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Updated: Jul 12, 2025
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Pulmonary Alveolar Proteinosis-associated Pulmonary Fibrosis: Evolutional Changes and Radiologic-Pathologic
Eileen Hu-Wang1, Lydia Chelala1, Luis Landeras1
1From the Departments of Cardiopulmonary Imaging (E.H.W., L.C., L.L., J.H.C.), Pathology (H.L., A.N.H.), Pulmonary Medicine (M.E.S.), and Radiology (L.C.), The University of Chicago Medical Center, 5841 S Maryland Ave, Chicago, IL 60637.
Abstract:
Pulmonary alveolar proteinosis (PAP) is a rare disease with frequently favorable outcomes. In a minority of patients with primary or secondary PAP, the disease course may be complicated by pulmonary fibrosis (PF) despite appropriate management. Imaging and histopathologic manifestations of uncomplicated PAP are well-known. In contrast, radiologic-pathologic descriptions of PAP-associated PF (PAP-PF) are limited. The current manuscript presents three cases of PAP-PF, each with serial high-resolution CT imaging demonstrating the longitudinal progression of this unusual complication, with concordant pathologic findings in two patients. Much remains to be known regarding adverse prognostic factors contributing to PAP-PF. Early recognition of radiologic-pathologic manifestations would allow timely diagnosis and management optimization. Keywords: CT, Lung, Inflammation, Pathology © RSNA, 2023.
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