SPR unknown #46-- FINAL

Peter Harri, MD

Bradley Wyly MD

Kiery Braithwaite MD

Emory University Department of Radiology and Children’s Hospital of Atlanta, Egleston Hospital

Keywords

SPR unknown 46, idiopathic pulmonary hemosiderosis, iph


Publication Date: 2011-02-25

History

7-year-old female who presented to pediatrician with two days of fever of 102 F, cough, and increased work of breathing. The fever subsided after acetaminophen was given, and her breathing got better after a dose of albuterol. She was noted to be tachypneic and sent to ED. While in the ED, the patient coughed up 1/2 teaspoon of reddish brown material.

Findings

Chest X-ray and Chest CT: Diffuse bilateral alveolar opacities, left greater than right, with interstitial thickening.

Diagnosis

Idiopathic Pulmonary Hemosiderosis (IPH)

Discussion

Pulmonary hemosiderosis is defined as abnormal accumulation of hemosiderin in alveolar macrophages due to repeated episodes of intra-alveolar bleeding, which leads eventually to the development of pulmonary fibrosis and severe anemia. Pulmonary hemosiderosis can be a primary lung disease or secondary systemic disease. Primary pulmonary hemosiderosis is more common in the pediatric population. There are three variants of primary pulmonary hemosiderosis (PPH): PPH due to Goodpature syndrome, PPH associated with hypersensitivity to cow’s milk (also known as Heiner syndrome), and idiopathic pulmonary hemosiderosis (IPH). PPH due to Good pasture syndrome is most common in young adult males. Heiner syndrome is most often seen in children 6 months old to 2 years old. IPH is most common in children 1-7 year olds.

IPH is a diagnosis of exclusion, which requires elimination of other causes of pulmonary hemosiderosis. In patients younger than 10 years in the United States, IPH shows an equal distribution between males and females. IPH can have an insidious onset, or can present with more fulminant course.

Radiographs in IPH often demonstrate diffuse, bilateral patchy air space opacities, which are often although not always relatively symmetric. There is a predilection for the lower lobes, often with relative sparing of the periphery. Early hemorrhage or less severe cases tend to have perihilar involvement. Lymphadenopathy and pleural effusions are rare. In severe cases, the chest radiograph can appear similar to edema or pneumonia in distribution. However, the cardiac silhouette will not be enlarged, pleural effusions will be absent, peribronchial cuffing will not be seen, and signs of interstitial edema, e.g. Kerley lines, will be absent. During remission, usually after 2 weeks, a reticular interstitial pattern can develop; and with repeated hemorrhages, the development of fibrosis. CT can be helpful in excluding other causes of pulmonary hemorrhage, such as endobronchial lesions or vascular malformations, as well as further characterizing the extent of disease

The above radiographic findings are helpful, but in isolation, can be non-specific. Often the triad of hemoptysis, iron deficiency anemia, and diffuse pulmonary opacities aids in the diagnosis. Bronchioalveolar lavage or lung biopsy is often helpful, revealing hemosiderin-laden macrophages, which are diagnostic for pulmonary hemosiderosis.

References

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  3. Dearborn DG. Pulmonary hemorrhage in infants and children. Curr Opin Pediatr 1997;9(3):219-224.
  4. Ioachimescu OC, Sieber S, Kotch A. Idiopathic pulmonary haemosiderosis revisited. Eur Respir J 2004;24(1):162-170
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