Highlights & Basics
- Eosinophilic granulomatosis with polyangiitis (also known as Churg-Strauss syndrome) is characterized by the pathologic triad of tissue eosinophilia, granulomatous inflammation, and vasculitis.
- The cornerstone of treatment is corticosteroids. Severe disease is treated in a similar manner to other antineutrophil cytoplasmic antibody associated vasculitides, with additional immunosuppressive agents.
- Cardiac involvement causes significant morbidity and mortality.
- Typically patients are initially diagnosed with asthma, and then months to years later develop manifestations due to tissue eosinophilia, followed by vasculitis.
Quick Reference
History & Exam
Key Factors
Other Factors
Diagnostics Tests
Treatment Options
Definition
Epidemiology
Etiology
Pathophysiology
Images
CT scan of the chest obtained (A) during symptom exacerbation, revealing pulmonary infiltrates in the right inferior lobe (A3) and the left inferior lobe (A4). Re-evaluation 15 days later (B) revealed the migratory character of the lesions, with complete disappearance of the previously described infiltrates and the presence of new areas of ground glass opacities within the right inferior lobe (B2) and the left lingular and left inferior lobe (B4). (C) Six months after treatment, all the lung lesions described had healed completely
Histologic evaluation of the lung biopsy specimen, revealing the presence of an inflammatory infiltrate composed predominantly of eosinophils found within both the vascular lumen and the vascular wall
Cytologic examination of the bronchoalveolar lavage fluid, revealing the presence of a vast predominance of eosinophils (A), representing 27% of the cellular elements. The other cellular elements found were macrophages (C) and lymphocytes (B)
Citations
Ntatsaki E, Carruthers D, Chakravarty K, et al; BSR and BHPR Standards, Guidelines and Audit Working Group. BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis. Rheumatology (Oxford). 2014 Dec;53(12):2306-9.[Abstract][Full Text]
Groh M, Pagnoux C, Baldini C, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management. Eur J Intern Med. 2015 Sep;26(7):545-53.[Abstract][Full Text]
Chung SA, Langford CA, Maz M, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol. 2021 Aug;73(8):1366-83.[Abstract][Full Text]
Hellmich B, Sanchez-Alamo B, Schirmer JH, et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Ann Rheum Dis. 2023 Mar 16:ard-2022-223764.[Abstract][Full Text]
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