Late-Presenting Eosinophilic Granulomatosis with Polyangiitis as Cause of Pulmonary Fibrosis: A Case-Based Review.
Mediterr J Rheumatol
; 35(1): 172-178, 2024 Mar.
Article
em En
| MEDLINE
| ID: mdl-38736966
ABSTRACT
Introduction:
Eosinophilic granulomatosis with polyangiitis (eGPA) is a necrotising vasculitis of small and medium calibre vessels, which affects mostly patients in their fourth to sixth decade of life, and it is a very uncommon aetiology for pulmonary fibrosis. Clinical case A Hispanic 72-year-old female patient presents with a history of lower extremities pain, paraesthesia, oedema, and occasional macroscopic haematuria. During her hospitalisation, the patient presents, and images showed findings compatible with pulmonary fibrosis and alveolar haemorrhage, which require a biopsy, establishing the diagnosis of an eGPA.Discussion:
eGPA is a low-incidence autoimmune vasculitis, with a high number of phenotypes which explain the broad clinical spectrum, but recent advances has helped to understand the physiopathology and its link with other conditions like pulmonary fibrosis.Conclusion:
Early diagnosis and management of this condition is mandatory because it is the only factor that change the outcome of the patients.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Idioma:
En
Ano de publicação:
2024
Tipo de documento:
Article