Hemosiderosis: Causes, Symptoms, and Treatment
Medically reviewed by Harsha Moole, MD, Internal Medicine. Last reviewed: October 23, 2026.
What kind of doctor treats Hemosiderosis?
Hemosiderosis that affects the lungs is usually cared for by a pulmonologist (a lung doctor). A hematologist (a blood doctor) may help with anemia, and a rheumatologist (a doctor for immune system diseases) may join the care team. A direct primary care or concierge doctor can be a first stop for an evaluation and can refer you to a specialist when needed.
What is hemosiderosis?
Hemosiderosis means that hemosiderin has built up in the body. Hemosiderin is a storage form of iron. The information below focuses on one type, idiopathic pulmonary hemosiderosis (IPH). "Idiopathic" means the cause is not yet known. "Pulmonary" means it involves the lungs.
IPH is a rare lung disease. It mostly affects children under 10 years old. People with IPH have bleeding into the lungs. This causes repeated episodes of shortness of breath and coughing, often with blood.
Other conditions can also cause too much iron to build up in the body. One example is hemochromatosis. Secondary hemochromatosis is usually the result of something else, such as anemia, thalassemia, liver disease, or blood transfusions. It is a different disease from IPH.
Symptoms
Some people have no symptoms early in the disease. The most common symptoms of IPH include:
- Coughing up blood.
- Shortness of breath.
- A long cough that does not stop.
- Anemia, which comes from bleeding in the lungs and low iron.
- Fatigue.
- Fever.
- Chest pain.
- Poor growth in children, called failure to thrive.
- An enlarged liver and spleen.
Ongoing bleeding often leads to iron deficiency anemia. In children, this causes tiredness and poor growth. The disease can also injure the lungs. This leads to inflammation and, in some people, scarring that does not go away.
Causes and who gets it
The cause of IPH is not known. It is thought to be an autoimmune disease, where the body's defense system harms the body's own tissue. Many patients also have celiac disease. The two together are called Lane-Hamilton syndrome. In most of these patients, a gluten-free diet also improved the IPH symptoms.
IPH has been reported in siblings and children of people with IPH. Patients with Down syndrome may be at higher risk. Secondhand smoke and mold exposure may also play a part. NORD says more research is needed.
About 80% of cases occur in children, mostly under 10 years old. About 20% occur in adults, and most of them are under 30.
How doctors diagnose it
IPH is rare, so doctors must rule out other causes of bleeding or scarring in the lungs. Tests may include blood tests, sputum tests (tests of the mucus you cough up), and imaging such as x-rays and CT scans.
A pulmonologist uses a camera, called a bronchoscope, to look in the lungs. The scope takes samples of fluid from the lungs. This is called bronchoalveolar lavage. The doctor also takes small tissue samples, called biopsies, to confirm the diagnosis under a microscope.
A team often helps with diagnosis. It may include pulmonologists and rheumatologists. Early recognition and treatment can help avoid serious complications.
Treatment
NORD says there is no known cure for IPH. The goal of treatment is to calm the immune response and prevent repeated bleeding in the lungs. There is no gold standard treatment yet. Doctors choose therapies based on their experience and the research. Commonly accepted therapies include:
- Steroids, which may control bleeding in the lungs and later scarring.
- Immunosuppressants, which are medicines that quiet the immune system and help prevent bleeding in the lungs.
- Blood transfusions, which replace blood lost from lung bleeding and may help the symptoms of anemia.
- Extracorporeal membrane oxygenation (ECMO), a temporary life support system during acute severe bleeding or end-stage lung disease.
- Lung transplant, which is a final resort for severe IPH.
Stem cell transplant is an experimental therapy for IPH. If a person has celiac disease, a gluten-free diet may also help.
When to see a doctor
See a doctor if you or your child has a long cough that does not go away, fatigue, or poor growth. These can be signs of IPH or of other lung problems.
Call 911 or go to the emergency room if you or your child is coughing up blood, or has trouble breathing, bluish or grey skin, chest pain lasting two minutes or more, or fainting.
How a direct primary care or concierge doctor fits in
A direct primary care doctor charges a flat monthly fee instead of billing insurance, while a concierge doctor usually charges more and often works alongside insurance. Either type of doctor can start the evaluation and order basic tests. They can also help you keep track of care and refer you to a lung specialist.
Common questions
Can idiopathic pulmonary hemosiderosis be cured?
No, there is no known cure. NORD says treatment aims to calm the immune response and prevent repeated bleeding in the lungs. Therapies include steroids and immunosuppressants.
Does idiopathic pulmonary hemosiderosis only affect children?
No. About 80% of cases occur in children, mostly under 10 years old. About 20% occur in adults.
Is celiac disease linked to hemosiderosis in the lungs?
Yes, in some people. Many patients with IPH also have celiac disease. Together they are called Lane-Hamilton syndrome.
What does idiopathic mean?
It means the cause is not yet known. Doctors do not yet know exactly why IPH starts, although it is thought to be an autoimmune disease.
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Medical Disclaimer
This content is for general educational and informational purposes only and is not medical advice. It is not a substitute for professional medical advice, diagnosis, or treatment, and reading it does not create a doctor-patient relationship. Always seek the advice of your physician or another qualified health provider with any questions about a medical condition, and never disregard or delay seeking professional advice because of something you read here. If you think you may have a medical emergency, call 911 (or your local emergency number) immediately.
