Distinct effects of corticosteroid/immunossupressive therapy on the different manifestations of Churg-Strauss syndrome

Authors

  • Maria João Nunes da Silva Assistente Hosptalar Graduada de Medicina Interna, Serviço de Medicina 2, Hospital Universitário de Santa Maria, Lisboa
  • Nídia Garrido Assistente Hospitalar de Infecciologia, Serviço de Medicina 2, Hospital Universitário de Santa Maria, Lisboa
  • Patrícia Alves Assistente Hospitalar Graduada de Gastrenterologia, Serviço de Medicina 2, Hospital Universitário de Santa Maria, Lisboa
  • Rui M. M. Victorino Director do Serviço de Medicina Serviço de Medicina 2, Hospital Universitário de Santa Maria, Lisboa

Keywords:

Churg-Strauss, Immunosupresive therapy, Neurological involvement, resistance to therapy

Abstract

We report the case of a 64-year-old female with a history of
late presentation asthma of 10 years duration, who presented
with high fever and severe mononeuritis multiplex, accompanied
by hypereosinophilia, in whom the diagnosis of Churg-Strauss
Syndrome (CSS) was established. On immunosupressive therapy
(prednisone and cyclophosphamide), the fever, asthma and hypereosinophilia disappeared rapidly, but the neurological symptoms
persisted although with prolonged therapy the neuropathy fi nely
improved . This case illustrates the distinct effects of the therapy
on the different components of the Churg-Strauss Syndrome,
probably as a consequence of the different immunopathogenic
mechanisms involved in these two manifestations of the CSS..

Downloads

Download data is not yet available.

References

Noth I, StrecK M, Leff A. Churg-Strauss Syndrome. Lancet 2003;361:587-594.

LanhamJG , Elkon KB , Pusey CD, Hughes GR . Systemic Vasculitis With Asthma and Eosinophilia: A Clinical Aproach to the Churg-Strauss Syndrome. Medicine (Baltimore) 1984; 63 : 65-81.

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 1999; 78 : 26-37.

Masi AT, Hunter GG, Lie JT, Michel BA, Bloch DA, Areud WP et al. The American College of Rheumatology 1990 Criteria for the Classification of Churg-Strauss Syndrome (Allergic Granulomatosis and Angiitis). Arthritis Rheum, 1990; 33 :1094-1100.

Jennette JC et al. Nomenclature of Systemic Vasculitides: Proposal of an International Consensus Conference. Arthritis Rheum 1994;37:187-192.

Marazzi R , Pareyson D , Boiardi A , Corbo M , Scaioli V , Sghirlanzoni. Peripheral Nerve Involvement in Churg-Strauss Syndrome. J Neurol 1992 ; 239 : 317-321.

Gross WL , Csemok E , Helmchen U , Antineutrophil Cytoplasma Autoantibodies, Autoantigens and Systemic Vasculitis. APMIS 1995 ; 103 : 81-87.

Schultz DR, Torman EC. Antineutrophil Cytoplasmic Antibodies: Major Autoantigens, Pathophysiology and Disease Associations. Sem-Arthritis Rheum, 1995 ; 25 :143-159.

Sneller MC, Fauci AS. Pathogenesis of Vasculitis Syndromes. Med Clin North Am 1997 ; 1 : 221-242.

Carol L. Treatment of Polyarteritis Nodosa, Microscopic Polyangiitis, and Churg-Strauss Syndrome: Where do we stand ?. Arthritis Reum 2001 ;44 :508-512.

.Guillevin L, Lhote F, Jarrousse B, Faun O. Treatment of Polyarteritis Nodosa And Churg-Strauss Syndrome. A Meta Analysis of 3 prospective

controled trials including 182 Patients over 12 years. Ann-Med-Interne-Paris 1992 ;142 : 405-416.

Gayraud M , Guillevin L , Cohen P , Lhote F , Cacoub P, Deblois P et al. Treatment of Good-prognosis Polyarteritis Nodosa and Churg-Strauss Syndrome: Comparision of Steroids and oral or pulse Cyclophosphamide in 25 Patients. French Cooperative Study Group for Vasculitidis. Br J Rheumatol 1997 ; 36 :1290- 1297.

Guillevin L , Lhote F , Gayraud M , Cohen P , Jarrousse B , Lortholary O , Thibult N, Cassasus P. Prognostic Factors in Polyarteritis Nodosa and Churg-Strauss Syndrome: A Prospective Study in 342 Patients. Medicine (Baltimore) 1996 ; 75 :17-28

Guillevin L. Treatments for systemic vasculitidis. European Journal of Internal Medicine 2001 ; 12 :412-419.

Gayraud M , Guillevin L, Toumelin P , Cohen P , Lhote F , Cassasus P Jarrousse B and the French Vasculitis Study Group. Long-Term Followup of Polyarteritis Nodosa, Microscopic Poliangiitis, and Churg-Strauss Syndrome. Analysis of Four Prospective Trials Including 278 Patients. Arthritis Reum 2001 ; 44 : 666-675.

Allen NB , Bressler PB . Diagnosis and Treatment of the Systemic and Cutaneous Necrotizing Vasculitis Syndromes . Med Clin North Am 1997 ; 81 : 244-259.

Tsurikisawa N. et al Treatment of Churg-Strauss Syndrome with high dose intravenous immunoglobulin. Ann Allergy Asthma Immunol 2004 ;92 :80-87.

Tatsis E et al. Interferon � treatment of four patients with the Churg-Strauss Syndrome. Ann Intern Med 1998; 129:370-374.

Arbach O et al. Treatment of refractory Churg-Strauss-Syndrome(CSS) by TNFalpha blockade. Immunobiology 2002 ;206:496-501.

Shegal M, Swanson JW, Deremee RA, Colby TV . Neurological Manifestations of Churg-Strauss Syndrome. Mayo Clin Proc 1995 ; 70 : 337-341.

Schatz M. et al Eosinophils and Immunologic Lung Disease. Med Clin North Am 1981;65:1055-1071.

Monaco et al Polyneuropaty in hypereosinophilic syndrome. Neurology 1988; 38:494-496.

Hattori N et al. Clinicopathological features of Churg-Strauss Syndrome-associated neuropathy. Brain 1999;122:427-439

Additional Files

Published

2005-09-30

How to Cite

1.
Nunes da Silva MJ, Garrido N, Alves P, M. M. Victorino R. Distinct effects of corticosteroid/immunossupressive therapy on the different manifestations of Churg-Strauss syndrome. RPMI [Internet]. 2005 Sep. 30 [cited 2024 Dec. 18];12(3):154-8. Available from: https://revista.spmi.pt/index.php/rpmi/article/view/1694

Issue

Section

Case Reports

Most read articles by the same author(s)