Methods: 109 patients with MPO-ANCA-associated vasculitis (MPO-AAV), 20 DPPs and 23 patients diagnosed with anti-GBM disease from a large center in China were included in this study. The ratio of patients with renal biopsy in three groups were 100%, 50% and 100%, respectively. Their clinical and pathological characteristics, and outcomes were analyzed. The intensity of immune deposits in the kidney at diagnosis was detected by immunofluorescence (IF). Furthermore, multivariate Cox hazard model analysis was used to assess the clinical and histological predictors of end-stage renal disease (ESRD) and death for DPPs. Results: In our study, we found that patients in the DPPs group were older than the other two groups (p = 0.007, MPO-AAV vs. DPPs; p < 0.001, DPPs vs. anti-GBM). The DPPs group had a higher value of serum creatinine (p = 0.041) and lower estimated glomerular filtration rate (eGFR) (p = 0.032) compared with MPO-AAV patients. On the contrary, the DPPs group had a lower serum creatinine (p = 0.003) compared with patients with anti-GBM group. The proportion of patients with cardiac system involvement in the DPPs group was higher than anti-GBM patients (p = 0.014). Cellular crescents could be generally observed in renal biopsy of DPPs and patients with anti-GBM glomerulonephritis. In addition, Bowman's capsule rupture was more common in DPPs than MPO-AAV patients (p = 0.001). MPO-AAV had a better renal and overall survival outcome than DPPs (p < 0.001). There was no significant difference of renal and overall survival outcome between DPPs and patients with anti-GBM disease. The incidence of ESRD in DPPs was negatively associated with lymphocyte count (HR 0.153, 95% CI 0.027 to 0.872, p = 0.034) and eGFR (HR 0.847, 95% CI 0.726 to 0.989, p = 0.036). Elevated serum creatinine was confirmed as a risk factor of both renal (HR 1.003, 95% CI 1.000 to 1.005, p = 0.019) and patient survival in DPPs (HR1.461, 95% CI 1.050 to 2.033, p = 0.024). Conclusion: In summary, compared with anti-GBM disease, DPPs tended to involve multi-organ damage rather than limited to the kidney. It is highlighted that serologic DPPs have a worse renal and patient prognosis than MPO-AAV. Moreover, we found that the risk factors of renal survival of DPPs include low lymphocyte count, elevated serum creatinine and reduced eGFR, and serum creatinine can predict patient survival.

Case Report: An Unusual Presentation of Cardiovascular Involvement in Eosinophilic Granulomatosis With Polyangiitis.

Background: Because eosinophilic granulomatosis with polyangiitis (EGPA) is so rare and the symptoms so varied, it can be a challenge to get a correct diagnosis in clinical practice. Cardiovascular involvement is the main cause of death of EGPA. We are the first to report of cardiac magnetic resonance (CMR) findings about right-sided heart involvement in EGPA. Patient Findings: The initial abnormalities detected by CMR were Löffler endocarditis with extensive thrombosis and left ventricular (LV) dysfunction. After active treatment, LV systolic function recovered and endocarditis with thrombosis significantly improved, but there was rapidly progressive pulmonary hypertension, enlargement of right atrium and right ventricle and persistent right-sided heart failure. The patient eventually died of sudden cardiac death 6 months after hospital discharge. Conclusions: Löffler endocarditis and right-sided heart involvement are both rare presentations in patients with EGPA. CMR is a reliable non-invasive tool to precisely and comprehensively assess cardiovascular involvement in EGPA.

High levels of platelet-to-lymphocyte ratio may predict reduced risk of end stage of renal disease in Chinese patients with MPO-ANCA associated vasculitis. / 血小板/淋巴细胞比率比值升高可能预示中国MPO-ANCAç›¸å ³æ€§è¡€ç®¡ç‚Žæ‚£è€ è¿›å±•è‡³ç»ˆæœ«æœŸè‚¾ç— çš„é£Žé™©è¾ƒä½Ž.

OBJECTIVES: Platelet-to-lymphocyte ratio (PLR) has recently been investigated as a new inflammatory marker in many inflammatory diseases, including systemic lupus erythematosus and immunoglobulin A vasculitis. However, there were very few reports regarding the clinical role of PLR in patients with anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis. This study was thus undertaken to investigate the relationship between inflammatory response and disease activity in Chinese patients with myeloperoxidase-anti-neutrophil cytoplasmic antibody (MPO-ANCA) associated vasculitis. Furthermore, we evaluated whether PLR predicts the progression of end stage of renal disease (ESRD) and all-cause mortality. METHODS: The clinical, laboratory and pathological data, and the outcomes of MPO-ANCA associated vasculitis patients were collected. The Spearman correlation coefficient was computed to examine the association between 2 continuous variables. Cox regression analysis was used to estimate the association between PLR and ESRD or all-cause mortality. RESULTS: A total of 190 consecutive patients with MPO-ANCA associated vasculitis were included in this study. Baseline PLR was positively correlated with CRP (r=0.333, P<0.001) and ESR (r=0.218, P=0.003). PLR had no obvious correlation with Birmingham Vasculitis Activity Score (BVAS). Patients having PLR≥330 exhibited better cumulative renal survival rates than those having PLR<330 (P=0.017). However, there was no significant difference in the cumulative patient survival rates between patients with PLR≥330 and those with PLR<330 at diagnosis (P>0.05). In multivariate analysis, PLR is associated with the decreased risk of ESRD (P=0.038, HR=0.518, 95% CI 0.278 to 0.963). We did not find an association between PLR with all-cause mortality using multivariate analysis (HR=1.081, 95% CI 0.591 to 1.976, P=0.801). CONCLUSIONS: PLR is positively correlated with CRP and ESR. Furthermore, PLR may independently predict the risk of ESRD.

Clinical features and outcomes of anti-neutrophil cytoplasmic autoantibody-associated vasculitis in Chinese childhood-onset patients.

Data on anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) are limited in children. This study is to determine the clinical features and outcomes of childhood-onset AAV. A retrospective study was performed on patients who were diagnosed with AAV before 18 years old in Xiangya Hospital. Their medical records were analyzed by retrospective review. Sixteen patients were diagnosed with AAV before 18 years old in the past 9 years, with an average age of 13.3 ± 3.3 years and 13 of them were female. There were 15 patients with microscopic polyangiitis (MPA) and 1 with Wegener's granulomatosis. The interval between onset of disease and diagnosis of AAV was 2 (1.5-3) months. Most patients (15/16, 93.8%) had multi-organ involvement, and all patients had renal involvement with 7 (43.8%) patients requiring dialysis at presentation. Eleven patients underwent a renal biopsy, of which mixed class and sclerotic class were the most two common histological types. All patients received immunosuppressive therapy for induction therapy including intravenous administrations of methylprednisolone (MP) pulse therapy for 8 patients. 8 patients (50%) achieved remission after induction therapy. After a median follow-up of 46.3 ± 36.1 months, nine (56.3%) patients progressed to end-stage renal disease (ESRD) and 5 (31.3%) patients died. Childhood-onset AAV showed similar clinical and pathological features compared to those of adults, except that it usually occurs in girls. The most commonly involved organ was the kidney, and it had a high risk of progression to ESRD. Early diagnosis and initiation of appropriate immunomodulatory therapy would be important to improve outcomes.

Prevalence and risk factors of active tuberculosis in patients with rheumatic diseases: a multi-center, cross-sectional study in China.

Evidence of active tuberculosis (ATB) in patients with rheumatic diseases are research priorities but limited data from China have been reported. Research targeting patients not taking anti-TNF biologics are especially insufficient. We aimed to investigate the prevalence and risk factors of ATB in this at-risk population. We conducted a tertiary hospital-based, multi-center, cross-sectional study by using stratified multi-stage cluster sampling strategy to screen ATB in patients with rheumatic diseases. We estimated the prevalence of ATB in patients with rheumatic diseases and identified risk factors among those who were not taking anti-TNF biologic. A total of 13,550 eligible patients were enrolled, and the result showed the standardized prevalence of ATB according to the composition ratio of various types of rheumatic disease was 882/100000 (95% confidence interval (CI): 706-1057). Multivariable logistic regression analysis in patients not taking anti-TNF biologics showed that the independent risk factors of ATB were having systemic lupus erythematosus (SLE) (OR=2.722, 95% CI: 1.437-5.159, p=0.002), having Behcet's disease (BD) (OR= 5.261, 95% CI: 2.071-13.365, p<0.001), taking azathioprine(AZA) within the past two years (OR=2.095, 95% CI: 0.986-4.450, p=0.054), exposing to glucocorticoids ≥30mg/d for more than four weeks within the past two years (OR=2.031, 95% CI: 1.247-3.309, p=0.004) and having evidences of previous TB (OR= 6.185, 95% CI: 3.487-10.969, p<0.001). The prevalence of ATB was higher in patients with rheumatic diseases compared to the general population. Patients with SLE or BD, prolonged exposure to moderate to high dose of glucocorticoids and previous TB were independent risk factors for ATB.

Risk factors for serious infections in inpatients with systemic lupus erythematosus. / ç³»ç»Ÿæ€§çº¢æ–‘ç‹¼ç–®ä½é™¢æ‚£è€ åˆå¹¶ä¸¥é‡æ„ŸæŸ“çš„å±é™©å› ç´ .

OBJECTIVES: To investigate the risk factors for serious infections among hospitalized systemic lupus erythematosus (SLE) patients, and to provide the advice for preventing serious infections in SLE patients. METHODS: Information of SLE patients hospitalized from March 2017 to February 2019 at the Department of Rheumatology and Immunology, Xiangya Hospital, Central South University was obtained. The patients were assigned into a serious infection group and a non-serious infection group. The risk factors for serious infections among SLE inpatients were identified by comparison between the 2 groups and multivariate logistic regression analysis. RESULTS: There were 463 SLE inpatients in total, and 144 were in the serious infection group and 319 in the non-serious infection group. Multivariate logistic regression analysis showed that age ≥54.50 years old (OR=4.958, P<0.001), cardiovascular involvement (OR=6.287, P<0.001), hematologic involvement (OR=2.643, P=0.003), serum albumin <20 g/L (OR=2.340, P=0.036), C-reaction protein (CRP)/erythrocyte sedimentation rate (ESR)≥0.12 (OR=2.430, P=0.002), glucocorticoid dose ≥8.75 mg/d prednisone-equivalent (OR=2.465, P=0.002), and the combined use of immunosuppressive agents (OR=2.847, P=0.037) were the risk factors for serious infections in SLE inpatients. CONCLUSIONS: SLE patients with older age, cardiovascular involvement, hematologic involvement, low serum albumin are prone to suffering serious infections. Increased CRP/ESR ratio indicates serious infections in SLE inpatients. High-dose glucocorticoid and the combined use of immunosuppressive agents can increase the risk of serious infections in SLE inpatients.

Systemic immune-inflammation index predicts a reduced risk of end-stage renal disease in Chinese patients with myeloperoxidase-anti-neutrophil cytoplasmic antibody-associated vasculitis: A retrospective observational study.

Chronic inflammation has been indicated to be important in the pathogenesis of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). The systemic immune-inflammation index (SII) is a novel marker of inflammation. The present study was thus performed to explore the association between the SII at diagnosis and inflammatory response and disease activity in Chinese patients with myeloperoxidase (MPO)-AAV. Furthermore, it was evaluated whether the SII is able to predict the progression to end-stage renal disease (ESRD) and patient survival. A total of 190 patients with MPO-AAV were included in the present study. The baseline SII was positively correlated with C-reactive protein (CRP; r=0.274, P<0.0001) and the erythrocyte sedimentation rate (ESR; r=0.481, P<0.0001). However, the SII had no obvious correlation with the Birmingham vasculitis activity score. Patients with SII≥2,136.45 exhibited better cumulative renal survival rates than those with SII<2,136.45 (P=0.001). However, no significant difference in patient survival was indicated between patients with SII≥2,136.45 and those with SII<2,136.45 at diagnosis. In conclusion, the SII was positively correlated with CRP and ESR in Chinese patients with MPO-AAV. Furthermore, the SII may be an independent factor associated with a reduced risk of ESRD.

ECMO Rescues Patients With Acute Respiratory Failure Related to GPA.

Granulomatosis with polyangiitis (GPA) is a subtype of anti-neutrophil cytoplasmic antibody-associated vasculitis with a wide range of clinical symptoms related to the systemic involvement of small blood vessels. The respiratory system is one of the most frequently involved, and life-threatening acute respiratory failure could occur due to diffusive alveolar hemorrhage and tracheal stenosis. When maximum mechanical ventilation is unable to maintain oxygenation, extracorporeal membrane oxygenation (ECMO) should be considered as the final respiratory supportive method, if available. Here we present a 32-year-old male patient with acute respiratory failure (ARF) related to GPA, who was rescued by winning time for accurate diagnosis and appropriate treatment. Additionally, we reviewed more than 60 GPA-related ARF cases on multiple online databases, summarized the clinical manifestations of these patients, and concluded that ECMO plays an important role in further respiratory support for ARF patients with GPA and assists in accurate and timely diagnosis and appropriate treatment, thus helping them recuperate.

Glomerular Immune Deposition in MPO-ANCA Associated Glomerulonephritis Is Associated With Poor Renal Survival.

Background: Rapidly progressive glomerulonephritis caused by antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is typically characterized as pauci-immune glomerulonephritis. However, immune complex (IC) deposition in the glomerulus has been reported in a growing number of studies. Here, we assess the presence of glomerular immune deposits alongside renal outcome in myeloperoxidase (MPO)-ANCA associated glomerulonephritis (MPO-ANCA GN). Methods: Clinical and histopathologic characteristics of 97 patients with MPO-ANCA GN classified by renal biopsy from January 2008 to December 2019 were extracted retrospectively from electronic medical records. The extent of immune deposits in the kidney (C3, C4, C1q, IgA, IgG, IgM) at diagnosis were analyzed by immunofluorescence (IF). Patients were followed up for a median period of 15 months. The response to treatment and outcomes of renal and histological lesion changes were also assessed. Results: In our study, 41% (40/97) of patients showed positive IF (≥2+) for at least one of the six immunoglobulin or complement components tested. Patients with IC deposits showed higher levels of serum creatinine (p=0.025), lower platelet counts (p=0.009), lower serum complement C3 (sC3) (≤790 ml/L) (p=0.013) and serum IgG (p=0.018) than patients with pauci-immune (PI) deposition at diagnosis. End-stage renal disease was negatively associated with eGFR (HR 0.885, 95% CI 0.837 to 0.935, p<0.0001), platelet count (HR 0.996, 95% CI 0.992 to 1.000, p=0.046) and serum globulin (HR 0.905, 95% CI 0.854 to 0.959, p=0.001). Patients with lower sC3 levels showed a worse renal outcome than the patients with normal sC3 at diagnosis (p=0.003). Analysis of the components of the renal deposits found that patients with IgG deposits exhibited a poorer renal outcome compared to patients that were IgG negative (p=0.028). Moreover, Bowman's capsule rupture occurred less frequently in patients with IgM deposition compared with IgM negative counterparts (p=0.028). Vascular lesions and granuloma-like lesions had been seen more frequently in cases with IgA deposition than those without IgA deposition (p=0.03 and 0.015, respectively). Conclusion: In conclusion, patients with immune complex deposits in the kidney showed less platelet count, lower sC3 and sIgG levels, and higher serum creatinine levels. Patients with low sC3 at initial and with continued low sC3 during the treatment displayed a trend toward poorer kidney survival. Moreover, the IC group showed a worse renal outcome than the PI group, further enforcing the present strategy of introducing complement targeted therapies in AAV.

Differences between myeloperoxidase-antineutrophil cytoplasmic autoantibody (ANCA) and proteinase 3-ANCA associated vasculitis: A retrospective study from a single center in China.

In antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAV), the two major target antigens of ANCA are proteinase 3 (PR3) and myeloperoxidase (MPO). Evidence is accumulating that there are distinct differences between patients with PR3-AAV and those with MPO-AAV. In the present study, the clinicopathological features and prognosis of patients with PR3-AAV and MPO-AAV from a single center in China were retrospectively analyzed. A total of 212 Chinese patients with AAV were recruited in the present study; 189/212 (89.15%) patients were classified as having MPO-AAV and 23/212 (10.85%) patients as having PR3-AAV. Compared with those in the PR3-AAV group, patients in the MPO-AAV group were older and less frequently had ear, nose and throat or ophthalmic involvement. MPO-AAV patients had higher levels of serum creatinine and proteinuria at baseline. No significant difference was observed with regard to the pathological changes of the glomeruli and tubulointerstitium between the two groups. The probability of developing end-stage renal disease was significantly higher in patients with MPO-AAV compared with that in patients with PR3-AAV. There was no significant difference in the one-year patient survival rate between the two groups. However, differences in certain clinical characteristics and outcomes were observed between MPO-AAV and PR3-AAV patients. A large national investigation of AAV is required to confirm the concept that PR3-AAV and MPO-AAV are distinct disease entities.

Clinical features and outcomes of anti-neutrophil cytoplasmic autoantibody-associated vasculitis in Chinese elderly and very elderly patients.

PURPOSE: Anti-neutrophil cytoplasmic autoantibody-associated vasculitis (AAV) is predominantly a disease of the elderly, and the incidence increases with age. However, there are few data focusing on the clinical features in elderly onset AAV, especially in very elderly onset AAV in China. The aim of this study was to explore whether elderly onset AAV shows any specific clinical features and outcomes in Chinese patients. METHODS: We performed a retrospective study in Xiangya Hospital, a mixed tertiary medical center in south China. A total of 177 patients presenting with AAV were included between January 1, 2010 and December 31, 2017. Patients were divided into younger group (age < 65 years) and older group (age ≥ 65 years) which was sub-divided into elderly group (age 65-74 years) and very elderly group (age ≥ 75 years). And their medical records were analyzed by retrospective review. RESULTS: We found patients in the very elderly group had more chest and cardiovascular involvement (P = 0.033 and P = 0.017). Older AAV patients had less renal involvement and lower serum C4 level (P = 0.013 and P = 0.003). Very elderly AAV patients had lower platelet counts. Patients in the younger group had a higher level of BVAS among three groups (P < 0.05 younger group vs. very elderly group; P < 0.05 younger group vs. elderly group). There were no significant difference in the proportion of ESRD patients among the three groups (P = 0.473). Patients in the very elderly group had the poorest patient survival (P = 0.002). CONCLUSION: Older AAV patients had less renal involvement, lower serum C4 level and BVAS. The very elderly group got the most chest and cardiovascular involvement and had lower platelet counts. Older age is associated with higher mortality in AAV patients.

Clinical characteristics and prognosis in 269 patients with antineutrophil cytoplasimc antibody associated vasculitis. / 269ä¾‹æŠ—ä¸­æ€§ç²’ç»†èƒžèƒžæµ†æŠ—ä½“ç›¸å ³æ€§è¡€ç®¡ç‚Žæ‚£è€ çš„ä¸´åºŠç— ç†ç‰¹å¾åŠé¢„åŽ.

OBJECTIVES: To investigate the clinic-pathological characteristics, prognosis and its risk factors for antineutrophil cytoplasimc antibody (ANCA)-associated vasculitis (AAV). METHODS: The basic information and clinic-pathological characteristics of AAV patients, who was diagnosed from January 2010 to January 2018 in Xiangya Hospital, Central South University, were retrospectively collected. The renal survival and patient survival were regular followed up, and their clinical pathological, prognosis data and risk factors for renal were analyzed. RESULTS: Among 269 AAV patients, 225 patients (83.64%) were microscopic polyangiitis (MPA), 33 patients (12.27%) were granulomatosis with polyangiitis (GPA), and 11 patients (4.09%) were eosinophilic granulomatosis with polyangiitis (EGPA). Male-to-female ratio was almost 1∶1 for MPA (147/122), but GPA and EGPA was more frequent among man. The medium time from disease onset to diagnosis was 64.0 (32.5, 148.5) days, 65 patients (24.16%) were diagnosed within 30 days. A total of 94.67% patients had kidney involvement in MPA patients, which was significantly higher than that in patients with GPA (63.63%), but ear, nose, and throat manifestations were more frequent in GPA patients. The 1-year kidney survival rates of GPA and MPA patients in this study were 75% and 59.3%, respectively. The 1-year death rates were 16.7% and 16.9%, respectively. The multivariable logistic regression analysis revealed that the low platelet level, low globulin level, low immunoglobin G, and high serum creatinine level were independent risk factors for the 1-year renal survival rate in AAV patients. Multiple factor logistic regression analysis showed that the serum creatinine level was an independent risk factor for all-cause mortality. CONCLUSIONS: MPA is the priority in the AAV patients. The proportion of patients with advanced end-stage renal disease and mortality is still high, and the early diagnosis and treatment in time is crucial for the improvement of prognosis for AAV.

The association of neutrophil-to-lymphocyte ratio with all-cause mortality in Chinese patients with MPO-ANCA associated vasculitis.

Neutrophil-to-lymphocyte ratio (NLR) has been recently reported to be a promising inflammatory marker to assess systemic inflammation in many disorders. However, there are only a few studies looking at NLR in patients with myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). This study was thus undertaken to explore the relationship between NLR at diagnosis with inflammatory response and disease activity among MPO-AAV patients in a single Chinese center. Furthermore, we evaluated whether NLR could predict the renal prognosis and patient outcome. 188 patients with MPO-AAV were included in this study. Baseline NLR was positively correlated with CRP (r = 0.404, P < 0.001) and negatively with serum levels of C3 (r = - 0.163, P = 0.035), but it had no obvious correlation with Birmingham Vasculitis Activity Score (BVAS). Patients with MPO-AAV having NLR ≥ 9.53 exhibited higher risk for all-cause mortality than those having NLR < 9.53 (P < 0.0001). However, no significant difference was found in the kidney survival between patients having NLR ≥ 9.53 and those NLR < 9.53 at diagnosis. In multivariate analysis, NLR was positively associated with all-cause mortality (P = 0.037, HR = 1.98, 95% CI 1.04-3.78). There was no association between NLR with ESRD observed using univariate analysis or multivariate analysis. This large retrospective study of MPO-AAV patients in a single Chinese center demonstrates that NLR positively correlates with CRP and negatively correlates with serum levels of C3 in Chinese patients with MPO-AAV. Importantly, higher NLR predicts increased mortality and is, therefore, a useful independent prognostic in MPO-AAV.

Risk factors for treatment resistance and relapse of Chinese patients with MPO-ANCA-associated vasculitis.

Identification of risk factors for treatment resistance and relapse would be crucial to personalization therapy in patients with myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (MPO-AAV). Current evidence with regard to the risk factors for treatment resistance and relapse remains limited and inconclusive. We aimed to assess the predictors for treatment resistance and relapse in a single-center cohort of Chinese patients with MPO-AAV in this study. In total, 184 patients with MPO-AAV were included. Treatment resistance occurred in 64 (34.9%) of 184 patients and was positively associated with lung involvement (odds ratio [OR] 3.581, 95% CI 1.137-11.278, p = 0.029) and the initial serum creatinine level (OR 1.004, 95% CI 1.001-1.007, p = 0.010) and was negatively associated with platelet (OR 0.992, 95% CI 0.987-0.998, p = 0.007) and serum C3 levels (OR 0.998, 95% CI 0.996-0.999, p = 0.004). Relapse occurred in 29 (24.17%) of 120 patients in whom remission was achieved and was independently associated with lung involvement (hazard ratio [HR] 4.595, 95% CI 1.272-16.599, p = 0.020) and cardiovascular involvement (HR 3.689, 95% CI 1.237-11, p = 0.019,). The serum globulin was demonstrated to be negatively associated with relapse independently (HR 0.876; 95% CI 0.806-0.953; p = 0.002). This retrospective study of MPO-AAV patients in a single Chinese center suggests that treatment resistance was positively associated with lung involvement and the initial serum creatinine level and was negatively associated with platelet and serum C3 levels. Lung involvement and cardiovascular involvement were associated with an increased risk of relapse, while the higher serum globulin was demonstrated to be in association with a decreased risk of relapse.

[Clinical characteristics for connective tissue disease complicated with cryptococcal meningitis].

OBJECTIVE: To investigate the clinical characteristics and prognosis for connective tissue disease (CTD) with cryptococcal meningitis (CM). 
 Methods: Clinical data of 18 patients with CTD complicated with cryptococcal meningitis diagnosed by Rheumatology and Immunology Department, Xiangya Hospital, Central South University from January 2000 to January 2017, were retrospectively analyzed.
 Results: The common symptoms of CTD patients with CM were headache, fever, nausea, and vomiting. Patients with severe clinical manifestations, such as convulsions and disturbance of consciousness, all died. Logistic regression analysis showed that disturbance of consciousness and decreased peripheral blood lymphocyte count might be the related factors of poor prognosis of CTD patients with CM (P<0.05). The mortality rate of CTD with CM was 61.11%, and the effective rate of treatment for this disease was 38.89%.
 Conclusion: CTD patients with cryptococcal meningitis have a high risk of death. Severe clinical symptoms, such as disturbance of consciousness and lower peripheral blood lymphocyte count, are associated with its poor prognosis.