Eosinophilic granulomatosis with polyangiitis: case report and literature review
Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg–Strauss syndrome, is a multisystem disorder characterised by asthma, blood and tissue eosinophilia and small-vessel vasculitis. Eosinophilic tissue infiltration and extravascular granuloma formation can lead to damage in...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
| Published: |
European Respiratory Society
2022-12-01
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| Series: | Breathe |
| Online Access: | http://breathe.ersjournals.com/content/18/4/220170.full |
| _version_ | 1811162577398398976 |
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| author | Vardah Alam Alexandra M. Nanzer |
| author_facet | Vardah Alam Alexandra M. Nanzer |
| author_sort | Vardah Alam |
| collection | DOAJ |
| description | Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg–Strauss syndrome, is a multisystem disorder characterised by asthma, blood and tissue eosinophilia and small-vessel vasculitis. Eosinophilic tissue infiltration and extravascular granuloma formation can lead to damage in any organ, but it is classically seen to cause pulmonary infiltrates, sino-nasal disease, peripheral neuropathy, renal and cardiac involvement, and rashes. EGPA is part of the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis syndromes, with the antibody being detected in ∼30–40% of cases and mostly against myeloperoxidase. Two genetically and clinically distinct phenotypes, defined by the presence or absence of ANCA have been identified. Treatment for EGPA focuses on inducing and maintaining disease remission. To date, oral corticosteroids remain first-line agents whilst second-line treatments include immunosuppressants such as cyclophosphamide, azathioprine, methotrexate, rituximab and mycophenolate mofetil. However, long-term steroid usage results in multiple and well-known adverse health effects and new insights into the pathophysiology of EGPA have allowed for the development of targeted biologic therapies, like the anti-eosinophilic, anti-interleukin-5 monoclonal antibodies. |
| first_indexed | 2024-04-10T06:31:43Z |
| format | Article |
| id | doaj.art-3e26b8a2ceba4ba4b97c2770d64ae0e1 |
| institution | Directory Open Access Journal |
| issn | 1810-6838 2073-4735 |
| language | English |
| last_indexed | 2024-04-10T06:31:43Z |
| publishDate | 2022-12-01 |
| publisher | European Respiratory Society |
| record_format | Article |
| series | Breathe |
| spelling | doaj.art-3e26b8a2ceba4ba4b97c2770d64ae0e12023-03-01T07:06:38ZengEuropean Respiratory SocietyBreathe1810-68382073-47352022-12-0118410.1183/20734735.0170-20220170-2022Eosinophilic granulomatosis with polyangiitis: case report and literature reviewVardah Alam0Alexandra M. Nanzer1 Guy's and St Thomas’ Hospitals NHS Trust, London, UK Guy's and St Thomas’ Hospitals NHS Trust, London, UK Eosinophilic granulomatosis with polyangiitis (EGPA), previously known as Churg–Strauss syndrome, is a multisystem disorder characterised by asthma, blood and tissue eosinophilia and small-vessel vasculitis. Eosinophilic tissue infiltration and extravascular granuloma formation can lead to damage in any organ, but it is classically seen to cause pulmonary infiltrates, sino-nasal disease, peripheral neuropathy, renal and cardiac involvement, and rashes. EGPA is part of the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis syndromes, with the antibody being detected in ∼30–40% of cases and mostly against myeloperoxidase. Two genetically and clinically distinct phenotypes, defined by the presence or absence of ANCA have been identified. Treatment for EGPA focuses on inducing and maintaining disease remission. To date, oral corticosteroids remain first-line agents whilst second-line treatments include immunosuppressants such as cyclophosphamide, azathioprine, methotrexate, rituximab and mycophenolate mofetil. However, long-term steroid usage results in multiple and well-known adverse health effects and new insights into the pathophysiology of EGPA have allowed for the development of targeted biologic therapies, like the anti-eosinophilic, anti-interleukin-5 monoclonal antibodies.http://breathe.ersjournals.com/content/18/4/220170.full |
| spellingShingle | Vardah Alam Alexandra M. Nanzer Eosinophilic granulomatosis with polyangiitis: case report and literature review Breathe |
| title | Eosinophilic granulomatosis with polyangiitis: case report and literature review |
| title_full | Eosinophilic granulomatosis with polyangiitis: case report and literature review |
| title_fullStr | Eosinophilic granulomatosis with polyangiitis: case report and literature review |
| title_full_unstemmed | Eosinophilic granulomatosis with polyangiitis: case report and literature review |
| title_short | Eosinophilic granulomatosis with polyangiitis: case report and literature review |
| title_sort | eosinophilic granulomatosis with polyangiitis case report and literature review |
| url | http://breathe.ersjournals.com/content/18/4/220170.full |
| work_keys_str_mv | AT vardahalam eosinophilicgranulomatosiswithpolyangiitiscasereportandliteraturereview AT alexandramnanzer eosinophilicgranulomatosiswithpolyangiitiscasereportandliteraturereview |