scholarly journals Systematic Histological Scoring Reveals More Prominent Interstitial Inflammation in Myeloperoxidase-ANCA Compared to Proteinase 3-ANCA Glomerulonephritis

2021 ◽  
Vol 10 (6) ◽  
pp. 1231
Author(s):  
Samy Hakroush ◽  
Ingmar Alexander Kluge ◽  
Philipp Ströbel ◽  
Peter Korsten ◽  
Désirée Tampe ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Clinical Presentation ◽  
Systemic Vasculitis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Laboratory Findings ◽  
Treatment Regimens ◽  
Anca Associated Vasculitis ◽  
Kidney Involvement ◽  
Tubulointerstitial Lesions ◽  
Severe Deterioration

Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic vasculitis, most frequently presenting as microscopic polyangiitis (MPA) or granulomatosis with polyangiitis (GPA). Kidney involvement is a common and severe complication of ANCA AAV which is observed in a considerable subset of patients, mainly affecting glomeruli. However, tubulointerstitial lesions have also been described in ANCA glomerulonephritis (GN). Therefore, we aim to describe active and chronic tubulointerstitial lesions in ANCA GN subtypes by systematic scoring analogous to the Banff scoring system while also utilizing clinical and laboratory findings. Methods: A total of 49 kidney biopsies with ANCA GN were retrospectively included in a single-center cohort study between 2015–2020. Results: We report that MPO-ANCA GN is associated with more severe deterioration of kidney function independent of systemic markers of AAV disease activity, and is also associated with increased proteinuria in MPO-ANCA GN and a decreased fraction of normal glomeruli. Finally, MPO-ANCA GN showed distinct, active, and chronic tubulointerstitial lesions. Conclusion: New insights into the pathophysiology of both entities, as well as differences in the clinical presentation of MPO- versus PR3-ANCA GN, could potentially pave the way for more precise treatment regimens. Therefore, it is important to understand the differences in histopathological presentation, especially in yet underestimated active tubulointerstitial lesions of ANCA GN subtypes. This research could further improve our understanding of distinct pathophysiological mechanisms.

Association of venous thromboembolic events with skin, pulmonary and kidney involvement in ANCA-associated vasculitis: a multinational study

Rheumatology ◽  
2021 ◽  
Author(s):  
Sergey Moiseev ◽  
Andreas Kronbichler ◽  
Egor Makarov ◽  
Nikolay Bulanov ◽  
Matija Crnogorac ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Thromboembolic Events ◽  
The European Union ◽  
Similar Frequency ◽  
Anca Associated Vasculitis ◽  
Kidney Involvement ◽  
Venous Thromboembolic Events ◽  
Venous Thromboembolic

Abstract Objective To investigate the occurrence of venous thromboembolic events (VTE) in a large cohort of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) across the European Union, Turkey, Russia, UK and North America. Methods Patients with a definite diagnosis of AAV who were followed for at least 3 months and had sufficient documentation were included. Data on VTE, including either deep vein thrombosis or pulmonary embolism, were collected retrospectively from tertiary vasculitis centres. Univariate and multivariate regression models were used to estimate odds ratios (ORs) and 95% CIs. Results Over a median follow-up of 63 (interquartile range: 29, 101) months, VTE occurred in 278 (9.7%) of 2869 AAV patients with a similar frequency across different countries (from 6.3% to 13.7%), and AAV subtype [granulomatosis with polyangiitis: 9.8% (95% CI: 8.3, 11.6%); microscopic polyangiitis: 9.6% (95% CI: 7.9, 11.4%); and eosinophilic granulomatosis with polyangiitis: 9.8% (95% CI: 7.0, 13.3%)]. Most VTE (65.6%) were reported in the first-year post-diagnosis. Multiple factor logistic regression analysis adjusted for sex and age showed that skin (OR 1.71, 95% CI: 1.01, 2.92), pulmonary (OR 1.78, 95% CI: 1.04, 3.14) and kidney [eGFR 15–60 ml/min/1.73 m2, OR 2.86 (95% CI: 1.27, 6.47); eGFR <15 ml/min/1.73 m2, OR 6.71 (95% CI: 2.94, 15.33)] involvement were independent variables associated with a higher occurrence of VTE. Conclusion Two-thirds of VTE occurred during the initial phase of active disease. We confirmed previous findings from smaller studies that a decrease in kidney function, skin involvement and pulmonary disease are independently associated with VTE.

Prevalence and Risk Factors for Major Infections in Patients with Antineutrophil Cytoplasmic Antibody–associated Vasculitis: Influence on the Disease Outcome

2019 ◽  
Vol 47 (3) ◽  
pp. 407-414 ◽  
Author(s):  
Eloi Garcia-Vives ◽  
Alfons Segarra-Medrano ◽  
Ferran Martinez-Valle ◽  
Irene Agraz ◽  
Roser Solans-Laque
Keyword(s):  
Bacterial Infections ◽  
Granulomatosis With Polyangiitis ◽  
Opportunistic Infections ◽  
Disease Onset ◽  
Antineutrophil Cytoplasmic Antibody ◽  
First Year ◽  
Laboratory Findings ◽  
Anca Associated Vasculitis ◽  
Tertiary Center ◽  
Tract Infections

Objective.To analyze the role that infections play on the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) outcome.Methods.A retrospective study of adult patients with AAV diagnosed in a tertiary center. Clinical features, laboratory findings, treatment, relapses, major infections, and outcome were evaluated.Results.Included were 132 patients [51 microscopic polyangiitis (MPA), 52 granulomatosis with polyangiitis (GPA), 29 eosinophilic GPA (EGPA)] with a mean followup of 140 (96–228) months. ANCA were positive in 85% of cases. A total of 300 major infections, mainly bacterial (85%), occurred in 60% patients during the followup. Lower respiratory tract (64%) and urinary tract infections (11%) were the most frequent, followed by bacteremia (10%). A total of 7.3% opportunistic infections were observed, most due to systemic mycosis. Up to 46% of all opportunistic infections took place in the first year of diagnosis, and 55% of them under cyclophosphamide (CYC) treatment. Bacterial infections were associated with Birmingham Vasculitis Activity Score (version 3) > 15 at the disease onset, a total cumulative CYC dose > 8.65 g, dialysis, and development of leukopenia during the followup. Leukopenia was the only factor independently related to opportunistic infections. Forty-four patients died, half from infection. Patients who had major infections had an increased mortality from any cause.Conclusion.Our results confirm that major infections are the main cause of death in patients with AAV.

scholarly journals A case of ANCA associated vasculitis presented with mononeuritis multiplex

2019 ◽  
Vol 9 (3) ◽  
pp. 257-260
Author(s):  
Tanbin Rahman ◽  
Md Rashedul Islam ◽  
Mohammad Sakhawat Hossen Khan ◽  
Sharif Mohammad Ruhul Quddus ◽  
Dilruba Alam ◽  
...  
Keyword(s):  
Inflammatory Cell ◽  
Granulomatosis With Polyangiitis ◽  
Microscopic Polyangiitis ◽  
Systemic Vasculitis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Mononeuritis Multiplex ◽  
Good Clinical Response ◽  
Standard Regimen ◽  
Anca Associated Vasculitis ◽  
Common Manifestation

Mononeuritis multiplex is a common manifestation of many illnesses which includes diabetes, leprosy, malignancy and certain types of systemic vasculitis. The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of rare diseases which show typical characteristic inflammatory cell infiltration and blood vessel wall necrosis. AAV syndromes include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilicgranulomatosis with polyangiitis (EGPA). Here we present a patient who presented with mononeuritis multiplex and had features ofEGPA. The patient was treated with standard regimen of steroids and pulsed cyclophosphamide and she achieved good clinical response. Birdem Med J 2019; 9(3): 257-260

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.”

Morbidity and mortality associated with subglottic laryngotracheal stenosis in granulomatosis with polyangiitis (Wegener's granulomatosis): a single-centre experience in the United Kingdom

2014 ◽  
Vol 128 (9) ◽  
pp. 831-837 ◽  
Author(s):  
N P Jordan ◽  
H Verma ◽  
A Siddiqui ◽  
G A Morrison ◽  
D P D'Cruz
Keyword(s):  
United Kingdom ◽  
Wegener’S Granulomatosis ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Wegener's Granulomatosis ◽  
Laryngotracheal Stenosis ◽  
Surgical Interventions ◽  
The United Kingdom ◽  
Single Centre Experience

AbstractObjectives:We aimed to determine the prevalence of symptomatic subglottic laryngotracheal stenosis in patients with granulomatosis with polyangiitis (Wegener's granulomatosis); we also wanted to characterise the clinical outcomes and surgical interventions required, and the relapse rate in our cohort.Methods:We undertook a retrospective clinical review of all granulomatosis with polyangiitis patients with symptomatic subglottic laryngotracheal stenosis attending St Thomas' Hospital, London, United Kingdom.Results:Symptomatic subglottic laryngotracheal stenosis developed in 16 per cent of granulomatosis with polyangiitis patients attending our clinic. The median age of patients at diagnosis was 44 years (range: 34–81 years); 78 per cent of those presenting with subglottic laryngotracheal stenosis were women and 22 per cent were men. All patients were white; 67 per cent of patients were proteinase 3-antineutrophil cytoplasmic antibody-positive and 67 per cent developed relapsing disease requiring repeated surgical intervention. Subglottic laryngotracheal stenosis relapse was not associated with active systemic vasculitis elsewhere.Conclusion:Subglottic laryngotracheal stenosis is an uncommon but significant complication of granulomatosis with polyangiitis. Management of subglottic laryngotracheal stenosis requires a multi-disciplinary approach, with both rheumatological and otolaryngological expertise involved, given the relapsing nature of the disease.

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 Peripheral neuropathy in antineutrophil cytoplasmic antibody-associated vasculitides

2019 ◽  
Vol 6 (6) ◽  
pp. e615 ◽  
Author(s):  
Antje Bischof ◽  
Veronika K. Jaeger ◽  
Robert D. M. Hadden ◽  
Raashid A. Luqmani ◽  
Anne-Katrin Pröbstel ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Nerve Biopsy ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Vasculitic Neuropathy ◽  
Diagnostic Certainty ◽  
Primary Systemic Vasculitis ◽  
Organ Manifestations ◽  
Eosinophilic Granulomatosis

ObjectiveReported prevalence of vasculitic neuropathy (VN) in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is highly variable, and associations with other organ manifestations have not been studied systematically while accounting for diagnostic certainty of VN.MethodsData of all patients with AAV within the Diagnostic and Classification criteria for primary systemic VASculitis study were analyzed cross-sectionally. VN was categorized as definite (histology proven), probable (multiple mononeuropathy or nerve biopsy consistent with vasculitis), or possible (all others). Associations with other organ manifestations were compared in patients with and without VN.ResultsNine hundred fifty-five patients (mean age 57 years, range 18–91 years, 51% female) were identified. Of these, 572 had granulomatosis with polyangiitis (GPA), 218 microscopic polyangiitis (MPA), and 165 eosinophilic granulomatosis with polyangiitis (EGPA). The prevalence of VN was 65% in EGPA, 23% in MPA, and 19% in GPA. Nerve biopsy was performed in 32/269 (12%) patients, demonstrating definite vasculitis in 17/32 (53%) of patients. VN was associated with myeloperoxidase-ANCA positivity (p = 0.004) and skin (p < 0.001), musculoskeletal, (p < 0.001) and cardiovascular (p = 0.005) involvement. Patients with VN were less likely to have renal (p < 0.001), eye (p < 0.001), and gastrointestinal (p = 0.023) involvement.ConclusionsOur study provides comprehensive insights into the prevalence and organ associations of VN in a large, systematically collected AAV cohort. VN is most commonly associated with skin, musculoskeletal, and cardiovascular manifestations. In routine clinical practice, diagnosis of VN is infrequently confirmed by the gold standard of nerve biopsy but rather supported by the clinical setting of active systemic AAV.

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.

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