Alpha-1 Antitrypsin Deficiency Presenting with MPO-ANCA Associated Vasculitis and Aortic Dissection
Joint Authors
Voorzaat, Bram M.
van Schaik, Jan
Crobach, Stijn L. P.
van Rijswijk, Catharina S. P.
Rotmans, Joris I.
Source
Issue
Vol. 2017, Issue 2017 (31 Dec. 2017), pp.1-4, 4 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2017-03-06
Country of Publication
Egypt
No. of Pages
4
Main Subjects
Abstract EN
The combination of alpha-1 antitrypsin (AAT) deficiency, ANCA-vasculitis, and aortic aneurysm has been rarely described in literature.
We report an eventually fatal case in a 70-year-old patient who initially presented with giant cell arteritis and ANCA associated glomerulonephritis.
Several years later, he presented with aortic dissection due to large vessel vasculitis, raising the suspicion of AAT deficiency, as two first-line relatives had chronic obstructive pulmonary disease, while they never smoked.
This diagnosis was confirmed by AAT electrophoresis and immunohistochemistry on a temporal artery biopsy.
Considering AAT deficiency in these cases might lead to a more timely diagnosis.
American Psychological Association (APA)
Voorzaat, Bram M.& van Schaik, Jan& Crobach, Stijn L. P.& van Rijswijk, Catharina S. P.& Rotmans, Joris I.. 2017. Alpha-1 Antitrypsin Deficiency Presenting with MPO-ANCA Associated Vasculitis and Aortic Dissection. Case Reports in Medicine،Vol. 2017, no. 2017, pp.1-4.
https://search.emarefa.net/detail/BIM-1146870
Modern Language Association (MLA)
Voorzaat, Bram M.…[et al.]. Alpha-1 Antitrypsin Deficiency Presenting with MPO-ANCA Associated Vasculitis and Aortic Dissection. Case Reports in Medicine No. 2017 (2017), pp.1-4.
https://search.emarefa.net/detail/BIM-1146870
American Medical Association (AMA)
Voorzaat, Bram M.& van Schaik, Jan& Crobach, Stijn L. P.& van Rijswijk, Catharina S. P.& Rotmans, Joris I.. Alpha-1 Antitrypsin Deficiency Presenting with MPO-ANCA Associated Vasculitis and Aortic Dissection. Case Reports in Medicine. 2017. Vol. 2017, no. 2017, pp.1-4.
https://search.emarefa.net/detail/BIM-1146870
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1146870