Article Text

PDF

Implication of ANCA status in Churg-Strauss syndrome
  1. S Gareeboo
  1. Specialist Registrar, Queen Elizabeth II Hospital, Welwyn Garden City, UK; sgareeboo{at}hotmail.com

Statistics from Altmetric.com

Churg-Strauss syndrome is a small vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA) in a significant proportion of patients. In this study a cross sectional analysis was performed of the clinical and histological features of patients with Churg-Strauss syndrome in relation to their ANCA status.

A cohort of 112 patients who were enrolled in multicentre treatment trials was selected. They were diagnosed with Churg-Strauss syndrome on the basis of current classifications and were less than 75 years of age. The presence of ANCA was detected by indirect immunofluorescence in 43 patients (38%), 39 of whom had ANCA of the perinuclear type (p-ANCA).

Patients with ANCA had a higher frequency of renal involvement (35% v 4%) and peripheral neuropathy (84% v 65%) than those without ANCA. In addition, vasculitis was more often observed in the biopsy specimens of the ANCA positive patients (79% v 39%). On the other hand, patients without ANCA were more likely to have fever (55% v 30%) and cardiac disease (49% v 12%). One limitation of the study was that patients who were initially ANCA negative were not retested.

The authors hypothesise the presence of two phenotypes of Churg-Strauss syndrome on the basis of the ANCA status. They conclude that the latter reflects the underlying pathophysiology of the disease, with the presence of ANCA favouring the likelihood of a vasculitis affecting certain organs. Further work is required to determine what effect these findings might have on treatment.

View Abstract

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.