Now processing ... 
 Now searching ... 
 Now loading ... 

EGPAの治療アルゴリズム

出典
imgimg
1: Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis.
著者: Giacomo Emmi, Alessandra Bettiol, Elena Gelain, Ingeborg M Bajema, Alvise Berti, Stella Burns, Maria C Cid, Jan W Cohen Tervaert, Vincent Cottin, Eugenia Durante, Julia U Holle, Alfred D Mahr, Marcos Martinez Del Pero, Chiara Marvisi, John Mills, Sergey Moiseev, Frank Moosig, Chetan Mukhtyar, Thomas Neumann, Iacopo Olivotto, Carlo Salvarani, Benjamin Seeliger, Renato A Sinico, Camille Taillé, Benjamin Terrier, Nils Venhoff, George Bertsias, Loïc Guillevin, David R W Jayne, Augusto Vaglio
雑誌名: Nat Rev Rheumatol. 2023 Jun;19(6):378-393. doi: 10.1038/s41584-023-00958-w. Epub 2023 May 9.
Abstract/Text: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, characterized by asthma, eosinophilia and granulomatous or vasculitic involvement of several organs. The diagnosis and management of EGPA are often challenging and require an integrated, multidisciplinary approach. Current practice relies on recommendations and guidelines addressing the management of ANCA-associated vasculitis and not specifically developed for EGPA. Here, we present evidence-based, cross-discipline guidelines for the diagnosis and management of EGPA that reflect the substantial advances that have been made in the past few years in understanding the pathogenesis, clinical subphenotypes and differential diagnosis of the disease, as well as the availability of new treatment options. Developed by a panel of European experts on the basis of literature reviews and, where appropriate, expert opinion, the 16 statements and five overarching principles cover the diagnosis and staging, treatment, outcome and follow-up of EGPA. These recommendations are primarily intended to be used by healthcare professionals, pharmaceutical industries and drug regulatory authorities, to guide clinical practice and decision-making in EGPA. These guidelines are not intended to limit access to medications by healthcare agencies, nor to impose a fixed order on medication use.

© 2023. Springer Nature Limited.
Nat Rev Rheumatol. 2023 Jun;19(6):378-393. doi: 10.1038/s41584-023-009...

原発性血管炎の分類

2012年チャペルヒルコンセンサス会議での血管炎の分類の図
出典
img
1: Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1‐11.(p4, figure2)

ANCA関連血管炎 指定難病受給者証給付数

参考文献:
難病情報センターホームページを参照に作成 [https://www.nanbyou.or.jp/entry/5354特定医療費(指定難病)受給者証所持者数]
出典
img
1: 著者提供

ANCA関連血管炎

出典
img
1: Jennette JC, Falk RJ. Pathogenesis of antineutrophil cytoplasmic autoantibody-mediated disease. Nat Rev Rheumatol. 2014;10(8):463‐473.(p469, figure5)

日本人でのANCA関連血管炎の特徴

出典
img
1: 著者提供

GPAにおける多発結節影の一例

出典
img
1: 著者提供

GPA診断基準

出典
img
1: 難病情報センターホームページ:[https://www.nanbyou.or.jp/entry/4012 多発血管炎性肉芽腫症(指定難病44)](2020年8月現在)より転載

MPA診断基準

出典
img
1: 難病情報センターホームページ:[https://www.nanbyou.or.jp/entry/245 顕微鏡的多発血管炎(指定難病43)](2020年8月現在)より転載

EGPA診断基準

出典
img
1: 難病情報センターホームページ:[https://www.nanbyou.or.jp/entry/3878 好酸球性多発血管炎性肉芽腫症(指定難病45)](2020年8月現在)より転載

GPAに対するPR3-ANCA陽性の診断意義

出典
imgimg
1: The diagnostic utility of c-ANCA in Wegener's granulomatosis.
著者: C A Langford
雑誌名: Cleve Clin J Med. 1998 Mar;65(3):135-40. doi: 10.3949/ccjm.65.3.135.
Abstract/Text: Anti-neutrophil cytoplasmic antibodies with a cytoplasmic staining pattern (c-ANCA) have been found to have a high degree of sensitivity and specificity for Wegener's granulomatosis. Nevertheless, despite the attraction of using this autoantibody as a diagnostic test, in almost all instances it should not be used in place of a biopsy to diagnose Wegener's granulomatosis.
Cleve Clin J Med. 1998 Mar;65(3):135-40. doi: 10.3949/ccjm.65.3.135.

腎炎のみの場合のANCAの診断意義

出典
imgimg
1: Diagnostic predictive value of ANCA serology.
Kidney Int. 1998 Mar;53(3):796-8. doi: 10.1038/ki.1998.36.

ANCAの抗体価と症状による検査前確率と検査後確率

ANCAの結果はEuro-Diagnosticaの検査を使用している。
出典
imgimg
1: A multicentre study to improve clinical interpretation of proteinase-3 and myeloperoxidase anti-neutrophil cytoplasmic antibodies.
著者: Xavier Bossuyt, Niels Rasmussen, Pieter van Paassen, Bernard Hellmich, Bo Baslund, Pieter Vermeersch, Daniel Blockmans, Jan-Willem Cohen Tervaert, Elena Csernok, Jan Damoiseaux
雑誌名: Rheumatology (Oxford). 2017 Sep 1;56(9):1533-1541. doi: 10.1093/rheumatology/kex170.
Abstract/Text: OBJECTIVE: The objective of this multicentre study was to improve the clinical interpretation of PR3- and MPO-ANCAs as an adjunct for the diagnosis of ANCA-associated vasculitis (AAV) by defining thresholds and test result intervals based on predefined specificities and by calculating test result interval-specific likelihood ratios (LRs).
METHODS: Eight different PR3- and MPO-ANCA immunoassays from seven companies were evaluated using 251 diagnostic samples from AAV patients and 924 diseased controls.
RESULTS: Thresholds for antibody levels were determined based on predefined specificities (95, 97.5, 99 and 100%) and used to delimit test result intervals. Test result interval-specific LRs were determined. For all assays, the LR for AAV increased with increasing antibody level. For all but one immunoassay, high antibodies levels (associated with LR >55) were found in a substantial fraction (>65%) of patients. The area under the curve (AUC) of receiver operating characteristics analysis of a diagnostic approach in which positive results were confirmed by IIF or another immunoassay was not substantially higher than the AUC of performing immunoassay only. The highest AUC was found when immunoassay was combined with another immunoassay or with IIF.
CONCLUSION: To diagnose AAV based on PR3- and MPO-ANCA, it is useful to define thresholds for antibody levels and to assign test result interval-specific LRs. Higher antibody levels are associated with a higher likelihood for disease. Such information improves clinical interpretation.

© The Author 2017. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com
Rheumatology (Oxford). 2017 Sep 1;56(9):1533-1541. doi: 10.1093/rheuma...

ANCAが陽性になりうる感染症

出典
imgimg
1: Infections and antineutrophil cytoplasmic antibodies: triggering mechanisms.
著者: Konstantin N Konstantinov, Constance J Ulff-Møller, Antonios H Tzamaloukas
雑誌名: Autoimmun Rev. 2015 Mar;14(3):201-3. doi: 10.1016/j.autrev.2014.10.020. Epub 2014 Nov 4.
Abstract/Text: The precise cause of the antineutrophil cytoplasmic antibodies (ANCA) autoimmunity is not known and is likely to be multifactorial. Infections may trigger formation of ANCA and a fraction of the patients with infection-triggered ANCA develop ANCA-associated vasculitis. Here we discuss some of the proposed mechanisms of ANCA formation during the course of infection. They include initiation of autoimmune response by microbial peptides that are complementary to autoantigens; epigenetic silencing and antigen complementarity leading to upregulation of autoantigen genes; molecular mimicry between bacterial and self-antigens; formation of neutrophil extracellular traps that stimulate immune processes including production of ANCA; and interaction of bacterial components with Toll-like receptors, which leads to formation of mediators affecting the immune responses to infections and can trigger ANCA production. Further work is needed to clarify these mechanisms and develop preventive measures and therapeutic interventions.

Published by Elsevier B.V.
Autoimmun Rev. 2015 Mar;14(3):201-3. doi: 10.1016/j.autrev.2014.10.020...

ANCAが陽性になりうる自己免疫性疾患

出典
img
1: 著者提供

CYCLOPS試験によるシクロフォスファミドの用量

参考文献:
de Groot K, Harper L, Jayne DR, Flores Suarez LF, Gregorini G, Gross WL, Luqmani R, Pusey CD, Rasmussen N, Sinico RA, Tesar V, Vanhille P, Westman K, Savage CO; EUVAS (European Vasculitis Study Group). Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a ranomized trial. Ann Intern Med. 2009 May 19;150(10):670-80. doi: 10.7326/0003-4819-150-10-200905190-00004. PMID: 19451574.
出典
img
1: 著者提供

PEXIVASにおけるグルココルチコイド減量のレジメン

出典
imgimg
1: Plasma exchange and glucocorticoid dosing in the treatment of anti-neutrophil cytoplasm antibody associated vasculitis (PEXIVAS): protocol for a randomized controlled trial.
著者: Michael Walsh, Peter A Merkel, Chen Au Peh, Wladimir Szpirt, Loïc Guillevin, Charles D Pusey, Janak De Zoysa, Natalie Ives, William F Clark, Karen Quillen, Jeffrey L Winters, Keith Wheatley, David Jayne, PEXIVAS Investigators
雑誌名: Trials. 2013 Mar 14;14:73. doi: 10.1186/1745-6215-14-73. Epub 2013 Mar 14.
Abstract/Text: BACKGROUND: Granulomatosis with polyangiitis (GPA, Wegener's) and microscopic polyangiitis (MPA) are small vessel vasculitides collectively referred to as anti-neutrophil cytoplasm antibody-associated vasculitis (AAV). AAV is associated with high rates of morbidity and mortality due to uncontrolled disease and treatment toxicity. Small randomized trials suggest adjunctive plasma exchange may improve disease control, while observational evidence suggests that current oral glucocorticoid doses are associated with severe infections in patients with AAV. A randomized study of both plasma exchange and glucocorticoids is required to evaluate plasma exchange and oral glucocorticoid dosing in patients with AAV.
METHODS/DESIGN: PEXIVAS is a two-by-two factorial randomized trial evaluating adjunctive plasma exchange and two oral glucocorticoid regimens in severe AAV. Five hundred patients are being randomized at centers across Europe, North America, Asia, and Australasia to receive plasma exchange or no plasma exchange, and to receive standard or reduced oral glucocorticoid dosing. All patients receive immunosuppression with either cyclophosphamide or rituximab. The primary outcome is the time to the composite of all-cause mortality and end-stage renal disease.PEXIVAS is funded by the National Institute of Health Research (UK), the Food and Drug Administration (USA), the National Institutes of Health (USA), the Canadian Institute of Health Research (Canada), the National Health and Medical Research Council (Australia), and Assistance Publique (France). Additional in-kind supplies for plasma exchange are provided by industry partners (TerumoBCT, Gambro Australia, and Fresenius Australia).
DISCUSSION: This is the largest trial in AAV undertaken to date. PEXIVAS will inform the future standard of care for patients with severe AAV. The cooperation between investigators, funding agencies, and industry provides a model for conducting studies in rare diseases.
TRIAL REGISTRATION: Current Controlled Trials: (ISRCTN07757494) and clinicaltrials.gov: (NCT00987389).
Trials. 2013 Mar 14;14:73. doi: 10.1186/1745-6215-14-73. Epub 2013 Mar...

Five Factor Score

参考文献:
  1. Guillevin L, Lhote F, Gayraud M, Cohen P, Jarrousse B, Lortholary O, Thibult N, Casassus P. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore). 1996 Jan;75(1):17-28. doi: 10.1097/00005792-199601000-00003. PMID: 8569467.
  1. Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P; French Vasculitis Study Group (FVSG). The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore). 2011 Jan;90(1):19-27. doi: 10.1097/MD.0b013e318205a4c6. PMID: 21200183.
出典
img
1: 著者提供

EGPAの鑑別診断

出典
imgimg
1: Eosinophilia in Rheumatologic/Vascular Disorders.
著者: Hiromichi Tamaki, Soumya Chatterjee, Carol A Langford
雑誌名: Immunol Allergy Clin North Am. 2015 Aug;35(3):453-76. doi: 10.1016/j.iac.2015.05.001. Epub 2015 Jun 17.
Abstract/Text: Peripheral and tissue eosinophilia can be a prominent feature of several unique rheumatologic and vascular diseases. These diseases span a wide range of clinical features, histologic findings, therapeutic approaches, and outcomes. Despite the rare nature of these entities--which makes large-scale studies challenging--knowledge has continued to grow regarding their epidemiology, pathophysiology, and management. This review compares and contrasts 5 rheumatologic and vascular conditions in which eosinophilia can be seen: eosinophilic granulomatosis with polyangiitis (Churg-Strauss), immunoglobulin G4-related disease, diffuse fasciitis with eosinophilia, eosinophilia-myalgia syndrome, and eosinophilic myositis.

Copyright © 2015 Elsevier Inc. All rights reserved.
Immunol Allergy Clin North Am. 2015 Aug;35(3):453-76. doi: 10.1016/j.i...

2022ACR/EULARの多発血管炎性肉芽腫症(GPA)の分類基準

出典
imgimg
1: 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis.
著者: Joanna C Robson, Peter C Grayson, Cristina Ponte, Ravi Suppiah, Anthea Craven, Andrew Judge, Sara Khalid, Andrew Hutchings, Richard A Watts, Peter A Merkel, Raashid A Luqmani, DCVAS Investigators
雑誌名: Ann Rheum Dis. 2022 Mar;81(3):315-320. doi: 10.1136/annrheumdis-2021-221795. Epub 2022 Feb 2.
Abstract/Text: OBJECTIVE: To develop and validate revised classification criteria for granulomatosis with polyangiitis (GPA).
METHODS: Patients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in five phases: (1) identification of candidate criteria items using consensus methodology, (2) prospective collection of candidate items present at the time of diagnosis, (3) data-driven reduction of the number of candidate items, (4) expert panel review of cases to define the reference diagnosis and (5) derivation of a points-based risk score for disease classification in a development set using least absolute shrinkage and selection operator logistic regression, with subsequent validation of performance characteristics in an independent set of cases and comparators.
RESULTS: The development set for GPA consisted of 578 cases of GPA and 652 comparators. The validation set consisted of an additional 146 cases of GPA and 161 comparators. From 91 candidate items, regression analysis identified 26 items for GPA, 10 of which were retained. The final criteria and their weights were as follows: bloody nasal discharge, nasal crusting or sino-nasal congestion (+3); cartilaginous involvement (+2); conductive or sensorineural hearing loss (+1); cytoplasmic antineutrophil cytoplasmic antibody (ANCA) or anti-proteinase 3 ANCA positivity (+5); pulmonary nodules, mass or cavitation on chest imaging (+2); granuloma or giant cells on biopsy (+2); inflammation or consolidation of the nasal/paranasal sinuses on imaging (+1); pauci-immune glomerulonephritis (+1); perinuclear ANCA or antimyeloperoxidase ANCA positivity (-1); and eosinophil count ≥1×109 /L (-4). After excluding mimics of vasculitis, a patient with a diagnosis of small- or medium-vessel vasculitis could be classified as having GPA if the cumulative score was ≥5 points. When these criteria were tested in the validation data set, the sensitivity was 93% (95% CI 87% to 96%) and the specificity was 94% (95% CI 89% to 97%).
CONCLUSION: The 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for GPA demonstrate strong performance characteristics and are validated for use in research.

© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.
Ann Rheum Dis. 2022 Mar;81(3):315-320. doi: 10.1136/annrheumdis-2021-2...

2022ACR/EULARの顕微鏡的多発血管炎(MPA)の分類基準

出典
imgimg
1: 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for microscopic polyangiitis.
著者: Ravi Suppiah, Joanna C Robson, Peter C Grayson, Cristina Ponte, Anthea Craven, Sara Khalid, Andrew Judge, Andrew Hutchings, Peter A Merkel, Raashid A Luqmani, Richard A Watts, DCVAS INVESTIGATORS
雑誌名: Ann Rheum Dis. 2022 Mar;81(3):321-326. doi: 10.1136/annrheumdis-2021-221796. Epub 2022 Feb 2.
Abstract/Text: OBJECTIVE: To develop and validate classification criteria for microscopic polyangiitis (MPA).
METHODS: Patients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in five phases: (1) identification of candidate items using consensus methodology, (2) prospective collection of candidate items present at the time of diagnosis, (3) data-driven reduction of the number of candidate items, (4) expert panel review of cases to define the reference diagnosis and (5) derivation of a points-based risk score for disease classification in a development set using least absolute shrinkage and selection operator logistic regression, with subsequent validation of performance characteristics in an independent set of cases and comparators.
RESULTS: The development set for MPA consisted of 149 cases of MPA and 408 comparators. The validation set consisted of an additional 142 cases of MPA and 414 comparators. From 91 candidate items, regression analysis identified 10 items for MPA, 6 of which were retained. The final criteria and their weights were as follows: perinuclear antineutrophil cytoplasmic antibody (ANCA) or anti-myeloperoxidase-ANCA positivity (+6), pauci-immune glomerulonephritis (+3), lung fibrosis or interstitial lung disease (+3), sino-nasal symptoms or signs (-3), cytoplasmic ANCA or anti-proteinase 3 ANCA positivity (-1) and eosinophil count ≥1×109/L (-4). After excluding mimics of vasculitis, a patient with a diagnosis of small- or medium-vessel vasculitis could be classified as having MPA with a cumulative score of ≥5 points. When these criteria were tested in the validation data set, the sensitivity was 91% (95% CI 85% to 95%) and the specificity was 94% (95% CI 92% to 96%).
CONCLUSION: The 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for MPA are now validated for use in clinical research.

© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.
Ann Rheum Dis. 2022 Mar;81(3):321-326. doi: 10.1136/annrheumdis-2021-2...

2022ACR/EULARの好酸球性多発血管炎性肉芽腫症(EGPA)の分類基準

出典
imgimg
1: 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis.
著者: Peter C Grayson, Cristina Ponte, Ravi Suppiah, Joanna C Robson, Anthea Craven, Andrew Judge, Sara Khalid, Andrew Hutchings, Raashid A Luqmani, Richard A Watts, Peter A Merkel, DCVAS Study Group
雑誌名: Ann Rheum Dis. 2022 Mar;81(3):309-314. doi: 10.1136/annrheumdis-2021-221794. Epub 2022 Feb 2.
Abstract/Text: OBJECTIVE: To develop and validate revised classification criteria for eosinophilic granulomatosis with polyangiitis (EGPA).
METHODS: Patients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in five phases: (1) identification of candidate criteria items using consensus methodology, (2) prospective collection of candidate items present at the time of diagnosis, (3) data-driven reduction of the number of candidate items, (4) expert panel review of cases to define the reference diagnosis and (5) derivation of a points-based risk score for disease classification in a development set using least absolute shrinkage and selection operator logistic regression, with subsequent validation of performance characteristics in an independent set of cases and comparators.
RESULTS: The development set for EGPA consisted of 107 cases of EGPA and 450 comparators. The validation set consisted of an additional 119 cases of EGPA and 437 comparators. From 91 candidate items, regression analysis identified 11 items for EPGA, 7 of which were retained. The final criteria and their weights were as follows: maximum eosinophil count ≥1×109/L (+5), obstructive airway disease (+3), nasal polyps (+3), cytoplasmic antineutrophil cytoplasmic antibody (ANCA) or anti-proteinase 3-ANCA positivity (-3), extravascular eosinophilic predominant inflammation (+2), mononeuritis multiplex/motor neuropathy not due to radiculopathy (+1) and haematuria (-1). After excluding mimics of vasculitis, a patient with a diagnosis of small- or medium-vessel vasculitis could be classified as having EGPA if the cumulative score was ≥6 points. When these criteria were tested in the validation data set, the sensitivity was 85% (95% CI 77% to 91%) and the specificity was 99% (95% CI 98% to 100%).
CONCLUSION: The 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis demonstrate strong performance characteristics and are validated for use in research.

© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.
Ann Rheum Dis. 2022 Mar;81(3):309-314. doi: 10.1136/annrheumdis-2021-2...

EGPA診断のワークアップ

出典
imgimg
1: Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis.
著者: Giacomo Emmi, Alessandra Bettiol, Elena Gelain, Ingeborg M Bajema, Alvise Berti, Stella Burns, Maria C Cid, Jan W Cohen Tervaert, Vincent Cottin, Eugenia Durante, Julia U Holle, Alfred D Mahr, Marcos Martinez Del Pero, Chiara Marvisi, John Mills, Sergey Moiseev, Frank Moosig, Chetan Mukhtyar, Thomas Neumann, Iacopo Olivotto, Carlo Salvarani, Benjamin Seeliger, Renato A Sinico, Camille Taillé, Benjamin Terrier, Nils Venhoff, George Bertsias, Loïc Guillevin, David R W Jayne, Augusto Vaglio
雑誌名: Nat Rev Rheumatol. 2023 Jun;19(6):378-393. doi: 10.1038/s41584-023-00958-w. Epub 2023 May 9.
Abstract/Text: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, characterized by asthma, eosinophilia and granulomatous or vasculitic involvement of several organs. The diagnosis and management of EGPA are often challenging and require an integrated, multidisciplinary approach. Current practice relies on recommendations and guidelines addressing the management of ANCA-associated vasculitis and not specifically developed for EGPA. Here, we present evidence-based, cross-discipline guidelines for the diagnosis and management of EGPA that reflect the substantial advances that have been made in the past few years in understanding the pathogenesis, clinical subphenotypes and differential diagnosis of the disease, as well as the availability of new treatment options. Developed by a panel of European experts on the basis of literature reviews and, where appropriate, expert opinion, the 16 statements and five overarching principles cover the diagnosis and staging, treatment, outcome and follow-up of EGPA. These recommendations are primarily intended to be used by healthcare professionals, pharmaceutical industries and drug regulatory authorities, to guide clinical practice and decision-making in EGPA. These guidelines are not intended to limit access to medications by healthcare agencies, nor to impose a fixed order on medication use.

© 2023. Springer Nature Limited.
Nat Rev Rheumatol. 2023 Jun;19(6):378-393. doi: 10.1038/s41584-023-009...

MPA、GPAの治療レジメンの選択

出典
img
1: 厚生労働科学研究費補助金 難治性疾患等政策研究事業難治性血管炎の医療水準・患者のQOL向上に資する研究班 針谷正祥ほか編:ANCA関連血管炎診療ガイドライン2023、pp.xv、診断と治療社、2023

EGPAの治療アルゴリズム

出典
imgimg
1: Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis.
著者: Giacomo Emmi, Alessandra Bettiol, Elena Gelain, Ingeborg M Bajema, Alvise Berti, Stella Burns, Maria C Cid, Jan W Cohen Tervaert, Vincent Cottin, Eugenia Durante, Julia U Holle, Alfred D Mahr, Marcos Martinez Del Pero, Chiara Marvisi, John Mills, Sergey Moiseev, Frank Moosig, Chetan Mukhtyar, Thomas Neumann, Iacopo Olivotto, Carlo Salvarani, Benjamin Seeliger, Renato A Sinico, Camille Taillé, Benjamin Terrier, Nils Venhoff, George Bertsias, Loïc Guillevin, David R W Jayne, Augusto Vaglio
雑誌名: Nat Rev Rheumatol. 2023 Jun;19(6):378-393. doi: 10.1038/s41584-023-00958-w. Epub 2023 May 9.
Abstract/Text: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, characterized by asthma, eosinophilia and granulomatous or vasculitic involvement of several organs. The diagnosis and management of EGPA are often challenging and require an integrated, multidisciplinary approach. Current practice relies on recommendations and guidelines addressing the management of ANCA-associated vasculitis and not specifically developed for EGPA. Here, we present evidence-based, cross-discipline guidelines for the diagnosis and management of EGPA that reflect the substantial advances that have been made in the past few years in understanding the pathogenesis, clinical subphenotypes and differential diagnosis of the disease, as well as the availability of new treatment options. Developed by a panel of European experts on the basis of literature reviews and, where appropriate, expert opinion, the 16 statements and five overarching principles cover the diagnosis and staging, treatment, outcome and follow-up of EGPA. These recommendations are primarily intended to be used by healthcare professionals, pharmaceutical industries and drug regulatory authorities, to guide clinical practice and decision-making in EGPA. These guidelines are not intended to limit access to medications by healthcare agencies, nor to impose a fixed order on medication use.

© 2023. Springer Nature Limited.
Nat Rev Rheumatol. 2023 Jun;19(6):378-393. doi: 10.1038/s41584-023-009...

原発性血管炎の分類

2012年チャペルヒルコンセンサス会議での血管炎の分類の図
出典
img
1: Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1‐11.(p4, figure2)