ANCA associated vasculitis (AAV): a review for internists

References

• Jennette JC, Falk RJ, Bacon PA, et al. Revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum. 2012;65(1):1–11.
   Web of Science ®Google Scholar
• Leavitt RY, Fauci AS, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum. 1990;33(8):1101–1107.
   PubMed Web of Science ®Google Scholar
• Robson JC, Grayson PC, Ponte C, et al. American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis. Ann Rheum Dis. 2022;81(3):315–320.
   PubMed Web of Science ®Google Scholar
• Suppiah R, Robson JC, Grayson PC, et al. American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for microscopic polyangiitis. Ann Rheum Dis. 2022;81(3):321–326.
   PubMed Web of Science ®Google Scholar
• Wegener F. Uber generalisierte, septische Gefasserkrankungen [On generalized, septic vascular diseases]. Verhandlungen Dtsch Pathol Gesselschaft. 1936;29:202–210.
   Google Scholar
• Godman GC, Churg J. Wegener’s granulomatosis: pathology and review of the literature. AMA Arch Pathol. 1954;58(6):533–553.
   PubMedGoogle Scholar
• Falk RJ, Gross WL, Guillevin L, et al. Granulomatosis with polyangiitis (Wegener’s): an alternative name for Wegener’s granulomatosis. J Am Soc Nephrol JASN. 2011;22(4):587–588.
   PubMed Web of Science ®Google Scholar
• Davson J, Ball J, Platt R. The kidney in periarteritis nodosa. Q J Med. 1948;17(67):175–202.
   PubMedGoogle Scholar
• Kitching AR, Anders HJ, Basu N, et al. ANCA-associated vasculitis. Nat Rev Dis Primer. 2020;6(1):71.
   PubMed Web of Science ®Google Scholar
• Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992;116(6):488–498.
   PubMed Web of Science ®Google Scholar
• Seo P, Stone JH. The antineutrophil cytoplasmic antibody-associated vasculitides. Am J Med. 2004;117(1):39–50.
   PubMed Web of Science ®Google Scholar
• Jayne D. The diagnosis of vasculitis. Best Pract Res Clin Rheumatol. 2009;23(3):445–453.
   PubMed Web of Science ®Google Scholar
• Koldingsnes W, Jacobsen EA, Sildnes T, et al. Pulmonary function and high‐resolution CT findings five years after disease onset in patients with Wegener’s granulomatosis. Scand J Rheumatol. 2005;34(3):220–228.
   PubMed Web of Science ®Google Scholar
• Fauci AS, Haynes BF, Katz P, et al. Wegener’s granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med. 1983;98(1):76–85.
   PubMed Web of Science ®Google Scholar
• Kempf HG. Ear involvement in Wegener’s granulomatosis. Clin Otolaryngol Allied Sci. 1989;14(5):451–456.
   PubMed Web of Science ®Google Scholar
• Felicetti M, Cazzador D, Padoan R, et al. Ear, nose and throat involvement in granulomatosis with polyangiitis: how it presents and how it determines disease severity and long-term outcomes. Clin Rheumatol. 2018;37(4):1075–1083.
   PubMed Web of Science ®Google Scholar
• Solans-Laqué R, Bosch-Gil J, Canela M, et al. Clinical features and therapeutic management of subglottic stenosis in patients with Wegener’s granulomatosis. Lupus. 2008;17(9):832–836.
   PubMed Web of Science ®Google Scholar
• Quinn KA, Gelbard A, Sibley C, et al. Subglottic stenosis and endobronchial disease in granulomatosis with polyangiitis. Rheumatol Oxf Engl. 2019;58(12):2203–2211.
   PubMed Web of Science ®Google Scholar
• Cordier JF, Valeyre D, Guillevin L, et al. Pulmonary Wegener’s granulomatosis. A clinical and imaging study of 77 cases. Chest. 1990;97(4):906–912.
   PubMed Web of Science ®Google Scholar
• Ananthakrishnan L, Sharma N, Kanne JP. Wegener’s granulomatosis in the chest: high-resolution CT findings. AJR Am J Roentgenol. 2009;192(3):676–682.
   PubMed Web of Science ®Google Scholar
• Tzelepis GE, Kokosi M, Tzioufas A, et al. Prevalence and outcome of pulmonary fibrosis in microscopic polyangiitis. Eur Respir J. 2010;36(1):116–121.
   PubMed Web of Science ®Google Scholar
• Schnabel A, Reuter M, Csernok E, et al. Subclinical alveolar bleeding in pulmonary vasculitides: correlation with indices of disease activity. Eur Respir J. 1999;14(1):118–124.
   PubMed Web of Science ®Google Scholar
• Gómez-Puerta JA, Hernández-Rodríguez J, López-Soto A, et al. Antineutrophil cytoplasmic antibody-associated vasculitides and respiratory disease. Chest. 2009;136(4):1101–1111.
   PubMed Web of Science ®Google Scholar
• Kubaisi B, Abu Samra K, Foster CS. Granulomatosis with polyangiitis (Wegener’s disease): an updated review of ocular disease manifestations. Intractable Rare Dis Res. 2016;5(2):61–69.
   PubMed Web of Science ®Google Scholar
• Bullen CL, Liesegang TJ, McDonald TJ, et al. Ocular complications of Wegener’s granulomatosis. Ophthalmology. 1983;90(3):279–290.
   PubMed Web of Science ®Google Scholar
• Schmidt J, Pulido JS, Matteson EL. Ocular manifestations of systemic disease: antineutrophil cytoplasmic antibody-associated vasculitis. Curr Opin Ophthalmol. 2011;22(6):489–495.
   PubMed Web of Science ®Google Scholar
• Moiseev SV. Classification, diagnosis and treatment of ANCA-associated vasculitis. World J Rheumatol. 2015;5(1):36.
   Google Scholar
• Booth AD, Almond MK, Burns A, et al. Outcome of ANCA-associated renal vasculitis: a 5-year retrospective study. Am J Kidney Dis Off J Natl Kidney Found. 2003;41(4):776–784.
   PubMed Web of Science ®Google Scholar
• Hanaoka H, Ota Y, Takeuchi T, et al. Poor renal outcomes in patients with anti-neutrophil cytoplasmic antibody-associated crescentic glomerulonephritis and normal renal function at diagnosis. Clin Rheumatol. 2016;35(2):495–500.
   PubMed Web of Science ®Google Scholar
• Francès C, Du LT, Piette JC, et al. Wegener’s granulomatosis. Dermatological manifestations in 75 cases with clinicopathologic correlation. Arch Dermatol. 1994;130(7):861–867.
   PubMedGoogle Scholar
• Miłkowska-Dymanowska J, Laskowska P, Rzuczkowski M, et al. Untypical manifestations of granulomatosis with polyangiitis—a review of the literature. SN Compr Clin Med. 2019;1(8):616–626.
   Google Scholar
• Napier SS, Allen JA, Irwin CR, et al. Strawberry gums: a clinicopathological manifestation diagnostic of Wegener’s granulomatosis? J Clin Pathol. 1993;46(8):709–712.
   PubMed Web of Science ®Google Scholar
• Hajj-Ali R, Butler R, Langford C, et al. Neurologic involvement in ANCA-associated vasculitis: data from multicenter longitudinal observational study [abstract]. American College of Rheumatology; 2021. https://acrabstracts.org/abstract/neurologic-involvement-in-anca-associated-vasculitis-data-from-multicenter-longitudinal-observational-study
   Google Scholar
• Nishino H, Rubino FA, DeRemee RA, et al. Neurological involvement in Wegener’s granulomatosis: an analysis of 324 consecutive patients at the Mayo Clinic. Ann Neurol. 1993;33(1):4–9.
   PubMed Web of Science ®Google Scholar
• Laudien M, Lamprecht P, Hedderich J, et al. Olfactory dysfunction in Wegener’s granulomatosis. Rhinology. 2009;47(3):254–259.
   PubMed Web of Science ®Google Scholar
• Foster WP, Greene JS, Millman B. Wegener’s granulomatosis presenting as ophthalmoplegia and optic neuropathy. Otolaryngol–head neck surg Off J am acad otolaryngol-head neck surg. 1995;112(6):758–762.
   PubMed Web of Science ®Google Scholar
• Haynes BF, Fishman ML, Fauci AS, et al. The ocular manifestations of Wegener’s granulomatosis. Fifteen years experience and review of the literature. Am J Med. 1977;63(1):131–141.
   PubMed Web of Science ®Google Scholar
• Yong TY, Jyz L, Amato L, et al. Pituitary involvement in Wegener’s granulomatosis. Pituitary. 2008;11(1):77–84.
   PubMed Web of Science ®Google Scholar
• Murphy JM, Gomez-Anson B, Gillard JH, et al. Wegener granulomatosis: MR imaging findings in brain and meninges. Radiology. 1999;213(3):794–799.
   PubMed Web of Science ®Google Scholar
• Bischof A, Jaeger VK, Hadden RDM, et al. Peripheral neuropathy in antineutrophil cytoplasmic antibody-associated vasculitides: insights from the DCVAS study. Neurol Neuroimmunol Neuroinflammation. 2019;6(6):e615.
   PubMed Web of Science ®Google Scholar
• McGeoch L, Carette S, Cuthbertson D, et al. Cardiac involvement in granulomatosis with polyangiitis. J Rheumatol. 2015;42(7):1209–1212.
   PubMed Web of Science ®Google Scholar
• Forstot JZ, Overlie PA, Neufeld GK, et al. Cardiac complications of Wegener granulomatosis: a case report of complete heart block and review of the literature. Semin Arthritis Rheum. 1980;10(2):148–154.
   PubMed Web of Science ®Google Scholar
• Merkel PA, Lo GH, Holbrook JT, et al. Brief communication: high incidence of venous thrombotic events among patients with Wegener granulomatosis: the Wegener’s Clinical Occurrence Of Thrombosis (WeCLOT) study. Ann Intern Med. 2005;142(8):620.
   PubMed Web of Science ®Google Scholar
• Allenbach Y, Seror R, Pagnoux C, et al. High frequency of venous thromboembolic events in Churg-Strauss syndrome, Wegener’s granulomatosis and microscopic polyangiitis but not polyarteritis nodosa: a systematic retrospective study on 1130 patients. Ann Rheum Dis. 2009;68(4):564–567.
   PubMed Web of Science ®Google Scholar
• Pagnoux C, Mahr A, Cohen P, et al. Presentation and outcome of gastrointestinal involvement in systemic necrotizing vasculitides: analysis of 62 patients with polyarteritis nodosa, microscopic polyangiitis, Wegener granulomatosis, Churg-Strauss syndrome, or rheumatoid arthritis-associated vasculitis. Medicine (Baltimore). 2005;84(2):115–128.
   PubMed Web of Science ®Google Scholar
• Masiak A, Zdrojewski Ł, Zdrojewski Z, et al. Gastrointestinal tract involvement in granulomatosis with polyangiitis. Gastroenterol Rev. 2016;4:270–275.
   Google Scholar
• Bosch X, Guilabert A, Font J. Antineutrophil cytoplasmic antibodies. Lancet Lond Engl. 2006;368(9533):404–418.
   PubMed Web of Science ®Google Scholar
• Stone JH, Talor M, Stebbing J, et al. Test characteristics of immunofluorescence and ELISA tests in 856 consecutive patients with possible ANCA-associated conditions. Arthritis Care Res Off J Arthritis Health Prof Assoc. 2000;13(6):424–434.
   PubMed Web of Science ®Google Scholar
• Wiik A. What you should know about PR3-ANCA. An introduction. Arthritis Res. 2000;2(4):252–254.
   PubMedGoogle Scholar
• Hagen EC, Daha MR, Hermans J, et al. Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis. EC/BCR project for ANCA assay standardization. Kidney Int. 1998;53(3):743–753.
   PubMed Web of Science ®Google Scholar
• Damoiseaux J, Csernok E, Rasmussen N, et al. Detection of antineutrophil cytoplasmic antibodies (ANCAs): a multicentre European Vasculitis Study Group (EUVAS) evaluation of the value of indirect immunofluorescence (IIF) versus antigen-specific immunoassays. Ann Rheum Dis. 2017;76(4):647–653.
   PubMed Web of Science ®Google Scholar
• Finkielman JD, Merkel PA, Schroeder D, et al. Antiproteinase 3 antineutrophil cytoplasmic antibodies and disease activity in Wegener granulomatosis. Ann Intern Med. 2007;147(9):611–619.
   PubMed Web of Science ®Google Scholar
• Tomasson G, Grayson PC, Mahr AD, et al. Value of ANCA measurements during remission to predict a relapse of ANCA-associated vasculitis–a meta-analysis. Rheumatol Oxf Engl. 2012;51(1):100–109.
   PubMed Web of Science ®Google Scholar
• Katzenstein AL, Locke WK. Solitary lung lesions in Wegener’s granulomatosis. Pathologic findings and clinical significance in 25 cases. Am J Surg Pathol. 1995;19(5):545–552.
   PubMed Web of Science ®Google Scholar
• Travis WD, Hoffman GS, Leavitt RY, et al. Surgical pathology of the lung in Wegener’s granulomatosis. Review of 87 open lung biopsies from 67 patients. Am J Surg Pathol. 1991;15(4):315–333.
   PubMed Web of Science ®Google Scholar
• Guillevin L, Durand-Gasselin B, Cevallos R, et al. Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum. 1999;42(3):421–430.
   PubMed Web of Science ®Google Scholar
• Bajema IM, Hagen EC, Hermans J, et al. Kidney biopsy as a predictor for renal outcome in ANCA-associated necrotizing glomerulonephritis. Kidney Int. 1999;56(5):1751–1758.
   PubMed Web of Science ®Google Scholar
• Berden AE, Ferrario F, Hagen EC, et al. Histopathologic classification of ANCA-associated glomerulonephritis. J Am Soc Nephrol JASN. 2010;21(10):1628–1636.
   PubMed Web of Science ®Google Scholar
• Papiris SA, Manali ED, Kalomenidis I, et al. Bench-to-bedside review: pulmonary-renal syndromes–an update for the intensivist. Crit Care Lond Engl. 2007;11(3):213.
   PubMed Web of Science ®Google Scholar
• McGrath MM, Isakova T, Rennke HG, et al. Contaminated cocaine and antineutrophil cytoplasmic antibody-associated disease. Clin J Am Soc Nephrol CJASN. 2011;6(12):2799–2805.
   PubMed Web of Science ®Google Scholar
• Langlois V, Lesourd A, Girszyn N, et al. Antineutrophil cytoplasmic antibodies associated with infective endocarditis. Medicine (Baltimore). 2016;95(3):e2564.
   PubMed Web of Science ®Google Scholar
• Pakalniskis MG, Berg AD, Policeni BA, et al. The many faces of granulomatosis with polyangiitis: a review of the head and neck imaging manifestations. AJR Am J Roentgenol. 2015;205(6):W619–629.
   PubMed Web of Science ®Google Scholar
• Stone JH, Hoffman GS, Merkel PA, et al. A disease-specific activity index for Wegener’s granulomatosis: modification of the Birmingham vasculitis activity score. international network for the study of the systemic vasculitides (INSSYS). Arthritis Rheum. 2001;44(4):912–920.
   PubMed Web of Science ®Google Scholar
• Jayne D, Rasmussen N, Andrassy K, et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med. 2003;349(1):36–44.
   PubMed Web of Science ®Google Scholar
• Walsh M, Merkel PA, Peh CA, et al. Plasma exchange and glucocorticoids in severe ANCA-associated vasculitis. N Engl J Med. 2020;382(7):622–631.
   PubMed Web of Science ®Google Scholar
• Brilland B, Garnier AS, Chevailler A, et al. Complement alternative pathway in ANCA-associated vasculitis: two decades from bench to bedside. Autoimmun Rev. 2020;19(1):102424.
   PubMed Web of Science ®Google Scholar
• Merkel PA, Niles JL, Jimenez R, et al. A randomized clinical trial of CCX168, an orally administered C5aR inhibitor for treatment of patients with ANCA-associated vasculitis; 2016.
   Google Scholar
• Jayne DRW, Bruchfeld AN, Harper L, et al. Randomized trial of C5a receptor inhibitor avacopan in ANCA-associated vasculitis. J Am Soc Nephrol JASN. 2017;28(9):2756–2767.
   PubMed Web of Science ®Google Scholar
• Jayne DRW, Merkel PA, Schall TJ, et al. Avacopan for the treatment of ANCA-associated vasculitis. N Engl J Med. 2021;384(7):599–609.
   PubMed Web of Science ®Google Scholar
• Walton EW. Giant-cell granuloma of the respiratory tract (Wegener’s granulomatosis). Br Med J. 1958;2(5091):265–270.
   PubMed Web of Science ®Google Scholar
• de Groot K, Harper L, Jayne DRW, et al. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial. Ann Intern Med. 2009;150(10):670–680.
   PubMed Web of Science ®Google Scholar
• Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010;363(3):221–232.
   PubMed Web of Science ®Google Scholar
• Jones RB, Tervaert JWC, Hauser T, et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med. 2010;363(3):211–220.
   PubMed Web of Science ®Google Scholar
• Specks U, Merkel PA, Seo P, et al. Efficacy of remission-induction regimens for ANCA-associated vasculitis. N Engl J Med. 2013;369(5):417–427.
   PubMed Web of Science ®Google Scholar
• De Groot K, Rasmussen N, Bacon PA, et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2005;52(8):2461–2469.
   PubMed Web of Science ®Google Scholar
• Jones RB, Hiemstra TF, Ballarin J, et al. Mycophenolate mofetil versus cyclophosphamide for remission induction in ANCA-associated vasculitis: a randomised, non-inferiority trial. Ann Rheum Dis. 2019;78(3):399–405.
   PubMed Web of Science ®Google Scholar
• Hiemstra TF, Walsh M, Mahr A, et al. Mycophenolate mofetil vs azathioprine for remission maintenance in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized controlled trial. JAMA. 2010;304(21):2381–2388.
   PubMed Web of Science ®Google Scholar
• Jayne DRW, Gaskin G, Rasmussen N, et al. Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol JASN. 2007;18(7):2180–2188.
   PubMed Web of Science ®Google Scholar
• Walsh M, Casian A, Flossmann O, et al. Long-term follow-up of patients with severe ANCA-associated vasculitis comparing plasma exchange to intravenous methylprednisolone treatment is unclear. Kidney Int. 2013;84(2):397–402.
   PubMed Web of Science ®Google Scholar
• Walsh M, Collister D, Zeng L, et al. The effects of plasma exchange in patients with ANCA-associated vasculitis: an updated systematic review and meta-analysis. Bmj. 2022;376:e064604.
   PubMed Web of Science ®Google Scholar
• Chung SA, Langford CA, Maz M, et al. American college of rheumatology/vasculitis foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Care Res. 2021;73(8):1088–1105.
   PubMed Web of Science ®Google Scholar
• Pagnoux C, Mahr A, Hamidou MA, et al. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med. 2008;359(26):2790–2803.
   PubMed Web of Science ®Google Scholar
• Guillevin L, Pagnoux C, Karras A, et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med. 2014;371(19):1771–1780.
   PubMed Web of Science ®Google Scholar
• Smith RM, Jones RB, Specks U, et al. Rituximab as therapy to induce remission after relapse in ANCA-associated vasculitis. Ann Rheum Dis. 2020;79(9):1243–1249.
   PubMed Web of Science ®Google Scholar
• Charles P, Terrier B, Perrodeau É, et al. 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(8):1143–1149.
   PubMed Web of Science ®Google Scholar
• Charles P, Perrodeau É, Samson M, et al. Long-term rituximab use to maintain remission of antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial. Ann Intern Med. 2020;173(3):179–187.
   PubMed Web of Science ®Google Scholar
• Flossmann O, Berden A, de Groot K, et al. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis. 2011;70(3):488–494.
   PubMed Web of Science ®Google Scholar
• Robson J, Doll H, Suppiah R, et al. Damage in the anca-associated vasculitides: long-term data from the European vasculitis study group (EUVAS) therapeutic trials. Ann Rheum Dis. 2015;74(1):177–184.
   PubMed Web of Science ®Google Scholar
• Kronbichler A, Jayne DRW, Mayer G. Frequency, risk factors and prophylaxis of infection in ANCA-associated vasculitis. Eur J Clin Invest. 2015;45(3):346–368.
   PubMed Web of Science ®Google Scholar
• Besada E, Koldingsnes W, Nossent JC. Serum immunoglobulin levels and risk factors for hypogammaglobulinaemia during long-term maintenance therapy with rituximab in patients with granulomatosis with polyangiitis. Rheumatol Oxf Engl. 2014;53(10):1818–1824.
   PubMed Web of Science ®Google Scholar
• Pagnoux C, Hogan SL, Chin H, et al. Predictors of treatment resistance and relapse in antineutrophil cytoplasmic antibody-associated small-vessel vasculitis: comparison of two independent cohorts. Arthritis Rheum. 2008;58(9):2908–2918.
   PubMed Web of Science ®Google Scholar
• Hogan SL, Falk RJ, Chin H, et al. Predictors of relapse and treatment resistance in antineutrophil cytoplasmic antibody-associated small-vessel vasculitis. Ann Intern Med. 2005;143(9):621–631.
   PubMed Web of Science ®Google Scholar
• Karras A, Pagnoux C, Haubitz M, et al. Randomised controlled trial of prolonged treatment in the remission phase of ANCA-associated vasculitis. Ann Rheum Dis. 2017;76(10):1662–1668.
   PubMed Web of Science ®Google Scholar