E2146. Radiological-Pathological Correlation of Idiopathic Pulmonary Hemosiderosis
  1. Peter Makhoul; Hospitals of University of Pennsylvania
  2. Nikolina Dioufa; Temple Health
  3. Friedrich Knollman; Hospitals of University of Pennsylvania
  4. Achala Donuru; Hospitals of University of Pennsylvania
Idiopathic pulmonary hemosiderosis (IPH) is a rare disease that primarily affects children and adolescents. IPH most commonly occurs between ages 1 and 7 years. It is characterized by episodic alveolar hemorrhage leading to pulmonary fibrosis. IPH remains a diagnosis of exclusion. The most common symptoms in adults are exertional dyspnea and fatigue due to pulmonary hemorrhage and iron deficiency anemia.

Educational Goals / Teaching Points
Radiographic findings include nonspecific opacities that sometimes have a central and lower lung distribution. Lung apices and costophrenic angles are typically spared unless disease is extensive. During the acute phase the chest radiographs show diffuse alveolar-type infiltrates, predominantly in the lower lung fields, with corresponding ground-glass attenuation on the high-resolution computer tomography. During the remission, the alveolar infiltrates tend to be reabsorbed and interstitial reticular and micronodular patterns ensue in the same areas, with variable degree of fibrosis. Isolated findings consistent with diffuse hemorrhage without associated findings and negative serologic test results are characteristic of IPH.

Key Anatomic/Physiologic Issues and Imaging Findings/Techniques
Specific imaging features, although nonspecific in isolation, may be identified on thoracic imaging studies, principally chest radiography and CT, depending on the phase of disease.

Diagnosis of idiopathic pulmonary hemosiderosis involves demonstration of a combination of characteristic clinical findings, iron deficiency anemia, and hemosiderin-laden macrophages in bronchoalveolar lavage (BAL) fluid or lung biopsy specimens. Corticosteroids may reduce the morbidity and mortality of acute episodes of alveolar bleeding and may control the disease progression of pulmonary fibrosis. Integration of clinical, radiologic, laboratory, and pathologic findings facilitates timely diagnosis.