Airway-centered interstitial fibrosis

Case contributed by Eva Gimenez
Diagnosis possible

Presentation

Asymptomatic non-smoker with rheumatoid arthritis. No history of exposure to inhaled antigens.

Patient Data

Age: 55 years
Gender: Female

Large volume lungs with mosaic attenuation. Bilateral peribronchovascular ground glass opacities, interstitial thickening, and traction bronchiectasis with thickened bronchial walls. No subpleural predominance or honeycombing.

No consolidation, lymphadenopathy or pleural effusion.

Case Discussion

Airway-centered interstitial fibrosis (ACIF) is an uncommonly recognized histological pattern of interstitial lung disease of variable etiology and relatively favorable outcome. It is characterized by fibrosis of the respiratory bronchioles and the peribronchiolar interstitium and moderate lymphocytes on bronchoalveolar lavage. Histologically confirmed ACIF is rare and can be associated with hypersensitivity pneumonitis, aspiration or endogenous autoimmune conditions such as rheumatoid arthritis. Long-term prognosis is relatively good on immunosuppressive therapy.

Thoracic CT showed a distinctive pattern of interstitial fibrosis that was centered and extended around the bronchioles. We suggested ACIF based on clinical and radiological characteristics, but histological confirmation was not obtained.

The maintained lung volume and mosaic attenuation could suggest constrictive bronchiolitis, however expiratory CT is not available.

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