Idiopathic pulmonary hemosiderosis: radiological findings in 13 patients

Purpose: To asses the imaging findings in idiopathic pulmonary hemosiderosis (IPH). Material and Methods: In this retrospective study we reviewed the chest X-ray and high resolution CT (HRCT) findings in 13 patients with diagnosis of IPH in a 15 year-period (1987-2002). The mean age at diagnosis was 7 years old (range 6 months-17 years). Half of patients were younger than 7 years. The incidence was higher in females (8/13, 62%). The diagnosis was made by open lung biopsy in 6 patients (46%), hemosiderin-laden macrophages in bronchoalveolar lavage (BAL) fluid in 5 patients (38%) and hemosiderin-laden macrophages in gastric aspirate in 2 patients (15%). We analyzed clinical data and imaging findings obtained by chest X-ray and high resolution CT with low dose mAs. Results: The chest X-ray showed interstitial infiltrates (12/13) and ill-defined bilateral nodular areas in 2/13 cases (15%). HRCT showed patterns of ground glass opacification in both lungs in 12/13 cases (92%), intra alveolar infiltrates in 2/13 cases (15%) and thickened interlobular septa with parenchymal distortion in 2/13 cases (15%). All these imaging findings were consistent with pulmonary hemorrhage. Conclusion: The correlation between clinical data and characteristic radiologic patterns allows an early diagnosis of this rare disease. HRCT findings are not pathognomonic and final diagnosis requires either open lung biopsy, BAL, or gastric fluid analysis. An accurate diagnosis of IPH may avoid severe complications. (author)