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Journal articles on the topic 'ANCA glomerulonephritis vasculitis'

Author: Grafiati

Published: 3 June 2025

Last updated: 1 August 2025

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1

Radwan, Yasser, Sarah Berini, Floranne Ernste, and Ashima Makol. "Proteinase 3 (PR3)-antineutrophil cytoplasmic antibody (ANCA)-associated vasculitic neuropathy in diffuse cutaneous systemic sclerosis: a rare duo." BMJ Case Reports 12, no. 11 (2019): e232987. http://dx.doi.org/10.1136/bcr-2019-232987.

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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 SS

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2

Savenkova, N. D. "Treatment strategy ANCA-associated renal vasculitides in children and adolescents." Nephrology (Saint-Petersburg) 23, no. 5 (2019): 107–15. http://dx.doi.org/10.24884/1561-6274-2019-23-5-107-115.

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The article presents the terminology and classification in accordance with the International Сhapel Hill Сonsensus Сonference nomenclature of vasculitides (2012), clinical, immunological and morphological manifestations, therapy strategy and outcome of Antineutrophil cytoplasmic antibody vasculitides (ANCA) renal associated vasculitis (microscopic polyanghiitis, granulomatosis with Wegener's polyangiitis, eosinophilic granulomatosis with polyangiitis Churg-Strauss) in children and adolescents. IgG class antibodies to MPO and PR3, histopathological changes in renal biopsy specimens are consider

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3

Jennette, J. Charles, and Patrick H. Nachman. "ANCA Glomerulonephritis and Vasculitis." Clinical Journal of the American Society of Nephrology 12, no. 10 (2017): 1680–91. http://dx.doi.org/10.2215/cjn.02500317.

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4

Trimeche Ajmi, Sihem, Rim Braham, Sarra Toumi, et al. "Benzylthiouracil-Induced Glomerulonephritis." Case Reports in Medicine 2009 (2009): 1–4. http://dx.doi.org/10.1155/2009/687285.

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Vasculitis is a rare complication of antithyroid drugs (ATDs). It was first described with Propylthiouracil (PTU). We report a new case of antineutrophil cytoplasmic antibody (ANCA) vasculitis with glomerulonephritis induced by Benzylthiouracile (BTU). A 50-year-old man with Graves disease treated with BTU developed general malaise and haematuria without skin rash or respiratory involvement. Laboratory data revealed acute renal failure with proteinuria and haematuria. An indirect immunofluorescence test for ANCA was positive, showing a perinuclear pattern with specificity antimyeloperoxidase (

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5

Drouzas, Konstantinos, Petros Kalogeropoulos, George Liapis, and Sofia Lionaki. "Current Understanding of the Pathogenesis of ANCA-Associated Vasculitis and Novel Treatment Options Targeting Complement Activation." Life 15, no. 5 (2025): 756. https://doi.org/10.3390/life15050756.

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Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (ANCA-vasculitis) is an autoimmune disease characterized by inflammation and necrosis of small or medium vessels. In the past, the role of the complement in the pathogenesis of ANCA-vasculitis has been underestimated, due to the paucity of the complement at sites of injured glomeruli. Following evidence from animal models of the major role of the complement in pathogenesis of ANCA-vasculitis, the complement has again attracted interest. Immunohistology analysis of pauci-immune glomerulonephritis—ANCA glomerulonephritis (ANCA-GN)

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Preto, Clara, Armandina Silva, Sandra Alves, et al. "The Diagnosing Challenge of a Positive ANCA Vasculitis in the Paediatric Age." Case Reports in Pediatrics 2017 (2017): 1–5. http://dx.doi.org/10.1155/2017/2962794.

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ANCA-positive systemic vasculitides, rare in paediatric age, present multiorganic involvement. A female teenager presented with a history of subglottic stenosis diagnosed at the age of 12. From the investigation carried out, we highlight hematoproteinuria and negative ANCAs. At 15 years old, she was admitted for gastrointestinal symptoms and respiratory distress. She presented poor peripheral perfusion, pulmonary haemorrhage, respiratory failure, and severe renal insufficiency. She was started mechanical ventilation and emergency haemodialysis. The immunological study revealed ANCA MPO positiv

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7

Kao, Lily, and Cornelia Weyand. "Vasculitis in Systemic Sclerosis." International Journal of Rheumatology 2010 (2010): 1–9. http://dx.doi.org/10.1155/2010/385938.

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Systemic sclerosis (SSc) is a multiorgan connective tissue disease characterized by autoantibody production and fibroproliferative stenosis of the microvasculature. The vascoluopathy associated with SSc is considered to be noninflammatory, yet frank vasculitis can complicate SSc, posing diagnostic and therapeutic challenges. Here, we have reviewed the literature for reports of small-, medium-, and large-vessel vasculitis occurring in SSc. Amongst 88 reported cases of vasculitis in SSc, patients with ANCA-associated vasculitis appear to present a unique subclass in that they combined typical fe

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8

Van Haare Heijmeijer, Sophie, Dunja Wilmes, Selda Aydin, Caroline Clerckx, and Laura Labriola. "Necrotizing ANCA-Positive Glomerulonephritis Secondary to Culture-Negative Endocarditis." Case Reports in Nephrology 2015 (2015): 1–5. http://dx.doi.org/10.1155/2015/649763.

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Infective endocarditis (IE) and small-vessel vasculitis may have similar clinical features, including glomerulonephritis. Furthermore the association between IE and ANCA positivity is well documented, making differential diagnosis between IE- and ANCA-associated vasculitis particularly difficult, especially in case of culture-negative IE. We report on one patient with glomerulonephritis secondary to culture-negative IE caused byBartonella henselaewhich illustrates this diagnostic difficulty.

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9

Lamprecht, P., W. H. Schmitt, and W. L. Gross. "Mixed cryoglobulinemia, glomerulonephritis, and ANCA: essential cryoglobulinaemic vasculitis or ANCA-associated vasculitis?" Nephrology Dialysis Transplantation 13, no. 1 (1998): 213–21. http://dx.doi.org/10.1093/ndt/13.1.213.

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10

Endo, Akiko, Junichi Hoshino, Tatsuya Suwabe, et al. "Significance of Small Renal Artery Lesions in Patients with Antineutrophil Cytoplasmic Antibody-associated Glomerulonephritis." Journal of Rheumatology 41, no. 6 (2014): 1140–46. http://dx.doi.org/10.3899/jrheum.130657.

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Objective.Antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis is a vasculitis affecting the glomerular capillaries and small renal arteries. Although crescent formation has been reported to be characteristic of this condition, the significance of coexisting vasculitis affecting the small renal arteries has not been investigated.Methods.Fifty patients with ANCA-positive rapidly progressive glomerulonephritis whose renal biopsy specimens contained arterioles and/or interlobular arteries were retrospectively evaluated. Cellular crescents and/or necrotizing glomerulonephritis

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11

Benhadda, Selim, Manal Nmili, Nada Nassiri, Loubna Benamar, Naima Ouzeddoun, and Tarik Bouattar. "Double-Positive Anti-GBM and ANCA Vasculitis: 2 Case Reports and Review of the Literature." Journal of Clinical Nephrology 9, no. 1 (2025): 009–12. https://doi.org/10.29328/journal.jcn.1001145.

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Double-Positive Patients (DPPs), characterized by the simultaneous presence of Anti-Neutrophil Cytoplasmic Antibody (ANCA) and anti-Glomerular Basement Membrane (anti-GBM) antibodies, represent a rare subset in systemic vasculitis. We present two cases of DPPs with renal involvement and review the existing literature to elucidate the clinical characteristics, histopathological findings, management strategies, and prognostic outcomes associated with this condition. Both cases exhibited renal involvement with rapidly progressive glomerulonephritis, requiring renal replacement therapy. Renal biop

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Capusa, Cristina, Ana-Maria Mehedinti, Claudia Toma, and Violeta Bojinca. "ATYPICAL PRESENTATION IN A CASE OF GRANULOMATOSIS WITH POLYANGIITIS." Romanian Journal of Rheumatology 25, no. 1 (2016): 37–42. http://dx.doi.org/10.37897/rjr.2016.1.5.

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Granulomatosis with polyangiitis (GPA) is part of the anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis, mostly being ANCA-c (anti proteinase 3) positive. Primarily it involves the upper respiratory tract and kidneys having an increased mortality in the absence of early diagnosis and correct treatment. The most common renal involvement is pauci-immune crescentic glomerulonephritis. We present the case of a patient with GPA with a particular onset of interstitial nephritis, possible by vasa recta vasculitis, in the absence of glomerulonephritis.

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Hilhorst, Marc, Alexandre TJ Maria, Niloufar Kavian, et al. "Impact of MPO-ANCA-mediated oxidative imbalance on renal vasculitis." American Journal of Physiology-Renal Physiology 315, no. 6 (2018): F1769—F1776. http://dx.doi.org/10.1152/ajprenal.00111.2018.

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Glomerulonephritis is a severe complication of microscopic polyangiitis (MPA), a small-vessel vasculitis associated with anti-myeloperoxidase antibodies (MPO-ANCA). We previously showed the pathogenic effects of MPO-ANCA that activate MPO to trigger an oxidative burst mainly through HOCl production, contributing to endothelial injury and lung fibrosis. The aim of this study was to investigate the relationship between MPO-induced oxidative stress, anti-oxidant defenses and renal histological lesions in MPA patients. We therefore analyzed histological data from a prospective cohort of ANCA-assoc

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Gordovskaya, Nadezhda B., E. M. Shilov, Yu V. Korotchaeva, E. V. Stavrovskaya, and S. V. Roshchupkin. "For fulminant rapidly progressive glomerulonephritis in a 17-year-old female patient with ANCA-associated vasculitis." Clinical Medicine (Russian Journal) 96, no. 2 (2018): 180–85. http://dx.doi.org/10.18821/0023-2149-2018-96-2-180-185.

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A case of rapidly progressive glomerulonephritis in a 17-year-old patient associated with antibodies against the cytoplasm of neutrophils (ANCA) vasculitis - ANCA-associated vasculitis is associated with antibodies to proteinase-3 and morphological picture extracapillar glomerulonephritis with sclerotic lesion of up to 80% of the glomeruli. The peculiarity of the case is the presence of morphologically confirmed when alloimmune rapidly progressive glomerulonephritis type III a pronounced glow-focal granular nature of immunoglobulin classes G and M on the basement membrane of capillaries. The a

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15

Ward, Nicholas D., Diane E. Cosner, Colleen A. Lamb, et al. "Top Differential Diagnosis Should Be Microscopic Polyangiitis in ANCA-Positive Patient with Diffuse Pulmonary Hemorrhage and Hemosiderosis." Case Reports in Pathology 2014 (2014): 1–5. http://dx.doi.org/10.1155/2014/286030.

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A rat model of antineutrophil cytoplasmic antibody (ANCA) associated vasculitides reveals crescentic glomerulonephritis as seen in human renal biopsies and diffuse lung hemorrhage that is not well documented in human lung biopsies. A 64-year-old male, with shortness of breath and mild elevation of serum creatinine, was found to have a positive serum test for ANCA, but negative antiglomerular basement membrane antibody. A renal biopsy showed pauci-immune type of crescentic glomerulonephritis and focal arteritis. The prior lung wedge biopsy was retrospectively reviewed to show diffuse hemorrhage

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16

Freitas, Joana, Inês Sala, João Oliveira, et al. "Proteinase 3 (PR3)-Antineutrophil Cytoplasmic Antibody in a Patient with Minimal Change Disease: An Unusual Finding." Portuguese Journal of Nephrology & Hypertension 63, no. 2 (2022): 89–92. http://dx.doi.org/10.32932/pjnh.2022.06.183.

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Antineutrophil cytoplasmic antibodies (ANCA) are useful as markers for systemic vasculitis. PR3-ANCA antibodies have been also identified in association with chronic inflammatory and infectious conditions, other autoimmune diseases, malignancy, and certain drugs. The association of PR3 and minimal change disease (MCD) without vasculitis is not an expected finding. We report a case of MCD with PR3-ANCA positive title and no histopathological findings of vasculitis. This reports to an 86-year-old Caucasian woman without relevant past medical history and a normal renal function one month before p

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Syed, Rafeel, Amina Rehman, Gautam Valecha, and Suzanne El-Sayegh. "Pauci-Immune Crescentic Glomerulonephritis: An ANCA-Associated Vasculitis." BioMed Research International 2015 (2015): 1–8. http://dx.doi.org/10.1155/2015/402826.

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Rapidly progressive glomerulonephritis(RPGN) is a syndrome signified by a precipitous loss of renal function, with features of glomerulonephritis including dysmorphic erythrocyturia and glomerular proteinuria. RPGN is associated with extensive crescent formation, and, thus, the clinical term RPGN is often used interchangeably with the pathologic termcrescentic glomerulonephritis(CGN). From an immunopathologic standpoint, primary RPGN is divided intopauci-immune GN(PICG), anti-GBM GN, and immune complex GN. PICG, the most common etiology of primary RPGN, refers to a necrotizing glomerulonephrit

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Sinclair, David, and Judith M. Stevens. "Role of antineutrophil cytoplasmic antibodies and glomerular basement membrane antibodies in the diagnosis and monitoring of systemic vasculitides." Annals of Clinical Biochemistry: International Journal of Laboratory Medicine 44, no. 5 (2007): 432–42. http://dx.doi.org/10.1258/000456307781646049.

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Systemic vasculitis, although rare, is often diagnosed late and long after the onset of symptoms. The small vessel vasculitides are recognized clinically by their multisystem presentation, markers of inflammation and evidence for an acute glomerulonephritis (GN), with the most apparent organ involved directing referral to secondary care. Routine laboratory tests are usually non-specific in systemic vasculitis but the use of anti-neutrophil cytoplasmic antibodies (ANCAs) and glomerular basement membrane (GBM) antibodies can aid diagnosis, treatment and monitoring decisions. These antibodies are

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Salabei, Joshua K., Sara Khan, Afzal Khan, et al. "ANCA-Associated Intrahepatic Duct Injury Associated with Levamisole-Adulterated Cocaine." Case Reports in Hepatology 2020 (December 22, 2020): 1–3. http://dx.doi.org/10.1155/2020/8867183.

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Damage to the liver or kidney can occur through direct toxic effects; however, damage can also be drug-induced immune-mediated. Levamisole-adulterated cocaine (LAC) is known to cause antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitis and glomerulonephritis leading to acute kidney injury and end-stage renal disease. It remains unclear whether LAC is associated with hepatic duct damage. Here, we report a case with biopsy-proven evidence of intrahepatic duct damage months after being diagnosed with ANCA-associated crescentic and sclerosing glomerulonephritis caused by LAC use. Thi

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Laskova, A., B. Mohammed, F. Ali, and B. Syritsa. "AB0629 Glomerulonephritis associated with ANCA after COVID-19 vaccine: a systematic review." Annals of the Rheumatic Diseases 81, Suppl 1 (2022): 1441–42. http://dx.doi.org/10.1136/annrheumdis-2022-eular.3379.

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BackgroundThe COVID-19 pandemic has led to widespread vaccination with effective results and a great safety profile. However, as the vaccination rate has increased, more cases of autoimmune diseases after the COVID-19 vaccine have been described. We present a systematic review of ANCA-associated glomerulonephritis after the COVID-19 vaccine.ObjectivesTo summarize the existing evidence on ANCA-associated glomerulonephritis after the COVID-19 vaccine.MethodsWe searched all studies from inception till January 27, 2022, that described ANCA-associated biopsy-proven glomerulonephritis after COVID-19

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Bell, Stephen, Natalia Lattanzio, Qassem Abdelal, et al. "A Rare Case of Double Antibody-Positive Rapidly Progressive Glomerulonephritis: A Therapeutic Challenge." Journal of Investigative Medicine High Impact Case Reports 9 (January 2021): 232470962110131. http://dx.doi.org/10.1177/23247096211013193.

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Crescentic glomerulonephritis, also known as rapidly progressive glomerulonephritis, is a syndrome characterized by progressive and rapid deterioration of renal function over the course of weeks to months. Oliguria, hematuria, azotemia, and hypertension are characteristic features of this condition. Crescentic glomerulonephritis is further classified according to the staining pattern on immunofluorescence. In rare instances, a mixed pattern of injury is encountered as in the case of double antibody-positive rapidly progressive glomerulonephritis (RPGN). This case illustrates the challenge in t

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Hakroush, Samy, Désirée Tampe, Peter Korsten, Philipp Ströbel, and Björn Tampe. "Complement Components C3 and C4 Indicate Vasculitis Manifestations to Distinct Renal Compartments in ANCA-Associated Glomerulonephritis." International Journal of Molecular Sciences 22, no. 12 (2021): 6588. http://dx.doi.org/10.3390/ijms22126588.

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Acute kidney injury (AKI) is a common and severe complication of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) causing progressive chronic kidney disease (CKD), end-stage renal disease (ESRD) or death. Pathogenic ANCAs, in particular proteinase 3 (PR3) and myeloperoxidase (MPO), trigger a deleterious immune response resulting in pauci-immune necrotizing and crescentic glomerulonephritis (GN), a common manifestation of glomerular injury in AAV. However, there is growing evidence that activation of the complement pathway contributes to the pathogenesis and progression

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Arnold, S., J. Mahrhold, A. Kerstein-Staehle, et al. "POS0829 SPECTRUM OF ANCA-SPECIFICITIES IN EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS IN A RETROSPECTIVE MULTICENTER STUDY." Annals of the Rheumatic Diseases 81, Suppl 1 (2022): 705–6. http://dx.doi.org/10.1136/annrheumdis-2022-eular.3083.

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BackgroundAnti-neutrophil cytoplasmic autoantibodies specific for myeloperoxidase (MPO-ANCA) are found in 10-70% of the patients with eosinophilic granulomatosis with polyangiitis (EGPA) depending on disease activity, methodological aspects and cohort examined [1-3]. Recently, a higher prevalence of anti-pentraxin 3 (PTX3)-ANCA has been reported in EGPA compared to granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) [4].ObjectivesTo investigate the spectrum of ANCA specificities in a multicenter cohort of patients with EGPA and identify novel ANCA antigens.MethodsWe condu

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Lhotta, Karl, Wolfgang Vogel, Thomas Meisl та ін. "α1-Antitrypsin Phenotypes in Patients with Anti-Neutrophil Cytoplasmic Antibody-Positive Vasculitis". Clinical Science 87, № 6 (1994): 693–95. http://dx.doi.org/10.1042/cs0870693.

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1. The genetic background of anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis remains largely unknown. Recently a very high prevalence of medium and severe deficiency of α1-antitrypsin was described in a small group of patients with Wegener's granulomatosis and c-ANCA. c-ANCAs are autoantibodies against proteinase 3, and α1-antitrypsin is the main inhibitor of this enzyme. 2. α1-Antitrypsin phenotypic polymorphism was determined by isoelectric focusing in 32 patients with c-ANCA-associated systemic vasculitis. Twenty-nine patients had Wegener's disease, two had micros

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Kancharla, Pragnan, B. K. Surapaneni, M. Goldfinger, K. Hennrick, and David J. Ozeri. "Paraneoplastic Seronegative Pauci-Immune Glomerulonephritis Associated with Lung Adenocarcinoma Responds to Rituximab: A Case Report." Case Reports in Oncology 11, no. 2 (2018): 372–77. http://dx.doi.org/10.1159/000490101.

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Anti-neutrophil cytoplasmic antibodies (ANCA) play an important role in the pathogenesis of pauci-immune renal vasculitis. However, in 10% of the cases, ANCA are absent. We present a case of a 64-year-old man with a chronic untreated hepatitis C virus infection and Middle Eastern thalassemia who was ANCA-negative when he was hospitalized due to acute kidney injury and accounts for an uncommon presentation of renal vasculitis. The patient had earlier reported to have undergone local lobectomy and adjuvant chemotherapy (carboplatin/pemetrexed) for lung adenocarcinoma a month prior. IL-6 has been

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Klimkina, I., A. Skvortsov, M. Litvinova, S. Moiseev, and N. Bulanov. "Outcomes in ANCA-associated vasculitides." Clinical pharmacology and therapy 35, no. 3 (2024): 50–56. http://dx.doi.org/10.32756/0869-5490-2024-3-50-56.

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Immunosuppressive therapy significantly improved survival of patients with ANCA-associated vasculitides (AAV). However, the adverse effects of therapy, particularly glucocorticosteroids, along with the accumulation of organ damage and a deterioration in the quality of life, have become the leading factors contributing to the disease burden. The development of a core outcome set and assessment tools is a current problem of research methodology to improve their quality and uniformity of data. Disease activity and damage assessed by the Birmingham Vasculitis Activity Score (BVAS v.3) and the Vasc

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OBrien, Shane, Brenda Griffin, Anne Marie McLaughlin, and Joseph Keane. "ANCA associated glomerulonephritis in tuberculosis: a paradoxical reaction." BMJ Case Reports 14, no. 6 (2021): e241904. http://dx.doi.org/10.1136/bcr-2021-241904.

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We present a case of antineutrophil cytoplasmic antibodies (ANCA)-associated rapidly progressive glomerulonephritis in the context of treatment of pulmonary tuberculosis (TB). A 42-year-old woman was treated for drug-susceptible pulmonary TB and represented with paradoxical worsening of symptoms and radiological features. She was HIV negative. A severe acute kidney injury with features of glomerulonephritis was evident on admission. Perinuclear ANCA and antimyeloperoxidase antibodies were present in serum and renal biopsy was consistent with ANCA-associated vasculitis. The patient was successf

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Hakroush, S., and B. Tampe. "POS0247 INTERSTITIAL ANCA-ASSOCIATED VASCULITIS ASSOCIATES WITH SEVERE KIDNEY INJURY INDEPENDENT OF GLOMERULONEPHRITIS." Annals of the Rheumatic Diseases 80, Suppl 1 (2021): 345.2–345. http://dx.doi.org/10.1136/annrheumdis-2021-eular.4296.

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Background:Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a small vessel vasculitis affecting multiple organ systems, including the kidney. Small vessels in the kidney include small-sized arteries (interlobular artery, afferent and efferent arteriole), capillaries (glomerular and peritubular capillary) and venules.Objectives:Although crescentic ANCA glomerulonephritis (GN) is a common histological finding reflecting glomerular small vessel vasculitis, it is reasonable that manifestation of AAV could also contribute to interstitial small vessel vasculitis. Therefore,

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Tampe, B. "POS1437 INTERSTITIAL ANCA-ASSOCIATED VASCULITIS ASSOCIATES WITH SEVERE KIDNEY INJURY INDEPENDENT OF GLOMERULONEPHRITIS." Annals of the Rheumatic Diseases 82, Suppl 1 (2023): 1072.2–1072. http://dx.doi.org/10.1136/annrheumdis-2023-eular.4595.

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BackgroundAntineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a small vessel vasculitis affecting multiple organ systems, including the kidney. Small vessels in the kidney include small-sized arteries (interlobular artery, afferent and efferent arteriole), capillaries (glomerular and peritubular capillary) and venules.ObjectivesAlthough crescentic ANCA glomerulonephritis (GN) is a common histological finding reflecting glomerular small vessel vasculitis, it is reasonable that manifestation of AAV could also contribute to interstitial small vessel vasculitis. Therefore, we

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Kluth, DC, and J. Hughes. "ANCA-associated systemic vasculitis (AASV)." Journal of the Royal College of Physicians of Edinburgh 37, no. 2 (2007): 128–34. https://doi.org/10.1177/1478271520073702015.

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Anti-neutrophil cytoplasmic antibody-associated systemic vasculitis is a multi-system disease that can present to a number of medical specialties, and where early identification and treatment improves outcome. The vasculitides are commonly divided into WG, MPA, and CSS. This review will revise the common clinical presentations of these diseases and initial investigations. Anti-neutrophil cytoplasmic antibodies have provided a valuable test to aid in diagnosis and there is increasing clinical and experimental evidence that suggests they have an important role in the pathogenesis of disease. Ant

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Carlos, E. Jiménez-López, N. García-Mata Fiama, Z. Flores-Cervantes Jonadab, et al. "Microscopic Polyangiitis, Revisiting ANCA-Associated Vasculitis: Case Report and Review." International Journal of Medical Science and Clinical Research Studies 05, no. 02 (2025): 385–88. https://doi.org/10.5281/zenodo.14934912.

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Microscopic polyangiitis (MPA) is a systemic autoimmune necrotizing vasculitis belonging to the group of antineutrophil cytoplasmatic antibody (ANCA)-associated vasculitis (AAV). Characterized by pauci-inmune crescentic glomerulonephritis without systemic involvement. AAV is an uncommon disease with an incidence of about 20 per million population per year in Europe and North America. The kidney is involved in more than 90% of MPA cases. Rapidly progressive glomerulonephritis is the most common clinical presentation or renal vasculitis, revealed by rapid deterioration of renal function, low-gra

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Jukes, P., F. D’acquisto, R. Pepper, et al. "Investigating Annexin-A1 in ANCA vasculitis and glomerulonephritis." La Presse Médicale 42, no. 4 (2013): 689. http://dx.doi.org/10.1016/j.lpm.2013.02.090.

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Thoms, Brendan L., Varun Agrawal, Elvira R. Umyarova, Pamela C. Gibson, and Richard J. Solomon. "Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis with Kidney Involvement in a Patient with AL Amyloidosis." Case Reports in Nephrology and Dialysis 11, no. 2 (2021): 183–89. http://dx.doi.org/10.1159/000517142.

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Antineutrophil cytoplasmic autoantibody (ANCA) vasculitis has occasionally been associated with other systemic glomerulonephritis, such as anti-glomerular basement membrane disease. Here, we report the first clinical case of ANCA-associated crescentic glomerulonephritis with AL amyloidosis. An 81-years-old gentleman presented to the hospital with acute kidney injury (serum creatinine 4.7 mg/dL) on a background of chronic kidney disease and volume overload. Autoimmune serology was remarkable for p-ANCA and myeloperoxidase positivity. A renal biopsy confirmed pauci-immune glomerulonephritis and

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Javadi Parvaneh, Vadood, Arezoo Shirzani, Khosro Rahmani, and Reza Shiari. "Pediatric Granulomatosis With Polyangiitis Mimicking IgA Vasculitis: A Case Report." Clinical Medicine Insights: Arthritis and Musculoskeletal Disorders 13 (January 2020): 117954412096737. http://dx.doi.org/10.1177/1179544120967371.

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Background: Granulomatosis with polyangiitis (GPA) is a systemic vasculitis of the upper and lower respiratory tract along with glomerulonephritis and is very rare in childhood. Its renal manifestations similarity with IgA vasculitis can be misleading. Case presentation: Herein, we report a 12-years-old girl with the clinical picture of IgA vasculitis and renal involvement at the time of presentation, over time, elevated cytoplasmic Anti-neutrophil Cytoplasmic Antibody (C-ANCA) and tissue biopsy confirmed GPA. Conclusion: In the case of a patient with an unusual presentation of IgA vasculitis,

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Suzuki, Kazuo, and Andreas Kronbichler. "Highlights from the breakout session: cellular and molecular mechanisms of disease (II)." Rheumatology 64, Supplement_1 (2025): i106—i108. https://doi.org/10.1093/rheumatology/keae427.

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Abstract The steps leading to key pathogenetic aspect of ANCA-associated vasculitis, such as lung nodules in granulomatosis with polyangiitis (GPA), onset of glomerulonephritis (GN) in experimental myeloperoxidase (MPO)-ANCA models and the implications of carrying staphylococcal superantigens, such as toxic shock syndrome toxin-1 (tsst-1), in GPA remain to be elucidated. The session ‘Cellular and molecular mechanisms of disease (II)’ was devoted to close some of these knowledge gaps and underlined that research in ANCA-associated vasculitis leads to more granular understanding of these complex

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Tanaka, Marenao, Norihito Moniwa, Tomohiro Mita, et al. "A Case of Crescentic Glomerulonephritis Complicated with Hypocomplementemic Urticarial Vasculitis Syndrome and ANCA-Associated Vasculitis." Case Reports in Nephrology and Dialysis 7, no. 3 (2017): 144–53. http://dx.doi.org/10.1159/000484476.

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Systemic urticaria in a 64-year-old woman was diagnosed as leukocytoclastic vasculitis by a punch biopsy of the skin. Her physical findings improved after prescription of prednisolone at a dose of 20 mg/day, but the skin rash relapsed with renal dysfunction, proteinuria, and hematuria when the dose of prednisolone was reduced over a period of 9 months to 1 mg/day. She was admitted to our institute for further examination, when urinary protein and plasma creatinine levels were 0.8 g/day and 1.7 mg/dL, respectively. Complement analysis showed that levels of total hemolytic component, component C

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Kannan, Lakshmi, and UmmeRubab Syeda. "Overlapping Syndromes: Drug-Induced Vasculitis From Hydralazine With Positive Antinuclear Antibodies." Clinical Medicine Insights: Case Reports 16 (January 2023): 117954762211469. http://dx.doi.org/10.1177/11795476221146906.

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Hydralazine is an antihypertensive medication that has been associated with drug-associated autoimmune syndromes like interstitial lung disease, pauci-immune glomerulonephritis, and hypocomplementemia. Hydralazine-induced ANCA-associated vasculitis and hydralazine induced positive antinuclear antibodies are rare. Clinical manifestations range from arthralgia, petechiae, or rash to multiorgan involvement. When presented as pulmonary-renal syndrome, it can be rapidly progressive and fatal. Here, we describe a case of hydralazine-associated vasculitis with rapidly progressive glomerulonephritis a

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Baptiste, Gillian G., Anastasia-Stefania Alexopoulos, Tahsin Masud, and Joanna M. Bonsall. "Systemic Levamisole-Induced Vasculitis in a Cocaine User without Cutaneous Findings: A Consideration in Diagnosis." Case Reports in Medicine 2015 (2015): 1–3. http://dx.doi.org/10.1155/2015/547023.

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Levamisole is a known immunomodulating agent frequently used as a cutting agent in cocaine consumed in the United States today. Numerous cases of anti-neutrophil cytoplasmic antibody (ANCA) vasculitis connected with the use of levamisole-adulterated cocaine have previously been reported in the literature, classically characterized by a retiform purpuric rash. We report a case of a crack-cocaine user without cutaneous abnormalities who developed ANCA-associated glomerulonephritis that progressed to renal failure. This case demonstrates the difficulties in solidifying the diagnosis of levamisole

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Akshaya, R. C., R. Sathyanarayanan, C. R. V. Narasimhalu, and Sonti Sulochana. "ANCA Associated vasculitis- A case of microscopic polyangiitis with proliferative glomerulonephritis." Biomedicine 41, no. 3 (2021): 686–89. http://dx.doi.org/10.51248/.v41i3.1208.

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Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are a collection of diseases, characterised by destruction and inflammation of small and medium vessels. Microscopic Polyangiitis (MPA) is part of an ANCA-associated vasculitis (AAV).The clinical signs diverge and disturba number of organs such as the kidneys, lungs, stomach and intestine. Skin manifestations such as purpuric, urticarial, nodular, ulcerative, livedoid and necrotic skin lesions were common as in other vaso-occlusive disorder. Morphology and added features aid the diagnostic approach. Here, we report a diagno

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Horai, Yoshiro, Shota Kurushima, and Atsushi Kawakami. "Current Diagnosis and Treatment of Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: A Review Including a Comparison of Characteristics in Europe and Japan." Journal of Clinical Medicine 14, no. 5 (2025): 1724. https://doi.org/10.3390/jcm14051724.

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Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a disease entity characterized by systemic vasculitis positive for ANCAs, which often leads to severe organ damage such as diffuse bronchoalveolar hemorrhage and rapidly progressive glomerulonephritis. It is known that the incidence and characteristics of AAV vary depending on region, and differences in the peak age of onset, the ratio of positive rates of MPO-ANCA to PR3-ANCA, and occurrence rates of GPA and MPA may have resulted in different approaches to clinical practice. It may also be necessary to modify therapeut

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Pieri, Christopher, Benjamin Challoner, Konstantinos Giaslakiotis, Mark Hawthorne, Michael Sheaff, and Ravindra Rajakariar. "Relapsing ANCA-associated vasculitis presenting as a mediastinal mass." BMJ Case Reports 18, no. 1 (2025): e262481. https://doi.org/10.1136/bcr-2024-262481.

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Anti-neutrophil cytoplasmic antibody-associated vasculitides (AAV) represent a heterogeneous multisystem group of disorders typified by necrotising inflammation of smaller blood vessels, classically yielding a pauci-immune, crescentic glomerulonephritis. Without prompt treatment, there is a significant risk of irreversible damage and ensuing renal impairment.Diagnosis is often challenging, exacerbated by the disorder’s often vague and insidious presentation. Even with known AAV, relapse can mimic other similarly serious conditions including infection or malignancy, with significant ramificatio

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Siller Jr., Alfredo, Joseph Jebain, Yomna Amer, Christopher T. Haley, Leon Chen, and Stephen K. Tyring. "Painful Retiform Purpura with Cutaneous Necrosis." SKIN The Journal of Cutaneous Medicine 4, no. 6 (2020): 595–98. http://dx.doi.org/10.25251/skin.4.6.18.

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Levamisole-induced vasculitis is characterized by a painful, purpuric rash in a retiform or stellate pattern with or without central necrosis that commonly involves the trunk, extremities, digits, distal nose, cheeks, and ear helices. This clinical syndrome is associated with the use of levamisole-adultered cocaine. Histologic findings for levamisole-induced vasculitis are not disease specific but may include leukocytoclastic vasculitis, microvascular thrombosis, and panniculitis. Laboratory findings of levamisole-induced toxicity include agranulocytosis, neutropenia, hepatotoxicity, glomerulo

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Trivioli, Giorgio, Benjamin Terrier, and Augusto Vaglio. "Eosinophilic granulomatosis with polyangiitis: understanding the disease and its management." Rheumatology 59, Supplement_3 (2020): iii84—iii94. http://dx.doi.org/10.1093/rheumatology/kez570.

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Abstract Eosinophilic granulomatosis with polyangiitis is characterized by asthma, blood and tissue eosinophilia and small-vessel vasculitis. The clinical presentation is variable, but two main clinic-pathologic subsets can be distinguished: one hallmarked by positive ANCA and predominant ‘vasculitic’ manifestations (e.g. glomerulonephritis, purpura and mononeuritis multiplex) and the other by negative ANCA and prominent ‘eosinophilic’ manifestations (e.g. lung infiltrates and cardiomyopathy). The pathogenesis is not fully understood but probably results from the interplay between T and B cell

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Albers, Jan, Harald Seeger, David Jayne, and Andrea Elisabeth Fisler. "ANCA-associated vasculitis with slowly progressive renal dysfunction: a little-known but treatable disease." BMJ Case Reports 17, no. 11 (2024): e260740. http://dx.doi.org/10.1136/bcr-2024-260740.

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The clinical spectrum of antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) with renal involvement includes forms with a slowly progressive course. These forms are poorly recognised and, therefore, often associated with misdiagnosis and delayed treatment. We present here a case of slowly progressive AAV with renal involvement. A patient in her 50s with long-standing hypertension was evaluated for chronic renal impairment. Laboratory diagnostics revealed mild glomerular disease with relevant proteinuria and glomerular microhaematuria. Furthermore, significantly elevated AN

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Vanga, Amaresh, Amna Z. Rana, Jolanta Kowalewska, and Harlan Rust. "Pericardial Tamponade: An Uncommon Clinical Presentation in cANCA Related Vasculitis and Glomerulonephritis in Association with Very High Titres of ANA." Case Reports in Nephrology 2019 (June 13, 2019): 1–5. http://dx.doi.org/10.1155/2019/4983139.

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ANCA (anti-neutrophil cytoplasmic antibody) vasculitides are systemic autoimmune diseases in which anti-neutrophilic cytoplasmic antibodies activate primed neutrophils, thereby generating an inflammatory cascade resulting in the damage of small sized blood vessels in various organs of the body, including the heart. Pleuropericardial involvement is underrecognized as a complication of ANCA vasculitis and is highlighted in this case report of a 51-year-old male who presented with an initial symptomatic presentation of pleuropericardial effusion progressing to pericardial tamponade in the setting

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Sellares, Jacobo, and Sergio Prieto-González. "Highlights from the plenary session: end-organ damage and repair." Rheumatology 64, Supplement_1 (2025): i101—i103. https://doi.org/10.1093/rheumatology/keae424.

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Abstract The plenary session on “End organ damage and repair” at the 21st International Vasculitis Workshop Congress featured two lectures and three studies addressing the pathogenesis, diagnosis, and management of fibrosis in vasculitis. The studies presented at the session demonstrate the importance of cellular interplay in driving inflammation and fibrosis, like the B cell-fibroblast interactions in the aorta of giant cell arteritis patients and the potential fibrotic role of specific infiltrating macrophage subtypes in ANCA-associated glomerulonephritis. Moreover, organ damage, such as the

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Gaspar, Ana, Anna Lima, Afonso Santos, Catarina Brás, Rita Manso, and Karina Soto. "Systemic lupus erythematosus and ANCA-associated vasculitis overlap syndrome: A case report and literature review of poliautoimmunity." Portuguese Journal of Nephrology & Hypertension 35, no. 1 (2021): 47–50. http://dx.doi.org/10.32932/pjnh.2021.04.118.

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Systemic lupus erythematosus and ANCA-associated vasculitis overlap syndrome is rare and complex. Moreover, the mechanisms that explain the interaction between these two conditions are still unclear. The authors describe the case of a patient who had myeloperoxidase ANCA-associated vasculitis as the initial diagnosis, with biopsy-proven pauci immune crescentic glomerulonephritis, attaining complete remission after immunosuppression. Five years later, Systemic Lupus Erythematosus was diagnosed and a second kidney biopsy showed a pattern of lupus nephritis class III. While on immunosuppression t

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Bulanov, N., E. Stolyarevich, A. Zykova, et al. "AB0497 RENAL INVOLVEMENT IN ANCA-ASSOCIATED VASCULITIS: DO THE PRESENCE OF ANCA AND THEIR TYPE MATTER?" Annals of the Rheumatic Diseases 79, Suppl 1 (2020): 1546.2–1547. http://dx.doi.org/10.1136/annrheumdis-2020-eular.6262.

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Background:The role of ANCA type is well established for the risk of relapses of ANCA-associated vasculitis (AAV). However their association with renal involvement and its outcomes is less well understood.Objectives:To assess clinical and morphological features of ANCA-associated glomerulonephritis (ANCA-GN) and renal survival in ANCA-negative patients, proteinase-3-ANCA (pr3-ANCA) positive and myeloperoxidase-ANCA (MPO-ANCA) positive patients.Methods:We enrolled 53 patients with AAV, diagnosed according to Chapel Hill Consensus Conference (2012) definition and/or ACR (1990) criteria, with his

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Neves, Precil Diego Miranda de Menezes, Lucas Braga Mota, Cristiane Bitencourt Dias, et al. "Methimazole-Induced ANCA Vasculitis: A Case Report." Diagnostics 11, no. 9 (2021): 1580. http://dx.doi.org/10.3390/diagnostics11091580.

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Rapidly progressive glomerulonephritis (RPGN) is a syndrome which presents rapid loss of renal function. Vasculitis represents one of the major causes, often related to anti-neutrophil cytoplasmic antibodies (ANCA). Herein, we report a case of methimazole-induced ANCA-associated vasculitis. A 35-year-old woman complained of weight loss and fatigue for 2 weeks and attended the emergency room with alveolar hemorrhage. She had been diagnosed with Graves’ disease and had been taking methimazole in the past 6 months. Her physical examination showed pulmonary wheezing, hypertension and signs of resp

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Yeturi, Supraja, Mary Cronin, Robin Adam, Campbell Lorna, and Ann K. Rosenthal. "Pauci-Immune Crescentic Glomerulonephritis in Connective Tissue Disease." Case Reports in Rheumatology 2016 (2016): 1–6. http://dx.doi.org/10.1155/2016/9070487.

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Pauci-immune crescentic glomerulonephritis is commonly seen in ANCA-associated vasculitis but it is rarely seen during the course of other connective tissue diseases like lupus or Sjogren’s syndrome or MCTD. We report 3 cases of pauci-immune crescentic glomerulonephritis in patients with connective tissue disease other than vasculitis. We reviewed literature and made summary of previously reported cases of this rare entity. Clinical and laboratory features of these patients varied widely, but most of patients have met criteria for lupus. In this small population of patients there is no correla

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