A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg–Strauss, EGPA): monocentric experiences in 150 patients
Abstract
Objective To evaluate a vasculitis centre based management strategy for eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA). 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. Results 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. ≤7.5 mg) 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…
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Eosinophil-related autoimmune disorders are strongly linked to cardiovascular manifestations including myocarditis.
“eosinophil-related autoimmune disorders are strongly linked to cardiovascular manifestations including myocarditis.”
From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [202]
Over 40% of patients with eosinophilic granulomatosis with polyangiitis experience cardiovascular complications.
“over 40% of patients with eosinophilic granulomatosis with polyangiitis, also known as ChurgStrauss, experience cardiovascular complications.”
From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [202]
Approximately 20% to 50% of patients with hypereosinophilic syndrome exhibit inflammation or fibrosis in the heart.
“Approximately 20% to 50% of patients with hypereosinophilic syndrome exhibit inflammation or fibrosis in the heart”
From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [202]
Cites
Mepolizumab as a steroid-sparing treatment option in patients with Churg-Strauss syndrome
Kim, S., Marigowda, G., Oren, E., Israel, E., & Wechsler, M. E. (2010) · Journal of Allergy and Clinical Immunology · doi:10.1016/j.jaci.2010.03.028 · Cited by 280 · see citing works
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Groh, M., Pagnoux, C., Baldini, C., Bel, E., Bottero, P., Cottin, V., Dalhoff, K., Dunogué, B., Gross, W., Holle, J., et al. (2015) · European Journal of Internal Medicine · doi:10.1016/j.ejim.2015.04.022 · Cited by 476 · see citing works
Self-citationAutoimmune Myocarditis, Old Dogs and New Tricks.
Won, T., Song, E. J., Kalinoski, H. M., Moslehi, J. J., & Čiháková, D. (2024) · Circulation Research · doi:10.1161/circresaha.124.323816 · Cited by 51 · see citing works
Churg-Strauss Syndrome or Eosinophilic Granulomatosis with Polyangiitis
Izquierdo-Domínguez, A., Cordero Castillo, A., Alobid, I., & Mullol, J. (2015) · Sinusitis · doi:10.3390/sinusitis1010024 · Cited by 8 · see citing works
- Authors
- Moosig, F., Bremer, J. P., Hellmich, B., Holle, J. U., Holl-Ulrich, K., Laudien, M., Matthis, C., Metzler, C., Nölle, B., Richardt, G., & Gross, W. L.
- Affiliations
- University Hospital Schleswig-Holstein (DE)
- University of Tübingen (DE)
- Christian-Albrechts-Universität zu Kiel (DE)
- University of Lübeck (DE)
- Krankenhaus Barmherzige Brüder (DE)
- Segeberger Kliniken (DE)
- Corresponding author
- Moosig, Frank
- Venue
- Annals of the Rheumatic Diseases
- Publisher
- Elsevier BV
- Published
- 2013-06
- Volume
- 72
- Issue
- 6
- Pages
- 1011-1017
- ISSN
- 0003-4967 (ISSN-L)
- OpenAlex
- W2151789392
- Cited by
- 279
- References
- 1
- Citations by year
- 2026: 9 · 2025: 24 · 2024: 16 · 2023: 28 · 2022: 29 · 2021: 22 · 2020: 18 · 2019: 24 · 2018: 15 · 2017: 18 · 2016: 18 · 2015: 18 · 2014: 18 · 2013: 19 · 2012: 3
- FWCI
- 14.95
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Annals of the Rheumatic Diseases (publishedVersion)
- PubMed (publishedVersion)
- HighWire Press Open Archive (submittedVersion)
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Vasculitis and related conditions
- Health Sciences › Medicine › Rheumatology › Otitis Media and Relapsing Polychondritis
- Health Sciences › Medicine › Nephrology › Renal Diseases and Glomerulopathies
- Keywords
- eosinophilic granulomatosis with polyangiitis, multidisciplinary management, immunosuppressive therapy, cyclophosphamide, prednisolone dose, remission induction and maintenance, relapse, mortality, cardiac involvement, organ involvement
- MeSH terms
- Leflunomide, Anti-Inflammatory Agents, Azathioprine, Cyclophosphamide, Female, Humans, Immunosuppression Therapy, Immunosuppressive Agents, Isoxazoles, Male, Methotrexate, Middle Aged, Prednisolone, Remission Induction, Retrospective Studies, Churg-Strauss Syndrome, Cohort Studies, Treatment Outcome, Secondary Prevention
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