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Moxey, Jordan Maureen, Low, Emma Victoria and Turner, Alice Margaret (2016) Rare case of eosinophilic granulomatosis with polyangiitis in two patients with α-1-antitrypsin deficiency (PiSZ). BMJ case reports, 2016. ISSN 1757-790X. This article is available to all HEFT staff and students via ASK HEFT Discovery tool http://tinyurl.com/z795c8c using their HEFT Athens Login.
Full text not available from this repository.Abstract
We present two cases of eosinophilic granulomatosis with polyangiitis occurring with α-1-antitrypsin deficiency, both PiSZ phenotype. The simultaneous occurrence of these two conditions has seldom been described in the literature, despite evidence of an association between α-1-antitrypsin deficiency and other forms of vasculitis. Both patients had pulmonary involvement and reported intermittent exacerbations of vasculitic symptoms. Both patients were managed on low-dose oral steroids and azathioprine remaining well with occasional exacerbations. It is important to consider whether there is an association between eosinophilic granulomatosis with polyangiitis and α-1-antitrypsin deficiency, as this may lead to more severe pulmonary symptoms during exacerbations. If a genetic association between the two conditions is found, clinicians should be aware of the possible need to screen for α-1-antitrypsin deficiency in appropriate patients.
| Item Type: | Article |
|---|---|
| Additional Information: | This article is available to all HEFT staff and students via ASK HEFT Discovery tool http://tinyurl.com/z795c8c using their HEFT Athens Login. |
| Subjects: | WH Haemic and lymphatic systems. Haematology |
| Divisions: | Planned IP Care > Respiratory Medicine |
| Related URLs: | |
| Depositing User: | Mrs Adele Creak |
| Date Deposited: | 30 Jan 2017 14:06 |
| Last Modified: | 30 Jan 2017 14:06 |
| URI: | http://www.repository.uhblibrary.co.uk/id/eprint/1131 |
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