Clinical Trial: Eosinophilic Granulomatosis With Polyangiitis Cohort

Study Status: Recruiting
Recruit Status: Recruiting
Study Type: Observational

Official Title: Eosinophilic Granulomatosis With Polyangiitis Cohort

Brief Summary: EGPA (Eosinophilic Granulomatosis with Polyangiitis, previously Churg-Strauss syndrome) is a rare vasculitis, characterised by asthma, rhinosinusitis and eosinophilia. There has previously been confusion around diagnostic criteria, with multiple sets of classification criteria being used inappropriately for diagnosis. The ERS formed a taskforce to produce specific diagnostic criteria, and these divided the Churg-Strauss Syndrome cohort into two groups - vasculitic EGPA and tissular Hypereosinophilic Asthma with Systemic Manifestations (HASM). It is not known if the groups separated by the diagnostic criteria are also separated in clinical symptomatology, or if their disease course varies, and this could have significant impact on management and further research.

Detailed Summary:

Background Eosinophilic Granulomatosis with Polyangiitis (EGPA, previously Churg-Strauss Syndrome) is a rare, idiopathic, ANCA associated vasculitis (AAV), alongside Granulomatosis with Polyangiitis (GPA) and Microscopic Polyangiitis (MPA). It is an eosinophilic small/medium vessel vasculitis characterised by asthma, rhinosinusitis and eosinophilia. Patients can also suffer renal, cardiac, gastrointestinal and neurological involvement. First described by Churg and Strauss in 1951, exact epidemiology remains difficult because of a lack of clear diagnostic criteria. It appears to have a prevalence of around 11-13 per million population with a mean age of 50 years old and represents 10% of diagnosed vasculitides.

Following Churg and Strauss' original paper, the first attempt to provide diagnostic clarity was provided by John Lanham in 1984. Lanham listed symptoms seen in a set of patients with EGPA and suggested which symptoms were required for the disease and which symptoms were "additional". The American College of Rheumatology (ACR) in 1990 published a list of 6 symptoms to assist with classification of vasculitides, created by comparing 20 patients with EGPA with 787 patients with other forms of vasculitis. This was frequently used for diagnosis despite being intended for classification. In 1994, and revised in 2012, the International Chapel Hill Consensus Conference produced a nomenclature and definition for vasculitides, again frequently incorrectly used as diagnostic criteria. Finally, in 2014, diagnostic criteria were provided by the European Respiratory Society Churg Strauss Syndrome (ERS-CSS) taskforce; however published literature still uses a variety of criteria.

EGPA is recognised as a Th2 predominant disease with elevated levels of IL-4, IL-5, IL-13 and IgE according to a number of case control studies
Sponsor: Portsmouth Hospitals NHS Trust

Current Primary Outcome: Diagnosis according to various diagnostic criteria [ Time Frame: From date of diagnosis for 5 years ]

The diagnosis of each participant according to different diagnostic or classification criteria (ERS-CSS, Chapel Hill, ACR, Lanham) based on all test results available between the point of diagnosis and 60 months later


Original Primary Outcome: Same as current

Current Secondary Outcome:

  • Corticosteroid dose [ Time Frame: From date of diagnosis for 5 years ]
    Stable doses (taken for ≥4 weeks) of oral and inhaled corticosteroids at diagnosis and 12 monthly up to 5 years
  • Immunosuppressive medication [ Time Frame: From date of diagnosis for 5 years ]
    Which additional immunosuppressive medication had been given and when
  • Comorbid conditions [ Time Frame: From date of diagnosis for 5 years ]
    List of comorbidities
  • Biopsy findings [ Time Frame: From date of diagnosis for 5 years ]
    List of Biopsy results
  • Relapse rates [ Time Frame: From date of diagnosis for 5 years ]
    Rate of relapses or flares
  • Peak disease activity [ Time Frame: From date of diagnosis for 5 years ]
    maximum disease activity from diagnosis to 5 years as defined by BVAS score and VDI score


Original Secondary Outcome: Same as current

Information By: Portsmouth Hospitals NHS Trust

Dates:
Date Received: September 26, 2016
Date Started: September 2016
Date Completion: August 2017
Last Updated: January 25, 2017
Last Verified: January 2017