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Genetic and Rare Diseases Information Center (GARD)

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Idiopathic pulmonary hemosiderosis

Other Names for this Disease
  • Alveolar hypoventilation syndrome
  • Hemosiderosis, pulmonary, with deficiency of gamma-a globulin
  • Pulmonary hemosiderosis
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Your Question

What is the prognosis for individuals affected by idiopathic pulmonary hemosiderosis? Will a cure become available? Can the disease become terminal?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

How might idiopathic pulmonary hemosiderosis be treated?

The treatment of idiopathic pulmonary hemosiderosis is aimed at managing acute crises and providing long-term therapy. Acute therapies may include oxygen supplementation, blood transfusion, supportive respiratory therapy, mechanical ventilatory support, extracorporeal membrane oxygenation and immunosuppressive therapy. The use of immunosuppressive therapy for long-term management remains controversial.[1]

More detailed information about the treatment of idiopathic pulmonary hemosiderosis can be accessed through eMedicine. Click on the following links to learn more about Treatment & Management and Medications.

Last updated: 7/6/2011

Can idiopathic pulmonary hemosiderosis be cured?

There is no cure for idiopathic pulmonary hemosiderosis at this time.
Last updated: 7/6/2011

What is the prognosis for idiopathic pulmonary hemosiderosis?

The clinical course of idiopathic pulmonary hemosiderosis varies widely. Overall, the prognosis is poor, with a mean survival of 2.5 to 5 years following diagnosis. Death may result from acute massive hemorrhage or from progressive pulmonary insufficiency and right heart failure. Treatment does not appear to greatly improve survival. One study noted the following:[1]

  • The severity of the disease at onset did not correlate with the outcome,
  • Females survive longer than males,
  • Younger individuals exhibit a less favorable prognosis, and
  • Common therapies did not affect outcome.

Another study of children with idiopathic pulmonary hemosiderosis found that the presence of antineutrophil cytoplasmic antibodies or other autoantibodies translated to a poor prognosis.[1]

Last updated: 7/6/2011

Can idiopathic pulmonary hemosiderosis become terminal?

Yes. This condition may become terminal in some cases.[1]
Last updated: 7/6/2011