scholarly journals Increase of Antimyeloperoxidase Antineutrophil Cytoplasmic Antibody (ANCA) in Patients with Renal ANCA-associated Vasculitis: Association with Risk to Relapse

2015 ◽  
Vol 42 (10) ◽  
pp. 1853-1860 ◽  
Author(s):  
Makoto Yamaguchi ◽  
Masahiko Ando ◽  
Sawako Kato ◽  
Takayuki Katsuno ◽  
Noritoshi Kato ◽  
...  
Keyword(s):  
Regression Models ◽  
Cox Regression ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Time Dependent ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Anca Associated Vasculitis ◽  
Level Increase ◽  
Diagnostic Values ◽  
Renal Vasculitis ◽  
Risk Of Disease

Objective.The diagnostic values of antiproteinase 3 and antimyeloperoxidase tests using antineutrophil cytoplasmic antibodies (ANCA) are well established. Our study determined whether an increase in ANCA level was a predictor of disease flareup.Methods.Our study included 126 patients with ANCA-associated renal vasculitis treated at 9 nephrology centers in Japan. The relationship between increased ANCA levels and relapse was assessed using time-dependent multivariate Cox regression models adjusted for clinically relevant factors. The outcome of interest was the time from remission to first relapse.Results.During the observation period [median 41 mos, interquartile range (IQR) 23–66 mos], 118 patients (95.8%) achieved remission at least once. After achieving remission, 34 patients relapsed (21.7%). Time-dependent multivariate Cox regression models revealed that lung involvement (adjusted HR 2.29, 95% CI 1.13–4.65, p = 0.022) and increased ANCA levels (adjusted HR 17.4, 95% CI 8.42–36.0, p < 0.001) were significantly associated with relapse. The median time from ANCA level increase to relapse was 0.6 months (IQR 0–2.1 mos).Conclusion.In our study, an increase in ANCA level during remission was associated with a risk of disease relapse. A rise in ANCA level may be useful for guiding treatment decisions in appropriate subsets of patients with ANCA-associated vasculitis.

scholarly journals Proteinase 3 (PR3)-antineutrophil cytoplasmic antibody (ANCA)-associated vasculitic neuropathy in diffuse cutaneous systemic sclerosis: a rare duo

2019 ◽  
Vol 12 (11) ◽  
pp. e232987 ◽  
Author(s):  
Yasser Radwan ◽  
Sarah Berini ◽  
Floranne Ernste ◽  
Ashima Makol
Keyword(s):  
Systemic Sclerosis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Proteinase 3 ◽  
Vasculitic Neuropathy ◽  
Anca Associated Vasculitis ◽  
Common Clinical Presentation ◽  
Risk Of Disease ◽  
Diffuse Cutaneous Systemic Sclerosis

Systemic sclerosis (SSc) is characterised by non-inflammatory vasculopathy, autoimmunity and widespread fibrosis. While the presence of antineutrophil cytoplasmic antibodies (ANCAs) has been reported in SSc, their association with ANCA-associated vasculitis is exceedingly rare. Myeloperoxidase ANCA is more common than proteinase-3 ANCA, and glomerulonephritis is the most common clinical presentation of ANCA-associated vasculitis in SSc. ANCAs have been associated with the adverse disease outcomes in SSc, including higher mortality per recent reports. A 65-year-old man with diffuse cutaneous SSc for 6 years presented with new-onset peripheral neuropathy. Workup revealed a positive proteinase-3 and cytoplasmic ANCA, and histopathology confirmed an inflammatory vasculitic neuropathy. The patient was successfully treated with rituximab. Our case highlights the importance of checking ANCA in SSc at baseline, given the risk of disease-related complications, even years later. Tissue biopsy is often warranted for confirmation of vasculitis and prompt treatment can optimise long-term outcomes.

scholarly journals An unusual precipitant of acute heart failure—ANCA-associated vasculitis in a patient with ischaemic cardiomyopathy: a case report

2020 ◽  
Author(s):  
Krishna Prasad ◽  
Pruthvi C Revaiah ◽  
Krishna Santosh Vemuri ◽  
Parag Barwad
Keyword(s):  
Heart Failure ◽  
Renal Failure ◽  
Acute Heart Failure ◽  
Acute Decompensated Heart Failure ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Left Ventricular ◽  
Alveolar Haemorrhage ◽  
Coronary Syndrome ◽  
Anca Associated Vasculitis ◽  
Renal Vasculitis

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.

scholarly journals Antineutrophil Cytoplasmic Antibody–associated Glomerulonephritis Complicated by Pneumatosis Intestinalis

2015 ◽  
Vol 8 ◽  
pp. CCRep.S26155 ◽  
Author(s):  
Saki Nakagawa ◽  
Tetsu Akimoto ◽  
Shin-ichi Takeda ◽  
Mari Okada ◽  
Atsushi Miki ◽  
...  
Keyword(s):  
Autoimmune Diseases ◽  
Bowel Wall ◽  
Pneumatosis Intestinalis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Current Case ◽  
Anca Associated Vasculitis ◽  
Characteristic Imaging

Pneumatosis intestinalis is a characteristic imaging phenomenon indicating the presence of gas in the bowel wall. The link between pneumatosis intestinalis and various kinds of autoimmune diseases has been reported anecdotally, while information regarding the cases with antineutrophil cytoplasmic antibodies (ANCA)–associated vasculitis complicated by concurrent pneumatosis intestinalis is lacking. In this report, we describe our serendipitous experience with one such case of pneumatosis intestinalis in a patient with ANCA-associated glomerulonephritis. We also discuss several therapeutic concerns that arose in the current case, which had an impact on the pathogenesis of the disease.

Severe Acute Kidney Injury and Mortality in Extremely Low Gestational Age Neonates

2021 ◽  
Vol 16 (6) ◽  
pp. 862-869
Author(s):  
Sangeeta Hingorani ◽  
Robert H. Schmicker ◽  
Patrick D. Brophy ◽  
Patrick J. Heagerty ◽  
Sandra E. Juul ◽  
...  
Keyword(s):  
Confidence Interval ◽  
Gestational Age ◽  
Regression Models ◽  
Cox Regression ◽  
Controlled Trial ◽  
Hazard Ratio ◽  
Time Dependent ◽  
Recombinant Erythropoietin ◽  
The Relationship ◽  
Extremely Low Gestational Age

Background and objectivesAKI is associated with poor short- and long-term outcomes. Questions remain about the frequency and timing of AKI, and whether AKI is a cause of death in extremely low gestational age neonates.Design, setting, participants, & measurementsThe Recombinant Erythropoietin for Protection of Infant Kidney Disease Study examines the kidney outcomes of extremely low gestational age neonates enrolled in the Preterm Epo Neuroprotection study, a randomized, placebo-controlled trial of recombinant human erythropoietin. We included 900 of 941 patients enrolled in Preterm Epo Neuroprotection. Baseline characteristics were compared by primary exposure (severe AKI versus none/stage 1 AKI) using unadjusted logistic regression models. Cox regression models estimated the relationship between severe AKI and death after adjustment for potential confounders. Time-dependent AKI was modeled as a binary outcome and a categorical variable by stage of AKI. We fit Cox models using time-dependent AKI status lagged by <7 days before death. Landmark analyses examined the relationship of death with development of severe AKI.ResultsSevere AKI occurred in 168 of 900 (19%, 95% confidence interval, 17% to 20%) neonates, and stage 3 AKI occurred in 60 (7%, 95% confidence interval, 5% to 8%). Stage 3 AKI occurring 7 days before death (hazard ratio, 3.88; 95% confidence interval, 1.26 to 11.96), intraventricular hemorrhage (hazard ratio, 2.01; 95% confidence interval, 1.01 to 3.99) and sepsis (hazard ratio, 2.85; 95% confidence interval, 1.12 to 7.22) were all independently associated with death. Severe AKI occurring 7 days before death (hazard ratio, 2.21; 95% confidence interval, 0.92 to 5.26) was associated with death but not statistically significant. In a landmark analysis, after adjusting for potential confounders, late (after day 14 and before day 28) severe AKI was strongly associated with higher hazard of death (hazard ratio, 4.57; 95% confidence interval, 1.82 to 11.5).ConclusionsSevere AKI occurs frequently in extremely low gestational age neonates. Stage 3 AKI is associated with mortality, and this association is present 7 days before death.

S10.2: Time dependent exposure and age effects in Cox regression models– a simulation study

2004 ◽  
Vol 46 (S1) ◽  
pp. 21-21
Author(s):  
Ingo Langner ◽  
Ralf Bender ◽  
Jürgen Wellmann
Keyword(s):  
Simulation Study ◽  
Regression Models ◽  
Cox Regression ◽  
Age Effects ◽  
Time Dependent

scholarly journals Digital infarct and mononeuritis in a middle aged female: always suspect Antineutrophil cytoplasmic antibodies associated vasculitis

2020 ◽  
Vol 7 (4) ◽  
pp. 707
Author(s):  
Avirup Majumdar ◽  
Virendra Atam ◽  
Saurabh Pandey ◽  
Prashant Singh ◽  
Himanshu Chauhan
Keyword(s):  
Antineutrophil Cytoplasmic Antibody ◽  
Organ Damage ◽  
Small Vessel ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Small Vessel Vasculitis ◽  
Multisystem Disease ◽  
Cutaneous Manifestations ◽  
Similar Treatment ◽  
Process Testing ◽  
Anca Associated Vasculitis

Vasculitis is a process caused by inflammation of blood vessel walls and results in a variety of disorders.  Small-vessel vasculitis (vasculitis involving arteries, venules and capillaries) should be suspected in any patient who presents with a multisystem disease that is not caused by an infectious or malignant process. Testing for Antineutrophil cytoplasmic antibody (ANCA) is the basis of classification of small vessel vasculitis into ANCA associated and non - ANCA associated vasculitis. Apart from cutaneous manifestations like palpable purpura and vasculitic urticaria, digital gangrene in a patient with evidence of mononeuritis multiplex is highly suggestive of ANCA associated vasculitis (AAV). Clinically most of these vasculitides have overlapping clinical presentations and similar treatment. Early diagnosis and rapid initiation of treatment of AAV is recommended rather than ordering for definitive tests (e.g. histopathology or angiograms) since delay in treatment can result in serious end organ damage (pulmonary or renal).

The impact of statin therapy after surgical or endovascular treatment of cerebral aneurysms

2020 ◽  
Vol 133 (1) ◽  
pp. 182-189
Author(s):  
Tae-Jin Song ◽  
Seung-Hun Oh ◽  
Jinkwon Kim
Keyword(s):  
Statin Therapy ◽  
Coil Embolization ◽  
Primary Outcome ◽  
Cox Regression ◽  
Cerebral Aneurysms ◽  
Time Dependent ◽  
Lipid Lowering ◽  
Surgical Clipping ◽  
The Impact

OBJECTIVECerebral aneurysms represent the most common cause of spontaneous subarachnoid hemorrhage. Statins are lipid-lowering agents that may expert multiple pleiotropic vascular protective effects. The authors hypothesized that statin therapy after coil embolization or surgical clipping of cerebral aneurysms might improve clinical outcomes.METHODSThis was a retrospective cohort study using the National Health Insurance Service–National Sample Cohort Database in Korea. Patients who underwent coil embolization or surgical clipping for cerebral aneurysm between 2002 and 2013 were included. Based on prescription claims, the authors calculated the proportion of days covered (PDC) by statins during follow-up as a marker of statin therapy. The primary outcome was a composite of the development of stroke, myocardial infarction, and all-cause death. Multivariate time-dependent Cox regression analyses were performed.RESULTSA total of 1381 patients who underwent coil embolization (n = 542) or surgical clipping (n = 839) of cerebral aneurysms were included in this study. During the mean (± SD) follow-up period of 3.83 ± 3.35 years, 335 (24.3%) patients experienced the primary outcome. Adjustments were performed for sex, age (as a continuous variable), treatment modality, aneurysm rupture status (ruptured or unruptured aneurysm), hypertension, diabetes mellitus, household income level, and prior history of ischemic stroke or intracerebral hemorrhage as time-independent variables and statin therapy during follow-up as a time-dependent variable. Consistent statin therapy (PDC > 80%) was significantly associated with a lower risk of the primary outcome (adjusted hazard ratio 0.34, 95% CI 0.14–0.85).CONCLUSIONSConsistent statin therapy was significantly associated with better prognosis after coil embolization or surgical clipping of cerebral aneurysms.

scholarly journals Updating OMERACT Core Set of Domains for ANCA-associated Vasculitis: Patient Perspective Using the International Classification of Function, Disability, and Health

2019 ◽  
Vol 46 (10) ◽  
pp. 1415-1420 ◽  
Author(s):  
Nataliya Milman ◽  
Eilish McConville ◽  
Joanna C. Robson ◽  
Annelies Boonen ◽  
Peter Tugwell ◽  
...  
Keyword(s):  
The United States ◽  
International Classification ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Personal Factors ◽  
Disability And Health ◽  
Individual Interviews ◽  
The United Kingdom ◽  
Anca Associated Vasculitis ◽  
Core Set

Objective.Aspects of antineutrophil cytoplasmic antibodies–associated vasculitis (AAV) prioritized by patients with AAV were described using the International Classification of Function, Disability, and Health (ICF).Methods.Items identified during 14 individual interviews were incorporated into an ICF-based questionnaire administered to participants of 2 vasculitis patient symposia: 36 in the United Kingdom and 63 in the United States.Results.Categories identified as at least “moderately relevant” by ≥ 5% of subjects included 44 body functions, 14 body structures, 35 activities and participation, 31 environmental factors, and 38 personal factors.Conclusion.Identified categories differ from those identified by the current Outcome Measures in Rheumatology (OMERACT) core set and those prioritized by vasculitis experts.

scholarly journals ANCA Status or Clinical Phenotype — What Counts More?

2021 ◽  
Vol 23 (6) ◽  
Author(s):  
Martin Windpessl ◽  
Erica L. Bettac ◽  
Philipp Gauckler ◽  
Jae Il Shin ◽  
Duvuru Geetha ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Granulomatosis With Polyangiitis ◽  
Clinical Phenotype ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Cardiovascular Death ◽  
Genetic Associations ◽  
Ongoing Debate ◽  
The Past ◽  
Anca Associated Vasculitis

Abstract Purpose of Review There is ongoing debate concerning the classification of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. That is, whether classification should be based on the serotype (proteinase 3 (PR3)- or myeloperoxidase (MPO)-ANCA) or on the clinical phenotype (granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA)). To add clarity, this review focused on integration of the most recent literature. Recent Findings Large clinical trials have provided evidence that a serology-based risk assessment for relapses is more predictive than distinction based on the phenotype. Research conducted in the past decade indicated that a serology-based approach more closely resembles the genetic associations, the clinical presentation (i.e., lung involvement), biomarker biology, treatment response, and is also predicting comorbidities (such as cardiovascular death). Summary Our review highlights that a serology-based approach could replace a phenotype-based approach to classify ANCA-associated vasculitides. In future, clinical trials and observational studies will presumably focus on this distinction and, as such, translate into a “personalized medicine.”

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