| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Rheumatology 2001; 40: 763-771
© 2001 British Society for Rheumatology
Original Papers |
Churg–Strauss syndrome: outcome and long-term follow-up of 32 patients
Department of Internal Medicine,
1 Department of Pathology,
2 Department of Pneumology and
3 Department of Preventive Medicine, Vall d'Hebrón University General Hospital, 08035 Barcelona, Spain
Objectives. To study the clinical spectrum and evolution of Churg–Strauss syndrome in order to assess the clinicopathological features of the disease, the response to treatment and the long-term outcome.
Methods. Thirty-two patients with proven allergic and granulomatous angiitis (Churg–Strauss syndrome) and followed up at a single institution were evaluated. They were recruited between 1977 and 1999 from internal medicine departments. Data were obtained retrospectively from medical files in 15 cases and prospectively, using a standardized form, for the remaining patients.
Results. All patients had asthma and hypereosinophilia. The lungs, skin and peripheral nervous system were the organs most frequently involved. Antineutrophil cytoplasmic antibodies with antimyeloperoxidase specificity (MPO-ANCA) were detected in 77.8% of tested patients but they were not useful for monitoring disease activity. Extravascular granulomas were rarely seen in tissue biopsies. Forty per cent of the patients were treated with steroids alone. Immunosuppressive agents were added to the treatment when severe neurological, cardiac or gastrointestinal involvement was present. The outcome and long-term survival were good. Clinical relapse was rare after the first year of therapy. Dysaesthesiae of the distal limbs, neurophatic pain and cardiac failure were the most frequent sequelae.
Conclusions. Churg–Strauss syndrome is a rare disorder characterized by hypereosinophilia and systemic vasculitis occurring in patients with asthma and allergic rhinitis. Vasculitis commonly affects the lungs, skin and peripheral nervous system. Outcome and long-term survival is usually good with steroids alone or in combination with immunosuppressive agents. The syndrome has a low mortality rate compared with other systemic vasculitides.
KEY WORDS: Churg–Strauss syndrome, Clinicopathological findings, Survival, Long-term follow-up.
Correspondence to: R. Solans Laqué, Medicina Interna, 3a pares, Hospital General Universitario Vall d'Hebrón, Vall d'Hebrón 119-129, 08035 Barcelona, Spain
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  L. Mathew, K. Talbot, S. Love, S. Puvanarajah, and M. Donaghy Treatment of vasculitic peripheral neuropathy: a retrospective analysis of outcome QJM, January 1, 2007; 100(1): 41 - 51. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R. Sable-Fourtassou, P. Cohen, A. Mahr, C. Pagnoux, L. Mouthon, D. Jayne, D. Blockmans, J.-F. Cordier, P. Delaval, X. Puechal, et al. Antineutrophil Cytoplasmic Antibodies and the Churg-Strauss Syndrome Ann Intern Med, November 1, 2005; 143(9): 632 - 638. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 T. Kawakami, Y. Soma, K. Kawasaki, A. Kawase, and M. Mizoguchi Initial Cutaneous Manifestations Consistent With Mononeuropathy Multiplex in Churg-Strauss Syndrome Arch Dermatol, July 1, 2005; 141(7): 873 - 878. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S K Frankel, G P Cosgrove, and K K Brown Small vessel vasculitis of the lung Chronic Respiratory Disease, April 1, 2005; 2(2): 75 - 84. [Abstract] [PDF]
|
|
|
|
 |
|
 K. L. Loo, R. Ramachandran, S. K. Chow, E. M. L. Goh, and S. S. Yeap Mononeuritis in Churg-Strauss syndrome in Asians responding to intravenous cyclophosphamide Rheumatology, February 1, 2004; 43(2): 251 - 252. [Full Text] [PDF]
|
|
|
|
 |
|
 E.W Lau, D.M Carruthers, and N Prasad Churg-Strauss syndrome as an unusual cause of spontaneous atraumatic intra-pericardial thrombosis Eur J Echocardiogr, January 1, 2004; 5(1): 65 - 67. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Y. Hoi and E. F. Morand Churg-Strauss syndrome: the use of cyclophosphamide in mononeuritis Rheumatology, February 1, 2003; 42(2): 390 - 391. [Full Text] [PDF]
|
|
|
|
|
|
A. Della Rossa, C. Baldini, A. Tavoni, A. Tognetti, D. Neglia, G. Sambuceti, R. Puccini, C. Colangelo, and S. Bombardieri Churg-Strauss syndrome: clinical and serological features of 19 patients from a single Italian centre Rheumatology, November 1, 2002; 41(11): 1286 - 1294. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. J. Rios Blanco, J. Gomez Cerezo, I. Suarez, M. Gutierrez, J. J. Vazquez, F. J. Barbado, R. Solans, J. A. Bosch, C. Perez-Bocanegra, A. Selva, et al. Churg-Strauss syndrome: outcome and long-term follow-up of 32 patients. Comment on the article by Solans et al. Rheumatology, August 1, 2002; 41(8): 946 - 947. [Full Text] [PDF]
|
|
|
|
 |
|
 R Solans, J A Bosch, A Selva, R Orriols, and M Vilardell Montelukast and Churg-Strauss syndrome Thorax, February 1, 2002; 57(2): 183 - 185. [Abstract] [Full Text] [PDF]
|
|