Vasculitis: Again, Changing the Standard of Care
Authors: Gary S. Hoffman
Journal: Annals of Internal Medicine (2020)

Editorials4 August 2020Vasculitis: Again, Changing the Standard of CareGary S. Hoffman, MD, MS, MACRGary S. Hoffman, MD, MS, MACRCleveland Clinic, Cleveland, Ohio (G.S.H.)Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/M20-2853 SectionsSupplemental MaterialAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail If you do not know history…you are a leaf that does not know it is part of a tree.—Michael CrichtonThe history of medicine was first marked by descriptive accounts of illness—without meaningful therapeutic interventions—followed by empiric treatment, prospective observational studies, and finally controlled trials. Rare diseases have had a painfully slower progression through these stages. For noninfectious systemic vasculitides, the last stage of this journey was not achieved until the 1990s. Before 1970, we had little understanding of vasculitis etiology or why certain vasculitides favored particular organs and spared others, why some damaged only a single site, why some ...References1. Hoffman GS, Calabrese LH. Vasculitis: determinants of disease patterns. Nat Rev Rheumatol. 2014;10:454-62. [PMID: 24934189] doi:10.1038/nrrheum.2014.89 CrossrefMedlineGoogle Scholar2. Pryshchep O, Ma-Krupa W, Younge BR, et al. Vessel-specific Toll-like receptor profiles in human medium and large arteries. Circulation. 2008;118:1276-84. [PMID: 18765390] doi:10.1161/CIRCULATIONAHA.108.789172 CrossrefMedlineGoogle Scholar3. Fauci AS, Wolff SM. Wegener's granulomatosis: studies in eighteen patients and a review of the literature. Medicine (Baltimore). 1973;52:535-61. [PMID: 4748591] CrossrefMedlineGoogle Scholar4. Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992;116:488-98. [PMID: 1739240] LinkGoogle Scholar5. Hogan SL, Nachman PH, Wilkman AS, et al. Prognostic markers in patients with antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis. J Am Soc Nephrol. 1996;7:23-32. [PMID: 8808106] CrossrefMedlineGoogle Scholar6. Lyons PA, Rayner TF, Trivedi S, et al. Genetically distinct subsets within ANCA-associated vasculitis. N Engl J Med. 2012;367:214-23. [PMID: 22808956] doi:10.1056/NEJMoa1108735 CrossrefMedlineGoogle Scholar7. Stone JH, Merkel PA, Spiera R, et al; RAVE-ITN Research Group. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010;363:221-32. [PMID: 20647199] doi:10.1056/NEJMoa0909905 CrossrefMedlineGoogle Scholar8. Guillevin L, Pagnoux C, Karras A, et al; French Vasculitis Study Group. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med. 2014;371:1771-80. [PMID: 25372085] doi:10.1056/NEJMoa1404231 CrossrefMedlineGoogle Scholar9. Charles P, Terrier B, Perrodeau É, et al; French Vasculitis Study Group. Comparison of individually tailored versus fixed-schedule rituximab regimen to maintain ANCA-associated vasculitis remission: results of a multicentre, randomised controlled, phase III trial (MAINRITSAN2). Ann Rheum Dis. 2018;77:1143-1149. [PMID: 29695500] doi:10.1136/annrheumdis-2017-212878 CrossrefMedlineGoogle Scholar10. Charles P, Perrodeau É, Samson M, et al; French Vasculitis Study Group. Long-term rituximab use to maintain remission of antineutrophil cytoplasmic antibody–associated vasculitis. A randomized trial. Ann Intern Med. 2020;173:179-87. doi:10.7326/M19-3827 LinkGoogle Scholar Author, Article, and Disclosure InformationAffiliations: Cleveland Clinic, Cleveland, Ohio (G.S.H.)Disclosures: The author has disclosed no conflicts of interest. The form can be viewed at www.acponline.org/authors/icmje/ConflictOfInterestForms.do?msNum=M20-2853.Corresponding Author: Gary S. Hoffman, MD, MS, MACR, 30060 Bolingbrook Road, Pepper Pike, OH 44124; e-mail, [email protected]org.This article was published at Annals.org on 2 June 2020. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetailsSee AlsoLong-Term Rituximab Use to Maintain Remission of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis Pierre Charles , Élodie Perrodeau , Maxime Samson , Bernard Bonnotte , Antoine Néel , Christian Agard , Antoine Huart , Alexandre Karras , François Lifermann , Pascal Godmer , Pascal Cohen , Catherine Hanrotel-Saliou , Nicolas Martin-Silva , Grégory Pugnet , François Maurier , Jean Sibilia , Pierre-Louis Carron , Pierre Gobert , Nadine Meaux-Ruault , Thomas Le Gallou , Stéphane Vinzio , Jean-François Viallard , Eric Hachulla , Christine Vinter , Xavier Puéchal , Benjamin Terrier , Philippe Ravaud , Luc Mouthon , Loïc Guillevin , and Metrics Cited byThe eye is a common site of granulomatosis with polyangiitis. A collaborative study 4 August 2020Volume 173, Issue 3Page: 235-236KeywordsAntibodiesB cellsBiomarkersCorticosteroid therapyCyclophosphamideGenetic diseasesMorbidityRheumatoid arthritisSerious adverse eventsVasculitis ePublished: 2 June 2020 Issue Published: 4 August 2020 Copyright & PermissionsCopyright © 2020 by American College of Physicians. All Rights Reserved.PDF downloadLoading ...