Churg–Strauss syndrome: outcome and long-term follow-up of 32 patients. Comment on the article by Solans et al.

J. J. Ríos Blanco, J. Gómez Cerezo, I. Suárez, M. Gutiérrez1, J. J. Vázquez and F. J. Barbado

Servicios de Medicina Interna and 1Anatomía Patológica, Hospital Universitario La Paz, Madrid, Spain

SIR, We have read the interesting article by Solans et al. [1] reviewing the clinical characteristics and outcome of 32 patients with Churg–Strauss syndrome during 12 yr in a hospital in Cataluña, in the north-east of Spain. In 2000 we published our experience over two decades (1978–1998) [2] with 16 patients, which was the largest series published in Spain at that time. We wish to make several observations. (i) Epidemiologically, our hospital serves patients from the central part of Spain, but is also a reference centre for several other regions of Spain. (ii) Some of our findings are similar to the ones in the article by Solans et al.: most of the patients were female (81%), a proportion that differs from that in other previous studies [3, 4]. The lungs (100%), nervous system (63%) and heart (44%) were also the most frequently affected organs. (iii) However, some of our findings are different. Only 38% of our patients had cutaneous involvement, whereas Solans et al. found skin lesions in 81% of the patients [1]. The most frequent neurological complication in our patients was a symmetrical dystal polyneuropathy (80%), which was asymptomatic in many cases, and we did not find as many patients with mononeuritis multiplex. (iv) The diagnosis was histological in 88% of our patients. The most frequent findings were necrotizing vasculitis (11 cases) and infiltration of eosinophils (eight cases). The observation of granulomas is uncommon (six cases) and we also believe that the absence of granulomas should not rule out the diagnosis of Churg–Strauss syndrome. (v) The most striking difference between our series and Solan's is the frequency of relapses. Although Solans et al. found 28% of the patients had clinical relapse (14 episodes), we have not found a single case of vasculitic reactivation. It is also noteworthy that most of the relapses were preceded by precipitating factors such as cocaine consumption, influenza vaccination, administration of montelukast and pregnancy; some of them are possible aetiological factors of the disease. Therefore, we believe that these episodes should not be considered relapses.

Finally, we would like to point out that, in our experience, Churg–Strauss syndrome usually has only one episode (a ‘one-shot vasculitis’), because relapses are extremely uncommon. However, we definitely agree with Solans et al. in that persistent asthma after the vasculitic presentation is frequent (80% of our patients, many of whom are dependent on corticosteroids). The article by Solans et al. is a useful contribution to our knowledge of this disease, about which there are still many puzzling questions.

Notes

Correspondence to: J. J. Ríos Blanco, Servicio de Medicina Interna, Hospital Universitario La Paz, Paseo de la Castellana 261, Madrid 28046, Spain. Back

Accepted 20 February 2002

References

  1. Solans R, Bosch JA, Pérez-Bocanegra C et al. Churg–Strauss syndrome: outcome and long-term follow-up of 32 patients. Rheumatology2001;40:763–71.[Abstract/Free Full Text]
  2. Ríos JJ, Gómez Cerezo J, Súarez I, Gutiérrez M, Vázquez JJ, Barbado FJ. Churg–Strauss syndrome. Our two decade experience. Rev Clin Esp2000;200:597–601.[ISI][Medline]
  3. Chumbley LC, Harrison EG, De Remee RA. Allergic granulomatosis and angiitis (Churg–Strauss syndrome). Report and analysis of 30 cases. Mayo Clin Proc1977;52:477–84.[ISI][Medline]
  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;78:26–37.[ISI][Medline]

 

Reply

R. Solans, J. A. Bosch, C. Pérez-Bocanegra, A. Selva, P. Huguet, J. Alijotas, R. Orriols, L. Armadans and M. Vilardell

Servicio de Medicina Interna 3a planta pares, Hospital Vall d'Hebrón 119–129, Barcelona 08035, Spain

We have read with great attention the letter from Ríos-Blanco et al. [1] relating to our article [2], and we wish to make several observations. First, in the majority of large series of patients with Churg–Strauss syndrome (CSS), mononeuritis multiplex is more frequently reported than polyneuropathy, and is one of the most handicapping clinical features [3]. Secondly, clinical relapses are relatively frequent in CSS during the first months of the illness, although this type of vasculitis tends to respond better to the treatment than other necrotizing vasculitides of the polyarteritis nodosa group. In our series, 6/32 patients (18.7%) suffered a clinical relapse during the first year of therapy and only 3/32 patients (9.4%) did so later, and our findings are similar to those reported by other authors [3]. It is surprising that Ríos-Blanco et al. did not find any relapses in their series. Finally, recent vaccinations, anaesthesic procedures, cocaine consumption, ingestion of tryptophan or montelukast and rapid discontinuation of steroids have been reported as putative triggering factors not only in the onset of CSS, but also in its relapse [35].

In agreement with Ríos-Blanco et al. [1], in our experience the outcome and long-term survival is usually good in CSS, with a low mortality rate compared with other systemic vasculitides.

Notes

Correspondence to: R. Solans. Back

References

  1. Ríos Blanco JJ, Gómez Cerezo J, Suarez García I, Gutierrez Molina M, Vazquez Rodríguez JJ, Barbado Hernández FJ. Churg–Strauss syndrome: outcome and long-term follow-up of 32 patients. Comment on the article by Solans et al. Rheumatology 2002;41:946–7.[Free Full Text]
  2. Solans R, Bosch JA, Pérez-Bocanegra C et al. Churg–Strauss syndrome: outcome and long-term follow-up of 32 patients. Rheumatology2001;40:763–71.[Abstract/Free Full Text]
  3. Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrouse B, Casassus P. Churg–Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine (Baltimore) 1999;78:26–37.
  4. Orriols R, Muñoz X, Ferre J, Huguet P, Morell F. Cocaine-induced Churg–Strauss vasculitis. Eur Respir J1996;9:175–7.[Abstract/Free Full Text]
  5. Solans R, Bosch JA, Selva A, Orriols R, Vilardell M. Montelukast and Churg–Strauss syndrome. Thorax2002;57:183–5.[Abstract/Free Full Text]
Accepted 20 February 2002