scholarly journals Proteinase 3 (PR3)-antineutrophil cytoplasmic antibody (ANCA)-associated vasculitic neuropathy in diffuse cutaneous systemic sclerosis: a rare duo

2019 ◽  
Vol 12 (11) ◽  
pp. e232987 ◽  
Author(s):  
Yasser Radwan ◽  
Sarah Berini ◽  
Floranne Ernste ◽  
Ashima Makol
Keyword(s):  
Systemic Sclerosis ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Proteinase 3 ◽  
Vasculitic Neuropathy ◽  
Anca Associated Vasculitis ◽  
Common Clinical Presentation ◽  
Risk Of Disease ◽  
Diffuse Cutaneous Systemic Sclerosis

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 SSc for 6 years presented with new-onset peripheral neuropathy. Workup revealed a positive proteinase-3 and cytoplasmic ANCA, and histopathology confirmed an inflammatory vasculitic neuropathy. The patient was successfully treated with rituximab. Our case highlights the importance of checking ANCA in SSc at baseline, given the risk of disease-related complications, even years later. Tissue biopsy is often warranted for confirmation of vasculitis and prompt treatment can optimise long-term outcomes.

scholarly journals Increase of Antimyeloperoxidase Antineutrophil Cytoplasmic Antibody (ANCA) in Patients with Renal ANCA-associated Vasculitis: Association with Risk to Relapse

2015 ◽  
Vol 42 (10) ◽  
pp. 1853-1860 ◽  
Author(s):  
Makoto Yamaguchi ◽  
Masahiko Ando ◽  
Sawako Kato ◽  
Takayuki Katsuno ◽  
Noritoshi Kato ◽  
...  
Keyword(s):  
Regression Models ◽  
Cox Regression ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Time Dependent ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Anca Associated Vasculitis ◽  
Level Increase ◽  
Diagnostic Values ◽  
Renal Vasculitis ◽  
Risk Of Disease

Objective.The diagnostic values of antiproteinase 3 and antimyeloperoxidase tests using antineutrophil cytoplasmic antibodies (ANCA) are well established. Our study determined whether an increase in ANCA level was a predictor of disease flareup.Methods.Our study included 126 patients with ANCA-associated renal vasculitis treated at 9 nephrology centers in Japan. The relationship between increased ANCA levels and relapse was assessed using time-dependent multivariate Cox regression models adjusted for clinically relevant factors. The outcome of interest was the time from remission to first relapse.Results.During the observation period [median 41 mos, interquartile range (IQR) 23–66 mos], 118 patients (95.8%) achieved remission at least once. After achieving remission, 34 patients relapsed (21.7%). Time-dependent multivariate Cox regression models revealed that lung involvement (adjusted HR 2.29, 95% CI 1.13–4.65, p = 0.022) and increased ANCA levels (adjusted HR 17.4, 95% CI 8.42–36.0, p < 0.001) were significantly associated with relapse. The median time from ANCA level increase to relapse was 0.6 months (IQR 0–2.1 mos).Conclusion.In our study, an increase in ANCA level during remission was associated with a risk of disease relapse. A rise in ANCA level may be useful for guiding treatment decisions in appropriate subsets of patients with ANCA-associated vasculitis.

scholarly journals Clinical Utility of Serial Measurements of Antineutrophil Cytoplasmic Antibodies Targeting Proteinase 3 in ANCA-Associated Vasculitis

2020 ◽  
Vol 11 ◽  
Author(s):  
Gwen E. Thompson ◽  
Lynn A. Fussner ◽  
Amber M. Hummel ◽  
Darrell R. Schroeder ◽  
Francisco Silva ◽  
...  
Keyword(s):  
Clinical Utility ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Proteinase 3 ◽  
Anca Associated Vasculitis ◽  
Serial Measurements

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 053. CLINICAL UTILITY OF SERIAL MEASUREMENTS OF ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES TARGETING PROTEINASE 3 IN ANCA-ASSOCIATED VASCULITIS

Rheumatology ◽  
2019 ◽  
Vol 58 (Supplement_2) ◽  
Author(s):  
Gwen Thompson ◽  
Lynn Fussner ◽  
Amber Hummel ◽  
Darrell Schroeder ◽  
Francisco Silva ◽  
...  
Keyword(s):  
Clinical Utility ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Proteinase 3 ◽  
Anca Associated Vasculitis ◽  
Serial Measurements

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.

Induction of neutrophil responsiveness to myeloperoxidase antibodies by their exposure to supernatant of degranulated autologous neutrophils

Blood ◽  
2000 ◽  
Vol 96 (8) ◽  
pp. 2822-2827 ◽  
Author(s):  
Christoph Hess ◽  
Salima Sadallah ◽  
Jürg-Alfred Schifferli
Keyword(s):  
Monoclonal Antibody ◽  
Cell Surface ◽  
Oxygen Radicals ◽  
Disease Process ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Polymorphonuclear Neutrophils ◽  
Proteinase 3 ◽  
Clinical Expression ◽  
Anca Vasculitis ◽  
Anca Associated Vasculitis

Abstract Antibodies against myeloperoxidase (MPO) and proteinase 3 (PR3) are the predominant autoantibodies present in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Their binding to the corresponding antigen on the surface of polymorphonuclear neutrophils (PMNs) is believed to trigger the disease process. Cytokines released during an inflammatory reaction are thought to prime resting PMNs, making them responsive to autoantibodies. In the present study we found that MPO but not PR3 could be detected on the cell surface of unstimulated PMNs after incubation with the supernatants of activated autologous PMNs. MPO was shown to be acquired from these supernatants, because PMNs did not express MPO when the supernatants were specifically MPO-depleted. In addition, purified soluble MPO bound to unstimulated PMNs. Unstimulated PMNs that had passively acquired MPO released oxygen radicals when incubated with monoclonal antibody anti-MPO or the immunoglobulin G fraction of a patient with MPO-ANCA. The data presented here suggest that, in ANCA-associated vasculitis, soluble MPO released by activated PMNs may bind to unstimulated PMNs, thereby making them reactive to anti-MPO antibodies. This mechanism of dispersing PMN activation would be specific for MPO-ANCA and may explain differences in the pathologic and clinical expression of MPO-ANCA versus PR3-ANCA vasculitis.

Induction of neutrophil responsiveness to myeloperoxidase antibodies by their exposure to supernatant of degranulated autologous neutrophils

Blood ◽  
2000 ◽  
Vol 96 (8) ◽  
pp. 2822-2827 ◽  
Author(s):  
Christoph Hess ◽  
Salima Sadallah ◽  
Jürg-Alfred Schifferli
Keyword(s):  
Monoclonal Antibody ◽  
Cell Surface ◽  
Oxygen Radicals ◽  
Disease Process ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Polymorphonuclear Neutrophils ◽  
Proteinase 3 ◽  
Clinical Expression ◽  
Anca Vasculitis ◽  
Anca Associated Vasculitis

Antibodies against myeloperoxidase (MPO) and proteinase 3 (PR3) are the predominant autoantibodies present in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Their binding to the corresponding antigen on the surface of polymorphonuclear neutrophils (PMNs) is believed to trigger the disease process. Cytokines released during an inflammatory reaction are thought to prime resting PMNs, making them responsive to autoantibodies. In the present study we found that MPO but not PR3 could be detected on the cell surface of unstimulated PMNs after incubation with the supernatants of activated autologous PMNs. MPO was shown to be acquired from these supernatants, because PMNs did not express MPO when the supernatants were specifically MPO-depleted. In addition, purified soluble MPO bound to unstimulated PMNs. Unstimulated PMNs that had passively acquired MPO released oxygen radicals when incubated with monoclonal antibody anti-MPO or the immunoglobulin G fraction of a patient with MPO-ANCA. The data presented here suggest that, in ANCA-associated vasculitis, soluble MPO released by activated PMNs may bind to unstimulated PMNs, thereby making them reactive to anti-MPO antibodies. This mechanism of dispersing PMN activation would be specific for MPO-ANCA and may explain differences in the pathologic and clinical expression of MPO-ANCA versus PR3-ANCA vasculitis.

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