Hemosiderosis

Alternate Names
idiopathic pulmonary hemosiderosis
IPH
pulmonary hemorrhage

Definition of 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 for no known reason
• 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 signs and symptoms of the condition?

Symptoms include:

• cough, producing blood-tinged sputum
• rapid breathing
• wheezing, or shortness of breath
• airway obstruction
• acute otitis media, or ear infection
• runny nose
• stomach upset, diarrhea, vomiting, and abdominal distress
• failure to thrive, a condition in which an infant or child does not gain weight and grow normally
• cyanosis, a blue tinge in the skin color
• anemia, a low red blood cell count that causes skin pallor, or paleness
• fatigue
• weakness
• lethargy, a feeling of excessive tiredness
• liver disease, such as cirrhosis

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 and is equally common in boys and girls. When it begins in adulthood, it is more likely to affect males.

What can be done to prevent the condition?

Prevention is based on treating the underlying cause, if one can be found.

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
• chest x-ray
• lung scan
• liver scan
• lung biopsy
• pulmonary function tests
• sputum culture
• culture of stool sample

An important part of the diagnosis is to rule out known causes of bleeding into the lungs.

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
• diabetes
• heart disease
• congestive heart failure, a condition in which the heart is not able to pump enough blood to body tissues
• death

What are the risks to others?

Hemosiderosis is not contagious. If the underlying cause is an infection, that infection may be contagious.

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 professional 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.