Evolving Challenges in Diagnosis of Renal Vasculitis (original) (raw)
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Renal Limited Anti-Neutrophil Cytoplasmic Antibody Associated Vasculitis: A Case Report
Journal of Clinical Medical Research, 2024
Background: Renal Limited Vasculitis (RLV) is a localized autoimmune vascular inflammatory disorder that is part of the Pauci Immune Glomerulonephritis (PIGN) spectrum. Over 90% of PIGN patients have circulating Anti-Neutrophil Cytoplasmic Antibodies (ANCA Ab). Anti-Neutrophil Cytoplasmic Antibody (ANCA) associated vasculitides are a heterogeneous group of multisystemic autoimmune disorders with distinct pathological findings. They are characterized by widespread inflammation of vessels according to their size, their location, and their serotypes based on the presence or absence of ANCA antibodies, namely Myeloperoxidase (MPO-ANCA), Proteinase-3 (PR3-ANCA) or simply ANCA negative, if no antibodies are found. RLV is characterized by antibody positivity to myeloperoxidase in the majority of cases. Case Report: A 77-year-old Caucasian female was admitted with altered mental status and laboratory evidence of renal function compromise consistent with non-oliguric acute renal failure. Imaging and renal biopsy resulted in a diagnosis of pauci-immune glomerulonephritis consistent with MPO-ANCA vasculitis with a subtype that was associated with necrotizing arteritis, a severe form of the disease. She was placed on hemodialysis, intravenous cyclophosphamide, and oral prednisone, and entered remission. Conclusion: A high level of suspicion and familiarity with clinical signs and symptoms are critical in distinguishing primary RPGN subtypes from other types of ANCA-associated small vessel vasculitis. If left untreated, renal limited vasculitis can progress to widespread systemic involvement and become fatal.
Clinical Kidney Journal
Background Although rapidly progressive glomerulonephritis is the main renal phenotype of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), slow renal disease progression is sometimes observed. These forms have been rarely discussed; we analysed their prevalence, clinico-pathological characteristics and outcome. Methods We screened patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis followed at seven referral centres and selected those with estimated glomerular filtration rate (eGFR) reduction <50% over a 6-month period preceding diagnosis. Data regarding patient features and response to treatment were retrieved. Results Of 856 patients, 41 (5%) had slowly progressive renal AAV. All had MPA and all but one was P-ANCA/myeloperoxidase (MPO) ANCA-positive. At diagnosis, the median age was 70 years [interquartile range (IQR) 64–78] and extra-renal manifestations were absent or subclinical (interstitial lung lesions in 10, 24%). Th...
Clinical and Experimental Rheumatology, 2020
In ANCA-associated vasculitis (AAV), renal relapses are cause of concern as they are unpredictable and predictors of end-stage renal disease (ESRD). We aimed to assess the frequency of major renal (MR) relapses in AAV and to identify independent base-line predictors. We performed a retrospective monocentric observational cohort study of patients affected by granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and renal limited vasculitis (RLV), diagnosed from 2000 to 2019, and who achieved clinical remission defined as Birmingham Vasculitis Activity Index version 3 (BVASv3)=0 and/or clinical judgment. MR relapse was defined as the occurrence of major items of renal BVASv3. Univariate and multivariable analysis was performed with competitive risk analysis. We included 96 patients: 73 GPA, 21 MPA and 2 RLV. Eighty-five (90%) patients were ANCA-positive: 56 c-ANCA/PR3, 28 p-ANCA/MPO and 1 double positive. During the follow-up, 17/96 patients developed at least one MR ...
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.
Renal-limited vasculitis with elevated levels of multiple antibodies
CEN Case Reports, 2017
serological triple antibodies positivity in the absence of linear IgG deposition could benefit from the combination therapy regimen for plasma exchange and primary induction of remission against microscopic polyangiitis. Keywords MPO-ANCA • PR3-ANCA • Anti-GBM antibody • Case report Abbreviations ANCA Anti-neutrophil cytoplasmic antibody PR3-ANCA Proteinase 3 anti-neutrophil cytoplasmic antibody MPO-ANCA Myeloperoxidase anti-neutrophil cytoplasmic antibody GBM Glomerular basement membrane RLV Renal-limited vasculitis MPA Microscopic polyangiitis RPGN Rapidly progressive glomerulonephritis APTT Activated partial thromboplastin time IVCY Intravenous high-dose cyclophosphamide therapy PSC Primary sclerotic cholangitis Background Renal-limited vasculitis (RLV) is a type of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis that presents with only a renal manifestation; no other organs, including lungs, are involved. RLV is also suggested to be a subtype of microscopic polyangiitis (MPA) or granulomatosis with polyangiitis (GPA) based on the presence of myeloperoxidase (MPO)-ANCA and proteinase3 (PR3)-ANCA. RLV is also characterized as an anti-GBM-associated disease with no pulmonary manifestation [1].
Antineutrophil cytoplasmic antibody-associated vasculitis with renal involvement
International Journal of Evidence-Based Healthcare, 2010
Antineutrophil cytoplasmic antibody-associated vasculitis is an autoimmune disease involving small to medium blood vessels. It is an uncommon illness, but can have devastating consequences, particularly on kidney function and other vital organs. Exciting progress has been made in the treatment of the disease largely because of international collaboration in randomised clinical trials. Patient survival has improved dramatically with advancements in disease diagnosis and medical treatment. The long-term morbidity from the disease, although improving, remains substantial with up to 10% of survivors requiring dialysis or kidney transplantation. Clinical trials are underway using more specifically targeted immunosuppressants in the hope to improve the long-term patient outcomes. Advancements are also being made in understanding the pathogenesis of the disease and this will further assist disease treatment and outcomes in the future.
Antineutrophilic cytoplasmic antibody-associated vasculitis and the kidney
Current Opinion in Pediatrics, 2021
Objectives: ANCA-associated vasculitis are severe autoimmune pathologies that are characterized by inflammation and necrosis of the small vessels. The physiopathological mechanisms are complex and have yet to be totally elucidated. Several environmental factors have been described as being associated: medications, infectious agents.. . and rarely, neoplasms. Methods: We performed a retrospective multicenter study over a period of 12 years with a view to describing the association of ANCA-associated vasculitis and malignant hemopathies, excluding hemopathies secondary to vasculitis treatment. Results: Sixteen patients with ANCA-associated vasculitis with an hemopathy were identified. The gender ratio was 7 and the mean age was 65 years. The frequency of this association is estimated at 1%. The ANCA-associated vasculitis were micropolyangiitis (n = 7), followed by granulomatous polyangiitis (n = 4), vasculitis limited to the kidney (n = 3), and eosinophilic granulomatous polyangiitis (n = 2). The associated malignant hemopathies were mainly non-Hodgkin's lymphoma in seven cases and myelodysplasia in five cases. The other hemopathies were: Hodgkin's disease, hypereosinophilic syndrome, and Waldenström's macroglobulinemia. Hemopathy treatment was associated with vasculitis treatment in seven cases. Conclusion: The association of ANCA-associated vasculitis and malignant hemopathy is rare but must nevertheless be recognized because: (i) the clinical signs of both pathologies are not specific, (ii) the survival scores that are used for ANCA-associated vasculitis do not appear to be applicable, (iii) both pathologies must be taken into account in order to implement an effective therapeutic strategy that limits the inherent risks.
Nephrology Dialysis Transplantation, 2003
Antineutrophil cytoplasmic antibody (ANCA)positive renal vasculitis is the most common cause of rapidly progressive (crescentic) glomerulonephritis. Its life-threatening natural course may be modified substantially by current treatment modalities. The European Vasculitis Study Group (EUVAS) developed a subclassification of ANCA-positive vasculitides based on the disease severity at presentation, and have organized (so far) two waves of clinical trials. The first wave of randomized clinical trials had the aim of optimizing the existing therapeutic regimens; the second wave concentrated on testing some newer therapeutic approaches. Here, the design and available results of the first wave and the design of some second wave trials are reviewed briefly. The potential of the new targeted approaches (e.g. anti-tumour necrosis factor therapy) is also briefly mentioned.
Cureus
Granulomatosis with polyangiitis (GPA), or Wegener's granulomatosis as it was formerly referred to, is an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). GPA is characterized as a necrotizing vasculitis with few or no immune deposits termed pauci-immune deposits, predominantly affecting small and medium arterial vessels, involving the upper and lower respiratory tract as well as glomeruli. Renal manifestations are of critical importance because of the progression that may ensue following onset. Glomerulonephritis (primarily rapidly progressive crescentic glomerulonephritis) is quite common, which eventually leads to chronic kidney disease or end-stage renal disease. Usually, patients with GPA and rapidly progressive glomerulonephritis have an elevated plasma creatinine level and urinalysis revealing dysmorphic hematuria, red cell casts, and sub-nephrotic levels of proteinuria. We present a case of a 44-year-old male whose biopsy demonstrated crescentic glomerulonephritis, pauci-immune type proteinase 3 antineutrophil cytoplasmic antibody (PR3-ANCA) consistent with GPA, as well as profound proteinuria, an atypical manifestation.
Pediatric nephrology (Berlin, Germany), 2017
The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a group of disorders characterized by necrotizing inflammation of the small to medium vessels in association with autoantibodies against the cytoplasmic region of the neutrophil. Included in this definition are granulomatosis with polyangiitis (GPA, formerly known as Wegener's granulomatosis), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome). AAV are chronic, often relapsing diseases that can be organ or life threatening. Despite immunosuppression, the morbidity and mortality remain high. Renal involvement contributes significantly to the morbidity with high numbers of patients progressing to end-stage kidney disease. Current therapies have enabled improvements in renal function in the short term, but evidence for long-term protection is lacking. In MPA, renal involvement is common at presentation (90%) and often follows a m...