What is idiopathic pulmonary haemosiderosis?
In idiopathic pulmonary haemosiderosis (IPH), small blood vessels in the lungs repeatedly leak blood into the alveoli, the tiny air sacs where oxygen is absorbed. The iron from this blood is taken up by scavenger cells called macrophages and stays trapped in the lungs, so the body cannot reuse it. Over time this causes iron-deficiency anaemia, and repeated episodes can lead to lung scarring.
IPH is a form of diffuse alveolar haemorrhage. It is called idiopathic because no cause such as vasculitis, anti-GBM disease, heart disease or a clotting problem is found. It is ultra-rare and is diagnosed most often in children under ten, although adults can also be affected. Children are managed jointly with paediatric pulmonology.
Symptoms
IPH classically causes a triad of features:
- Coughing up blood (haemoptysis): this may be absent, especially in young children who swallow sputum.
- Iron-deficiency anaemia with pallor, tiredness and poor growth, sometimes without any obvious source of blood loss.
- Shadows on the chest X-ray that come and go with each bleed.
During an acute episode there may be cough, breathlessness, fever and low oxygen levels. Between episodes some people feel well, while others have persistent cough and fatigue. Unexplained anaemia that does not fully respond to iron is an important clue.
Causes and associations
The exact cause is unknown. An immune mechanism is suspected because many patients respond to immunosuppression and some later develop autoimmune features. Recognised associations include:
- Coeliac disease: in a subset of patients, and a gluten-free diet may reduce bleeding (sometimes called Lane-Hamilton syndrome).
- Cow's milk protein sensitivity in infants (Heiner syndrome).
- Environmental exposures such as mould in damp homes have been proposed but are not proven.
How IPH is diagnosed
The diagnosis requires proof of bleeding into the lungs and exclusion of every other cause.
| Test | What it shows |
|---|---|
| Full blood count and iron studies | Microcytic iron-deficiency anaemia |
| Chest X-ray and CT | Ground-glass or patchy shadowing during bleeds, and fine fibrosis with repeated episodes |
| Bronchoscopy with bronchoalveolar lavage | Progressively bloodier washings and haemosiderin-laden macrophages, confirming alveolar bleeding, and also excludes infection |
| Lung function tests | A raised gas transfer (DLCO) during fresh bleeding, and restriction if scarring develops |
| Autoantibodies (ANCA, anti-GBM, ANA) | Vasculitis, anti-GBM disease or lupus, which must be excluded |
| Urine tests and kidney function | Kidney involvement that would suggest a pulmonary-renal syndrome |
| Coeliac serology | Associated coeliac disease |
| Echocardiography and clotting tests | Heart or bleeding disorders as alternative causes |
Lung biopsy is sometimes required to exclude small-vessel inflammation (capillaritis). In India, haemoptysis is often first attributed to tuberculosis, so sputum and lavage testing help avoid an incorrect diagnosis. See bronchoscopy for what the procedure involves.
Treatment
Acute bleeding episodes
Systemic corticosteroids are the mainstay of treatment for an acute bleed, alongside oxygen and, where needed, blood transfusion. Severe episodes may require hospital admission and breathing support.
Long-term control
To prevent relapses and limit steroid side effects, steroid-sparing immunosuppressants such as azathioprine, hydroxychloroquine or others are often used for long periods. Inhaled steroids have been used in some patients as maintenance.
Diet and anaemia
Iron replacement corrects anaemia. A strict gluten-free diet is advised where coeliac disease is present, and avoiding cow's milk is considered in infants with suspected milk sensitivity.
Coordinated care
Care is shared with paediatric pulmonology for children, and with haematology, gastroenterology and rheumatology as needed.
Outlook and follow-up
The course ranges from a single episode to repeated bleeding over many years. With early diagnosis and long-term immunosuppression, outcomes have improved considerably compared with older reports. Follow-up includes blood counts, lung function with gas transfer, and imaging. Some patients later develop autoimmune disease, so new symptoms such as joint pain, rash or kidney changes are reported promptly. Patients and families in Hyderabad are seen at Apollo Hospitals, Financial District.
When to seek urgent care
Seek emergency care for coughing up more than a small amount of blood, sudden worsening of breathlessness, bluish lips, severe pallor, dizziness or fainting. In children, fast breathing, poor feeding or unusual drowsiness also need urgent assessment.
Frequently asked questions
Can IPH occur without coughing up blood?
Yes. Many children swallow their sputum, so the first sign may be unexplained iron-deficiency anaemia or tiredness. Lung shadows on X-ray and lavage findings confirm the bleeding.
Is IPH inherited?
IPH is not usually inherited, although a few families with more than one affected member have been described.
Why is a bronchoscopy needed?
Washings from the lungs show blood and iron-laden cells that prove bleeding is occurring in the air sacs, and they also help exclude infection such as tuberculosis.
What is the link with coeliac disease?
Some patients with IPH also have coeliac disease. In these patients a strict gluten-free diet can reduce or stop the lung bleeding.
How long does treatment continue?
Steroids are used for acute bleeds, and steroid-sparing medicines are often continued for years to prevent relapse. Treatment is reviewed regularly and reduced gradually when the disease is quiet.
Can children with IPH lead normal lives?
Many children do well with treatment and attend school and play normally, with regular follow-up shared between the adult and paediatric lung teams.
Related conditions and procedures
Consult Dr. Gogineni Ratnakara Rao
Apollo Hospitals, Financial District: Monday to Saturday, 8:30 AM to 5:00 PM. Anything in Lung Clinic, Chanda Nagar: Monday to Saturday (Thursday closed), 6:00 PM to 9:00 PM.