CTICasesInterstitial Pneumonia with Autoimmune Features
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Imaging findings
Chest radiograph and CT demonstrate extensive, bilateral, geographic ground-glass opacities and confluent consolidation predominating in the lower lung zones, along with a pleural effusion. Explant or surgical lung biopsy reveals a dense lymphoplasmacytic infiltrate.
Key takeaways
Interstitial pneumonia with autoimmune features (IPAF) is a clinical classification for patients with an interstitial pneumonia who have features suggesting an autoimmune etiology (such as Raynaud's phenomenon or specific histological features like a dense lymphoplasmacytic infiltrate) but do not meet formal diagnostic criteria for a defined connective tissue disease. Recognizing this clinical entity can help avoid unnecessary surgical lung biopsies and guide early management with immunosuppressive therapy.
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