CTICasesRapidly Progressive Fibrosis in Sjogren Syndrome
Imaging findings
Serial CT chest scans over 6 months show rapid progression of bilateral NSIP-like reticulation, ground-glass opacities, and lower-lobe traction bronchiectasis, complicated by the development of spontaneous pneumomediastinum.
Key takeaways
Sjögren syndrome is classically associated with lymphoid interstitial pneumonia (LIP), but patients can also develop fibrotic NSIP or UIP patterns. Rapid progression of fibrosis is associated with poor prognosis and can be complicated by alveolar rupture leading to spontaneous pneumomediastinum.
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