CTICasesIdiopathic Pulmonary Hemosiderosis with PPFE-like Fibrosis
Imaging findings
A young woman with long-standing idiopathic pulmonary hemosiderosis (diagnosed in childhood) has a chest radiograph showing nodularity, fibrotic change, and hilar retraction, and CT showing lobular areas of high attenuation and ground glass with pronounced sparing of the septa, resembling chronic pulmonary hemorrhage, together with upper lobe fibrosis that has a pleuroparenchymal fibroelastosis-like appearance. She underwent transplant, with pulmonary hypertension found on explant but little to suggest it on imaging.
Key takeaways
Idiopathic pulmonary hemosiderosis produces a characteristic pattern of lobular ground-glass and high-attenuation change with septal sparing, reflecting chronic recurrent alveolar hemorrhage present for decades. An associated upper-lobe pleuroparenchymal fibroelastosis-like fibrosis has been observed in these cases, and the possible link (perhaps via ischemia or vascular changes) between hemosiderosis and PPFE-type fibrosis is not yet understood; associated pulmonary hypertension may be present.
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