Medical Central Resource
Idiopathic Pulmonary Hemosiderosis
Nusser, Christopher
11/27/96
Presentation
Six month old female presents with weakness and several episodes of hempotysis.
Imaging Technique
Plain X-ray
Imaging Findings
Parahilar linear interstial predominance
Diagnosis
Idiopathic Pulmonary Hemosiderosis
Discussion
Idiopathic Pulmonary Hemosiderosis:
- Clinical:
A rare probably autoimmune process which results in widespread hemosiderin
deposition in the lungs. The disorder is seen most commonly in children under
the age of 10 years and affects males and females equally. In adults, males are
affected more than females and the disease is usually less severe than in
children. Patients present with recurrent hemoptysis (acute attacks which last
2-4 days and is usually not massive), anemia, weakness, clubbing, and
hepatosplenomegaly (25%). Prognosis is poor with recurrent episodes and
remissions. Patients generally die within 5 years due to cor pulmonale. The
disorder is occasionally associated with celiac disease (disease improves on
gluten free diet) or immunoglobulin A gammopathy.
- X-ray:
Following acute hemorrhage there are patchy parenchymal infiltrates with a
perihilar and lower lung predominance that may resemble a pneumonia.
Bilateral, hazy parahilar opacities can be seen and may mimic pulmonary
edema. The infiltrates typically clear over 5 to 7 days. A reticulonodular
interstitial pattern develops as blood is cleared from the alveoli and
hemosiderin is deposited within the septa. Later there is interstitial fibrosis.
Adenopathy and pleural effusions are infrequent findings.
Submitted by: Christopher Nusser,Capt,USAF,MC,Wilford Hall Medical Center
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