α1-Antitrypsin deficiency in a patient diagnosed with granulomatosis with polyangiitis.
BMJ Case Rep
; 20132013 Apr 25.
Article
em En
| MEDLINE
| ID: mdl-23625671
ABSTRACT
Granulomatosis with polyangiitis is a rare type of vasculitis that affects small-sized and medium-sized vessels. Any organ system can become affected, but it most commonly affects the upper airways, lungs and kidneys. The α1-antitrypsin deficiency is another rare disease that involves a genetic deficiency in the enzyme antitrypsin, which is produced in the liver and protects the lung against proteinases. The simultaneous occurrence of these two diseases is very rare and has been described. We present a case of granulomatosis with polyangiitis limited to the upper airways, and α1-antitrypsin deficiency occurring in the same patient. The patient presented with recurrent upper airway infections. The patient was treated with steroids and azathioprine which prevented recurrence of symptoms. High clinical suspicion of the concomitant occurrence of α1-antitrypsin deficiency in patients with vasculitis is essential to provide patients with adequate screening and treatment.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Fragmentos de Peptídeos
/
Granulomatose Orofacial
/
Vasculite Sistêmica
Tipo de estudo:
Diagnostic_studies
Limite:
Adult
/
Female
/
Humans
Idioma:
En
Revista:
BMJ Case Rep
Ano de publicação:
2013
Tipo de documento:
Article