Alpha-1 Antitrypsin Deficiency Presenting with MPO-ANCA Associated Vasculitis and Aortic Dissection
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 l...
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| Format: | Article |
| Language: | English |
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Wiley
2017-01-01
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| Series: | Case Reports in Medicine |
| Online Access: | http://dx.doi.org/10.1155/2017/8140641 |
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| author | Bram M. Voorzaat Jan van Schaik Stijn L. P. Crobach Catharina S. P. van Rijswijk Joris I. Rotmans |
| author_facet | Bram M. Voorzaat Jan van Schaik Stijn L. P. Crobach Catharina S. P. van Rijswijk Joris I. Rotmans |
| author_sort | Bram M. Voorzaat |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-db249b7375e6472ea5bd3a7ace6375d0 |
| institution | Kabale University |
| issn | 1687-9627 1687-9635 |
| language | English |
| publishDate | 2017-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Medicine |
| spelling | doaj-art-db249b7375e6472ea5bd3a7ace6375d02025-02-03T05:52:19ZengWileyCase Reports in Medicine1687-96271687-96352017-01-01201710.1155/2017/81406418140641Alpha-1 Antitrypsin Deficiency Presenting with MPO-ANCA Associated Vasculitis and Aortic DissectionBram M. Voorzaat0Jan van Schaik1Stijn L. P. Crobach2Catharina S. P. van Rijswijk3Joris I. Rotmans4Department of Nephrology, Leiden University Medical Center, Leiden, NetherlandsDepartment of Surgery, Leiden University Medical Center, Leiden, NetherlandsDepartment of Pathology, Leiden University Medical Center, Leiden, NetherlandsDepartment of Radiology, Leiden University Medical Center, Leiden, NetherlandsDepartment of Nephrology, Leiden University Medical Center, Leiden, NetherlandsThe 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.http://dx.doi.org/10.1155/2017/8140641 |
| spellingShingle | Bram M. Voorzaat Jan van Schaik Stijn L. P. Crobach Catharina S. P. van Rijswijk Joris I. Rotmans Alpha-1 Antitrypsin Deficiency Presenting with MPO-ANCA Associated Vasculitis and Aortic Dissection Case Reports in Medicine |
| title | Alpha-1 Antitrypsin Deficiency Presenting with MPO-ANCA Associated Vasculitis and Aortic Dissection |
| title_full | Alpha-1 Antitrypsin Deficiency Presenting with MPO-ANCA Associated Vasculitis and Aortic Dissection |
| title_fullStr | Alpha-1 Antitrypsin Deficiency Presenting with MPO-ANCA Associated Vasculitis and Aortic Dissection |
| title_full_unstemmed | Alpha-1 Antitrypsin Deficiency Presenting with MPO-ANCA Associated Vasculitis and Aortic Dissection |
| title_short | Alpha-1 Antitrypsin Deficiency Presenting with MPO-ANCA Associated Vasculitis and Aortic Dissection |
| title_sort | alpha 1 antitrypsin deficiency presenting with mpo anca associated vasculitis and aortic dissection |
| url | http://dx.doi.org/10.1155/2017/8140641 |
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