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Current Rheumatology Reports

Erschienen in:

01.04.2012 | PEDIATRIC RHEUMATOLOGY (TJA LEHMAN, SECTION EDITOR)

Granulomatosis with Polyangiitis in Childhood

verfasst von: Marinka Twilt, Susanne Benseler, David Cabral

Erschienen in: Current Rheumatology Reports | Ausgabe 2/2012

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Abstract

Granulomatosis with polyangiitis (GPA) is a rare yet frequently organ- or life-threatening systemic vasculitis affecting small- to medium-sized arteries in multiple organs. It characteristically leads to alveolar hemorrhage and destructive, pauci-immune glomerulonephritis. GPA is also characterized by granulomas in the upper and lower respiratory tract causing erosive sinusitis and nodular or even cavitating lesions in the respiratory tract. Antineutrophil cytoplasmic antibodies, a hallmark of GPA, are likely integral to the pathogenesis and recently have become a therapeutic target. International collaborations in childhood vasculitis have led to the development and validation of childhood vasculitis classification criteria, advanced our understanding of the clinical phenotype at presentation of GPA, and improved our ability to capture disease activity and determine treatment choices. Treatment efficacy and safety data continue to be largely derived from adult GPA studies. This review focuses on the recent publications on epidemiology, pathogenesis, and treatment in childhood GPA and relevant publications from the adult GPA literature.

van der Woude FJ, Rasmussen N, Lobatto S, Wiik A, Permin H, van Es LA, et al. Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker of disease activity in Wegener’s granulomatosis. L. 1985;1(8426):425–9.

Kallenberg CG. Pathogenesis of PR3-ANCA associated vasculitis. J Autoimmun. 2008;30(1–2):29–36.PubMedCrossRef

•• Falk RJ, Gross WL, Guillevin L, Hoffman GS, Jayne DR, Jennette JC, et al. Granulomatosis with polyangiitis (Wegener’s): an alternative name for Wegener’s granulomatosis. Arthritis Rheum. 2011;63(4):863–4. It is important for this nomenclature change to be recognized promptly and adopted universally.PubMedCrossRef

Radhakrishna SM, Shaham B, Marzan KAB, Brown DE, Reiff AO. Paediatric rheumatic disease in the intensive care unit: lessons learned from 15 years experience in a tertiary care paediatric hospital. Arthritis Rheum. 2010;62(10, supplement):S879.

Akikusa JD, Schneider R, Harvey EA, Hebert D, Thorner PS, Laxer RM, et al. Clinical features and outcome of pediatric Wegener’s granulomatosis. Arthritis Rheum. 2007;57(5):837–44.PubMedCrossRef

Rottem M, Fauci AS, Hallahan CW, Kerr GS, Lebovics R, Leavitt RY, et al. Wegener granulomatosis in children and adolescents: clinical presentation and outcome. J Pediatr. 1993;122(1):26–31.PubMedCrossRef

• Arulkumaran N, Jawad S, Smith SW, Harper L, Brogan P, Pusey CD, et al. Long- term outcome of paediatric patients with ANCA vasculitis. Pediatr Rheumatol Online J. 2011;9:12. This was a very small series of pediatric-onset GPA patients but with very long-term follow-up, with some valuable lessons to be taken from outcome findings.PubMedCrossRef

•• Cabral DA, Uribe AG, Benseler S, O’Neil KM, Hashkes PJ, Higgins G, et al. Classification, presentation, and initial treatment of Wegener’s granulomatosis in childhood. Arthritis Rheum. 2009;60(11):3413–24. This is the largest pediatric cohort of GPA elected to date and provides relatively contemporary time-of-diagnosis data.PubMedCrossRef

Wilkinson NM, Page J, Uribe AG, Espinosa V, Cabral DA. Establishment of a pilot pediatric registry for chronic vasculitis is both essential and feasible: a Childhood Arthritis and Rheumatology Alliance (CARRA) survey. J Rheumatol. 2007;34(1):224–6.PubMed

10.

• Eleftheriou D, Brogan PA. Vasculitis in children. Best Pract Res Clin Rheumatol. 2009;23(3):309–23. This is a good recent overview of childhood vasculitis in general and also GPA specifically.PubMedCrossRef

11.

Watts RA, Scott DG, Lane SE. Epidemiology of Wegener’s granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome. Cleve Clin J Med. 2002;69 Suppl 2:SII84–6.PubMedCrossRef

12.

Watts RA, Lane SE, Bentham G, Scott DG. Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom. Arthritis Rheum. 2000;43(2):414–9.PubMedCrossRef

13.

Watts RA, Gonzalez-Gay MA, Lane SE, Garcia-Porrua C, Bentham G, Scott DG. Geoepidemiology of systemic vasculitis: comparison of the incidence in two regions of Europe. Ann Rheum Dis. 2001;60(2):170–2.PubMedCrossRef

14.

Hall SL, Miller LC, Duggan E, Mauer SM, Beatty EC, Hellerstein S. Wegener granulomatosis in pediatric patients. J Pediatr. 1985;106(5):739–44.PubMedCrossRef

15.

Mahr AD, Neogi T, Merkel PA. Epidemiology of Wegener’s granulomatosis: lessons from descriptive studies and analyses of genetic and environmental risk determinants. Clin Exp Rheumatol. 2006;24(2 Suppl 41):S82–91.PubMed

16.

Koldingsnes W, Nossent H. Epidemiology of Wegener’s granulomatosis in northern Norway. Arthritis Rheum. 2000;43(11):2481–7.PubMedCrossRef

17.

Koldingsnes W, Gran JT, Omdal R, Husby G. Wegener’s granulomatosis: long-term follow-up of patients treated with pulse cyclophosphamide. Br J Rheumatol. 1998;37(6):659–64.PubMedCrossRef

18.

Cotch MF, Hoffman GS, Yerg DE, Kaufman GI, Targonski P, Kaslow RA. The epidemiology of Wegener’s granulomatosis. Estimates of the five-year period prevalence, annual mortality, and geographic disease distribution from population-based data sources. Arthritis Rheum. 1996;39(1):87–92.PubMedCrossRef

19.

O’Donnell JL, Stevanovic VR, Frampton C, Stamp LK, Chapman PT. Wegener’s granulomatosis in New Zealand: evidence for a latitude-dependent incidence gradient. Intern Med J. 2007;37(4):242–6.PubMedCrossRef

20.

Belostotsky VM, Shah V, Dillon MJ. Clinical features in 17 paediatric patients with Wegener granulomatosis. Pediatr Nephrol. 2002;17(9):754–61.PubMedCrossRef

21.

Grisaru S, Yuen GW, Miettunen PM, Hamiwka LA. Incidence of Wegener’s granulomatosis in children. J Rheumatol. 2010;37(2):440–2.PubMedCrossRef

22.

Fries JF, Hunder GG, Bloch DA, Michel BA, Arend WP, Calabrese LH, et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis. Summary. Arthritis Rheum. 1990;33(8):1135–6.PubMedCrossRef

23.

Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum. 1990;33(8):1101–7.PubMedCrossRef

24.

Ozen S, Ruperto N, Dillon MJ, Bagga A, Barron K, Davin JC, et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis. 2006;65(7):936–41.PubMedCrossRef

25.

•• Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, et al. EULAR/PRINTO/PRES criteria for Henoch-Schonlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis. 2010;69(5):798–806. This is an important milestone that recognizes the differences between childhood and adulthood presentations, and therefore classification, of GPA.PubMedCrossRef

26.

• Basu N, Watts R, Bajema I, Baslund B, Bley T, et al. EULAR points to consider in the development of classification and diagnostic criteria in systemic vasculitis. Ann Rheum Dis. 2010;69(10):1744–50. This is an important overview that acknowledges the limitations of the existing classification systems for vasculitis.PubMedCrossRef

27.

Watts RA, Suppiah R, Merkel PA, Luqmani R. Systemic vasculitis—is it time to reclassify? Rheumatology (Oxford) 2010.

28.

• Uribe AG, Cabral DA, Morishita K, et al. Relative performance of two validated classification systems for Wegener’s granulomatosis among patients with ANCA-associated vasculitis in a registry of children with vasculitis (ARChiVe). Arthritis Rheum. 2010;62(10):S705. This abstract characterizes some pediatric-specific difficulties with the existing classification systems for vasculitis.

29.

Liu LJ, Chen M, Yu F, Zhao MH, Wang HY. Evaluation of a new algorithm in classification of systemic vasculitis. Rheumatology (Oxford). 2008;47(5):708–12.CrossRef

30.

Linder R, Orth I, Hagen EC, van der Woude FJ, Schmitt WH. Differentiation between Wegener’s granulomatosis and microscopic polyangiitis by an artificial neural network and by traditional methods. J Rheumatol. 2011;38(6):1039–47.PubMedCrossRef

31.

Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W, 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(2):222–7.PubMedCrossRef

32.

Finkielman JD, Lee AS, Hummel AM, Viss MA, Jacob GL, Homburger HA, et al. ANCA are detectable in nearly all patients with active severe Wegener’s granulomatosis. Am J Med. 2007;120(7):643.e9–643.e14.CrossRef

33.

Savige JA, Davies DJ, Gatenby PA. Anti-neutrophil cytoplasmic antibodies (ANCA): their detection and significance: report from workshops. Pathology. 1994;26(2):186–93.CrossRef

34.

Csernok E, Holle J, Hellmich B, Willem J, Tervaert C, Kallenberg CG, et al. Evaluation of capture ELISA for detection of antineutrophil cytoplasmic antibodies directed against proteinase 3 in Wegener’s granulomatosis: first results from a multicentre study. Rheumatology (Oxford). 2004;43(2):174–80.CrossRef

35.

Xiao H, Heeringa P, Hu P, Liu Z, Zhao M, Aratani Y, et al. Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice. J Clin Invest. 2002;110(7):955–63.PubMed

36.

Little MA, Smyth L, Salama AD, Mukherjee S, Smith J, Haskard D, et al. Experimental autoimmune vasculitis: an animal model of anti-neutrophil cytoplasmic autoantibody-associated systemic vasculitis. Am J Pathol. 2009;174(4):1212–20.PubMedCrossRef

37.

•• Kallenberg CG. Pathophysiology of ANCA-associated small vessel vasculitis. Curr Rheumatol Rep. 2010;12(6):399–405. This is a relatively contemporary review of existing knowledge regarding the pathophysiology of AAV.PubMedCrossRef

38.

Tervaert JW. Proteinase 3: A cofactor for the binding of antineutrophil cytoplasm antibodies (ANCA) to endothelial cells? Kidney Int. 2000;57(5):2171–2.PubMedCrossRef

39.

Richter AG, Perkins GD, Chavda A, Sapey E, Harper L, Thickett DR. Neutrophil chemotaxis in Wegener’s granulomatosis and idiopathic pulmonary fibrosis. Eur Respir J 2011.

40.

• Kessenbrock K, Krumbholz M, Schonermarck U, Back W, Gross WL, Werb Z, et al. Netting neutrophils in autoimmune small-vessel vasculitis. Nat Med. 2009;15(6):623–5. This article includes important observations about the role of neutrophils in disease pathogenesis.PubMedCrossRef

41.

Cao Y, Schmitz JL, Yang J, Hogan SL, Bunch D, Hu Y, et al. DRB1*15 allele is a risk factor for PR3-ANCA disease in African Americans. J Am Soc Nephrol. 2011;22(6):1161–7.PubMedCrossRef

42.

• Tadema H, Heeringa P, Kallenberg CG. Bacterial infections in Wegener’s granulomatosis: mechanisms potentially involved in autoimmune pathogenesis. Curr Opin Rheumatol. 2011;23(4):366–71. This is a good relatively contemporary review of existing knowledge specifically regarding the role of infection in the pathophysiology of AAV.PubMedCrossRef

43.

Stegmayr BG, Gothefors L, Malmer B, Muller Wiefel DE, Nilsson K, Sundelin B. Wegener granulomatosis in children and young adults. A case study of ten patients. Pediatr Nephrol. 2000;14(3):208–13.PubMedCrossRef

44.

Eustaquio ME, Chan KH, Deterding RR, Hollister RJ. Multilevel airway involvement in children with Wegener’s granulomatosis: clinical course and the utility of a multidisciplinary approach. Arch Otolaryngol Head Neck Surg. 2011;137(5):480–5.PubMedCrossRef

45.

• Holle JU, Gross WL, Latza U, Nolle B, Ambrosch P, Heller M, et al. Improved outcome in 445 patients with Wegener’s granulomatosis in a German vasculitis center over four decades. Arthritis Rheum. 2011;63(1):257–66. This article provides important information about the long-term outcome of adult GPA patients and trends toward improvement over time that likely is associated with changing treatment strategies.PubMedCrossRef

46.

Flossmann O, Berden A, de GK, Hagen C, Harper L, Heijl C, et al. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis. 2011;70(3):488–94.PubMedCrossRef

47.

• Heijl C, Harper L, Flossmann O, Stucker I, Scott DG, Watts RA, et al. Incidence of malignancy in patients treated for antineutrophil cytoplasm antibody-associated vasculitis: follow-up data from European Vasculitis Study Group clinical trials. Ann Rheum Dis. 2011;70(8):1415–21. A contemporary review of malignancy risks is always relevant to clinical practice.PubMedCrossRef

48.

Hajj-Ali RA, Wilke WS, Calabrese LH, Hoffman GS, Liu X, Bena J, et al. Pilot study to assess the frequency of fibromyalgia, depression, and sleep disorders in patients with granulomatosis with polyangiitis (Wegener’s). Arthritis Care Res (Hoboken). 2011;63(6):827–33.CrossRef

49.

Walsh M, Mukhtyar C, Mahr A, Herlyn K, Luqmani R, Merkel PA, et al. Health-related quality of life in patients with newly diagnosed antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Care Res (Hoboken). 2011;63(7):1055–61.CrossRef

50.

Wierzbicka M, Szyfter W, Puszczewicz M, Borucki L, Bartochowska A. Otologic symptoms as initial manifestation of wegener granulomatosis: diagnostic dilemma. Otol Neurotol. 2011;32(6):996–1000.PubMedCrossRef

51.

Fasunla JA, Hundt W, Lutz J, Forger F, Thurmel K, Steinbach S. Evaluation of smell and taste in patients with Wegener’s granulomatosis. Eur Arch Otorhinolaryngol 2011.

52.

Miszalski-Jamka T, Szczeklik W, Sokolowska B, Miszalski-Jamka K, Karwat K, Grzadziel G, et al. Cardiac involvement in Wegener’s granulomatosis resistant to induction therapy. Eur Radiol. 2011;21(11):2297–304.PubMedCrossRef

53.

• Suppiah R, Judge A, Batra R, Flossmann O, Harper L, Hoglund P, et al. A model to predict cardiovascular events in patients with newly diagnosed Wegener’s granulomatosis and microscopic polyangiitis. Arthritis Care Res (Hoboken). 2011;63(4):588–96. This article is a good reminder that cardiovascular events are an important and not uncommon consequence of GPA and its treatment, and there are some predictors of this.CrossRef

54.

Fauci AS, Wolff SM. Wegener’s granulomatosis: studies in eighteen patients and a review of the literature. 1973. Medicine (Baltimore). 1994;73(6):315–24.

55.

Moorthy AV, Chesney RW, Segar WE, Groshong T. Wegener granulomatosis in childhood: prolonged survival following cytotoxic therapy. J Pediatr. 1977;91(4):616–8.PubMedCrossRef

56.

Fauci AS, Haynes BF, Katz P, Wolff SM. Wegener’s granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med. 1983;98(1):76–85.PubMed

57.

Reinhold-Keller E, Beuge N, Latza U, de GK, Rudert H, Nolle B, et al. An interdisciplinary approach to the care of patients with Wegener’s granulomatosis: long-term outcome in 155 patients. Arthritis Rheum. 2000;43(5):1021–32.PubMedCrossRef

58.

Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med. 1992;116(6):488–98.PubMed

59.

•• Langford CA. Cyclophosphamide as induction therapy for Wegener’s granulomatosis and microscopic polyangiitis. Clin Exp Immunol. 2011;164 Suppl 1:31–4. This is a good overview of the ever-changing landscape of recommended therapies for GPA.PubMedCrossRef

60.

Talar-Williams C, Hijazi YM, Walther MM, Linehan WM, Hallahan CW, Lubensky I, et al. Cyclophosphamide-induced cystitis and bladder cancer in patients with Wegener granulomatosis. Ann Intern Med. 1996;124(5):477–84.PubMed

61.

Turnbull J, Harper L. Adverse effects of therapy for ANCA-associated vasculitis. Best Pract Res Clin Rheumatol. 2009;23(3):391–401.PubMedCrossRef

62.

Sneller MC, Hoffman GS, Talar-Williams C, Kerr GS, Hallahan CW, Fauci AS. An analysis of forty-two Wegener’s granulomatosis patients treated with methotrexate and prednisone. Arthritis Rheum. 1995;38(5):608–13.PubMedCrossRef

63.

Langford CA, Talar-Williams C, Barron KS, Sneller MC. A staged approach to the treatment of Wegener’s granulomatosis: induction of remission with glucocorticoids and daily cyclophosphamide switching to methotrexate for remission maintenance. Arthritis Rheum. 1999;42(12):2666–73.PubMedCrossRef

64.

Stone JH, Tun W, Hellman DB. Treatment of non-life threatening Wegener’s granulomatosis with methotrexate and daily prednisone as the initial therapy of choice. J Rheumatol. 1999;26(5):1134–9.PubMed

65.

de Groot K, Muhler M, Reinhold-Keller E, Paulsen J, Gross WL. Induction of remission in Wegener’s granulomatosis with low dose methotrexate. J Rheumatol. 1998;25(3):492–5.PubMed

66.

Jayne D, Rasmussen N, Andrassy K, Bacon P, Tervaert JW, Dadoniene J, et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med. 2003;349(1):36–44.PubMedCrossRef

67.

Hiemstra TF, Walsh M, Mahr A, Savage CO, de GK, Harper L, et al. Mycophenolate mofetil vs azathioprine for remission maintenance in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized controlled trial. JAMA. 2010;304(21):2381–8.PubMedCrossRef

68.

• de Groot K, Harper L, Jayne DR, Flores Suarez LF, Gregorini G, Gross WL, 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):670–80. This is a good head-to-head comparison of oral and intravenous cyclophosphamide administration—an issue that has been contentious at times.PubMed

69.

Seror R, Pagnoux C, Ruivard M, Landru I, Wahl D, Riviere S, et al. Treatment strategies and outcome of induction-refractory Wegener’s granulomatosis or microscopic polyangiitis: analysis of 32 patients with first-line induction-refractory disease in the WEGENT trial. Ann Rheum Dis. 2010;69(12):2125–30.PubMedCrossRef

70.

Langford CA, Talar-Williams C, Sneller MC. Use of methotrexate and glucocorticoids in the treatment of Wegener’s granulomatosis. Long-term renal outcome in patients with glomerulonephritis. Arthritis Rheum. 2000;43(8):1836–40.PubMedCrossRef

71.

de Groot K, Rasmussen N, Bacon PA, Tervaert JW, Feighery C, Gregorini G, 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):2461–9.PubMedCrossRef

72.

Hu W, Liu C, Xie H, Chen H, Liu Z, Li L. Mycophenolate mofetil versus cyclophosphamide for inducing remission of ANCA vasculitis with moderate renal involvement. Nephrol Dial Transplant. 2008;23(4):1307–12.PubMedCrossRef

73.

Szpirt WM, Heaf JG, Petersen J. Plasma exchange for induction and cyclosporine A for maintenance of remission in Wegener’s granulomatosis—a clinical randomized controlled trial. Nephrol Dial Transplant. 2011;26(1):206–13.PubMedCrossRef

74.

Casian A, Jayne D. Plasma exchange in the treatment of Wegener’s granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and renal limited vasculitis. Curr Opin Rheumatol. 2011;23(1):12–7.PubMedCrossRef

75.

•• Jones RB, Tervaert JW, Hauser T, Luqmani R, Morgan MD, Peh CA, et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med. 2010;363(3):211–20. This was one of two landmark randomized controlled trials with rituximab use compared against traditional cyclophosphamide use, albeit with any short-term outcome results.PubMedCrossRef

76.

•• Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010;363(3):221–32. This was one of two landmark randomized controlled trials with rituximab use compared against traditional cyclophosphamide use, albeit with only short-term outcome results.PubMedCrossRef

77.

Rees F, Yazdani R, Lanyon P. Long-term follow-up of different refractory systemic vasculitides treated with rituximab. Clin Rheumatol. 2011;30(9):1241–5.PubMedCrossRef

78.

Roccatello D, Sciascia S, Rossi D, Alpa M, Naretto C, Russo A, et al. Long-term effects of rituximab added to cyclophosphamide in refractory patients with vasculitis. Am J Nephrol. 2011;34(2):175–80.PubMedCrossRef

79.

•• Guerry MJ, Brogan P, Bruce IN, D’Cruz DP, Harper L, Luqmani R et al. Recommendations for the use of rituximab in anti-neutrophil cytoplasm antibody-associated vasculitis. Rheumatology (Oxford) 2011. This article provides expert consensus recommendations from both rheumatologists and nephrologists on use of rituximab with AAV based on a systematic review of the literature and including some pediatric input.

80.

Jones RB, Ferraro AJ, Chaudhry AN, Brogan P, Salama AD, Smith KG, et al. A multicenter survey of rituximab therapy for refractory antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2009;60(7):2156–68.PubMedCrossRef

81.

• Mukhtyar C, Guillevin L, Cid MC, Dasgupta B, de GK, Gross W, et al. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2009;68(3):310–7. These recommendations for management of small vessel vasculitis require updating in light of recent rituximab trials and do not address pediatric patients.PubMedCrossRef

82.

Tzaribachev N, Koetter I, Kuemmerle-Deschner JB, Schedel J. Rituximab for the treatment of refractory pediatric autoimmune diseases: a case series. Cases J. 2009;2:6609.PubMedCrossRef

83.

Hugle B, Solomon M, Harvey E, James A, Wadhwa A, Amin R, et al. Pneumocystis jiroveci pneumonia following rituximab treatment in Wegener’s granulomatosis. Arthritis Care Res (Hoboken). 2010;62(11):1661–4.CrossRef

84.

Luqmani RA, Bacon PA, Moots RJ, Janssen BA, Pall A, Emery P, et al. Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis. QJM. 1994;87(11):671–8.PubMed

85.

Mukhtyar C, Lee R, Brown D, Carruthers D, Dasgupta B, Dubey S, et al. Modification and validation of the Birmingham Vasculitis Activity Score (version 3). Ann Rheum Dis. 2009;68(12):1827–32.PubMedCrossRef

86.

Exley AR, Bacon PA, Luqmani RA, Kitas GD, Gordon C, Savage CO, et al. Development and initial validation of the Vasculitis Damage Index for the standardized clinical assessment of damage in the systemic vasculitides. Arthritis Rheum. 1997;40(2):371–80.PubMedCrossRef

87.

Suppiah R, Flossman O, Mukhtyar C, Alberici F, Baslund B, Brown D, et al. Measurement of damage in systemic vasculitis: a comparison of the Vasculitis Damage Index with the Combined Damage Assessment Index. Ann Rheum Dis. 2011;70(1):80–5.PubMedCrossRef

88.

Merkel PA, Aydin SZ, Boers M, Direskeneli H, Herlyn K, Seo P, et al. The OMERACT core set of outcome measures for use in clinical trials of ANCA-associated vasculitis. J Rheumatol. 2011;38(7):1480–6.PubMedCrossRef

89.

• Morishita KA, Li SC, Muscal E, Spalding S, Guzman J, Uribe A, et al. Assessing the performance of the Birmingham Vasculitis Activity Score (BVAS v.3) at diagnosis for children with ANCA-associated vasculitis (AAV) in A Registry for Childhood Vasculitis (ARChiVe). J Rheumatol. 2012; accepted for publication. This article highlights the importance of developing and using pediatric-specific disease activity measurement tools, ultimately for both clincial practice and future clincial trials.

Titel: Granulomatosis with Polyangiitis in Childhood
verfasst von: Marinka Twilt
Susanne Benseler
David Cabral
Publikationsdatum: 01.04.2012
Verlag: Current Science Inc.
Erschienen in: Current Rheumatology Reports / Ausgabe 2/2012
Print ISSN: 1523-3774
Elektronische ISSN: 1534-6307
DOI: https://doi.org/10.1007/s11926-012-0238-6

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