scholarly journals Clinical implication of plasma exchange on life-threatening antineutrophil cytoplasmic antibody-associated vasculitis

2020 ◽  
Author(s):  
Pil Gyu Park ◽  
Byung-Woo Yoo ◽  
Jason Jungsik Song ◽  
Yong-Beom Park ◽  
Sang-Won Lee

Abstract Background: We assessed the rate of and the predictor for all-cause mortality in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients receiving plasma exchange (PLEX) and evaluated the survival-benefit of PLEX for diffuse alveolar haemorrhage (DAH) between AAV patients receiving PLEX and those not receiving. Methods: We retrospectively reviewed the medical records of 212 AAV patients. Demographic, clinical and laboratory data at the time of PLEX was collected from both 9 patients receiving PLEX and 10 AAV patients with DAH. The follow-up duration was defined as the period from the time of PLEX or DAH occurrence to death for the deceased patients and as that to the last visit for the survived patients. Results: The median age of 9 AAV patients receiving PLEX was 71.0 years and 5 patients were men. Four of 9 patients receiving PLEX died at a median follow-up duration of 92.0 days. Three died of sepsis and one died of no response to PLEX. When patients with DAH receiving PLEX and those not receiving were compared, there were no significant differences in variables between the two groups. The cumulative patients’ survival rate between patients with DAH receiving PLEX and those not receiving were also compared using the Kaplan-Meier survival analysis but no survival-benefit of PLEX for DAH was observed. Conclusion: The rate of all-cause mortality in 9 AAV patients receiving PLEX was assessed as 44.4% and it was controversial that PLEX is beneficial for the improvement of prognosis of AAV-related DAH.

2020 ◽  
Author(s):  
Pil Gyu Park ◽  
Byung-Woo Yoo ◽  
Jason Jungsik Song ◽  
Yong-Beom Park ◽  
Sang-Won Lee

Abstract Background We assessed the rate of and the predictor for all-cause mortality in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients receiving plasma exchange (PLEX) and evaluated the survival-benefit of PLEX for diffuse alveolar haemorrhage (DAH) between AAV patients receiving PLEX and those not receiving. Methods We retrospectively reviewed the medical records of 212 AAV patients. Demographic, clinical and laboratory data at the time of PLEX was collected from both 9 patients receiving PLEX and 10 AAV patients with DAH. The follow-up duration was defined as the period from the time of PLEX or DAH occurrence to death for the deceased patients and as that to the last visit for the survived patients. Results The median age of 9 AAV patients receiving PLEX was 71.0 years and 5 patients were men. Four of 9 patients receiving PLEX died at a median follow-up duration of 92.0 days. Three died of sepsis and one died of no response to PLEX. When patients with DAH receiving PLEX and those not receiving were compared, there were no significant differences in variables between the two groups. The cumulative patients’ survival rate between patients with DAH receiving PLEX and those not receiving were also compared using the Kaplan-Meier survival analysis but no survival-benefit of PLEX for DAH was observed. Conclusion The rate of all-cause mortality in 9 AAV patients receiving PLEX was assessed as 44.4% and it was controversial that PLEX is beneficial for the improvement of prognosis of AAV-related DAH.


Author(s):  
Andreas Kronbichler ◽  
Jae Il Shin ◽  
Chia-Shi Wang ◽  
Wladimir M Szpirt ◽  
Mårten Segelmark ◽  
...  

Abstract Plasma exchange (PLEX) is capable of removing significant amounts of circulating antibodies. In anti-neutrophil cytoplasmic antibody-associated vasculitis, PLEX was reserved for patients with severe presentation forms such as rapidly progressive glomerulonephritis and pulmonary haemorrhage. The Plasma Exchange and Glucocorticoids in Severe ANCA-Associated Vasculitis (PEXIVAS) trial included all comers with a glomerular filtration rate <50 mL/min/1.73 m2 and thus aimed to answer the question of whether PLEX is an option for patients with no relevant kidney function impairment or not. PEXIVAS revealed that after a follow-up of almost 3 years, routine administration of PLEX does not provide an additional benefit to reduce the rate of a composite comprising end-stage kidney disease or death. In the absence of histological parameters, it is tempting to speculate whether PLEX is effective or not in those with a potential for renal recovery. A subset of patients presented with alveolar haemorrhage, and there was a trend towards a better outcome of such cases receiving PLEX. This would be in line with observational studies reporting a recovery of alveolar haemorrhage following extracorporeal treatment. In this PRO part of the debate, we highlight the shortcomings of the PEXIVAS trial and stimulate further research paths, which in our eyes are necessary before abandoning PLEX from the therapeutic armamentarium.


2020 ◽  
Author(s):  
Soo Bin [email protected] ◽  
Hyeok Chan Kwon ◽  
Jung Yoon Pyo ◽  
Mi Il Kang ◽  
Jason Jungsik Song ◽  
...  

Abstract Objective: We investigated the prevalence of metabolic syndrome (MetS) in all or nonobese patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and compared it with age- and gender-matched controls. Also, we assessed the effect of variables at diagnosis on the risk of cardiovascular disease (CVD) in all or nonobese AAV patients. Methods: In this study, 173 AAV patients and 344 controls were included and MetS was defined by the National Cholesterol Education Program Adults Treatment Panel III criteria. The obesity based on BMI was defined as BMI ≥ 25 kg/m 2 . The follow-up duration was defined as the period from diagnosis to the last visit or to each poor outcome occurrence. Results: The median age of AAV patients was 58.7 years and 57 patients were men. The prevalence of MetS was 50.9% in all AAV patients and 46.5% in nonobese AAV patients, which were significantly higher than 37.8% in all controls and 28.2% in nonobese controls. In the Kaplan-Meier survival analysis, Mets at diagnosis significantly reduced the cumulative CVD-free survival rate in both all and nonobese AAV patients. In the multivariable Cox hazards model analysis, CVD during follow-up was significantly associated with both BVAS (HR 1.159) and MetS at diagnosis (HR 9.036) in nonobese AAV patients. Conclusions: The prevalence of MetS at diagnosis in all or nonobese AAV patients was significantly higher than those in all or nonobese controls. Furthermore, both BVAS and MetS at diagnosis increased the risk of CVD in nonobese AAV patients.


2021 ◽  
Author(s):  
Soo Bin Lee ◽  
Hyeok Chan Kwon ◽  
Jung Yoon Pyo ◽  
Mi Il Kang ◽  
Jason Jungsik Song ◽  
...  

Abstract Objective: We investigated the prevalence of metabolic syndrome (MetS) in all or nonobese patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and compared it with age- and gender-matched controls. Also, we assessed the effect of variables at diagnosis on the risk of cardiovascular disease (CVD) in all or nonobese AAV patients. Methods: In this study, 173 AAV patients and 344 controls were included and MetS was defined by the National Cholesterol Education Program Adults Treatment Panel III criteria. The obesity based on body mass index (BMI) was defined as BMI ≥ 25 kg/m2. The follow-up duration was defined as the period from diagnosis to the last visit or to each poor outcome occurrence.Results: The median age of AAV patients was 58.7 years and 57 patients were men. The prevalence of MetS was 50.9% in all AAV patients and 46.5% in nonobese AAV patients, which were significantly higher than 37.8% in all controls and 28.2% in nonobese controls. In the Kaplan-Meier survival analysis, Mets at diagnosis significantly reduced the cumulative CVD-free survival rate in both all and nonobese AAV patients. In the multivariable Cox hazards model analysis, CVD during follow-up was significantly associated with both BVAS (HR 1.159) and MetS at diagnosis (HR 9.036) in nonobese AAV patients.Conclusions: The prevalence of MetS at diagnosis in all or nonobese AAV patients was significantly higher than those in all or nonobese controls. Furthermore, both BVAS and MetS at diagnosis increased the risk of CVD in nonobese AAV patients.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1563.3-1563
Author(s):  
H. Tamaki ◽  
S. Fukui ◽  
T. Nakai ◽  
G. Kidoguchi ◽  
S. Kawaai ◽  
...  

Background:Currently it is hypothesized that many systemic autoimmune diseases occur due to environmental risk factors in addition to genetic risk factors. Anti-Neutrophil Cytoplasmic Antibody (ANCA) is mainly associated with three systemic autoimmune disease including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA). It is known that ANCA can be positive before clinical symptoms in patients with known diagnosis of GPA and ANCA titers rise before clinical manifestations appear. However, prevalence of ANCA among general population is not well known. It has not been described as well how many of people with positive ANCA eventually develop clinical manifestations of ANCA associated Vasculitis.Objectives:This study aims to estimate prevalence of ANCA in general population without ANCA associated Vasculitis. It also describes natural disease course of people with positive ANCA without ANCA associated Vasculitis. Risk factors for positive ANCA are also analyzed.Methods:This is a single center retrospective study at Center for Preventive Medicine of St. Luke’s International Hospital in Tokyo. ANCA was checked among the patients who wished to between 2018 and 2019. St. Luke’s Health Check-up Database (SLHCD) was utilized to collect the data. The patients whose serum was measured for ANCA were identified. The data for basic demographics, social habits, dietary habits and laboratory data were extracted. The charts of the patients with positive ANCA were reviewed.Results:Sera of total 1204 people were checked for ANCA. Of these 1204 people, 587 (48.8%) are male and the mean age was 55.8 years (32.6 to 79). There were total 11 patients with positive ANCA. Myeloperoxidase ANCA (MPO-ANCA) was positive for 3 patients and proteinase 3 ANCA (PR3-ANCA) was positive for 8 patients. Of these 11 patients, 5 were male (45.5%) and the mean age was 54.6 years. Two patients had history of autoimmune disease (primary biliary cirrhosis and ulcerative colitis). Five patients were evaluated by rheumatologists with the median follow-up period of 274 days. None of them developed clinical signs and symptoms of ANCA associated Vasculitis. Four out of five patients had ANCA checked later, two of which turned negative. The prevalence of ANCA in this cohort was 0.9% (95% confidence interval [95% CI]: 0.5% to 1.6%). Univariate analysis was performed to identify risk factors of positive ANCA. The variables analyzed include age, gender, body mass index (BMI), smoking habits, alcohol intake, dietary habits (fruits, fish, red meat), hypertension, dyslipidemia, and laboratory data. None of these variables demonstrated statistically significant differences except for positive rheumatoid factor (ANCA positive group: 33 % vs ANCA negative group: 9.1%, p value = 0.044).Conclusion:The prevalence of ANCA in this cohort was 0.9% (95% CI: 0.5% to 1.6%). None of them who had a follow-up developed ANCA associated Vasculitis during the follow-up period. Longer follow-up and more patients are necessary to determine natural course of people with positive ANCA.Disclosure of Interests:None declared


Author(s):  
Krishna Prasad ◽  
Pruthvi C Revaiah ◽  
Krishna Santosh Vemuri ◽  
Parag Barwad

Abstract Background Antineutrophil cytoplasmic antibody (ANCA)-associated pulmonary renal vasculitis is an uncommon disease entity. Its presentation as acute heart failure for the first time in a patient with established coronary artery disease (CAD) is even rarer. We present here a case of such an association and an approach to managing this clinical situation. Case summary A 60-year-old male patient presented to the emergency room with recent-onset dyspnoea New York Heart Association Class IV. He was having hypertension, uncontrolled diabetes mellitus, chronic kidney disease (CKD), and CAD. He also underwent a percutaneous coronary intervention to left anterior descending in the past for acute coronary syndrome and had moderate left ventricular dysfunction. He was being managed as a case of acute decompensated heart failure (ADHF) and was mechanically ventilated. Suddenly his ventilator requirement increased and endotracheal aspirate contained blood. The chest radiograph showed bilateral hilar infiltrates. Simultaneously he also had recurrent episodes of ventricular tachycardia (VT) requiring direct current (DC) cardioversion. Blood investigations showed deranged renal function and severe hyperkalaemia, but no evidence of coagulopathy. High-resolution computed tomography chest showed features of diffuse alveolar haemorrhage. Further investigations revealed high titres of c-ANCA and raised inflammatory biomarkers. A diagnosis of ANCA-associated vasculitis presenting as acute on CKD with dyselectrolytaemia (hyperkalaemia) leading to VT was made. Apart from standard management for associated illness, he was treated with plasma exchange, steroids, and cyclophosphamide to which he responded and was later on discharged. Discussion Antineutrophil cytoplasmic antibody-related pulmonary renal vasculitis can lead to rapidly progressing renal failure and may present as ADHF in a patient with existent CAD. The associated VT storm in our patient can be attributed to hyperkalaemia secondary to acute renal failure. A multidisciplinary approach is required for the successful management of such a complex clinical scenario.


2021 ◽  
Author(s):  
Susanne Bauer ◽  
Christina Strack ◽  
Ekrem Ücer ◽  
Stefan Wallner ◽  
Ute Hubauer ◽  
...  

Aim: We assessed the 10-year prognostic role of 11 biomarkers with different pathophysiological backgrounds. Materials & methods/results: Blood samples from 144 patients with heart failure were analyzed. After 10 years of follow-up (median follow-up was 104 months), data regarding all-cause mortality were acquired. Regarding Kaplan–Meier analysis, all markers, except TIMP-1 and GDF-15, were significant predictors for all-cause mortality. We created a multimarker model with nt-proBNP, hsTnT and IGF-BP7 and found that patients in whom all three markers were elevated had a significantly worse long-time-prognosis than patients without elevated markers. Conclusion: In a 10-year follow-up, a combination of three biomarkers (NT-proBNP, hs-TnT, IGF-BP7) identified patients with a high risk of mortality.


2020 ◽  
Author(s):  
Fifonsi Adjidossi GBEASOR-KOMLANVI ◽  
Martin Kouame TCHANKONI ◽  
Akila Wimima BAKOUBAYI ◽  
Matthieu Yaovi LOKOSSOU ◽  
Arnold SADIO ◽  
...  

Abstract Background: Assessing hospital mortality and its predictors is important as some of these can be prevented through appropriate interventions. Few studies have reported hospital mortality data among older adults in sub-Saharan Africa. The objective of this study was to assess the mortality and associated factors among hospitalized older adults in Togo.Methods: We conducted a prospective cohort study from February 2018 to September 2019 among patients ≥50 years admitted in medical and surgical services of six hospitals in Togo. Data were recorded during hospitalization and through telephone follow-up survey within 90 days after admission. The main outcome was all-cause mortality at 3 months. Survival curves were estimated using the Kaplan-Meier method and Cox regression analyses were performed to assess predictors of mortality.Results: The median age of the 650 older adults included in the study period was 61 years, IQR: [55-70] and at least one comorbidity was identified in 59.7% of them. The all-cause mortality rate of 17.2% (95%CI: 14.4-20.4) and the majority of death (93.7%) occurred in hospital. Overall survival rate was 85.5% and 82.8% after 30 and 90 days of follow-up, respectively. Factors associated with 3-month mortality were the hospital level in the health pyramid, hospitalization service, length of stay, functional impairment, depression and malignant diseases.Conclusion: Togolese health system needs to adjust its response to an aging population in order to provide the most effective care.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
I Warchol ◽  
A Lubinski ◽  
M Sterlinski ◽  
O Kowalski ◽  
K Goscinska-Bis ◽  
...  

Abstract Background In the Polish ICD Registry population secondary prevention recipients account for over 27%. Despite the evolution of indications for secondary prevention implantable cardioverter defibrillators (ICDs), recommendations regarding the use of ICDs for secondary prevention of sudden cardiac death (SCD) rely on information from a small number of randomized controlled trials that were performed decades ago, with mixed results. Moreover, research on the outcomes after implantations for secondary prevention of ICDs is limited. While dual-chamber devices offer theoretical advantage over single-chamber devices, dual-chamber ICDs (DC-ICDs) were announced not superior to single-chamber (SC-ICDs) in some research. Purpose Therefore, the aim of the study was to evaluate the all-cause mortality among patients from the Polish ICD Registry receiving either a single- or a dual-chamber device for secondary prevention in contemporary clinical practice. Methods All patients enrolled in the Polish ICD Registry from 1995 to 2016 were identified. Patients were included in the study if they were designated as receiving an ICD for secondary prevention of SCD after documented tachycardic arrest, sustained ventricular tachycardia (VT), or syncope. Kaplan-Meier survival analysis was used to assess all-cause mortality. Results In the study population of 3596 ICD recipients (mean age 69±12 years, 81% male, SC-ICD 61%, DC-ICD 39%), during mean follow-up of 79±43 months all-cause mortality rate was higher in the dual-chamber group than in the single chamber group, with a significant difference between the two groups as depicted in Kaplan-Meier curve (p<0,05). The median survival time was 98 months versus 110 months for SC and DC-ICD, respectively. Conclusions This study is the first to describe the characteristics of a national cohort of patients receiving a secondary prevention ICD in such a long follow-up period in contemporary practice. Implantation of a dual-chamber ICD was associated with higher all-cause mortality compared with single chamber devices.


Author(s):  
Abdonas Tamosiunas ◽  
Laura Sapranaviciute-Zabazlajeva ◽  
Dalia Luksiene ◽  
Dalia Virviciute ◽  
Martin Bobak

Background: The purpose of the study is to evaluate the association between cognitive function and risk of all-cause and cardiovascular disease mortality during 10 years of the follow-up. Methods: 7087 participants were assessed in the baseline survey of the Health Alcohol Psychosocial Factors in Eastern Europe (HAPIEE) study in 2006–2008. During 10 years of follow-up, all-cause and CVD mortality risk were evaluated. Results: During 10 years of follow-up, 768 (23%) men and 403 (11%) women died (239 and 107 from CVD). After adjustment for sociodemographic, biological, lifestyle factors, and illnesses, a decrease per 1 standard deviation in different cognitive function scores increased risk for all-cause mortality (by 13%–24% in men, and 17%–33% in women) and CVD mortality (by 19%–32% in men, and 69%–91% in women). Kaplan-Meier survival curves for all-cause and CVD mortality, according to tertiles of cognitive function, revealed that the lowest cognitive function (1st tertile) predicts shorter survival compared to second and third tertiles (p < 0.001). Conclusions: The findings of this follow-up study suggest that older participants with lower cognitive functions have an increased risk for all-cause and CVD mortality compared to older participants with a higher level of cognitive function.


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