Rheumatoid arthritis-associated interstitial lung disease (UIP pattern)

Case contributed by Sze Yuen Lee
Diagnosis almost certain

Presentation

Underlying rheumatoid arthritis with chronic cough.

Patient Data

Age: 35 years
Gender: Female

Exuberant honeycombing predominantly in the anterior upper lobes and bilateral lower lobes with peripheral and peribronchovascular involvement. Few soft tissue densities within the honeycomb cystic spaces of both upper lobes.

Features are suggestive of rheumatoid arthritis-associated interstitial lung disease (RA-ILD) with possible superimposed fungal infection in both upper lobes.

The yellow arrow demonstrates honeycombing in the anterior segment of right upper lobe (anterior upper lobe sign). The blue arrow shows the soft tissue density within the honeycomb cystic spaces.

Case Discussion

This case clearly demonstrates the signs that have been described to be more suggestive of connective tissue disorder 1 as compared to idiopathic pulmonary fibrosis (IPF) such as exuberant honeycombing sign (large, extensive honeycomb cysts involving large regions of a lobe) and anterior upper lobe sign (involvement of the anterior aspect of upper lobes with relative sparing of rest of the upper lobes). The straight edge sign (fibrosis of lower zones with sharp demarcation with normal lung in the craniocaudal plane on coronal view) is less apparent in this study.

Serum galactomannan came back positive and patient was treated for fungal infection.

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