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Thorax doi:10.1136/thx.2007.093955

Churg Strauss Syndrome and Leukotriene antagonist use: A respiratory perspective

  1. Nazim Nathani (n.nathani{at}nhs.net)
  1. City Hospital, United Kingdom
    1. Mark A Little (m.little{at}bham.ac.uk)
    1. University of Birmingham, United Kingdom
      1. Heinke Kunst (heinke.kunst{at}heartofengland.nhs.uk)
      1. Heartlands Hospital, United Kingdom
        1. Duncan Wilson (duncan.wilson{at}uhb.nhs.uk)
        1. University Hospitals Birmingham, United Kingdom
          1. David R Thickett (d.thickett{at}bham.ac.uk)
          1. University of Birmingham, United Kingdom
            • Published Online First 20 May 2008

            Abstract

            Background: Churg Strauss Syndrome (CSS) is a rare granulomatous small vessel vasculitis that occurs against a background of long-standing asthma. Leukotriene antagonists (LTAs) are used in the management of asthma and may facilitate a reduction in steroid dosage. Reports of the development of CSS in asthmatic patients following the initiation of LTA therapy suggest either a causal association or an unmasking of latent CSS as steroid doses fall. We have undertaken a systematic review to establish whether evidence of a drug induced syndrome exists.

            Methods: Systematic review searching Medline from database inception to August 2007 to identify cases with a possible association between LTAs and CSS. Hill’s criteria of causation were used to assess strength of causality.

            Results: 62 cases in which CSS developed after the introduction of LTA therapy were identified. Patients were divided into three groups: Group 1 had received no previous steroid therapy, group 2 had been treated with oral and / or inhaled corticosteroids, but had no change in steroid therapy following LTA introduction and group 3 had a clear reduction in steroid therapy following introduction of LTA therapy. The majority of patients from each group exhibited a clear temporal relationship between initiation of LTA and development of CSS with no evidence of pre-existing disease.

            Conclusions: Currently available evidence suggests an association between LTA and CSS that may be causal.

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