ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferences
Idiopathic pulmonary hemosiderosis
Print
Other Resources UpToDate PubMed

Idiopathic pulmonary hemosiderosis

Print Images (1)
Contributors: Casey Silver BA, Mary Anne Morgan MD, Michael W. Winter MD
Other Resources UpToDate PubMed

Synopsis

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease characterized by recurrent episodes of diffuse alveolar hemorrhage, or bleeding into the lungs. Patients typically present with shortness of breath, fever, cough, and hemoptysis. Chronic illness often results in malnutrition and failure to thrive, which is particularly pronounced in the pediatric patient population. Malaise from chronic blood loss anemia and iron deficiency can also be a hallmark symptom of IPH.

The etiology is poorly understood, but it is thought to result from immune dysregulation. IPH is very rare with an unknown prevalence. Most cases are described in children (80%) or young adults; however, patients of all ages have been diagnosed with IPH. Increased incidence among related individuals is suggestive of either environmental or genetic risk factors. To date, no known environmental or inheritable factor has been identified.

Although the body is able to reabsorb most of the blood from the lungs, a large amount of iron is often left behind. Over time, this iron can lead to pulmonary fibrosis and chronic lung disease. Severe anemia can also occur.

Codes

ICD10CM:
J84.03 – Idiopathic pulmonary hemosiderosis

SNOMEDCT:
40527005 – Idiopathic Pulmonary Hemosiderosis

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Other causes of diffuse alveolar hemorrhage include:

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Reviewed: 05/07/2019
Last Updated: 05/17/2019
Copyright © 2019 VisualDx®. All rights reserved.
Idiopathic pulmonary hemosiderosis
Print 1 Images
Idiopathic pulmonary hemosiderosis : Cough, Fever, Iron deficiency anemia, Dyspnea, Hemoptysis
Copyright © 2019 VisualDx®. All rights reserved.