Pathways to renal biopsy and diagnosis among patients with ANCA small-vessel vasculitis (original) (raw)
Related papers
Rheumatology Advances in Practice
Objectives Diagnosing patients with ANCA-associated vasculitis (AAV) can be challenging owing to its rarity and complexity. Diagnostic delay can have severe consequences, such as chronic organ damage or even death. Given that few studies have addressed diagnostic pathways to identify opportunities to improve, we performed a clinical audit to evaluate the diagnostic phase. Methods This retrospective, observational study of electronic medical records data in hospitals focused on diagnostic procedures during the first assessment until diagnosis. Results We included 230 AAV patients from nine hospitals. First assessments were mainly performed by a specialist in internal medicine (52%), pulmonology (14%), ENT (13%) or rheumatology (10%). The overall median time to diagnosis was 13 [interquartile range: 2–49] days, and in patients primarily examined by a specialist in internal medicine it was 6 [1–25] days, rheumatology 14 [4–45] days, pulmonology 15 [5–70] days and ENT 57 [16–176] days (...
Kidney Pathology and Outcomes in Anca-Associated Vasculitis: Retrospective Analysis of 85 Patients
2021
ANCA-associated vasculitis (AAV) pose a significant risk of kidney failure, kidney biopsy remains a key prognostic tool. Pathology classification of the AAV glomerulonephritis (GN) developed by Berden et al showed correlation between GN classes and kidney outcomes; ANCA Renal Risk Score (ARRS) included tubular atrophy and interstitial fibrosis (TA/IF) as an additional parameter for risk assessment. We aimed to evaluate kidney survival across AAV GN classes and ARRS groups. A single-center retrospective study included 85 adult patients with biopsy-proven AAV kidney disease followed in 2000-2020. Primary outcome was kidney survival at the end of 18 [5; 66] months follow-up, kidney death considered as CKD stage 5. We found significant difference in the kidney survival for sclerotic, mixed, crescentic and focal AAV GN classes: 19%, 76.2%, 91.7% and 100% respectively (p=0.009). Kidney survival was 0%, 75.6% and 100% for the high, median and low risk ARRS groups respectively (p<0.001);...
Histological and clinical predictors of early and late renal outcome in ANCA-associated vasculitis
Nephrology Dialysis Transplantation, 2005
Background. Renal involvement remains a major determinant in antineutrophil cytoplasmic autoantibodyassociated small vessel vasculitis (AASV). While some patients may develop persistent renal damage, others have a favourable outcome. Methods. To identify patients at risk for poor renal outcome, we evaluated 95 renal biopsies (67 initial biopsies and 28 repeat biopsies) of 67 patients with AASV for the presence and extent of active (AI) and chronic (CI) lesions, retrospectively. AI, CI, levels of proteinuria and dose of cyclophosphamide (CYC) were related to renal outcome. Results. Recovery of renal function in patients initially dialysis dependent was associated with a lower CI compared with patients who remained on dialysis (P<0.001), while AI did not differ significantly. In these patients, age <65 years revealed a positive predictive value of 85% for renal function recovery. Patients initially requiring dialysis exhibited a higher AI and CI compared with those who did not. Renal function in long-term follow-up correlated with CI and the amount of proteinuria. This relationship increased with time, exhibiting at 4 years a correlation coefficient of 0.607 for CI (P<0.01) and of 0.775 for proteinuria (P<0.001). Follow-up biopsies showed a more pronounced CI compared with initial biopsies (P<0.001). None of the investigated initial parameters was predictive for renal relapse. However, there was a relationship between dose and duration of CYC and time to relapse. Compared with the initial biopsy, repeat biopsies of eight patients with a creeping serum creatinine in clinical remission showed a decrease of AI (P<0.001) while CI increased rapidly. These patients also had less initial CYC (NS).
Repeat protocol renal biopsy in ANCA-associated renal vasculitis
Nephrology Dialysis Transplantation, 2014
http://ndt.oxfordjournals.org/ Downloaded from I N FO C U S R a r e t r a n s f o r m a t i o n b e t w e e n s e g m e n t a l a n d g l o b a l L N 2931 by vladimir tesar on July 18, 2014 http://ndt.oxfordjournals.org/ Downloaded from
Diagnosing ANCA-associated vasculitis in ANCA positive patients
Medicine, 2016
Currently no validated diagnostic system for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is available. Therefore, diagnosing AAV is often challenging. We aimed to identify factors that lead to a clinical diagnosis AAV in ANCA positive patients in a teaching hospital in The Netherlands. In this study, all patients that tested positive for ANCA proteinase 3 (PR3) and/or myeloperoxidase (MPO) between 2005 and 2015 were analysed. Patients with a clinical diagnosis of AAV were compared with patients without a clinical diagnosis of AAV. Clinical symptoms and laboratory variables at presentation, including the ANCA titre, were collected for both patients with and without AAV. Clinical and laboratory variables related with AAV were investigated, using multivariable logistic regression. Two hundred thirty seven consecutive patients with a positive ANCA were included, of whom 119 were clinically diagnosed with AAV. Of the 118 ANCA positive patients without AAV, 87 patients had an alternative diagnosis, including inflammatory bowel disease (n = 24), other rheumatic diseases (n = 23), infection (n = 11), malignancy (n = 4), and other diagnoses (n = 25). In a multivariable regression model, a high ANCA titre (odds ratio [OR] 14.16, 95% confidence interval [CI] 6.93-28.94) and a high number of affected organ systems (OR 7.67, 95% CI 3.69-15.94) were associated with AAV. MPO and PR3 ANCA can be positive in a variety of diseases that mimic AAV. A higher ANCA titre and multiple affected organ systems may help to discriminate between AAV and other systemic illnesses in anti-PR3 and anti-MPO positive patients. A diagnostic scoring system incorporating these factors should be considered.
From Guidelines to Missed Opportunities: A Critical Analysis of ANCA Vasculitis Management Practices
From Guidelines to Missed Opportunities: A Critical Analysis of ANCA Vasculitis Management Practices, 2024
Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) represents a collection of rare autoimmune disorders that primarily target small blood vessels. This group includes granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA). The diagnosis of AAV is multifaceted, relying on a combination of clinical assessments, biological markers, radiological imaging, and histopathological evaluations. Although the revised Chapel Hill classification has refined disease definitions, the absence of ANCAs in some patients can complicate diagnosis. The management of AAV typically begins with an induction phase aimed at inducing remission. This phase often incorporates corticosteroids (CS) or Avacopan in conjunction with immunosuppressants such as Rituximab (RTX) or Cyclophosphamide (CYC). Following this initial treatment, patients generally transition to maintenance therapy. Despite significant advancements in the understanding and treatment of AAV, challenges persist regarding accurate prognosis and therapeutic management. To evaluate disease severity and inform treatment decisions, clinicians utilize tools such as the Five-Factor Score (FFS) and the Birmingham Vasculitis Activity Score (BVAS). However, these assessment instruments have inherent limitations and may not fully encapsulate the complexity of the disease. Recent epidemiological research has underscored geographic variability in the incidence of AAV and highlighted the role of ANCAs as crucial diagnostic and prognostic markers. Evaluating long-term sequelae using indices such as the Vasculitis Damage Index (VDI) is essential, especially given improved survival rates and an increasing focus on enhancing patients' quality of life. Current treatment strategies aim to minimize relapses and manage complications, including infections and metabolic disturbances; however, there is a pressing need for more personalized approaches.This review emphasizes the importance of developing more sophisticated prognostic tools and activity scores to improve clinical management of AAV. It also advocates for continued research to optimize treatment strategies and enhance outcomes for patients affected by these challenging conditions.
Evolving Challenges in Diagnosis of Renal Vasculitis
Journal of Medical Cases, 2021
Renal-limited vasculitis is a rare anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis that presents only with a renal manifestation in the absence of other organs involvement. In this report, a 50-year-old female presented with nonspecific symptoms and anemia, who was subsequently discovered to have renal-limited vasculitis. After receiving a combination of steroid and immunosuppressive therapy, she recovered uneventfully without further relapse. A wide range of nonspecific presenting symptoms and the insidious nature of renal disease often delay in early recognition of renal-limited vasculitis. Keeping a lower threshold of initiating vasculitis workup helps detect the earlier diagnosis which is crucial in management with improved renal outcome.
Physiology International, 2021
BackgroundImmunosuppressive therapy has improved the outcome of ANCA-associated vasculitis (AAV), but infectious morbidity and mortality remained high. Recognizing its risk factors seems crucial for prevention, aiming to increase survival of these patients.MethodsWe investigated the incidence and types of infections and assessed predictive factors in 132 patients with severe systemic AAV.ResultsPatients with lower than median incidence of total infections/patient-year during induction had lower baseline serum creatinine, dialysis requirement and Charlson comorbidity index (CCI), compared to those with higher than median incidence (P = 0.037; P = 0.024; P = 0.001; respectively). In subgroups with below and above than median number of severe infections/patient-year during induction, differences were found in baseline creatinine (P = 0.002) and dialysis requirement (P = 0.001); comparing the same cohorts during maintenance immunosuppression, baseline dialysis requirement, diabetes, CCI...
Frontiers in Medicine, 2021
Aim Accumulating evidence supports the use of antineutrophil cytoplasmic antibody (ANCA) type to classify different clinical entities. We aimed to evaluate whether the presence and type of ANCA determine different diseases, based on clinical phenotypes, renal involvement, and response to treatment. Patients and Methods Differences in terms of clinical manifestations, disease activity, laboratory parameters, and histology were recorded between patients with focal necrotizing glomerulonephritis (FNGN) due to myeloperoxidase (MPO-), proteinase 3-ANCA(+) [PR3-ANCA(+)], and ANCA(-) disease at time of diagnosis. Patients were treated with the same protocol and followed-up for 24 months, in a scheduled basis of every month for the first year and every 3 months for the second year. Primary end points were: (i) Combined end-stage renal disease (ESRD) and/or death and (ii) The presence of major or minor relapse during follow-up and secondary endpoint was the combination of ESRD and reduction ...
ANCA-associated renal vasculitis--epidemiology, diagnostics and treatment
Prague medical report, 2004
The pauciimmune small-vessel vasculitides are multisystem diseases with frequent renal involvement. They are strongly associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCA). In this review we have focused on the ethiopathogenesis and the role of ANCA, clinical presentation and histopathologic findings of different ANCA - associated vasculitides (AAV). Current treatment strategies and the overall and renal outcome of patients with AAV are also discussed.