The Diagnostic and Clinical Utility of Autoantibodies in Systemic Vasculitis
Considerable progress has been made in understanding the role of autoantibodies in systemic vasculitides (SV), and consequently testing for anti-neutrophil cytoplasmic antibodies (ANCA), anti-glomerular basement membrane antibodies (anti-GBM), and anti-C1q antibodies is helpful and necessary in the...
| Main Author: | |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2019-05-01
|
| Series: | Antibodies |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2073-4468/8/2/31 |
| _version_ | 1819141855869140992 |
|---|---|
| author | Elena Csernok |
| author_facet | Elena Csernok |
| author_sort | Elena Csernok |
| collection | DOAJ |
| description | Considerable progress has been made in understanding the role of autoantibodies in systemic vasculitides (SV), and consequently testing for anti-neutrophil cytoplasmic antibodies (ANCA), anti-glomerular basement membrane antibodies (anti-GBM), and anti-C1q antibodies is helpful and necessary in the diagnosis, prognosis, and monitoring of small-vessel vasculitis. ANCA-directed proteinase 3 (PR3-) or myeloperoxidase (MPO-) are sensitive and specific serologic markers for ANCA-associated vasculitides (AAV), anti-GBM antibodies are highly specific for the patients with anti-GBM antibody disease (formerly Goodpasture’s syndrome), and autoantibodies to C1q are characteristic of hypocomlementemic urticarial vasculitis syndrome (HUVS; anti-C1q vasculitis). The results of a current EUVAS study have led to changes in the established strategy for the ANCA testing in small-vessel vasculitis. The revised 2017 international consensus recommendations for ANCA detection support the primary use PR3- and MPO-ANCA immunoassays without the categorical need for additional indirect immunofluorescence (IIF). Interestingly, the presence of PR3- and MPO-ANCA have led to the differentiation of distinct disease phenotype of AAV: PR3-ANCA-associated vasculitis (PR3-AAV), MPO-ANCA-associated vasculitis (MPO-AAV), and ANCA-negative vasculitis. Further studies on the role of these autoantibodies are required to better categorize and manage appropriately the patients with small-vessel vasculitis and to develop more targeted therapy. |
| first_indexed | 2024-12-22T12:01:05Z |
| format | Article |
| id | doaj.art-9b422257e4364b05a964f4ec797cdf78 |
| institution | Directory Open Access Journal |
| issn | 2073-4468 |
| language | English |
| last_indexed | 2024-12-22T12:01:05Z |
| publishDate | 2019-05-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Antibodies |
| spelling | doaj.art-9b422257e4364b05a964f4ec797cdf782022-12-21T18:26:37ZengMDPI AGAntibodies2073-44682019-05-01823110.3390/antib8020031antib8020031The Diagnostic and Clinical Utility of Autoantibodies in Systemic VasculitisElena Csernok0Department of Internal Medicine, Rheumatology and Immunology, Vasculitis-Center Tübingen-Kirchheim, Medius Klinik Kirchheim, University of Tübingen, 73230 Kirchheim-Teck, GermanyConsiderable progress has been made in understanding the role of autoantibodies in systemic vasculitides (SV), and consequently testing for anti-neutrophil cytoplasmic antibodies (ANCA), anti-glomerular basement membrane antibodies (anti-GBM), and anti-C1q antibodies is helpful and necessary in the diagnosis, prognosis, and monitoring of small-vessel vasculitis. ANCA-directed proteinase 3 (PR3-) or myeloperoxidase (MPO-) are sensitive and specific serologic markers for ANCA-associated vasculitides (AAV), anti-GBM antibodies are highly specific for the patients with anti-GBM antibody disease (formerly Goodpasture’s syndrome), and autoantibodies to C1q are characteristic of hypocomlementemic urticarial vasculitis syndrome (HUVS; anti-C1q vasculitis). The results of a current EUVAS study have led to changes in the established strategy for the ANCA testing in small-vessel vasculitis. The revised 2017 international consensus recommendations for ANCA detection support the primary use PR3- and MPO-ANCA immunoassays without the categorical need for additional indirect immunofluorescence (IIF). Interestingly, the presence of PR3- and MPO-ANCA have led to the differentiation of distinct disease phenotype of AAV: PR3-ANCA-associated vasculitis (PR3-AAV), MPO-ANCA-associated vasculitis (MPO-AAV), and ANCA-negative vasculitis. Further studies on the role of these autoantibodies are required to better categorize and manage appropriately the patients with small-vessel vasculitis and to develop more targeted therapy.https://www.mdpi.com/2073-4468/8/2/31autoantibodiesvasculitisdiagnostic and clinical significanceproteinase 3-/myeloperoxidase-ANCAnew recommendations |
| spellingShingle | Elena Csernok The Diagnostic and Clinical Utility of Autoantibodies in Systemic Vasculitis Antibodies autoantibodies vasculitis diagnostic and clinical significance proteinase 3-/myeloperoxidase-ANCA new recommendations |
| title | The Diagnostic and Clinical Utility of Autoantibodies in Systemic Vasculitis |
| title_full | The Diagnostic and Clinical Utility of Autoantibodies in Systemic Vasculitis |
| title_fullStr | The Diagnostic and Clinical Utility of Autoantibodies in Systemic Vasculitis |
| title_full_unstemmed | The Diagnostic and Clinical Utility of Autoantibodies in Systemic Vasculitis |
| title_short | The Diagnostic and Clinical Utility of Autoantibodies in Systemic Vasculitis |
| title_sort | diagnostic and clinical utility of autoantibodies in systemic vasculitis |
| topic | autoantibodies vasculitis diagnostic and clinical significance proteinase 3-/myeloperoxidase-ANCA new recommendations |
| url | https://www.mdpi.com/2073-4468/8/2/31 |
| work_keys_str_mv | AT elenacsernok thediagnosticandclinicalutilityofautoantibodiesinsystemicvasculitis AT elenacsernok diagnosticandclinicalutilityofautoantibodiesinsystemicvasculitis |