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Idiopathic Pulmonary Hemosiderosis


Joyce Lee

, MD, MAS, University of Colorado School of Medicine

Reviewed/Revised Oct 2023

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 causes damage (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 an autoimmune disorder is not known. Symptoms vary depending... 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 .

An important function of the immune system Overview of the Immune System The immune system is designed to defend the body against foreign or dangerous invaders. Such invaders include Microorganisms (commonly called germs, such as bacteria, viruses, and fungi) Parasites... read more is fighting off infections. To do this, the immune system recognizes microorganisms as foreign to the person and produces proteins (antibodies Antibodies One of the body's lines of defense ( immune system) involves white blood cells (leukocytes) that travel through the bloodstream and into tissues, searching for and attacking microorganisms and... read more Antibodies ) that bind to the microorganisms so they can be removed from the body. In autoimmune disorders 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 an autoimmune disorder is not known. Symptoms vary depending... read more , 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 Failure to Thrive in Children 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 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 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, a thin viewing tube with a light, has a camera at the... 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 of Idiopathic Pulmonary Hemosiderosis

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