Idiopathic pulmonary hemosiderosis is a rare disorder that involves episodes of bleeding into the lungs (diffuse alveolar hemorrhage). The red blood cells that have leaked into the lungs break down and release iron, which accumulates in lungs and damages them (see also Hemosiderosis). Idiopathic means there is no detectable underlying disorder. It occurs mainly in children younger than 10 years but can also affect young to middle-aged adults. It is thought to be due to an underlying problem in the lung cells responsible for the exchange of oxygen and carbon dioxide, possibly due to an autoimmune disorder. Some affected patients also have celiac disease.
Symptoms
Symptoms of idiopathic pulmonary hemosiderosis in children include repeated episodes of shortness of breath and cough. Cough is usually dry initially. Later, the person may cough up blood. Children with idiopathic pulmonary hemosiderosis may instead have failure to thrive and iron deficiency anemia. Children with failure to thrive are extremely underweight. Iron deficiency anemia causes fatigue, paleness, and weakness.
The most common symptoms in adults are shortness of breath during activity and the symptoms of iron deficiency anemia.
Diagnosis
In people who have symptoms, doctors do blood tests to detect anemia.
Diagnosis of idiopathic pulmonary hemosiderosis is confirmed by bronchoalveolar lavage. In this procedure, doctors insert a bronchoscope into the small airways of the lungs and then instill salt water through the instrument. The doctor then suctions the water out and sends it for examination in the laboratory. If examination shows evidence of excess iron and no other disorder explains this finding, the diagnosis is confirmed.
If the diagnosis is still uncertain, doctors may do a biopsy of the lungs to diagnose idiopathic pulmonary hemosiderosis.
Treatment
During an episode of bleeding, the person may need to be hospitalized and given oxygen or aid in breathing. A person with severe bleeding may need a blood transfusion.
People are given corticosteroids or other drugs to suppress the immune system.
People who also have celiac disease should follow a gluten-free diet.
