Cellular NSIP in IPAF
Imaging findings
CT shows multifocal, geographic bilateral ground-glass opacities and consolidations, predominantly in the lower lung zones. Surgical lung biopsy demonstrates cellular NSIP characterized by homogeneous interstitial lymphocytic infiltration and lymphoid follicles, with pleural thickening.
Key takeaways
Interstitial pneumonia with autoimmune features (IPAF) is a provisional classification for patients with ILD and clinical/serologic autoimmune features who do not meet full criteria for a defined CTD. Cellular NSIP with lymphoid follicles and pleural thickening is a classic histologic pattern.
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