Reply

A. Y. Hoi and E. F. Morand

Department of Rheumatology, Monash Medical Centre, Level 3, Block E, 246 Clayton Road, Clayton, Victoria 3168, Australia

Correspondence to: A. Y. Hoi. E-mail: ahoi{at}lambert.net.au

SIR, We were pleased to read the report of Loo et al. in relation to two cases of polyneuropathy associated with Churg–Strauss syndrome (CSS) responding to intravenous pulse cyclophosphamide. The primary purpose of our original article was to raise awareness that the presence of mononeuritis multiplex or, as in these cases, distal motor polyneuropathy, should be regarded as severe disease associated with significant long-term morbidity. In the current literature, the definition of severe disease in CSS is ambiguous as far as peripheral nerve involvement is concerned. While we acknowledge that CSS is associated with a better prognosis when compared with other primary small-vessel vasculitides [12], significant neuropathic involvement can result in short- and long-term disability. This may be prevented if we can treat appropriate patients with adequate therapy to induce rapid remission of disease and prevent relapse. Although steroid monotherapy can sometimes achieve this, in our two cases steroid alone was insufficient to achieve complete remission. The use of concomitant pulse cyclophosphamide therapy may also reduce the cumulative dose of corticosteroid required to maintain remission, thus avoiding the bone and metabolic complications of long-term steroid therapy. We agree with Loo et al. that prospective outcome studies that address the risk–benefit ratio should be done, especially to take account of functional outcome and quality of life issues in patients with Churg–Strauss syndrome.

The authors have declared no conflicts of interest.

References

  1. Gayraud M, Guillevin L, le Toumelin P et al. Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg–Strauss syndrome: analysis of four prospective trials including 278 patients. Arthritis Rheum 2001;44:666–75.[CrossRef][ISI][Medline]
  2. Hattori N, Mori K, Misu K, Koike H, Ichimura M, Sobue G. Mortality and morbidity in peripheral neuropathy associated Churg–Strauss syndrome and microscopic polyangiitis. J Rheumatol 2002;29:1408–14.[ISI][Medline]
Accepted 12 June 2003





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