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- Title
Successful α1-antitrypsin replacement therapy in a patient with α1-antitrypsin deficiency and granulomatosis with polyangiitis.
- Authors
Hernández Pérez, José María; Fumero García, Sergio; Alvarez Pío, Alberto
- Abstract
The authors discuss a case of an 84-year-old woman with a history of arterial hypertension and ischaemic cardiopathy presented with an air flow limitation and a predicted forced expiratory volume in 1 second (FEV1) of 63 percent. The patient had dehydration, renal dysfunction, and vascular thrombus which led to the diagnosis of granulomatosis with polyangiitis. Treatment with methylprednisolone and replacement therapy with alpha-1-antitrypsin (A1AT) was started after which she recovered.
- Subjects
PULMONARY emphysema; ALPHA 1-antitrypsin; ALPHA 1-antitrypsin deficiency; AUTOANTIBODIES; BIOPSY; BLOOD testing; BLOOD vessels; GENES; HEALTH outcome assessment; PHYSICAL diagnosis; TOMOGRAPHY; EQUIPMENT &; supplies; TREATMENT effectiveness; METHYLPREDNISOLONE; PREVENTION; THERAPEUTICS
- Publication
Rheumatology, 2013, Vol 52, Issue 4, p755
- ISSN
1462-0324
- Publication type
Article
- DOI
10.1093/rheumatology/kes233