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Semin Respir Crit Care Med 2011; 32(3): 298-309
DOI: 10.1055/s-0031-1279826
© Thieme Medical Publishers

Churg-Strauss Syndrome: Clinical Symptoms, Complementary Investigations, Prognosis and Outcome, and Treatment

Bertrand Dunogué1 , 2 , Christian Pagnoux1 , 2 , Loïc Guillevin1 , 2
  • 1Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases (including Vasculitis, Scleroderma), Hôpital Cochin, Assistance Publique–Hôpitaux de Paris, Paris, France
  • 2Université Paris Descartes, Paris, France
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Publication History

Publication Date:
14 June 2011 (online)

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ABSTRACT

Churg-Strauss syndrome (CSS), first described in 1951, is a rare vasculitis of small- and medium-sized vessels. It is characterized by a constant association with asthma and eosinophilia, and by the presence of anti-myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibodies (ANCA) in ~40% of the patients. Vasculitis typically develops in a previously asthmatic and eosinophilic middle-aged patient and most frequently involves the peripheral nerves and skin. Other organs, however, may be affected and must be screened for vasculitis, especially those associated with a poorer prognosis, such as the heart, kidney, and gastrointestinal tract, as assessed by the recently revised Five-Factor Score (FFS). Overall survival of CSS patients is excellent, but relapses are not uncommon and require maintenance or steroid-sparing therapies, depending on the original FFS-based prognosis at diagnosis. All patients require corticosteroids, often for prolonged periods, combined with immunosuppressants [e.g., induction (cyclophosphamide) and maintenance therapy (azathioprine)], for those with poorer prognoses. Recent insights, especially concerning clinical differences associated with ANCA status, showed that CSS patients might constitute a heterogeneous group, both clinically and pathogenically. Future therapies might reflect these differences more strongly.

KEYWORDS

Churg-Strauss syndrome - vasculitis - ANCA - steroids - immunosuppressants

REFERENCES

  • 1 Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa.  Am J Pathol. 1951;  27 (2) 277-301
    PubMedGoogle Scholar
  • 2 Masi A T, Hunder G G, Lie J T et al.. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis).  Arthritis Rheum. 1990;  33 (8) 1094-1100
    CrossrefPubMedGoogle Scholar
  • 3 Jennette J C, Falk R J, Andrassy K et al.. Nomenclature of systemic vasculitides: proposal of an international consensus conference.  Arthritis Rheum. 1994;  37 (2) 187-192
    CrossrefPubMedGoogle Scholar
  • 4 Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P. Churg-Strauss syndrome: clinical study and long-term follow-up of 96 patients.  Medicine (Baltimore). 1999;  78 (1) 26-37
    CrossrefPubMedGoogle Scholar
  • 5 Sablé-Fourtassou R, Cohen P, Mahr A French Vasculitis Study Group et al. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome.  Ann Intern Med. 2005;  143 (9) 632-638
    CrossrefPubMedGoogle Scholar
  • 6 Sinico R A, Di Toma L, Maggiore U et al.. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome.  Arthritis Rheum. 2005;  52 (9) 2926-2935
    CrossrefPubMedGoogle Scholar
  • 7 Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P. French Vasculitis Study Group (FVSG) . The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort.  Medicine (Baltimore). 2011;  90 (1) 19-27
    CrossrefPubMedGoogle Scholar
  • 8 Lanham J G, Elkon K B, Pusey C D, Hughes G R. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome.  Medicine (Baltimore). 1984;  63 (2) 65-81
    CrossrefPubMedGoogle Scholar
  • 9 Guillevin L, Amouroux J, Arbeille B, Boura R. Churg-Strauss angiitis: arguments favoring the responsibility of inhaled antigens.  Chest. 1991;  100 (5) 1472-1473
    CrossrefPubMedGoogle Scholar
  • 10 Manger B J, Krapf F E, Gramatzki M et al.. IgE-containing circulating immune complexes in Churg-Strauss vasculitis.  Scand J Immunol. 1985;  21 (4) 369-373
    CrossrefPubMedGoogle Scholar
  • 11 Bottero P, Bonini M, Vecchio F et al.. The common allergens in the Churg-Strauss syndrome.  Allergy. 2007;  62 (11) 1288-1294
    CrossrefPubMedGoogle Scholar
  • 12 Falk R J, Terrell R S, Charles L A, Jennette J C. Anti-neutrophil cytoplasmic autoantibodies induce neutrophils to degranulate and produce oxygen radicals in vitro.  Proc Natl Acad Sci U S A. 1990;  87 (11) 4115-4119
    CrossrefPubMedGoogle Scholar
  • 13 Xiao H, Heeringa P, Hu P et al.. Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice.  J Clin Invest. 2002;  110 (7) 955-963
    CrossrefPubMedGoogle Scholar
  • 14 Heeringa P, Brouwer E, Tervaert J W, Weening J J, Kallenberg C G. Animal models of anti-neutrophil cytoplasmic antibody associated vasculitis.  Kidney Int. 1998;  53 (2) 253-263
    CrossrefPubMedGoogle Scholar
  • 15 Tsukadaira A, Okubo Y, Kitano K et al.. Eosinophil active cytokines and surface analysis of eosinophils in Churg-Strauss syndrome.  Allergy Asthma Proc. 1999;  20 (1) 39-44
    CrossrefPubMedGoogle Scholar
  • 16 Tai P C, Holt M E, Denny P, Gibbs A R, Williams B D, Spry C J. Deposition of eosinophil cationic protein in granulomas in allergic granulomatosis and vasculitis: the Churg-Strauss syndrome.  Br Med J (Clin Res Ed). 1984;  289 (6442) 400-402
    CrossrefPubMedGoogle Scholar
  • 17 Schmitt W H, Csernok E, Kobayashi S, Klinkenborg A, Reinhold-Keller E, Gross W L. Churg-Strauss syndrome: serum markers of lymphocyte activation and endothelial damage.  Arthritis Rheum. 1998;  41 (3) 445-452
    CrossrefPubMedGoogle Scholar
  • 18 Peen E, Hahn P, Lauwers G, Williams Jr R C, Gleich G, Kephart G M. Churg-Strauss syndrome: localization of eosinophil major basic protein in damaged tissues.  Arthritis Rheum. 2000;  43 (8) 1897-1900
    CrossrefPubMedGoogle Scholar
  • 19 Cottin V, Tardy F, Gindre D, Vernet G, Deviller P, Cordier J F. Urinary eosinophil-derived neurotoxin in Churg-Strauss syndrome.  J Allergy Clin Immunol. 1995;  96 (2) 261-264
    CrossrefPubMedGoogle Scholar
  • 20 Higashi N, Mita H, Taniguchi M et al.. Urinary eicosanoid and tyrosine derivative concentrations in patients with vasculitides.  J Allergy Clin Immunol. 2004;  114 (6) 1353-1358
    CrossrefPubMedGoogle Scholar
  • 21 Kiene M, Csernok E, Müller A, Metzler C, Trabandt A, Gross W L. Elevated interleukin-4 and interleukin-13 production by T cell lines from patients with Churg-Strauss syndrome.  Arthritis Rheum. 2001;  44 (2) 469-473
    CrossrefPubMedGoogle Scholar
  • 22 Taki H, Shinoda K, Hounoki H et al.. Serum interleukin-5 levels correlate with disease activity of Churg-Strauss syndrome in a patient treated with a leucotriene receptor antagonist, pranlukast, and inhaled corticosteroid.  Scand J Rheumatol. 2010;  39 (4) 341-343
    CrossrefPubMedGoogle Scholar
  • 23 Termeer C, Simon J C, Schöpf E. Churg-Strauss syndrome associated with fluticasone therapy.  Arch Dermatol. 2001;  137 (11) 1527-1528
    PubMedGoogle Scholar
  • 24 Terrier B, Bièche I, Maisonobe T et al.. Interleukin-25: a cytokine linking eosinophils and adaptive immunity in Churg-Strauss syndrome.  Blood. 2010;  116 (22) 4523-4531
    CrossrefPubMedGoogle Scholar
  • 25 Müschen M, Warskulat U, Perniok A et al.. Involvement of soluble CD95 in Churg-Strauss syndrome.  Am J Pathol. 1999;  155 (3) 915-925
    CrossrefPubMedGoogle Scholar
  • 26 Polzer K, Karonitsch T, Neumann T et al.. Eotaxin-3 is involved in Churg-Strauss syndrome—a serum marker closely correlating with disease activity.  Rheumatology (Oxford). 2008;  47 (6) 804-808
    CrossrefPubMedGoogle Scholar
  • 27 Hellmich B, Csernok E, Gross W L. Proinflammatory cytokines and autoimmunity in Churg-Strauss syndrome.  Ann N Y Acad Sci. 2005;  1051 121-131
    CrossrefPubMedGoogle Scholar
  • 28 Pagnoux C. Churg-Strauss syndrome: evolving concepts.  Discov Med. 2010;  9 (46) 243-252
    PubMedGoogle Scholar
  • 29 Saito H, Tsurikisawa N, Tsuburai T, Akiyama K. Involvement of regulatory T cells in the pathogenesis of Churg-Strauss syndrome.  Int Arch Allergy Immunol. 2008;  146 (suppl 1) 73-76
    CrossrefPubMedGoogle Scholar
  • 30 Saito H, Tsurikisawa N, Tsuburai T, Oshikata C, Akiyama K. Cytokine production profile of CD4 + T cells from patients with active Churg-Strauss syndrome tends toward Th17.  Int Arch Allergy Immunol. 2009;  149 (suppl 1) 61-65
    CrossrefPubMedGoogle Scholar
  • 31 Wieczorek S, Holle J U, Bremer J P et al.. Contrasting association of a non-synonymous leptin receptor gene polymorphism with Wegener's granulomatosis and Churg-Strauss syndrome.  Rheumatology (Oxford). 2010;  49 (5) 907-914
    CrossrefPubMedGoogle Scholar
  • 32 Wieczorek S, Holle J U, Epplen J T. Recent progress in the genetics of Wegener's granulomatosis and Churg-Strauss syndrome.  Curr Opin Rheumatol. 2010;  22 (1) 8-14
    CrossrefPubMedGoogle Scholar
  • 33 Mahr A, Guillevin L, Poissonnet M, Aymé S. Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener's granulomatosis, and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate.  Arthritis Rheum. 2004;  51 (1) 92-99
    CrossrefPubMedGoogle Scholar
  • 34 Haugeberg G, Bie R, Bendvold A, Larsen A S, Johnsen V. Primary vasculitis in a Norwegian community hospital: a retrospective study.  Clin Rheumatol. 1998;  17 (5) 364-368
    CrossrefPubMedGoogle Scholar
  • 35 Martin R M, Wilton L V, Mann R D. Prevalence of Churg-Strauss syndrome, vasculitis, eosinophilia and associated conditions: retrospective analysis of 58 prescription-event monitoring cohort studies.  Pharmacoepidemiol Drug Saf. 1999;  8 (3) 179-189
    CrossrefPubMedGoogle Scholar
  • 36 Watts R A, Lane S E, Bentham G, Scott D G. Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom.  Arthritis Rheum. 2000;  43 (2) 414-419
    CrossrefPubMedGoogle Scholar
  • 37 Watts R A, Lane S, Scott D G. What is known about the epidemiology of the vasculitides?.  Best Pract Res Clin Rheumatol. 2005;  19 (2) 191-207
    CrossrefPubMedGoogle Scholar
  • 38 Harrold L R, Andrade S E, Go A S et al.. Incidence of Churg-Strauss syndrome in asthma drug users: a population-based perspective.  J Rheumatol. 2005;  32 (6) 1076-1080
    PubMedGoogle Scholar
  • 39 Dietz A, Hübner C, Andrassy K. Macrolide antibiotic-induced vasculitis (Churg-Strauss syndrome) [in German].  Laryngorhinootologie. 1998;  77 (2) 111-114
    Article in Thieme ConnectPubMedGoogle Scholar
  • 40 Imai H, Nakamoto Y, Hirokawa M, Akihama T, Miura A B. Carbamazepine-induced granulomatous necrotizing angiitis with acute renal failure.  Nephron. 1989;  51 (3) 405-408
    CrossrefPubMedGoogle Scholar
  • 41 Mathur S, Dooley J, Scheuer P J. Quinine induced granulomatous hepatitis and vasculitis.  BMJ. 1990;  300 (6724) 613
    PubMedGoogle Scholar
  • 42 Guillevin L, Guittard T, Blétry O, Godeau P, Rosenthal P. Systemic necrotizing angiitis with asthma: causes and precipitating factors in 43 cases.  Lung. 1987;  165 (3) 165-172
    CrossrefPubMedGoogle Scholar
  • 43 Green R L, Vayonis A G. Churg-Strauss syndrome after zafirlukast in two patients not receiving systemic steroid treatment.  Lancet. 1999;  353 (9154) 725-726
    CrossrefPubMedGoogle Scholar
  • 44 Franco J, Artés M J. Pulmonary eosinophilia associated with montelukast.  Thorax. 1999;  54 (6) 558-560
    CrossrefPubMedGoogle Scholar
  • 45 Wechsler M E, Drazen J M. Zafirlukast and Churg-Strauss syndrome.  Chest. 1999;  116 (1) 266-267
    CrossrefPubMedGoogle Scholar
  • 46 Wechsler M E, Finn D, Gunawardena D et al.. Churg-Strauss syndrome in patients receiving montelukast as treatment for asthma.  Chest. 2000;  117 (3) 708-713
    CrossrefPubMedGoogle Scholar
  • 47 Wechsler M E, Wong D A, Miller M K, Lawrence-Miyasaki L. Churg-Strauss syndrome in patients treated with omalizumab.  Chest. 2009;  136 (2) 507-518
    CrossrefPubMedGoogle Scholar
  • 48 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 (2) 155-158
    CrossrefPubMedGoogle Scholar
  • 49 Puéchal X, Rivereau P, Vinchon F. Churg-Strauss syndrome associated with omalizumab.  Eur J Intern Med. 2008;  19 (5) 364-366
    CrossrefPubMedGoogle Scholar
  • 50 Hauser T, Mahr A, Metzler C et al.. The leucotriene receptor antagonist montelukast and the risk of Churg-Strauss syndrome: a case-crossover study.  Thorax. 2008;  63 (8) 677-682
    CrossrefPubMedGoogle Scholar
  • 51 Wechsler M E, Pauwels R, Drazen J M. Leukotriene modifiers and Churg-Strauss syndrome: adverse effect or response to corticosteroid withdrawal?.  Drug Saf. 1999;  21 (4) 241-251
    CrossrefPubMedGoogle Scholar
  • 52 Chumbley L C, Harrison Jr E G, DeRemee R A. Allergic granulomatosis and angiitis (Churg-Strauss syndrome): report and analysis of 30 cases.  Mayo Clin Proc. 1977;  52 (8) 477-484
    PubMedGoogle Scholar
  • 53 Gaskin G, Clutterbuck E J, Pusey C D. Renal disease in the Churg-Strauss syndrome: diagnosis, management and outcome.  Contrib Nephrol. 1991;  94 58-65
    PubMedGoogle Scholar
  • 54 Haas C, Geneau C, Odinot J M et al.. Angéïte allergique et granulomateuse: le syndrome de Churg–Strauss: Étude rétrospective de 16 cas.  Ann Med Interne (Paris). 1991;  142 (5) 335-342
    PubMedGoogle Scholar
  • 55 Abu-Shakra M, Smythe H, Lewtas J, Badley E, Weber D, Keystone E. Outcome of polyarteritis nodosa and Churg-Strauss syndrome: an analysis of twenty-five patients.  Arthritis Rheum. 1994;  37 (12) 1798-1803
    CrossrefPubMedGoogle Scholar
  • 56 Riva N, Cerri F, Butera C et al.. Churg Strauss syndrome presenting as acute neuropathy resembling Guillain Barré syndrome: case report.  J Neurol. 2008;  255 (11) 1843-1844
    CrossrefPubMedGoogle Scholar
  • 57 Guillevin L, Lhote F, Gayraud M et al.. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome: a prospective study in 342 patients.  Medicine (Baltimore). 1996;  75 (1) 17-28
    CrossrefPubMedGoogle Scholar
  • 58 Pagnoux C, Mahr A, Cohen P, Guillevin L. 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
    CrossrefPubMedGoogle Scholar
  • 59 Cottin V, Cordier J F. Eosinophilic pneumonias.  Allergy. 2005;  60 (7) 841-857
    CrossrefPubMedGoogle Scholar
  • 60 Bhagirath K M, Paulson K, Ahmadie R, Bhalla R S, Robinson D, Jassal D S. Clinical utility of cardiac magnetic resonance imaging in Churg-Strauss syndrome: case report and review of the literature.  Rheumatol Int. 2009;  29 (4) 445-449
    CrossrefPubMedGoogle Scholar
  • 61 Marmursztejn J, Cohen P, Duboc D et al.. Cardiac magnetic resonance imaging in Churg-Strauss-syndrome: impact of immunosuppressants on outcome assessed in a prospective study on 8 patients.  Clin Exp Rheumatol. 2010;  28 (1, suppl 57) 8-13
    PubMedGoogle Scholar
  • 62 Marmursztejn J, Vignaux O, Cohen P et al.. Impact of cardiac magnetic resonance imaging for assessment of Churg-Strauss syndrome: a cross-sectional study in 20 patients.  Clin Exp Rheumatol. 2009;  27 (1, suppl 52) S70-S76
    PubMedGoogle Scholar
  • 63 Androudi S, Iaccheri B, Brazitikos P, Papadaki T, Foster C S. Bilateral chronic anterior uveitis & neuro-ophthalmologic manifestations in a patient with Churg-Strauss syndrome: an unusual ocular presentation.  Ocul Immunol Inflamm. 2004;  12 (1) 59-63
    CrossrefPubMedGoogle Scholar
  • 64 Fernandez A J. Ureteral granuloma in Churg-Strauss syndrome.  Mayo Clin Proc. 1978;  53 (9) 618
    PubMedGoogle Scholar
  • 65 Modigliani R, Muschart J M, Galian A, Clauvel J P, Piel-Desruisseaux J L. Allergic granulomatous vasculitis (Churg-Strauss Syndrome): report of a case with widespread digestive involvement.  Dig Dis Sci. 1981;  26 (3) 264-270
    CrossrefPubMedGoogle Scholar
  • 66 Généreau T, Lortholary O, Pottier M A French Vasculitis Study Group et al. Temporal artery biopsy: a diagnostic tool for systemic necrotizing vasculitis.  Arthritis Rheum. 1999;  42 (12) 2674-2681
    CrossrefPubMedGoogle Scholar
  • 67 Lie J T, Bayardo R J. Isolated eosinophilic coronary arteritis and eosinophilic myocarditis: a limited form of Churg-Strauss syndrome.  Arch Pathol Lab Med. 1989;  113 (2) 199-201
    PubMedGoogle Scholar
  • 68 Lie J T. Limited forms of Churg-Strauss syndrome.  Pathol Annu. 1993;  28 (Pt 2) 199-220
    PubMedGoogle Scholar
  • 69 Steinfeld S, Golstein M, De Vuyst P. Chronic eosinophilic pneumonia (CEP) as a presenting feature of Churg-Strauss syndrome (CSS).  Eur Respir J. 1994;  7 (11) 2098
    PubMedGoogle Scholar
  • 70 Terrier B, Saadoun D, Sene D et al.. Anti-myeloperoxidase antibodies are a useful marker of disease activity in ANCA-associated vasculitides.  Ann Rheum Dis. 2008;  68 1564-1578
    PubMedGoogle Scholar
  • 71 Wallaert B, Gosset P, Prin L, Bart F, Marquette C H, Tonnel A B. Bronchoalveolar lavage in allergic granulomatosis and angiitis.  Eur Respir J. 1993;  6 (3) 413-417
    PubMedGoogle Scholar
  • 72 Finan M C, Winkelmann R K. The cutaneous extravascular necrotizing granuloma (Churg-Strauss granuloma) and systemic disease: a review of 27 cases.  Medicine (Baltimore). 1983;  62 (3) 142-158
    PubMedGoogle Scholar
  • 73 Reid A J, Harrison B D, Watts R A, Watkin S W, McCann B G, Scott D G. Churg-Strauss syndrome in a district hospital.  QJM. 1998;  91 (3) 219-229
    CrossrefPubMedGoogle Scholar
  • 74 Roufosse F, Cogan E, Goldman M. The hypereosinophilic syndrome revisited.  Annu Rev Med. 2003;  54 169-184
    CrossrefPubMedGoogle Scholar
  • 75 Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa.  Am J Pathol. 1951;  27 (2) 277-301
    PubMedGoogle Scholar
  • 76 Hoffman G S, Kerr G S, Leavitt R Y et al.. Wegener granulomatosis: an analysis of 158 patients.  Ann Intern Med. 1992;  116 (6) 488-498
    CrossrefPubMedGoogle Scholar
  • 77 Cohen P, Pagnoux C, Mahr A French Vasculitis Study Group 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 (4) 686-693
    CrossrefPubMedGoogle Scholar
  • 78 Bourgarit A, Le Toumelin P, Pagnoux C French Vasculitis Study Group et al. Deaths occurring during the first year after treatment onset for polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: a retrospective analysis of causes and factors predictive of mortality based on 595 patients.  Medicine (Baltimore). 2005;  84 (5) 323-330
    CrossrefPubMedGoogle Scholar
  • 79 Ribi C, Cohen P, Pagnoux C French Vasculitis Study Group 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 (2) 586-594
    CrossrefPubMedGoogle Scholar
  • 80 Guillevin L. Clinical trials on systemic necrotizing vasculitides.  Presse Med. 2010;  39 (6) 653-659
    CrossrefPubMedGoogle Scholar
  • 81 Gayraud M, Guillevin L, Cohen P French Cooperative Study Group for Vasculitides 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;  36 (12) 1290-1297
    CrossrefPubMedGoogle Scholar
  • 82 Metzler C, Hellmich B, Gause A, Gross W L, 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-S61
    PubMedGoogle Scholar
  • 83 Pagnoux C, Guillevin L. Churg-Strauss syndrome: evidence for disease subtypes?.  Curr Opin Rheumatol. 2010;  22 (1) 21-28
    CrossrefPubMedGoogle Scholar
  • 84 Koukoulaki M, Smith K G, Jayne D R. Rituximab in Churg-Strauss syndrome.  Ann Rheum Dis. 2006;  65 (4) 557-559
    CrossrefPubMedGoogle Scholar
  • 85 Pepper R J, Fabre M A, Pavesio C 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 (7) 1104-1105
    CrossrefPubMedGoogle Scholar
  • 86 Bouldouyre M A, Cohen P, Guillevin L. Severe bronchospasm associated with rituximab for refractory Churg-Strauss syndrome.  Ann Rheum Dis. 2009;  68 (4) 606
    CrossrefPubMedGoogle Scholar
  • 87 Metzler C, Schnabel A, Gross W L, Hellmich B. A phase II study of interferon-alpha for the treatment of refractory Churg-Strauss syndrome.  Clin Exp Rheumatol. 2008;  26 (3, suppl 49) S35-S40
    PubMedGoogle Scholar
  • 88 Giavina-Bianchi P, Agondi R, Kalil J. One year administration of anti-IgE to a patient with Churg-Strauss syndrome.  Int Arch Allergy Immunol. 2008;  146 (2) 176
    CrossrefPubMedGoogle Scholar
  • 89 Hamilos D L, Christensen J. Treatment of Churg-Strauss syndrome with high-dose intravenous immunoglobulin.  J Allergy Clin Immunol. 1991;  88 (5) 823-824
    CrossrefPubMedGoogle Scholar
  • 90 Rutberg S A, Ward D E, Roth B J. Churg-Strauss syndrome and pregnancy: successful treatment with intravenous immunoglobulin.  J Clin Rheumatol. 2002;  8 (3) 151-156
    CrossrefPubMedGoogle Scholar
  • 91 Taniguchi M, Tsurikisawa N, Higashi N et al.. Treatment for Churg-Strauss syndrome: induction of remission and efficacy of intravenous immunoglobulin therapy.  Allergol Int. 2007;  56 (2) 97-103
    CrossrefPubMedGoogle Scholar
  • 92 Jayne D R, Gaskin G, Rasmussen N European Vasculitis Study Group et al. Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis.  J Am Soc Nephrol. 2007;  18 (7) 2180-2188
    CrossrefPubMedGoogle Scholar
  • 93 Jayne D R, 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. 2007;  18 (7) 2180-2188
    CrossrefPubMedGoogle Scholar

Loïc GuillevinM.D. 

Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases (including Vasculitis, Scleroderma), INSERM U1060, Hôpital Cochin, Assistance Publique–Hôpitaux de Paris, Université Paris Descartes

27, rue du faubourg Saint-Jacques, 75679 Paris Cedex 14, France

Email: loic.guillevin@cch.aphp.fr

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