Review series
Rare diseases
11
Churg-Strauss syndrome
Matthew Conron, Huw L C Beynon
Department of
Rheumatology, Royal Free Hospital, Pond Street, London NW3 2QG, UK
Correspondence to: Dr H L C Beynon
| The first 150 words of the full text of this article appear below. |
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Introduction |
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In 1951 the pathologists Churg and Strauss identified 13 patients who presented with a clinical syndrome characterised by asthma, hypereosinophilia, and evidence of vasculitis affecting a
number of organs.1 The three main histological features
found on pathological examination of these cases were extravascular granulomas, tissue eosinophilia, and necrotising vasculitis (fig 1).
Most of these patients had been previously diagnosed with "periarteritis nodosa". After reviewing a number of cases of
periarteritis nodosa without asthma and finding no evidence of an
eosinophilic granulomatous process, Churg and Strauss suggested that
their 13 cases represented a separate disease process and coined the term "allergic granulomatosis and angiitis". Later "Churg-Strauss syndrome" (CSS) became the accepted title of this distinctive form of
systemic vasculitis. More recent pathological case series involving
patients with CSS have highlighted the fact that not all patients have
the three main histological features originally described by Churg and
Strauss.2 3 Given the absence of a
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