Late-Presenting Eosinophilic Granulomatosis with Polyangiitis as Cause of Pulmonary Fibrosis: A Case-Based Review

TL;DR

This case review discusses a rare autoimmune condition, eGPA, which can cause pulmonary fibrosis and highlights the importance of early diagnosis for better patient outcomes.

Contribution

The paper presents a case of late-presenting eGPA as a rare cause of pulmonary fibrosis and emphasizes the need for early diagnosis.

Findings

eGPA is a rare cause of pulmonary fibrosis and can present with atypical symptoms.

Early diagnosis and management significantly improve patient outcomes in eGPA.

The condition has a broad clinical spectrum due to its multiple phenotypes.

Abstract

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.

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.

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.

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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