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Idiopathic Pulmonary Hemosiderosis

By

Joyce Lee

, MD, MAS, University of Colorado Denver

Last full review/revision May 2020| Content last modified May 2020
Click here for the Professional Version

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 Diffuse Alveolar Hemorrhage Diffuse alveolar hemorrhage is a syndrome of recurrent or persistent bleeding into the lungs, caused most often by an autoimmune disorder. Common symptoms are difficulty breathing and coughing... read more ). 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 Hemosiderosis Hemosiderosis is a term used for excessive accumulation of iron deposits called hemosiderin in the tissues. (See also Overview of Iron Overload.) The lungs and kidneys are often sites of hemosiderosis... read more ). 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 Autoimmune Disorders An autoimmune disorder is a malfunction of the body's immune system that causes the body to attack its own tissues. What triggers autoimmune disorders is not known. Symptoms vary depending on... read more . Some affected patients also have celiac disease Celiac Disease Celiac disease is a hereditary intolerance to gluten (a protein found in wheat, barley, and rye) that causes characteristic changes in the lining of the small intestine, resulting in malabsorption... read more 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 Failure to Thrive Failure to thrive is a delay in weight gain and physical growth that can lead to delays in development and maturation. Medical disorders and a lack of proper nutrition are causes of failure... read more and iron deficiency anemia Iron Deficiency Anemia Iron deficiency anemia results from low or depleted stores of iron, which is needed to produce red blood cells. Excessive bleeding is the most common cause. People may be weak, short of breath... read more . 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

  • 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 Procedures Done With Bronchoscopy Bronchoscopy is a direct visual examination of the voice box (larynx) and airways through a viewing tube (a bronchoscope). A bronchoscope has a camera at the end that allows a doctor to look... read more Procedures Done With Bronchoscopy . 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

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Test your knowledge

Goodpasture Syndrome
Goodpasture syndrome is an uncommon autoimmune disorder in which bleeding into the lungs and progressive kidney failure occur. People with the syndrome usually have difficulty breathing and cough up blood. Which of the following groups is most likely to developing Goodpasture syndrome?
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