CTICasesIdiopathic Pulmonary Hemosiderosis with Severe Pulmonary Hypertension
Imaging findings
CT in a young non-smoking male demonstrates diffuse bilateral cystic spaces and areas of ground-glass opacity that could be described as emphysematous spaces though the patient is a lifetime non-smoker. There is a markedly enlarged main pulmonary artery indicating severe pulmonary hypertension, with pulmonary artery pressures measured in the 120s-130s mmHg. The central pulmonary arteries show evidence of intimal thickening and calcification from chronic pulmonary arterial hypertension superimposed on what appears to be chronic thromboembolic disease or intimal hyperplasia. A second case with nearly identical CT findings is shown for comparison. Both patients are young non-smoking males with the same pattern of lung disease diagnosed as pulmonary hemosiderosis.
Key takeaways
Chronic recurrent pulmonary hemorrhage producing hemosiderosis can result in cystic or emphysematous-appearing lung destruction in young non-smoking patients, associated with severe secondary pulmonary hypertension. The CT findings of bilateral low-attenuation cystic spaces in a young non-smoker combined with severe pulmonary hypertension should raise idiopathic pulmonary hemosiderosis in the differential. These cases may be mistaken for smoking-related emphysema or other cystic lung diseases. The characteristic pattern with severe PAH in young non-smokers is distinctive.
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