Top

27-04-2016 | Small-vessel vasculitis | Review | Article

Management of Small Vessel Vasculitides

Journal: Current Rheumatology Reports

Authors: Giuseppe Lopalco, Donato Rigante, Vincenzo Venerito, Giacomo Emmi, Maria Grazia Anelli, Giovanni Lapadula, Florenzo Iannone, Luca Cantarini

Publisher: Springer US

Abstract

Inflammation mediated by cells of the immune system and necrosis are the most striking features observed at the histologic level in patients with vasculitides, clinical entities classified according to pathologic findings involving different organs, to etiology, or to size of vessels involved. Small vessel vasculitides (SVV) are a peculiar group of systemic disorders electively involving small intraparenchymal arteries, arterioles, capillaries, or venules and leading to different levels of vascular obstruction, tissue ischemia and risk of infarction; they can be divided into anti-neutrophil cytoplasmic antibody-associated vasculitides and immune complex vasculitides. Despite the significant advances in understanding the whole disease process and pathophysiology of SVV, strong efforts are still needed to draft, share and spread guidelines in the therapeutic management of these protean disorders. After an accurate evaluation of different open or double-blind trials and cohort studies in this review, we analyze the actual medical tools suggested for treating granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, Henoch-Schönlein purpura, cryoglobulinemic vasculitis, anti-glomerular basement membrane disease and hypocomplementemic urticarial vasculitis.

Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med. 1997;337:1512–23.CrossRefPubMed

2.••

Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65:1–11. This paper represents the latest effort to improve nomenclature, to change names and definitions as appropriate, and to add important categories of vasculitis that were not included in the first International Chapel Hill Consensus Conference. CrossRefPubMed

Flossmann O, Berden A, de Groot K, Hagen C, Harper L, Heijl C, et al. European Vasculitis Study Group. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis. 2011;70:488–94.CrossRefPubMed

Walton EW. Giant-cell granuloma of the respiratory tract (Wegener's granulomatosis). Br Med J. 1958;2(5091):265–70.CrossRefPubMedPubMedCentral

Mukhtyar C, Guillevin L, Cid MC, Dasgupta B, de Groot K, Gross W, et al. European Vasculitis Study Group. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2009;68:310–7.CrossRefPubMed

Guillevin L, Lhote F, Gayraud M, Cohen P, Jarrousse B, Lortholary O, et al. Prognostic factors in polyarteritis nodosa and Churg–Strauss Syndrome. A prospective study in 342 patients. Medicine (Baltimore). 1996;75:17–28.CrossRef

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;68:1827–32.CrossRefPubMed

8.••

Holle JU, Gross WL. Treatment of ANCA-associated vasculitides (AAV). Autoimmun Rev. 2013;12:483–6. This article summarizes the current evidence for the use of conventional therapy and biologics in ANCA-associated vasculitis. CrossRefPubMed

9.•

Walsh M, Casian A, Flossmann O, Westman K, Höglund P, Pusey C, 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;84:397–402. This study evaluates a comparison between plasma exchange and intravenous methylprednisolone in improving renal disease. CrossRefPubMed

10.••

Walsh M, Merkel PA, Peh CA, Szpirt W, Guillevin L, Pusey CD, et al. Plasma exchange and glucocorticoid dosing in the treatment of anti-neutrophil cytoplasm antibody associated vasculitis (PEXIVAS): protocol for a randomized controlled trial. Trials. 2013;73:14. A trial to determine whether plasma exchange as well as immunosuppressive therapy are effective in reducing death and end-stage renal disease in ANCA-associated vasculitis.

11.

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;52:2461–9.CrossRefPubMed

12.•

Jones RB, Harper L, Ballarin J, Brogan P, Bruchfeld A, et al. A randomised trial of mycophenolate mofetil versus cyclophosphamide for remission induction of ANCA associated vasculitis. Presse Med. 2013;42:678. These preliminary data attest that mycophenolate mofetil is effective as intravenous cyclophosphamide pulses for induction of remission. CrossRef

13.

Comarmond C, Cacoub P. Granulomatosis with polyangiitis (Wegener): clinical aspects and treatment. Autoimmun Rev. 2014;13:1121–5.CrossRefPubMed

14.•

Pagnoux C, Quéméneur T, Ninet J, Diot E, Kyndt X, de Wazières B, et al. Treatment of systemic necrotizing vasculitides in patients aged sixty-five years or older: results of a multicenter, open-label, randomized controlled trial of corticosteroid and cyclophosphamide-based induction therapy. Arthritis Rheumatol. 2015;67:1117–27. New therapeutic strategy for induction of remission in older patients with systemic necrotizing vasculitides, capable of reducing serious adverse events within the first 3 years of follow-up. CrossRefPubMed

15.

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;150:670–80.CrossRefPubMed

16.

Weiner GJ. Rituximab: mechanism of action. Semin Hematol. 2010;47:115–23.CrossRefPubMedPubMedCentral

17.

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;363:211–20.CrossRefPubMed

18.

Jones RB, Walsh M, Jayne DRW, On behalf of the European Vasculitis Study Group. Two-year follow-up results from a randomized trial of RTX versus CyP for ANCA-associated renal vasculitis: RITUXVAS. Clin Exp Immunol. 2011;164 Suppl 1:57–8.

19.

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;363:221–32.CrossRefPubMedPubMedCentral

20.••

Specks U, Merkel PA, Seo P, Spiera R, Langford CA, Hoffman GS, et al. Efficacy of remission-induction regimens for ANCA-associated vasculitis. N Engl J Med. 2013;369:417–27. A single course of rituximab was as effective as continuous conventional immunosuppressive therapy for the induction and maintenance of remission over the course of 18 months. CrossRefPubMed

21.

de Menthon M, Cohen P, Pagnoux C, Buchler M, Sibilia J, Détrée F, et al. Infliximab or rituximab for refractory Wegener's granulomatosis: long-term follow up. A prospective randomised multicentre study on 17 patients. Clin Exp Rheumatol. 2011;29(1 Suppl 64):S63–71.PubMed

22.

Booth A, Harper L, Hammad T, Bacon P, Griffith M, Levy J, et al. Prospective study of TNF(alpha) blockade with infliximab in anti-neutrophil cytoplasmic antibody-associated systemic vasculitis. J Am Soc Nephrol. 2004;15:717–21.CrossRefPubMed

23.

Booth AD, Jayne DR, Kharbanda RK, McEniery CM, Mackenzie IS, Brown J, et al. Infliximab improves endothelial dysfunction in systemic vasculitis: a model of vascular inflammation. Circulation. 2004;109:1718–23.CrossRefPubMed

24.

Morgan MD, Drayson MT, Savage CO, Harper L. Addition of infliximab to standard therapy for ANCA-associated vasculitis. Nephron Clin Pract. 2011;117:c89–97.CrossRefPubMed

25.

Laurino S, Chaudhry A, Booth A, Conte G, Jayne D. Prospective study of TNF-alpha blockade with adalimumab in ANCA-associated systemic vasculitis with renal involvement. Nephrol Dial Transplant. 2010;25:3307–14.CrossRefPubMed

26.••

Langford CA, Monach PA, Specks U, Seo P, Cuthbertson D, McAlear CA, et al. An open-label trial of abatacept (CTLA4-IG) in non-severe relapsing granulomatosis with polyangiitis (Wegener's). Ann Rheum Dis. 2014;73:1376–9. The first report of an open-label trial of abatacept in non-severe relapsing granulomatosis with polyangiitis. CrossRefPubMedPubMedCentral

27.••

Berti A, Cavalli G, Campochiaro C, Guglielmi B, Baldissera E, Cappio S, et al. Interleukin-6 in ANCA-associated vasculitis: Rationale for successful treatment with tocilizumab. Semin Arthritis Rheum. 2015;45:48–54. The finding of an activated IL-6 pathway in patients with ANCA-associated vasculitis provides a rationale for the successful treatment with tocilizumab. CrossRefPubMed

28.

Takenaka K, Ohba T, Suhara K, Sato Y, Nagasaka K. Successful treatment of refractory aortitis in antineutrophil cytoplasmic antibody-associated vasculitis using tocilizumab. Clin Rheumatol. 2014;33:287–9.CrossRefPubMed

29.

Sumida K, Ubara Y, Suwabe T, Hayami N, Hiramatsu R, Hasegawa E. Complete remission of myeloperoxidase-anti-neutrophil cytoplasmic antibody-associated crescentic glomerulonephritis complicated with rheumatoid arthritis using a humanized anti-interleukin 6 receptor antibody. Rheumatology (Oxford). 2011;50:1928–30.CrossRef

30.

Schreiber A, Pham CT, Hu Y, Schneider W, Luft FC, Kettritz R. Neutrophil serine proteases promote IL-1β generation and injury in necrotizing crescentic glomerulonephritis. J Am Soc Nephrol. 2012;23:470–82.CrossRefPubMedPubMedCentral

31.

Chen M, Daha MR, Kallenberg CG. The complement system in systemic autoimmune disease. J Autoimmun. 2010;34:J276–86.CrossRefPubMed

32.••

Jayne DR, Bruchfeld A, Schaier M, Ciechanowski K, Harper L, Jadoul M, et al. Oral C5A receptor antagonist CCX168 phase 2 clinical trial in ANCA-associated renal vasculitis. Ann Rheum Dis. 2014;73(S2):148. The most recent report that supports CCX168 efficacy combined with cyclophosphamide for the management of ANCA-associated renal vasculitis. CrossRef

33.

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

34.

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

35.

Pagnoux C, Mahr A, Hamidou MA, Boffa JJ, Ruivard M, Ducroix JP, et al. AZA or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med. 2008;359:2790–803.CrossRefPubMed

36.

Metzler C, Miehle N, Manger K, Iking-Konert C, de Groot K, Hellmich B, et al. Elevated relapse rate under oral methotrexate versus leflunomide for maintenance of remission in Wegener's granulomatosis. Rheumatology. 2007;46:1087–91.CrossRefPubMed

37.

Popa ER, Tervaert JW. The relation between Staphylococcus aureus and Wegener's granulomatosis: current knowledge and future directions. Intern Med. 2003;42:771–80.CrossRefPubMed

38.

Stegeman CA, Tervaert JW, de Jong PE, Kallenberg CG. Trimethoprim-sulfamethoxazole (co-trimoxazole) for the prevention of relapses of Wegener's granulomatosis. Dutch Co-Trimoxazole Wegener Study Group. N Engl J Med. 1996;335:16–20.CrossRefPubMed

39.

Zycinska K, Wardyn KA, Zielonka TM, Krupa R, Lukas W. Co-trimoxazole and prevention of relapses of PR3-ANCA positive vasculitis with pulmonary involvement. Eur J Med Res. 2009;14 Suppl 4:265–7.CrossRefPubMedPubMedCentral

40.

Holle JU, Gross WL, Holl-Ulrich K, Ambrosch P, Noelle B, Both M, et al. Prospective long-term follow-up of patients with localized Wegener's granulomatosis: does it occur as persistent disease stage? Ann Rheum Dis. 2010;69:1934–9.CrossRefPubMed

41.

Pagnoux C, Stubbe M, Lifermann F, Decaux O, Pavic C, Bérezné, et al. Wegener's granulomatosis strictly and persistently localized to one organ is rare: assessment of 16 patients from the French Vasculitis Study Group database. J Rheumatol. 2011;38:475–8.CrossRefPubMed

42.

Jayne DR, Davies MJ, Fox CJ, Black CM, Lockwood CM. Treatment of systemic vasculitis with pooled intravenous immunoglobulin. Lancet. 1991;337:11379.CrossRef

43.

Jayne DR, Lockwood CM. Pooled intravenous immunoglobulin in the management of systemic vasculitis. Adv Exp Med Biol. 1993;336:469–72.CrossRefPubMed

44.

Jayne DR, Chapel H, Adu D, Misbah S, O'Donoghue D, Scott D, et al. Intravenous immunoglobulin for ANCA-associated systemic vasculitis with persistent disease activity. QJM. 2000;93:433–9.CrossRefPubMed

45.

Martinez V, Cohen P, Pagnoux C, Vinzio S, Mahr A, Mouthon L, et al. Intravenous immunoglobulins for relapses of systemic vasculitides associated with antineutrophil cytoplasmic autoantibodies: results of a multicenter, prospective, open-label study of twenty-two patients. Arthritis Rheum. 2008;58:308–17.CrossRefPubMed

46.•

Crickx E, Machelart I, Lazaro E, Kahn JE, Cohen-Aubart F, Martin T, et al. Intravenous immunoglobulin as immunomodulating agent in ANCA-associated vasculitides: a French nationwide study of 92 patients. Arthritis Rheumatol. 2015. doi:10.1002/art.39472. The most recent study that shows the clinical benefit of intravenous immunoglobulin as adjunctive therapy for treating ANCA-associated vasculitides.

47.

Smith RM, Jones RB, Guerry MJ, Laurino S, Catapano F, Chaudhry A, et al. Rituximab for remission maintenance in relapsing antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2012;64:3760–9.CrossRefPubMed

48.••

Guillevin L, Pagnoux C, Karras A, Khouatra C, Aumaître O, Cohen P, et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med. 2014;371:1771–80. In the present study, rituximab was superior to azathioprine to maintain remission of ANCA-associated vasculitis. CrossRefPubMed

49.

Stone JH, Uhlfelder ML, Hellmann DB, Crook S, Bedocs NM, Hoffman GS. Etanercept combined with conventional treatment in Wegener's granulomatosis: a six-month open-label trial to evaluate safety. Arthritis Rheum. 2001;44:1149–54.CrossRefPubMed

50.

Wegener's Granulomatosis Etanercept Trial (WGET) Research Group. Etanercept plus standard therapy for Wegener's granulomatosis. N Engl J Med. 2005;352:351–61.CrossRef

51.

Silva F, Seo P, Schroeder DR, Stone JH, Merkel PA, Hoffman GS, et al. Solid malignancies among etanercept-treated patients with granulomatosis with polyangiitis (Wegener's): long-term follow up of a multicentre longitudinal cohort. Arthritis Rheum. 2011;63:2495–503.CrossRefPubMedPubMedCentral

52.

Krumbholz M, Specks U, Wick M, Kalled SL, Jenne D, Meinl E. BAFF is elevated in serum of patients with Wegener's granulomatosis. J Autoimmun. 2005;25:298–302.CrossRefPubMed

53.

Ribi C, Cohen P, Pagnoux C, Mahr A, Arene JP, Lauque D, et al. Treatment of Churg–Strauss syndrome without poor-prognosis factors: a multicenter, prospective, randomized, open-label study of seventy-two patients. Arthritis Rheum. 2008;58:586–94.CrossRefPubMed

54.

Comarmond C, Pagnoux C, Khellaf M, Cordier JF, Hamidou M, Viallard JF, et al. Eosinophilic granulomatosis with polyangiitis (Churg–Strauss): clinical characteristics and long-term follow-up of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis Rheum. 2013;65:270–81.CrossRefPubMed

55.••

Groh M, Pagnoux C, Baldini C, Bel E, Bottero P, Cottin V, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. Eur J Intern Med. 2015;26:545–53. This paper provides useful tools for an effective individual management of patients with eosinophilic granulomatosis with polyangiitis. CrossRefPubMed

56.

Cohen P, Pagnoux C, Mahr A, Arene JP, Mouthon L, Le Guern V, et al. Churg–Strauss syndrome with poor-prognosis factors: a prospective multicenter trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in forty-eight patients. Arthritis Rheum. 2007;57:686–93.CrossRefPubMed

57.

Gayraud M, Guillevin L, Cohen P, Lhote F, Cacoub P, Deblois P, et al. Treatment of good-prognosis polyarteritis nodosa and Churg–Strauss syndrome: comparison of steroids and oral or pulse cyclophosphamide in 25 patients. Br J Rheumatol. 1997;35:1290–7.CrossRef

58.

Pagnoux C, Guilpain P, Guillevin L. Churg–Strauss syndrome. Curr Opin Rheumatol. 2007;19:25–32.CrossRefPubMed

59.

Metzler C, Hellmich B, Gause A, Gross WL, de Groot K. Churg Strauss syndrome--successful induction of remission with methotrexate and unexpected high cardiac and pulmonary relapse ratio during maintenance treatment. Clin Exp Rheumatol. 2004;22(6 Suppl 36):S52–61.PubMed

60.

Herrmann K, Gross WL, Moosig F. Extended follow-up after stopping mepolizumab in relapsing/refractory Churg-Strauss syndrome. Clin Exp Rheumatol. 2012;30(1 Suppl 70):S62–5.PubMed

61.

Cartin-Ceba R, Keogh KA, Specks U, Sethi S, Fervenza FC. Rituximab for the treatment of Churg–Strauss syndrome with renal involvement. Nephrol Dial Transplant. 2011;26:2865–71.CrossRefPubMedPubMedCentral

62.

Jones RB, FerraroAJ CAN, Brogan P, Salama AD, Smith KG, et al. A multicenter survey of rituximab therapy for refractory antineutrophil cytoplasmic antibody associated vasculitis. Arthritis Rheum. 2009;60:2156–68.CrossRefPubMed

63.•

Thiel J, Hassler F, Salzer U, Voll RE, Venhoff N. Rituximab in the treatment of refractory or relapsing (Churg-Strauss syndrome). Arthritis Res Ther. 2013;15:R133. Demonstration that B cell-depleting therapy with rituximab is effective in ANCA-positive eosinophilic granulomatosis with polyangiitis.

64.

Pepper RJ, Fabre MA, Pavesio C, Gaskin G, Jones RB, Jayne D, et al. Rituximab is effective in the treatment of refractory Churg-Strauss syndrome and is associated with diminished T-cell interleukin-5 production. Rheumatology (Oxford). 2008;47:1104–5.CrossRef

65.•

Mohammad AJ, Hot A, Arndt F, Moosig F, Guerry MJ, Amudala N, et al. Rituximab for the treatment of eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Ann Rheum Dis. 2014. doi:10.1136/annrheumdis-2014-206095. Rituximab induced a high rate of clinical improvement and reduced requirement of prednisolone.

66.

Danieli MG, Cappelli M, Malcangi G, Logullo F, Salvi A, Danieli G. Long term effectiveness of intravenous immunoglobulin in Churg–Strauss syndrome. Ann Rheum Dis. 2004;63:1649–54.CrossRefPubMedPubMedCentral

67.

Tatsis E, Schnabel A, Gross WL. Interferon-alpha treatment of four patients with the Churg–Strauss syndrome. Ann Intern Med. 1998;129:370–4.CrossRefPubMed

68.

Pavord ID, Korn S, Howarth P, Bleecker ER, Buhl R, Keene ON, et al. Mepolizumab for severe eosinophilic asthma (DREAM): a multicentre, double-blind, placebo-controlled trial. Lancet. 2012;380(9842):651–9.CrossRefPubMed

69.

Kim S, Marigowda G, Oren E, Israel E, Wechsler ME. Mepolizumab as a steroid-sparing treatment option in patients with Churg-Strauss syndrome. J Allergy Clin Immunol. 2010;125:1336–43.CrossRefPubMed

70.

Moosig F, Gross WL, Herrmann K, Bremer JP, Hellmich B. Targeting interleukin-5 in refractory and relapsing Churg–Strauss syndrome. Ann Intern Med. 2011;155:341–3.CrossRefPubMed

71.

Giavina-Bianchi P, Giavina-Bianchi M, Agondi R, Kalil J. Administration of anti-IgE to a Churg-Strauss syndrome patient. Int Arch Allergy Immunol. 2007;144:155–8.CrossRefPubMed

72.

Gardner-Medwin JMM, Dolezalova P, Cummins C, Southwood TR. Incidence of Henoch-Schönlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet. 2002;360:1197–202.CrossRefPubMed

73.

Haroon M. Should children with Henoch-Schönlein purpura and abdominal pain be treated with steroids? Arch Dis Child. 2005;90:1196–8.CrossRefPubMedPubMedCentral

74.

Ronkainen J, Koskimies O, Ala-Houhala M, Antikainen M, Merenmies J, Rajantie J, et al. Early prednisone therapy in Henoch-Schönlein purpura: a randomized, double-blind, placebo-controlled trial. J Pediatr. 2006;149:241–7.CrossRefPubMed

75.

Dudley J, Smith G, Llewellyn-Edwards A, Tizard E. Randomised placebo controlled trial to assess the role of early prednisolone on the development and progression of Henoch-Schönlein purpura nephritis. Pediatr Nephrol. 2007;22:1457.

76.

KDIGO. Clinical Practice Guideline for Glomerulonephritis. Chapter 10: immunoglobulin a nephropathy. Kidney Int Suppl. 2012;2:209–17.CrossRef

77.

Park JM, Won SC, Shin JI, Yim H, Pai KS. Cyclosporin A therapy for Henoch-Schönlein nephritis with nephrotic-range proteinuria. Pediatr Nephrol. 2011;26:411–7.CrossRefPubMed

78.

Du Y, Hou L, Zhao C, Han M, Wu Y. Treatment of children with Henoch-Schönlein purpura nephritis with mycophenolate mofetil. Pediatr Nephrol. 2012;27:765–71.CrossRefPubMed

79.

Fuentes Y, Valverde S, Valesquez-Jones L. Comparison of azathioprine vs mofetil mycophenolate for Henoch-Schönlein nephritis treatment. Pediatr Nephrol. 2010;25:1802.

80.

Kawasaki Y, Suzuki J, Suzuki H. Efficacy of methylprednisolone and urokinase pulse therapy combined with or without cyclophosphamide in severe Henoch-Schönlein nephritis: a clinical and histopathological study. Nephrol Dial Transplant. 2004;19:858–64.CrossRefPubMed

81.

Tarshish P, Bernstein J, Edelmann Jr CM. Henoch-Schönlein purpura nephritis: course of disease and efficacy of cyclophosphamide. Pediatr Nephrol. 2004;19:51–6.CrossRefPubMed

82.

Donnithorne KJ, Atkinson TP, Hinze CH, Nogueira JB, Saeed SA, Askenazi DJ, et al. Rituximab therapy for severe refractory chronic Henoch-Schönlein purpura. J Pediatr. 2009;155:136–9.CrossRefPubMed

83.

Pillebout E, Rocha F, Fardet L, Rybojad M, Verine J, Glotz D. Successful outcome using rituximab as the only immunomodulation in Henoch-Schonlein purpura: case report. Nephrol Dial Transplant. 2011;26:2044–6.CrossRefPubMed

84.•

El-Husseini A, Ahmed A, Sabucedo A, Fabulo E. Refractory Henoch-Schönlein purpura: atypical aetiology and management. J Ren Care. 2013;39:77–81. Evidence of the promising use of rituximab in patients with HSP who do not respond to conventional therapies. CrossRefPubMed

85.

Pindi Sala T, Michot JM, Snanoudj R, Dollat M, Estève E, Marie B, et al. Successful outcome of a cortico-dependent Henoch-Schönlein purpura adult with rituximab. Case Rep Med. 2014;2014:619218.PubMedPubMedCentral

86.

De Maddi F, Dinardo R, Buonocore MC, Dinardo M, Bartolomei B, Rigante D. Intravenous immunoglobulin in Henoch-Schönlein purpura complicated by cerebral hemorrhage. Rheumatol Int. 2013;33:2451–3.CrossRefPubMed

87.••

Stagnaro C, Cioffi E, Talarico R, Della Rossa A. Systemic vasculitides: a critical digest of the most recent literature. Clin Exp Rheumatol. 2015;33(2 Suppl 89):S-145-54. Overview of the most relevant studies regarding the epidemiology, pathogenesis and management of systemic vasculitis.

88.

Terrier B, Krastinova E, Marie I, Launay D, Lacraz A, Belenotti P, et al. Management of noninfectious mixed cryoglobulinemia vasculitis: data from 242 cases included in the CryoVas survey. Blood. 2012;119:5996–6004.CrossRefPubMed

89.

Terrier B, Launay D, Kaplanski G, Hot A, Larroche C, Cathébras P, et al. Safety and efficacy of rituximab in non-viral cryoglobulinemia vasculitis: data from the French autoimmunity and rituximab registry. Arthritis Care Res (Hoboken). 2010;62:1787–95.CrossRef

90.

Lahmer T, Heemann U. Anti-glomerular basement membrane antibody disease: a rare autoimmune disorder affecting the kidney and the lung. Autoimmun Rev. 2012;12:169–73.CrossRefPubMed

91.

Levy JB, Turner AN, Rees AJ, Pusey CD. Long-term outcome of anti-glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression. Ann Intern Med. 2001;134:1033–42.CrossRefPubMed

92.

Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Pagliuca G, et al. Goodpasture's syndrome: a clinical update. Autoimmun Rev. 2015;14:246–53.CrossRefPubMed

93.••

Touzot M, Poisson J, Faguer S, Ribes D, Cohen P, Geffray L, et al. Rituximab in anti-GBM disease: a retrospective study of 8 patients. J Autoimmun. 2015;60:74–9. Demostration of rituximab efficacy as additional and/or alternative therapy in the induction treatment of anti-glomerular basement membrane disease. CrossRefPubMed

94.

Jara LJ, Navarro C, Medina G, Vera-Lastra O, Saavedra MA. Hypocomplementemic urticarial vasculitis syndrome. Curr Rheumatol Rep. 2009;11:410–5.CrossRefPubMed

95.•

Jachiet M, Flageul B, Deroux A, Le Quellec A, Maurier F, Cordoliani F, et al. The clinical spectrum and therapeutic management of hypocomplementemic urticarial vasculitis: data from a French nationwide study of fifty-seven patients. Arthritis Rheumatol. 2015;67:527–34. The present study includes the largest series of patients with hypocomplementemic urticarial vasculitis reported so far in the literature. CrossRefPubMed

96.

Rigante D, Valentini P, Rizzo D, Leo A, De Rosa G, Onesimo R, et al. Responsiveness to intravenous immunoglobulins and occurrence of coronary artery abnormalities in a single-center cohort of Italian patients with Kawasaki syndrome. Rheumatol Int. 2010;30:841–6.CrossRefPubMed

97.

De Rosa G, Pardeo M, Rigante D. Current recommendations for the pharmacologic therapy in Kawasaki syndrome and management of its cardiovascular complications. Eur Rev Med Pharmacol Sci. 2007;11:301–8.PubMed

Medicine Matters Rheumatology

Abstract