Idiopathic pulmonary hemosiderosis
Other Names for this Disease
- Alveolar hypoventilation syndrome
- Hemosiderosis, pulmonary, with deficiency of gamma-a globulin
- Pulmonary hemosiderosis
Your QuestionWhat is the prognosis for individuals affected by idiopathic pulmonary hemosiderosis? Will a cure become available? Can the disease become terminal?
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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.
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:
- 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.
- Napchan GD, Talmaciu I. Hemosiderosis. eMedicine. 2009; http://emedicine.medscape.com/article/1002002-overview. Accessed 7/6/2011.