Granulomatosis with polyangiitis: news in diagnosis and treatment
Authors:
E. Lokočová; P. Horák; M. Skácelová
Authors place of work:
III. interní klinika NRE LF UP a FN, Olomouc
Published in the journal:
Čes. Revmatol., 27, 2019, No. 3, p. 126-135.
Category:
Review Article
Summary
Granulomatosis with polyangiitis (GPA) is a necrotizing vasculitis which belongs to ANCA associated vasculitides. It is characterized by the granulomas, most commonly affecting the nasal cavities, upper respiratory tract, lungs and kidneys. 82–94% of pacients with GPA are ANCA positive (antineutrophil cytoplasmic antibodies), especially type c-ANCA (specifically proteinase 3), in 20% p-ANCA (anti myeloperoxidase antibody) and ANCA negative in 10%. In edition to basic imaging we use as a standard lung HRCT (high resolution computed tomography) and brain and paranasal sinuses MRI (magnetic resonance imaging). Renal biopsy is important for diagnoses and assessing the severity of the disease. The Birmingham Vasculitis Activity Score (BVAS) is used to assess disease activity, both for initial diagnosis and for subsequent monitoring of treatment effect and relapse. Inicial therapy of GPA consists of high-dose glucocorticoids and cyclophosphamide or rituximab. Recently, a comparable effect has been demonstrated for mycophenolate mofetil. Plasma exchange is used for patients with rapidly progressing renal or pulmonary failure with pulmonary haemorrhage. Maintenance therapy of GPA consists of azathioprine, rituximab, methotrexate, mycophenolate mofetil, alternatively may be used leflunomide. Avacopan – C5a receptor inhibitor is new effective drug in replacing high-dose glucocorticoids.
Keywords:
azathioprine – rituximab – granulomatosis with polyangiitis – ANCA-associated vasculitis – Birmingham Vasculitis Activity Score – Cyclophosphamide – mycophenolate mofetil – Methotrexate – avacopan
Zdroje
1. Morović-Vergles J, Culo MI, et al. Clinical manifestation of antineutrophil cytoplazmic antibodies associated vasculitis. Lijec Vjesn 2014; 136: 228–231.
2. Seo P, Stone JH. The antineutrophil cytoplasmic antibody-associated vasculitides. Am J Med 2004; 117: 39.
3. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65: 1.
4. 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: 1101.
5. Watts R, Lane S, Hanslik T, et al. Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 2007; 66: 222.
6. Stone JH, Taylor M, Stebbing, et al. Test characteristics of of immunofluorescence and ELISA tests in 865 consecutive patients with possible ANCA-associated conditions. Arthritis Care Res 2000; 13: 424.
7. Hoffman GS, Specks U. Antineutrophil cytoplasmic antibodies. Arthritis Rheum 1998; 41: 1521.
8. Mahr A, Batteux F, Tubiana S, et al. Brief report: prevalence of antineutrophil cytoplasmic antibodies in inefective andocarditis. Arthritis Rheumatol 2014; 66: 1672.
9. Draibe JB, Fulladosa W, Cruzado M, et al. Current and novel biomarkers in anti-neutrophil cytoplasm-associated vasculitis. Clin Kidney J 2016; 9(4): 547–551.
10. Cordier JF, Valeyre D, Guillevin L, et al. Pulmonary Wegener’s granulomatosis. A clinical and imaging study of 77 cases. Chest 1990; 97: 906.
11. Ananthakrishnan L, Sharma N, Kanne JP. Wegener’s granulomatosis in the chest: high-resolution CT findings. AJR Am J Roentgenol 2009; 192: 676.
12. Silvera S, Vignaux O, Legmann P. Sinonasal and cerebral imaging findings in Wegener’s granulomatosis. Presse Med 2007; 36: 913.
13. Thompson G, Klecka M, Roden AC, et al. Biopsy-proven pulmonary capillaritis: A retrospective study of aetiologies including an in-depth look at isolated pulmonary capillaritis. Respirology 2016; 21: 734.
14. Borner U, Landis BN, Banz Y, et al. Diagnostic value of biopsies in identifying cytoplasmic antineutrophil cytoplasmic antibody-negative localized Wegener’s granulomatosis presenting primarily with sinonasal disease. Am J Rhinol Allergy 2012; 26: 475.
15. Yates M, Watts RA, Bajema IM, et al. EULAR/ERA-EDTCA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis 2016; 75(9): 1583–1594.
16. Hauer HA, Bajema IM, Van Houwelingen HC, et al. Determinants of outcome in ANCA-associated glomerulonephritis: a prospective clinico-histopathological analysis of 96 patients. Kidney Int 2002; 62: 1732.
17. Lind van Wijngaarden RA, Hauer HA, Wolterbeek R, et al. Clinical and histologic determinants of renal outcome in ANCA-associated vasculitis: A prospective analysis of 100 patients with severe renal involvement. J Am Soc Nephrol 2006; 17: 2264.
18. Berden AE, Ferrario F, Hagen EC, et al. Histopathologic classification of ANCA-associated glomerulonephritis. J Am Soc Nephrol 2010; 21: 1628.
19. Mukhtyar C, Lee R, Brown D, Carruthers D, et al. Modification and validation of the Birmingham Vasculitis Activity Score (version 3). Ann Rheum Dis 2009; 68(12): 1827–1832.
20. Stone JH, Merkel PA, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 2010; 363: 221.
21. Jones RB, Furuta S, Tervaert JW, et al. Rituximab versus cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial. Ann Rheum Dis 2015; 74: 1178.
22. Harper L, Morgan MD, Walsh M, et al. Pulse versus daily oral cycloclophosphamide for induction of remission in ANCA-associated vasculitis: long-term follow up. Ann Rheum Dis 2012; 71: 955.
23. Jones BR, Hiemstra T, Balladrin J, et al. Mycophenolate mofetil versus cyclophosphamide for remission induction in ANCA-assciated vasculitis: a randomised, non-inferiority trial. Ann Rheum Dis 2019; 78(3): 399–405.
24. Faurschou M, Westman K, Rasmussen N, et al. Brief report: long-term outcome of a randomized clinical trial comparing methotrexate to cyclophosphamide for remission induction in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum 2012; 64: 3472–3477.
25. Zadražil J, Horák P, Karásek D, a kol. Moderní farmakoterapie autoimunitních chorob, 2. vydání. Praha: Maxdorf 2019; 31.
26. Guillevin L, Pagnoux C, Karras A, et al. Rituximab versus azathioprine for mainetance in ANCA-associated vasculitis. N Engl J Med 2014; 371: 1771–1780.
27. 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: 2381–2388.
28. Springer J, Nutter B, Langford CA, et al. Granulomatosis with polyangiitis: impact of maintenance therapy duration. Medicine (Baltimore) 2014; 93: 82–90.
29. Jayne D, Bruchfeld A, et al. Randomized trial of C5a receptor inhibitor avacopan in ANCA-associated vasculitis. J Am Soc Nephrol 2017; 28(9): 2756–2767.
30. WGET. Etanercept plus standard therapy for Wegener’s granulomatosis. NEJM 2005; 352: 351–361.
31. Jayne D, Blockmans, Raashid L, et al. Efficacy and safety of belimumab and azathioprine for maintenance of remission in antineutrophil cytoplasmic antibody –associated vasculitis: A randomized controlled study. Arthritis Rheumatol 2019; 71(6): 952–963.
32. Savige J, Gillis D, Benson E, et al. International consensus statement on testing and reporting of antineutrophil cytoplasmic antibodies (ANCA). Am J Clin Pathol 1999; 111: 507–513.
33. Savige J, Dimech W, Fritzler M, et al. Addendum to the International Consensus Statement on testing and reporting of Antineutrophil Cytoplasmic antibodies. Quality control guidelines, comments, and recommendations for testing in other autoimmune diseases. Am J Clin Pathol 2003; 120: 312–318.
34. Finkielman JD, Lee AS, Hummel AM, et al. ANCA are detectable in nearly all patients with active severe Wegener’s granulomatosis. Am J Med 2007; 120: 643.e9–14.
35. Herlyn K, Gross WL, Reinhold-Keller E. Longitudinal effects of structured patient education programs for vasculitis patients. Z Rheumatol 2008; 67: 206–211.
36. Langford CA, Kerr GS. Pregnancy in vasculitis. Curr Opin Rheumatol 2002; 14: 36.
Štítky
Dermatology & STDs Paediatric rheumatology RheumatologyČlánok vyšiel v časopise
Czech Rheumatology
2019 Číslo 3
Najčítanejšie v tomto čísle
- Chronic recurrent multifocal osteomyelitis (CRMO) in childhood – review and original results
- Granulomatosis with polyangiitis: news in diagnosis and treatment
- Steroid myopathy
- Prenatal diagnosis of cardiac manifestation associated with lupus neonatorum?