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Rare case of eosinophilic granulomatosis with polyangiitis in two patients with α-1-antitrypsin deficiency (PiSZ).

Moxey, Jordan Maureen and 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.

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Official URL: http://casereports.bmj.com/content/2016/bcr-2015-2...

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: Miss Adele Creak
Date Deposited: 30 Jan 2017 14:06
Last Modified: 30 Jan 2017 14:06
URI: http://www.repository.heartofengland.nhs.uk/id/eprint/1131

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