Idiopathic Pulmonary Hemosiderosis – Hemosiderosis
Hemosiderosis is a rare, often fatal, condition in which iron builds up in the lungs. The iron is in the form of hemosiderin, a pigment in blood. Hemosiderosis results from bleeding into the lungs, also known as pulmonary hemorrhage.
Pulmonary hemosiderosis is often broken down into four categories:
idiopathic, which means it occurs by itself
occurs along with pancreatic or heart disease
occurs along with a milk sensitivity
occurs along with a kidney disease called glomerulonephritis
What is going on in the body?
This condition usually shows up between the ages of 6 months and 20 years. Blood that leaks out of the capillaries is taken up by scavenger cells in the lung. The breakdown products of the blood irritate the lung and lead to scarring.
What are the causes and risks of the condition?
Hemosiderosis is often linked with anemia and chronic infections. It may also be caused by problems with the heart or the immune system.
Pulmonary hemosiderosis is more common in children under 10 years old. It is equally common in boys and girls. When it begins in adulthood, it is more likely to affect males.
Symptoms & Signs
What are the signs and symptoms of the condition?
cough, producing blood-tinged sputum
wheezing, or shortness of breath
acute otitis media, or ear infection
stomach upset, diarrhea, vomiting, and abdominal distress
failure to thrive, a condition in which the child does not gain weight and grow normally
cyanosis, or a blue tinge in the skin color
anemia, a low red blood cell count that causes skin pallor, or paleness
liver disease, such as cirrhosis
Diagnosis & Tests
How is the condition diagnosed?
Diagnosis begins with a medical history and physical exam. Diagnostic tests may include:
blood tests, such as a complete blood count, or CBC, and liver function tests
pulmonary function tests
culture of stool sample
The diagnosis is often made by ruling out other causes of bleeding into the lungs.
Prevention & Expectations
What can be done to prevent the condition?
Prevention is based on treating the underlying cause. If another condition causes bleeding into the lungs, hemosiderosis can develop.
What are the long-term effects of the condition?
If hemosiderosis is not effectively treated, long-term effects from the bleeding and kidney damage can include:
glomerulonephritis, or progressive destruction of the filtering system of the kidneys
cirrhosis of the liver, a condition of continued scarring and decreased liver function
congestive heart failure, a condition in which the heart is not able to pump enough blood to body tissues
What are the risks to others?
Hemosiderosis is not contagious. If the underlying cause is an infection, that infection may be contagious.
Treatment & Monitoring
What are the treatments for the condition?
The treatment depends on the cause. Blood transfusions may be needed for blood loss. Supplemental oxygen is given for low levels of oxygen in the blood. Corticosteroids and medications that suppress the immune system are often used. If the cause is sensitivity to cow’s milk, a milk-free diet may be recommended.
What are the side effects of the treatments?
Side effects depend on the treatment used. Side effects of steroids may include stomach upset, jittery feelings, muscle weakness, bloating, and weight gain.
What happens after treatment for the condition?
Pulmonary bleeding can recur from time to time and will need to be evaluated.
How is the condition monitored?
This condition is monitored for the rest of the person’s life. Any further bleeding in the lungs must be evaluated. The healthcare provider should be told about any new or worsening symptoms. Blood tests, pulmonary function tests, and chest x-rays may also help monitor the course of the disease.