Idiopathic pulmonary hemosiderosis is a rare disease that causes recurrent diffuse alveolar hemorrhage Diffuse Alveolar Hemorrhage Diffuse alveolar hemorrhage is persistent or recurrent pulmonary hemorrhage that originates from the lung parenchyma (ie, the alveoli) as opposed to the airways. There are numerous causes, but... read more with no detectable underlying disorder; it occurs mainly in children < 10 years. In patients with idiopathic pulmonary hemosiderosis, repeated alveolar bleeding can eventually result in pulmonary hemosiderosis and fibrosis.
The disease is thought to be due to a defect in the alveolar capillary endothelium, possibly due to autoimmune injury. Many affected patients have celiac disease Celiac Disease Celiac disease is an immunologically mediated disease in genetically susceptible people caused by intolerance to gluten, resulting in mucosal inflammation and villous atrophy, which causes malabsorption... read more .
Symptoms and Signs of Idiopathic Pulmonary Hemosiderosis
Symptoms and signs of idiopathic pulmonary hemosiderosis in children include recurrent episodes of dyspnea Dyspnea Dyspnea is unpleasant or uncomfortable breathing. It is experienced and described differently by patients depending on the cause. Although dyspnea is a relatively common problem, the pathophysiology... read more and cough Cough in Children Cough is a reflex that helps clear the airways of secretions, protects the airway from foreign body aspiration, and can be the manifesting symptom of a disease. Cough is one of the most common... read more , particularly nonproductive cough initially. Hemoptysis occurs later. Children with idiopathic pulmonary hemosiderosis may present with only failure to thrive Failure to Thrive (FTT) in Children Failure to thrive in children is weight consistently below the 3rd to 5th percentile for age and sex, progressive decrease in weight to below the 3rd to 5th percentile, or a decrease in 2 major... read more and iron deficiency anemia Iron Deficiency Anemia Iron deficiency is the most common cause of anemia and usually results from blood loss; malabsorption, such as occurs in celiac disease, is a much less common cause. Symptoms are usually nonspecific... read more . The most common symptoms in adults are exertional dyspnea and fatigue due to pulmonary hemorrhage and iron deficiency anemia.
Diagnosis of Idiopathic Pulmonary Hemosiderosis
Diagnosis of idiopathic pulmonary hemosiderosis involves demonstration of a combination of characteristic clinical findings, iron deficiency anemia, and hemosiderin-laden macrophages in bronchoalveolar lavage (BAL) fluid or lung biopsy specimens plus no evidence of small-vessel vasculitis (pulmonary capillaritis) or another explanatory diagnosis.
Diagnosis is confirmed by lung biopsy if other findings are inconclusive.
Treatment of Idiopathic Pulmonary Hemosiderosis
Corticosteroids may reduce the morbidity and mortality of acute episodes of alveolar bleeding and may control the disease progression of pulmonary fibrosis. Some patients may require additional immunosuppressants.
Patients with celiac disease should be on a gluten-free diet.