CTICasesFamilial Pulmonary Fibrosis with a Non-UIP Fibrotic Pattern
Imaging findings
Serial CTs several years apart in a patient with known interstitial fibrosis show diffuse, bilateral reticulation with focal areas of dense peripheral upper lobe opacity containing traction bronchiectasis, relatively sparing the subpleural lower lung zones, and demonstrate clear interval worsening of fibrosis between the two studies -- a pattern atypical for usual interstitial pneumonia/IPF.
Key takeaways
A non-UIP pattern of progressive fibrosis, particularly with peripheral upper-lobe-predominant traction bronchiectasis, should prompt a family history inquiry -- this patient's mother and two siblings also had pulmonary fibrosis, supporting a diagnosis of familial pulmonary fibrosis, which classically shows more heterogeneous fibrotic patterns than sporadic IPF.
AI-assisted summary — may contain errors. Verify against the source video. Learn more
More from this webinar
- Thrombosed Anomalous Systemic Arterial Supply to the Lung ("Lonely Bronchus" Sign)Vascular
- Smoking-Related Interstitial Lung Disease with Bizarre Cystic AirspacesILD
- Mediastinal Large B-Cell Lymphoma Demonstrating the Hilum Overlay SignNeoplastic
- Pulmonary Vein Thrombosis After Redo Lung Transplant Causing Venous CongestionVascular
- Atrioesophageal Fistula After Atrial Fibrillation Ablation with Embolic StrokesIatrogenic
- Pseudocoarctation of the Aorta with Bicuspid Aortic ValveCongenital
- Aortic Coarctation with Disproportionately Dilated Descending AortaCongenital
- Dynamic Free-Breathing CT Airway Evaluation Demonstrating Severe TracheomalaciaCongenital
See all cases from April 19, 2018 →
Related ILD cases
- Anti-Jo-1 Antisynthetase Syndrome
- Anti-PL-7 Antisynthetase Syndrome
- Fibrotic Hypersensitivity Pneumonitis (Indeterminate for UIP Pattern)
- Acute Hypersensitivity Pneumonitis from Vaping Marijuana
- Radiation-Induced Migratory Organizing Pneumonia After Breast Cancer Treatment
- Hypersensitivity Pneumonitis Misdiagnosed as Sarcoidosis