Idiopathic pulmonary hemosiderosis (IPH) is a disease of unknown etiology that primarily affects children and adolescents. It is characterized clinically by recurrent episodes of diffuse alveolar hemorrhage, leading to hemoptysis, dyspnea, and iron deficiency anemia. On chest X-rays, the typical findings include diffuse, ill-defined ground-glass opacities predominantly in the middle and lower lung fields.
The initial diagnosis is primarily based on clinical symptoms and imaging studies. Bronchoalveolar lavage (BAL) is routinely performed to confirm the presence of alveolar hemorrhage and to detect occult hemorrhage. The presence of free red blood cells or alveolar macrophages containing phagocytosed red blood cells in BAL fluid (BALF) suggests recent alveolar hemorrhage, while numerous hemosiderin-laden macrophages indicate chronic alveolar hemorrhage. Additionally, circulating autoantibodies (e.g., anti-GBM, ANCA, ANA) should be routinely tested to rule out other causes of diffuse alveolar hemorrhage.
In general, the clinical course of IPH is relatively mild, especially in adults, with spontaneous remission occurring in approximately 25% of cases. However, diffuse alveolar hemorrhage can be life-threatening. Treatment is primarily supportive. Glucocorticoid therapy is beneficial in improving outcomes during acute exacerbations and preventing recurrent hemorrhage, although definitive criteria for assessing therapeutic efficacy are not yet established.