A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA): monocentric experiences in 150 patients
Annals of Rheumatic Diseases, 08/21/2012
Moosig F et al. – Regular re–evaluation and target–orientated adaption of therapy may lead to normalization of life expectancy and attenuation of disease progression. Continued centre based interdisciplinary treatment should be standard of care.Methods
- A retrospective cohort study at a vasculitis referral centre was performed.
- All EGPA patients admitted from 1990 to 2009 were included.
- A structured interdisciplinary work-up for proof of diagnosis, Disease Extent Index and Birmingham Vasculitis Activity Score was performed.
- Immunosuppressive therapy was initiated and regularly adapted.
- Treatment targets were induction and maintenance of remission according to definitions given by the European League Against Rheumatism and the European Vasculitis Study Group.
- Outcomes were mortality, rate of remission, relapses, adverse events and prednisolone-dose.
- Out of 269 patients with suspected EGPA 150 fulfilled the inclusion criteria.
- Of those, 104 had more than one follow-up visit resulting in a mean follow up of 53±4.9 months.
- By using additional data sources the follow-up concerning survival was extended to 92±5 month.
- Severe organ manifestations occurred at heart (46%), kidney (18%) and lungs (10%).
- Cyclophosphamide was used in 107 patients (71%).
- The prednisolone-doses of all patients were within the targeted range (i.e. ≤7.5mg) in 69% of the total follow-up time; the median dose at end of follow-up was 5mg/d.
- The 10-year survival rate was 89% resulting in mortality comparable to the general population (SMR 1.29).
- Only patients with cardiac failure associated with EGPA had an increased mortality (SMR 3.06).