CTICasesMixed connective tissue disease-associated non-specific interstitial pneumonia
Imaging findings
Long-term follow-up CT scans over 18 years show evolution from a typical non-specific interstitial pneumonia pattern to extensive, subpleural-sparing, bronchocentric cystic airspaces in the lower lobes without typical traction bronchiectasis or honeycombing.
Key takeaways
Connective tissue disease can present as a non-specific interstitial pneumonia that mimics idiopathic pulmonary fibrosis due to the development of extensive cysts. Clues that differentiate this from typical honeycombing include subpleural sparing, lack of progressive traction bronchiectasis, and very slow temporal evolution over decades.
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