CTICasesPrimary Pulmonary Hemosiderosis
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Imaging findings
Initial chest radiograph shows bilateral, diffuse, ill-defined opacities and ground-glass attenuation with relative sparing of the left lower lung. Chest CT confirms a combination of patchy centrilobular, confluent lobular, and geographic ground-glass opacities, along with some chronic-appearing nodular densities. A follow-up CT scan years later shows nearly complete resolution of the ground-glass opacities, leaving only a very fine, low-grade background of grayness representing mild interstitial fibrosis.
Key takeaways
Primary pulmonary hemosiderosis is a rare, idiopathic disease characterized by recurrent episodes of diffuse alveolar hemorrhage, primarily affecting children and young adults. While acute episodes present as transient ground-glass opacities or consolidation that can completely clear, repeated and unremitting hemorrhage leads to hemosiderin deposition in the lung parenchyma, which can eventually stimulate progressive, irreversible pulmonary fibrosis. The disease is a diagnosis of exclusion and is typically managed with long-term corticosteroid or immunosuppressive therapy.
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