Idiopathic pulmonary hemosiderosis is a rare disorder that causes repeated episodes of bleeding into the lungs, leading to accumulation of iron (hemosiderosis) in the lungs.
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 causes damage (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.
An important function of the immune system is fighting off infections. To do this, the immune system recognizes microorganisms as foreign to the person and produces proteins (antibodies) that bind to the microorganisms so they can be removed from the body. In autoimmune disorders, the body mistakenly reacts against a person's own tissues as if they were foreign. In autoimmune disorders that involve the lungs, the immune system attacks and damages lung tissue. Autoimmune disorders that affect the lungs also often affect other organs, particularly the kidneys.
Symptoms of Idiopathic Pulmonary Hemosiderosis
Symptoms of idiopathic pulmonary hemosiderosis in children include repeated episodes of shortness of breath and coughing. 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 of Idiopathic Pulmonary Hemosiderosis
Chest imaging
Blood tests
Bronchoalveolar lavage
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 of Idiopathic Pulmonary Hemosiderosis
Corticosteroids
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 medications to suppress the immune system.
People who also have celiac disease should follow a gluten-free diet.
