scholarly journals Classification, epidemiology and clinical subgrouping of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis

2015 ◽  
Vol 30 (suppl_1) ◽  
pp. i14-i22 ◽  
Author(s):  
Richard A. Watts ◽  
Alfred Mahr ◽  
Aladdin J. Mohammad ◽  
Paul Gatenby ◽  
Neil Basu ◽  
...  
Keyword(s):  
Antineutrophil Cytoplasmic Antibody ◽  
Anca Associated Vasculitis

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.”

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 ANCA-Associated Glomerulonephritis and Anti-Phospholipid Syndrome in a Patient with SARS-CoV-2 Infection: Just a Coincidence?

2021 ◽  
pp. 214-220
Author(s):  
Federica Maritati ◽  
Maria Ilaria Moretti ◽  
Valentina Nastasi ◽  
Roberta Mazzucchelli ◽  
Manrico Morroni ◽  
...  
Keyword(s):  
Rapidly Progressive Glomerulonephritis ◽  
Kidney Injury ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Hemodynamic Instability ◽  
Tubular Necrosis ◽  
Complex Processes ◽  
Anca Associated Vasculitis ◽  
High Incidence ◽  
New Diagnosis ◽  
The Relationship

Many reports have described a high incidence of acute kidney injury (AKI) among patients with COVID-19. Acute tubular necrosis has been reported to be the most common damage in these patients, probably due to hemodynamic instability. However, other complex processes may be involved, related to the cytokine storm and the activation of innate and adaptive immunity. Here, we describe a patient who developed an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis with rapidly progressive glomerulonephritis and lung involvement and an antiphospholipid syndrome soon after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. After viral pneumonia was excluded by bronchoalveolar lavage, the patient has been treated with rituximab for amelioration of kidney function and resolution of thrombosis without any adverse event. We conclude that COVID-19 may trigger autoimmune diseases including ANCA-associated vasculitis. Thus, this diagnosis should be taken in consideration in COVID-19 patients, especially when they develop AKI with active urinary sediment. In addition, considering the relationship between these 2 diseases, SARS-CoV-2 infection should be excluded in all patients with a new diagnosis ANCA-associated vasculitis before starting immunosuppressive therapy.

scholarly journals Granulomatosis with Polyangiitis: Recurrence or Treatment Consequences?

2021 ◽  
Author(s):  
Andreia Diegues ◽  
Joana Tavares ◽  
Diogo Sá ◽  
João Oliveira ◽  
Diana Fernandes ◽  
...  
Keyword(s):  
Intracranial Hypertension ◽  
Clinical Improvement ◽  
Granulomatosis With Polyangiitis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Lung Nodules ◽  
Rare Form ◽  
Anca Associated Vasculitis

Granulomatosis with polyangiitis (GPA) is the most common antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We describe the case of a 38-year-old woman with relapsing GPA who presented with intracranial hypertension, followed by the appearance of cavitated lung nodules despite treatment with azathioprine. Clinical improvement and ANCA titre reduction were observed after rituximab treatment. We report a rare form of GPA relapse and highlight the challenge of following-up patients with GPA, in whom can be hard to distinguish relapse from the consequences of long-term immunosuppression.

scholarly journals Comorbidities and health-related quality of life in Patients with Antineutrophil Cytoplasmic Antibody (ANCA) - associated vasculitis

2021 ◽  
Vol 20 (1) ◽  
pp. 102708
Author(s):  
Cédric Mercuzot ◽  
Simon Letertre ◽  
Claire I Daien ◽  
Laetitia Zerkowski ◽  
Philippe Guilpain ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Health Related Quality ◽  
Anca Associated Vasculitis ◽  
Related Quality ◽  
Health Related

scholarly journals Granulomatosis with Polyangiitis Restricted to the Back Muscle: The First Case Report

Medicina ◽  
2020 ◽  
Vol 56 (9) ◽  
pp. 431
Author(s):  
Yu-Mi Lee ◽  
Sang Wan Chung
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Final Diagnosis ◽  
High Dose ◽  
Back Muscle ◽  
Anca Associated Vasculitis ◽  
First Case ◽  
Psoas Muscles ◽  
High Dose Methylprednisolone ◽  
Single Organ

Granulomatosis with polyangiitis (GPA) is an autoimmune disease which is a type of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis that frequently affects the lungs and kidneys. However, GPA limited to a single organ has also been reported. A 71-year-old man was admitted for back pain and fever. We detected elevated levels of inflammatory markers and myeloperoxidase-ANCA. Magnetic resonance imaging indicated diffuse inflammation of the back and psoas muscles. Histology showed degenerated muscle fibers and granulomatosis vasculitis with mixed lymphoplasma cell infiltration. High-dose methylprednisolone therapy improved his symptoms. A final diagnosis of GPA limited to the muscles was made.

scholarly journals Imbalance of Circulatory T Follicular Helper and T Follicular Regulatory Cells in Patients with ANCA-Associated Vasculitis

2019 ◽  
Vol 2019 ◽  
pp. 1-9
Author(s):  
Ying Xu ◽  
Hongmei Xu ◽  
Yu Zhen ◽  
Xueting Sang ◽  
Hao Wu ◽  
...  
Keyword(s):  
Immunosuppressive Treatment ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Healthy Controls ◽  
Regulatory Cells ◽  
Humoral Immune ◽  
Tfh Cells ◽  
Humoral Immune Responses ◽  
Follicular Helper ◽  
Anca Associated Vasculitis ◽  
Kidney Involvement

Antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV) is characterized by small-vessel inflammation in association with autoantibodies. Balance between T follicular helper (Tfh) cells and T follicular regulatory (Tfr) cells is critical for humoral immune responses. Accumulating evidence supports that Tfh and Tfr are involved in autoimmune diseases; however, their roles in AAV are unclear. In this study, we tested the changes of circulatory Tfh and Tfr in patients with AAV. Twenty patients with AAV and twenty healthy controls were enrolled. Sixteen AAV patients had kidney involvement. We found that the AAV patients had increased circulating Tfh cells (CD4+CXCR5+CD25−CD127interm-hi), decreased Tfr cells (CD4+CXCR5+CD25+CD127lo-interm), and elevated Tfh/Tfr ratios compared with healthy controls (P<0.01). The Tfh percentage and Tfh/Tfr ratio, but not Tfr percentage, were positively correlated to proteinuria levels and BVAS scores in patients with AAV (P<0.01). In addition, AAV patients had decreased circulating Tfh1 (CCR6-CXCR3+), but increased Tfh2 cells (CCR6-CXCR3-), compared with healthy controls (P<0.01), indicating a Tfh1-to-Tfh2 shift. Furthermore, remission achieved by immunosuppressive treatment markedly attenuated the increase of total Tfh (P<0.01) and Tfh2 cells (P<0.05), promoted the Tfh1 response (P<0.05), and recovered the balance between Tfh/Tfr cells (P<0.05) and between Tfh1/Tfh2 cells (P<0.05) in patients with AAV. Plasma levels of IL-21, a cytokine secreted by Tfh cells, were elevated in AAV patients compared with healthy controls (P<0.01), which was attenuated by immunosuppressive treatment (P<0.05). Taken together, our findings indicate that circulatory Tfh/Tfr ratios, Tfh2/Tfh1 shift, and plasma IL-21 levels are associated with AAV and disease activity.

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.

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