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1. International Consensus on ANCA Testing in Eosinophilic Granulomatosis with Polyangiitis

Author

Moiseev, S, Bossuyt, X, Arimura, Y, Blockmans, D, Csernok, E, Damoiseaux, J, Emmi, G, Flores-Suárez, L, Hellmich, B, Jayne, D, Jennette, J, Little, M, Mohammad, A, Moosig, F, Novikov, P, Pagnoux, C, Radice, A, Sada, K, Segelmark, M, Shoenfeld, Y, Sinico, R, Specks, U, Terrier, B, Tzioufas, A, Vaglio, A, Zhao, M, Cohen Tervaert, J, Moiseev, Sergey, Bossuyt, Xavier, Arimura, Yoshihiro, Blockmans, Daniel, Csernok, Elena, Damoiseaux, Jan, Emmi, Giacomo, Flores-Suárez, Luis Felipe, Hellmich, Bernhard, Jayne, David, Jennette, J Charles, Little, Mark A, Mohammad, Aladdin J, Moosig, Frank, Novikov, Pavel, Pagnoux, Christian, Radice, Antonella, Sada, Ken-Ei, Segelmark, Mårten, Shoenfeld, Yehuda, Sinico, Renato A, Specks, Ulrich, Terrier, Benjamin, Tzioufas, Athanasios G, Vaglio, Augusto, Zhao, Ming-Hui, Cohen Tervaert, Jan Willem, Moiseev, S, Bossuyt, X, Arimura, Y, Blockmans, D, Csernok, E, Damoiseaux, J, Emmi, G, Flores-Suárez, L, Hellmich, B, Jayne, D, Jennette, J, Little, M, Mohammad, A, Moosig, F, Novikov, P, Pagnoux, C, Radice, A, Sada, K, Segelmark, M, Shoenfeld, Y, Sinico, R, Specks, U, Terrier, B, Tzioufas, A, Vaglio, A, Zhao, M, Cohen Tervaert, J, Moiseev, Sergey, Bossuyt, Xavier, Arimura, Yoshihiro, Blockmans, Daniel, Csernok, Elena, Damoiseaux, Jan, Emmi, Giacomo, Flores-Suárez, Luis Felipe, Hellmich, Bernhard, Jayne, David, Jennette, J Charles, Little, Mark A, Mohammad, Aladdin J, Moosig, Frank, Novikov, Pavel, Pagnoux, Christian, Radice, Antonella, Sada, Ken-Ei, Segelmark, Mårten, Shoenfeld, Yehuda, Sinico, Renato A, Specks, Ulrich, Terrier, Benjamin, Tzioufas, Athanasios G, Vaglio, Augusto, Zhao, Ming-Hui, and Cohen Tervaert, Jan Willem

Abstract

An international consensus on anti-neutrophil cytoplasm antibodies (ANCA) testing in eosinophilic granulomatosis with polyangiitis (EGPA) is presented. ANCA, specific for myeloperoxidase (MPO), can be detected in 30-35% of EGPA patients. MPO-ANCA should be tested with antigen-specific immunoassays in any patient with eosinophilic asthma and clinical features suggesting EGPA, including constitutional symptoms, purpura, polyneuropathy, unexplained heart, gastrointestinal or kidney disease, and/or pulmonary infiltrates or hemorrhage. A positive MPO-ANCA result contributes to the diagnostic work‐up for EGPA. Patients with MPO-ANCA associated EGPA have more frequently vasculitis features, such as glomerulonephritis, neuropathy, and skin manifestations than patients with ANCA negative EGPA. However, the presence of MPO-ANCA is neither sensitive nor specific enough to identify whether a patient should be subclassified as having "vasculitic" or "eosinophilic" EGPA. At present, ANCA status cannot guide treatment decisions, that is, whether cyclophosphamide, rituximab or mepolizumab should be added to conventional glucocorticoid treatment. In EGPA, monitoring of ANCA is only useful when MPO-ANCA was tested positive at disease onset.

Published
2020

2. 2020 international consensus on ANCA testing beyond systemic vasculitis

Author

Moiseev, S, Tervaert, J, Arimura, Y, Bogdanos, D, Csernok, E, Damoiseaux, J, Ferrante, M, Flores-Suárez, L, Fritzler, M, Invernizzi, P, Jayne, D, Jennette, J, Little, M, Mcadoo, S, Novikov, P, Pusey, C, Radice, A, Salama, A, Savige, J, Segelmark, M, Shoenfeld, Y, Sinico, R, Sousa, M, Specks, U, Terrier, B, Tzioufas, A, Vermeire, S, Zhao, M, Bossuyt, X, Moiseev, Sergey, Tervaert, Jan Willem Cohen, Arimura, Yoshihiro, Bogdanos, Dimitrios P., Csernok, Elena, Damoiseaux, Jan, Ferrante, Marc, Flores-Suárez, Luis Felipe, Fritzler, Marvin J., Invernizzi, Pietro, Jayne, David, Jennette, J. Charles, Little, Mark, McAdoo, Stephen P., Novikov, Pavel, Pusey, Charles D., Radice, Antonella, Salama, Alan D., Savige, Judith A., Segelmark, Mårten, Shoenfeld, Yehuda, Sinico, Renato A., Sousa, Maria-José, Specks, Ulrich, Terrier, Benjamin, Tzioufas, Athanasios G., Vermeire, Severine, Zhao, Ming-Hui, Bossuyt, Xavier, Moiseev, S, Tervaert, J, Arimura, Y, Bogdanos, D, Csernok, E, Damoiseaux, J, Ferrante, M, Flores-Suárez, L, Fritzler, M, Invernizzi, P, Jayne, D, Jennette, J, Little, M, Mcadoo, S, Novikov, P, Pusey, C, Radice, A, Salama, A, Savige, J, Segelmark, M, Shoenfeld, Y, Sinico, R, Sousa, M, Specks, U, Terrier, B, Tzioufas, A, Vermeire, S, Zhao, M, Bossuyt, X, Moiseev, Sergey, Tervaert, Jan Willem Cohen, Arimura, Yoshihiro, Bogdanos, Dimitrios P., Csernok, Elena, Damoiseaux, Jan, Ferrante, Marc, Flores-Suárez, Luis Felipe, Fritzler, Marvin J., Invernizzi, Pietro, Jayne, David, Jennette, J. Charles, Little, Mark, McAdoo, Stephen P., Novikov, Pavel, Pusey, Charles D., Radice, Antonella, Salama, Alan D., Savige, Judith A., Segelmark, Mårten, Shoenfeld, Yehuda, Sinico, Renato A., Sousa, Maria-José, Specks, Ulrich, Terrier, Benjamin, Tzioufas, Athanasios G., Vermeire, Severine, Zhao, Ming-Hui, and Bossuyt, Xavier

Abstract

This document follows up on a 2017 revised international consensus on anti-neutrophil cytoplasm antibodies (ANCA) testing in granulomatosis with polyangiitis and microscopic polyangiitis and focuses on the clinical and diagnostic value of ANCA detection in patients with connective tissue diseases, idiopathic interstitial pneumonia, autoimmune liver diseases, inflammatory bowel diseases, anti-glomerular basement membrane (GBM) disease, infections, malignancy, and during drug treatment. Current evidence suggests that in certain settings beyond systemic vasculitis, ANCA may have clinical, pathogenic and/or diagnostic relevance. Antigen-specific ANCA targeting proteinase-3 and myeloperoxidase should be tested by solid phase immunoassays in any patient with clinical features suggesting ANCA-associated vasculitis and in all patients with anti-GBM disease, idiopathic interstitial pneumonia, and infective endocarditis associated with nephritis, whereas in patients with other aforementioned disorders routine ANCA testing is not recommended. Among patients with autoimmune liver diseases or inflammatory bowel diseases, ANCA testing may be justified in patients with suspected autoimmune hepatitis type 1 who do not have conventional autoantibodies or in case of diagnostic uncertainty to discriminate ulcerative colitis from Crohn's disease. In these cases, ANCA should be tested by indirect immunofluorescence as the target antigens are not yet well characterized. Many questions concerning the optimal use of ANCA testing in patients without ANCA-associated vasculitis remain to be answered.

Published
2020

3. AB0511 International Consensus on ANCA Testing and Interpretation Beyond Systemic Vasculitis

Author

Moiseev, S, Cohen Tervaert, J, Arimura, Y, Bogdanos, D, Elena, C, Damoiseaux, J, Ferrante, M, Flores-Suárez, L, Fritzler, M, Invernizzi, P, Jayne, D, Jennette, J, Little, M, Mcadoo, S, Novikov, P, Pusey, C, Radice, A, Salama, A, Savige, J, Segelmark, M, Shoenfeld, Y, Sinico, R, De Sousa, M, Specks, U, Terrier, B, Tzioufas, A, Vermeire, S, Zhao, M, Bossuyt, X, S Moiseev, JW Cohen Tervaert, Y Arimura, D Bogdanos, C Elena, J Damoiseaux, M Ferrante, LF Flores-Suárez, M Fritzler, P Invernizzi, D Jayne, JC Jennette, M Little, SP Mcadoo, P Novikov, CD Pusey, A Radice, AD Salama, J Savige, M Segelmark, Y Shoenfeld, RA Sinico, MJR De Sousa, U Specks, B Terrier, A Tzioufas, S Vermeire, MH Zhao, X Bossuyt, Moiseev, S, Cohen Tervaert, J, Arimura, Y, Bogdanos, D, Elena, C, Damoiseaux, J, Ferrante, M, Flores-Suárez, L, Fritzler, M, Invernizzi, P, Jayne, D, Jennette, J, Little, M, Mcadoo, S, Novikov, P, Pusey, C, Radice, A, Salama, A, Savige, J, Segelmark, M, Shoenfeld, Y, Sinico, R, De Sousa, M, Specks, U, Terrier, B, Tzioufas, A, Vermeire, S, Zhao, M, Bossuyt, X, S Moiseev, JW Cohen Tervaert, Y Arimura, D Bogdanos, C Elena, J Damoiseaux, M Ferrante, LF Flores-Suárez, M Fritzler, P Invernizzi, D Jayne, JC Jennette, M Little, SP Mcadoo, P Novikov, CD Pusey, A Radice, AD Salama, J Savige, M Segelmark, Y Shoenfeld, RA Sinico, MJR De Sousa, U Specks, B Terrier, A Tzioufas, S Vermeire, MH Zhao, and X Bossuyt

Abstract

Background: ANCA can be detected in sera from patients with autoimmune, inflammatory, infectious or neoplastic diseases. Objectives: To issue a Consensus Statement on ANCA testing and interpretation beyond systemic vasculitis. Methods: This Statement was prepared by a group of experts, based on the results of a comprehensive search in PubMed. Results: In certain settings beyond systemic vasculitis, ANCA may have diagnostic, clinical, and/or prognostic relevance. Testing for PR3- and MPO-ANCA by specific immunoassays should be performed in any patient with clinical features suggesting ANCA-associated vasculitis and in patients with anti-GBM disease and idiopathic interstitial pneumonia. Routine ANCA testing is not recommended in patients with connective tissue diseases (CTD), autoimmune liver diseases, inflammatory bowel diseases, infections, and/or malignancy unless there is evidence for small vessel vasculitis. ANCA testing by specific immunoassays may be useful in patients with rheumatoid arthritis, systemic sclerosis or primary Sjögren’s syndrome who have kidney disease with a nephritic sediment or in patients with systemic lupus erythematosus if a kidney biopsy shows prominent necrotizing and crescentic lesions or proliferative lupus nephritis. ANCA testing may be justified in patients with suspected autoimmune hepatitis type 1, who do not have conventional disease-related autoantibodies, or in patients with inflammatory bowel diseases in case of diagnostic uncertainty to discriminate ulcerative colitis from Crohn’s disease. In these cases, ANCA should be tested by indirect immunofluorescence since target antigens are not well characterized. ANCA against bactericidal/permeability-increasing protein may be a biomarker for deteriorating lung function and a poor prognosis in patients with cystic fibrosis. Conclusion: ANCA testing is clinically relevant not only in patients with manifestations suggesting systemic vasculitis, but also in patients with certain other

Published
2020

7. Revised 2017 international consensus on testing of ANCAs in granulomatosis with polyangiitis and microscopic polyangiitis [Position paper]

Author

Bossuyt, X, Cohen Tervaert, J, Arimura, Y, Blockmans, D, Flores Suárez, L, Guillevin, L, Hellmich, B, Jayne, D, Jennette, J, Kallenberg, C, Moiseev, S, Novikov, P, Radice, A, Savige, J, Sinico, R, Specks, U, van Paassen, P, Zhao, M, Rasmussen, N, Damoiseaux, J, Csernok, E, SINICO, RENATO ALBERTO, Csernok, E., Bossuyt, X, Cohen Tervaert, J, Arimura, Y, Blockmans, D, Flores Suárez, L, Guillevin, L, Hellmich, B, Jayne, D, Jennette, J, Kallenberg, C, Moiseev, S, Novikov, P, Radice, A, Savige, J, Sinico, R, Specks, U, van Paassen, P, Zhao, M, Rasmussen, N, Damoiseaux, J, Csernok, E, SINICO, RENATO ALBERTO, and Csernok, E.

Abstract

Anti-neutrophil cytoplasmic antibodies (ANCAs) are valuable laboratory markers used for the diagnosis of well-defined types of small-vessel vasculitis, including granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). According to the 1999 international consensus on ANCA testing, indirect immunofluorescence (IIF) should be used to screen for ANCAs, and samples containing ANCAs should then be tested by immunoassays for proteinase 3 (PR3)-ANCAs and myeloperoxidase (MPO)-ANCAs. The distinction between PR3-ANCAs and MPO-ANCAs has important clinical and pathogenic implications. As dependable immunoassays for PR3-ANCAs and MPO-ANCAs have become broadly available, there is increasing international agreement that high-quality immunoassays are the preferred screening method for the diagnosis of ANCA-associated vasculitis. The present Consensus Statement proposes that high-quality immunoassays can be used as the primary screening method for patients suspected of having the ANCA-associated vaculitides GPA and MPA without the categorical need for IIF, and presents and discusses evidence to support this recommendation.

Published
2017

10. Granulomatosis con poliangitis en imágenes de cabeza y cuello: lo que el radiólogo necesita saber.

Author

Delgado-Hernández, R. D., Reynoso-Topete, A., Tovar-Calderón, I. L., Higuera, V., Ruiz, N., Vázquez-Lamadrid, J., and Flores-Suárez, L. F.

Abstract

Granulomatosis with polyangiitis is a necrotising vasculitis with multisystem granulomatous inflammation which predominantly affects small blood vessels; it is associated with anti-neutrophil cytoplasmic antibodies (ANCAs). The purpose of this article is to describe the most common image findings in the areas of the head and the neck, in patients with granulomatosis with polyangiitis. To date no Mexican publications provide orientation on this point. The paranasal sinuses and the nasal septum are the best known areas, in head and neck, for this disease; however, disorders which may appear in eye sockets, ear, central nervous system, pituitary gland, and larynx, even without clinical manifestation at the time of image evaluation, should not be overlooked. It is important to opportunely report lesions due to granulomatosis with polyangiitis which suggest activity identified by imaging methods to the clinical physician and perform comparative studies to assess new lesions. In most cases active lesions identified by imaging are characterized by heightened signal intensity following administration of intravenous contrast medium, using T1 weighted sequences with fatty suppression. Image findings which suggest activity may be compared at different evolution times to determine possible remission or for histological confirmation. Long term, the presence of active lesions will help modify treatment, which may include starting corticoids or modifying or intensifying their use. [ABSTRACT FROM AUTHOR]

Published
2015

11. 2020 international consensus on ANCA testing beyond systemic vasculitis

Author

Judith Savige, Alan D. Salama, Maria José Rego Sousa, Pavel Novikov, Athanasios G. Tzioufas, Sergey Moiseev, Yehuda Shoenfeld, Elena Csernok, Xavier Bossuyt, Yoshihiro Arimura, Marc Ferrante, Marvin J. Fritzler, Benjamin Terrier, Luis Felipe Flores-Suárez, Jan Willem Cohen Tervaert, Mark A. Little, Ulrich Specks, Mårten Segelmark, J. Charles Jennette, Dimitrios P. Bogdanos, David Jayne, Charles D. Pusey, Stephen P. McAdoo, Jan Damoiseaux, Ming Hui Zhao, Pietro Invernizzi, Antonella Radice, Renato Alberto Sinico, Severine Vermeire, Moiseev, S, Tervaert, J, Arimura, Y, Bogdanos, D, Csernok, E, Damoiseaux, J, Ferrante, M, Flores-Suárez, L, Fritzler, M, Invernizzi, P, Jayne, D, Jennette, J, Little, M, Mcadoo, S, Novikov, P, Pusey, C, Radice, A, Salama, A, Savige, J, Segelmark, M, Shoenfeld, Y, Sinico, R, Sousa, M, Specks, U, Terrier, B, Tzioufas, A, Vermeire, S, Zhao, M, and Bossuyt, X

Subjects
0301 basic medicine, ANTI-SACCHAROMYCES-CEREVISIAE, MICROSCOPIC POLYANGIITIS, medicine.medical_specialty, Consensus, BACTERICIDAL/PERMEABILITY-INCREASING PROTEIN, Myeloblastin, Immunology, ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Autoimmune hepatitis, urologic and male genital diseases, PRIMARY SJOGRENS-SYNDROME, Inflammatory bowel disease, Antibodies, Antineutrophil Cytoplasmic, 03 medical and health sciences, 0302 clinical medicine, immune system diseases, Immunology and Allergy, Medicine, Humans, cardiovascular diseases, CLINICAL-SIGNIFICANCE, skin and connective tissue diseases, Idiopathic interstitial pneumonia, Anti-neutrophil cytoplasmic antibody, Peroxidase, 030203 arthritis & rheumatology, anti-neutrophil cytoplasm antibodies (ANCA), connective tissue diseases, idiopathic interstitial pneumonia, autoimmune liver diseases, inflammatory bowel diseases, anti-glomerular basement membrane (GBM) disease, infections, malignancy, business.industry, TUBULIN ISOTYPE 5, Granulomatosis with Polyangiitis, PRIMARY SCLEROSING CHOLANGITIS, medicine.disease, AUTOIMMUNE LIVER DISORDERS, Dermatology, RHEUMATOID-ARTHRITIS, respiratory tract diseases, Hepatitis, Autoimmune, 030104 developmental biology, business, Granulomatosis with polyangiitis, Vasculitis, Microscopic polyangiitis, INFLAMMATORY-BOWEL-DISEASE, Systemic vasculitis
Abstract

This document follows up on a 2017 revised international consensus on anti-neutrophil cytoplasm antibodies (ANCA) testing in granulomatosis with polyangiitis and microscopic polyangiitis and focuses on the clinical and diagnostic value of ANCA detection in patients with connective tissue diseases, idiopathic interstitial pneumonia, autoimmune liver diseases, inflammatory bowel diseases, anti-glomerular basement membrane (GBM) disease, infections, malignancy, and during drug treatment. Current evidence suggests that in certain settings beyond systemic vasculitis, ANCA may have clinical, pathogenic and/or diagnostic relevance. Antigen-specific ANCA targeting proteinase-3 and myeloperoxidase should be tested by solid phase immunoassays in any patient with clinical features suggesting ANCA-associated vasculitis and in all patients with anti-GBM disease, idiopathic interstitial pneumonia, and infective endocarditis associated with nephritis, whereas in patients with other aforementioned disorders routine ANCA testing is not recommended. Among patients with autoimmune liver diseases or inflammatory bowel diseases, ANCA testing may be justified in patients with suspected autoimmune hepatitis type 1 who do not have conventional autoantibodies or in case of diagnostic uncertainty to discriminate ulcerative colitis from Crohn's disease. In these cases, ANCA should be tested by indirect immunofluorescence as the target antigens are not yet well characterized. Many questions concerning the optimal use of ANCA testing in patients without ANCA-associated vasculitis remain to be answered.

Published
2020

12. AB0511 International Consensus on ANCA Testing and Interpretation Beyond Systemic Vasculitis

Author

Judith Savige, Mark A. Little, Charles D. Pusey, Sergey Moiseev, Renato Alberto Sinico, Yehuda Shoenfeld, Ming-Hui Zhao, C. Elena, D. R. W. Jayne, Benjamin Terrier, Mårten Segelmark, Marc Ferrante, Dimitrios P. Bogdanos, J. W. Cohen Tervaert, Antonella Radice, Pavel Novikov, Alan D. Salama, Marvin J. Fritzler, Pietro Invernizzi, Luis Felipe Flores-Suárez, A. G. Tzioufas, Stephen P. McAdoo, J. Damoiseaux, Ulrich Specks, Xavier Bossuyt, Yoshihiro Arimura, Severine Vermeire, John Charles Jennette, M. J. R. De Sousa, Moiseev, S, Cohen Tervaert, J, Arimura, Y, Bogdanos, D, Elena, C, Damoiseaux, J, Ferrante, M, Flores-Suárez, L, Fritzler, M, Invernizzi, P, Jayne, D, Jennette, J, Little, M, Mcadoo, S, Novikov, P, Pusey, C, Radice, A, Salama, A, Savige, J, Segelmark, M, Shoenfeld, Y, Sinico, R, De Sousa, M, Specks, U, Terrier, B, Tzioufas, A, Vermeire, S, Zhao, M, and Bossuyt, X

Subjects
ANCA, vasculitis, consensus, medicine.medical_specialty, business.industry, Immunology, Lupus nephritis, Autoantibody, Autoimmune hepatitis, medicine.disease, Ulcerative colitis, Dermatology, General Biochemistry, Genetics and Molecular Biology, Rheumatology, Internal medicine, Immunology and Allergy, Medicine, business, Vasculitis, Idiopathic interstitial pneumonia, Systemic vasculitis
Abstract

Background:ANCA can be detected in sera from patients with autoimmune, inflammatory, infectious or neoplastic diseases.Objectives:To issue a Consensus Statement on ANCA testing and interpretation beyond systemic vasculitis.Methods:This Statement was prepared by a group of experts, based on the results of a comprehensive search in PubMed.Results:In certain settings beyond systemic vasculitis, ANCA may have diagnostic, clinical, and/or prognostic relevance. Testing for PR3- and MPO-ANCA by specific immunoassays should be performed in any patient with clinical features suggesting ANCA-associated vasculitis and in patients with anti-GBM disease and idiopathic interstitial pneumonia. Routine ANCA testing is not recommended in patients with connective tissue diseases (CTD), autoimmune liver diseases, inflammatory bowel diseases, infections, and/or malignancy unless there is evidence for small vessel vasculitis. ANCA testing by specific immunoassays may be useful in patients with rheumatoid arthritis, systemic sclerosis or primary Sjögren’s syndrome who have kidney disease with a nephritic sediment or in patients with systemic lupus erythematosus if a kidney biopsy shows prominent necrotizing and crescentic lesions or proliferative lupus nephritis. ANCA testing may be justified in patients with suspected autoimmune hepatitis type 1, who do not have conventional disease-related autoantibodies, or in patients with inflammatory bowel diseases in case of diagnostic uncertainty to discriminate ulcerative colitis from Crohn’s disease. In these cases, ANCA should be tested by indirect immunofluorescence since target antigens are not well characterized. ANCA against bactericidal/permeability-increasing protein may be a biomarker for deteriorating lung function and a poor prognosis in patients with cystic fibrosis.Conclusion:ANCA testing is clinically relevant not only in patients with manifestations suggesting systemic vasculitis, but also in patients with certain other disorders, particularly in patients with anti-GBM disease or idiopathic interstitial pneumonia.Disclosure of Interests:Sergey Moiseev Grant/research support from: This work was supported by the 5-100 Project, Sechenov University, Moscow, Jan Willem Cohen Tervaert: None declared, Yoshihiro Arimura: None declared, Dimitrios Bogdanos: None declared, Csernok Elena: None declared, Jan Damoiseaux: None declared, Marc Ferrante: None declared, Luis Felipe Flores-Suárez: None declared, Marvin Fritzler: None declared, Pietro Invernizzi: None declared, David Jayne Grant/research support from: ChemoCentryx, GSK, Roche/Genentech, Sanofi-Genzyme, Consultant of: Astra-Zeneca, ChemoCentryx, GSK, InflaRx, Takeda, Insmed, Chugai, Boehringer-Ingelheim, J. Charles Jennette: None declared, Mark Little: None declared, Stephen P. McAdoo: None declared, Pavel Novikov Grant/research support from: This work was supported by the 5-100 Project, Sechenov University, Moscow, Charles D. Pusey: None declared, Antonella Radice: None declared, Alan D. Salama: None declared, Judith Savige: None declared, Mårten Segelmark: None declared, Yehuda Shoenfeld: None declared, Renato Alberto Sinico: None declared, Maria Jose Rego de Sousa: None declared, Ulrich Specks: None declared, Benjamin Terrier: None declared, Athanasios Tzioufas: None declared, Severine Vermeire: None declared, Ming-hui Zhao: None declared, Xavier Bossuyt: None declared

Published
2020

13. Severe infections in patients with ANCA-associated vasculitis treated with rituximab.

Author

Segelmark L, Flores-Suárez L, and Mohammad A

Subjects
Adult, Antirheumatic Agents adverse effects, Antirheumatic Agents therapeutic use, Female, Humans, Male, Middle Aged, Retrospective Studies, Risk Factors, Rituximab adverse effects, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis drug therapy, Infections etiology, Rituximab therapeutic use
Abstract

Objectives: Rituximab (RTX) is an anti-CD20 antibody that selectively depletes B cells and has emerged as a therapy for ANCA-associated vasculitis (AAV) during the past decade. This study sought to quantify and determine potential risk factors for severe infections in AAV patients treated with RTX at rheumatology clinics in Mexico City, Mexico and Lund, Sweden., Methods: The study consisted of a retrospective case-record review (2005-15) with standardized data collection related to the occurrence of severe infection in 46 patients with AAV in Mexico City (n = 20) and Lund (n = 26) treated with RTX during their disease course. Median duration of follow-up from first RTX dose to death or end of study was 26 months., Results: Eleven (24%) patients suffered a total of 18 severe infections (infection rate of 11.5/100 patient-years). Thirteen of the 18 infections (72%) occurred within the first year of treatment. Risk factors for severe infection were older age at RTX initiation and absence of ENT involvement at diagnosis. In multivariate analyses, age at RTX infusion was the only independent factor predicting severe infection. Four patients (9%) died during follow-up, all as a result of infection., Conclusion: Severe infections are common following RTX treatment, and mortality due to infection is a major concern. Most severe infections occur within the first year of RTX treatment. The negative correlation of ENT involvement with severe infection might reflect granulomatosis with polyangiitis phenotype heterogeneity. Older age at time of RTX treatment independently predicts severe infections., (© The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Published
2021
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14. Etiology of cutaneous vasculitis: utility of a systemic approach

Author

Chanussot-Deprez C, Vega-Memije ME, Flores-Suárez L, Ríos-Romero C, Cabiedes-Contreras J, Reyes E, and Rangel-Gamboa L

Subjects
Adolescent, Adult, Aged, Child, Female, Humans, Male, Middle Aged, Prospective Studies, Young Adult, Skin Diseases, Vascular etiology, Vasculitis etiology
Abstract

Cutaneous vasculities (CV) represents a diagnostic challenge, occurs as primary cutaneous disorder or as a manifestation of other entities., Objective: To search the cause of CV. Methods: Patients with CV were prospectively evaluated. In all patients, skin biopsies were drawn, and direct immunofluorescence was done in most of the patients. American College of Rheumatology (ACR) and Chapel Hill Consensus Conference Criteria (CHCC) were used for classification., Results: 32 patients were studied. There was female predominance (71.8%). Children presented drug-associated CV or Schönlein-Henoch púrpura (SHP). Adults presented more frequently SHP, systemic lupus erythematosus or paraneoplastic vasculitis, other diagnosis as polyarteritis nodosa, microscopic polyangiitis, thrombotic vasculitis (post-puerperal), antiphospholipid syndrome, Churg-Strauss syndrome, and drug-associated CV were presented. Using the ACR and CHCC criteria, 50% of cases were classified., Discussion: In our institution, during this work the etiologic diagnostic of CV increased more than twice. However, in the case of HSV or LA and SHP none of the proposed criteria had high specificity; other parameters were used to discern between both. Six patients remained as not classified. In our view, cryoglobulins and hepatitis serology do not seem useful unless patient’s history supports they need to be done. Unclassified patients were followed-up closely for 2 years., (Copyright: © 2018 SecretarÍa de Salud)

Published
2018
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15. Seven clinical conundrums in the treatment of ANCA-associated vasculitis.

Author

Alba MA and Flores-Suárez LF

Subjects
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis diagnosis, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis immunology, B-Lymphocytes drug effects, B-Lymphocytes immunology, Cyclophosphamide therapeutic use, Drug Administration Schedule, Drug Therapy, Combination, Evidence-Based Medicine, Glucocorticoids administration & dosage, Glucocorticoids adverse effects, Humans, Immunosuppressive Agents administration & dosage, Immunosuppressive Agents adverse effects, Methotrexate therapeutic use, Mycophenolic Acid analogs & derivatives, Mycophenolic Acid therapeutic use, Plasma Exchange, Remission Induction, Treatment Outcome, Tumor Necrosis Factor-alpha antagonists & inhibitors, Tumor Necrosis Factor-alpha metabolism, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis drug therapy, Glucocorticoids therapeutic use, Immunosuppressive Agents therapeutic use
Abstract

Granulomatosis with polyangiitis and microscopic polyangiitis are two autoimmune diseases characterised by necrotising small-vessel vasculitis and presence of antineutrophil cytoplasm autoantibodies (ANCA). Current immunosuppressive regimes that combine cyclophosphamide and glucocorticoids have dramatically improved the outcome for these patients. However, these treatments are associated with toxic effects and do not lead to permanent remission in the majority of cases. Newer approaches have been sought during the last 15 years, with improvement in medication protocols and inclusion of novel therapies. This review develops on seven clinical conundrums of evidence-based therapeutic strategies for ANCA-vasculitis, posed as questions on aspects such as the role of established drugs in both remission induction and maintenance: glucocorticoids (and its duration), oral cyclophosphamide, methotrexate, TNF-α blockers, plasma exchange, mycophenolate mofetil, plus one related to newer developments in treatment with agents blocking the complement system and the possible role of sequential or combined therapies, mainly directed against B cells.

Published
2013

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