Idiopathic pulmonary haemosiderosis

A rare lung disease characterized by recurrent bleeding into the lungs

Idiopathic pulmonary hemosiderosis
Synonyms	IPH
Specialty	Pulmonology (Respirology), Pediatrics
Symptoms	Chronic cough, hemoptysis (coughing up blood), dyspnea (shortness of breath), iron-deficiency anemia
Complications	Pulmonary fibrosis, chronic respiratory failure, severe anemia
Usual onset	Typically in childhood, but can occur at any age
Duration	Chronic, relapsing-remitting course
Types	Idiopathic (primary), or secondary to other autoimmune or systemic diseases
Causes	Unknown (idiopathic); may involve immune-mediated mechanisms
Risk factors	Possible association with autoimmune disorders, but no known definitive risk factors
Diagnosis	Chest X-ray, high-resolution CT scan, bronchoalveolar lavage, lung biopsy
Differential diagnosis	Goodpasture syndrome, vasculitis, systemic lupus erythematosus, hemosiderosis due to left heart failure
Prevention	None known
Treatment	Immunosuppressive therapy (e.g., corticosteroids), oxygen therapy, supportive care
Medication	Prednisone, other immunosuppressants (e.g., azathioprine, hydroxychloroquine)
Prognosis	Variable; some patients have long-term remission, others may progress to lung fibrosis or respiratory failure
Frequency	Very rare
Deaths	Can occur in severe or untreated cases due to respiratory failure or severe anemia

Idiopathic pulmonary haemosiderosis (IPH) is a rare lung disease characterized by recurrent episodes of bleeding into the alveoli, the tiny air sacs in the lungs. This condition leads to the accumulation of haemosiderin, an iron-storage complex, within the lung tissue, causing pulmonary fibrosis and impaired lung function over time.

Pathophysiology

The exact cause of idiopathic pulmonary haemosiderosis is unknown, which is why it is termed "idiopathic." The disease is characterized by the deposition of haemosiderin-laden macrophages in the alveoli. This occurs due to repeated episodes of alveolar hemorrhage, where blood leaks into the alveolar spaces. Over time, the iron from the blood is stored as haemosiderin, leading to tissue damage and fibrosis.

Clinical Presentation

Patients with idiopathic pulmonary haemosiderosis often present with symptoms such as:

• Hemoptysis (coughing up blood)
• Dyspnea (shortness of breath)
• Cough
• Fatigue
• Anemia due to chronic blood loss

In children, the disease may present with more severe symptoms and can be associated with failure to thrive.

Diagnosis

The diagnosis of idiopathic pulmonary haemosiderosis is challenging and often involves a combination of clinical evaluation, imaging studies, and laboratory tests. Key diagnostic tools include:

• Chest X-ray and CT scan: These imaging studies may show diffuse pulmonary infiltrates.
• Bronchoalveolar lavage: This procedure can reveal haemosiderin-laden macrophages in the alveolar fluid.
• Lung biopsy: A biopsy may be necessary to confirm the presence of haemosiderin deposits and to rule out other causes of pulmonary hemorrhage.

Treatment

The management of idiopathic pulmonary haemosiderosis primarily involves the use of corticosteroids to reduce inflammation and prevent further bleeding. In some cases, additional immunosuppressive agents such as azathioprine or cyclophosphamide may be used. Supportive care, including oxygen therapy and treatment of anemia, is also important.

Prognosis

The prognosis of idiopathic pulmonary haemosiderosis varies. Some patients experience a chronic course with recurrent episodes of bleeding, while others may have periods of remission. Long-term complications can include pulmonary fibrosis and respiratory failure.

Related pages

• Pulmonary hemorrhage
• Pulmonary fibrosis
• Anemia
• Corticosteroids

External links

• Hemosiderosis at eMedicine