Idiopathic pulmomary haemosiderosis (IPH) is a rare disease of unknown etiology with intermittent intrapulmonary haemorrhage. More than 200 cases have been reported internationally, and more than 120 cases have been reported domestically (up to 1994). After the destruction of erythrocytes in the alveoli, the bead protein is absorbed and iron-containing heme is deposited in the lung tissue causing the reaction. Iron deficiency anemia may develop secondary to repeated bleeding. Symptoms Detailed description of symptoms The disease is more common in children, mainly between the ages of 1 and 7 years, with 15% over 15 years of age, and the ratio of men to women in adults is 2:1, with no obvious familial pattern. Symptoms depend on the degree of intrapulmonary hemorrhage. Mild persistent chronic hemorrhage may include dry cough, weakness, pale skin, weight loss and even pestle fingers. During acute hemorrhage, blood in the sputum, hemoptysis, low-grade fever, and chest pain may occur. In the later stages, there may be dyspnea and even cardiac insufficiency. In the acute phase and secondary infection, a distinct wet rhotic sound may be heard. The stool is positive for occult blood. X-rays show multiple fused blotchy shadows with indistinct margins in the middle and lower fields of both lungs, and the shadows resolve well within a few weeks after the pulmonary hemorrhage stops. In cases of persistent moderate hemorrhage, the intrapulmonary lesions may be cornified. The etiology and pathogenesis are not known. It is presumed to be related to several factors: abnormal development of the pulmonary epithelium, the presence of recurrent bleeding due to mechanical instability of the interstitial capillaries; immune dysfunction, e.g., 1/8 patients have intrapulmonary eosinophilic infiltration, and mast cells, increased plasma cells, and some patients have positive condensation set tests. Some patients may have rheumatoid, polyarthritis, myocarditis, and Goodpasture syndrome. Other causes mentioned are animal protein intake and inhalation of toxic substances (e.g. organic pesticides). Pathological changes Increased lung weight, diffuse brown pigmentation on cut surface, alveolar epithelial necrosis and hyperplasia on microscopic examination, local capillary dilation, alveolar and interstitial macrophages phagocytosing iron-containing heme, and diffuse interstitial fibrosis in later stages. Electron microscopy suggested extensive capillary endothelial cell swelling with protein deposits in the intima. After lung tissue washing and drying, the iron content in the tissue is still 5 to 200 times higher than in normal lung. And it was proportional to the disease. Diagnosis The initial diagnosis can be made on the basis of recurrent hemoptysis, blood in the sputum, blotchy shadows with indistinct margins in the lungs, and secondary iron deficiency anemia. The diagnosis can be confirmed by finding blue iron-containing heme in phagocytes in sputum, bronchoalveolar lavage fluid, and lung biopsy and excluding cardiogenic (bruising) factors. Treatment In the acute phase, hormonal therapy can be tried, with prednisone 1 to 2 mg/(kg・d), which is reduced after 2 to 3 weeks and gradually increased to maintenance doses. Symptomatic management includes iron therapy for iron deficiency anemia and antibiotic therapy for secondary infection. The duration of disease varies widely, with 68 cases reported, 20 cases (29%) died within 3 years, 17 cases (25%) were active, 12 cases (18%) were stable, and 19 cases (28%) returned to normal. Safety of medication 1. There is no special treatment for idiopathic pulmonary ferritin-containing hyperplasia. Anemia is effectively treated with iron. Treatment with corticosteroids and immunosuppressants such as cyclophosphamide and azathioprine can temporarily reduce the symptoms in some cases. 2. The main symptoms of idiopathic pulmonary ferritinosis include cough, hemoptysis, shortness of breath, weakness, pallor, and no weight gain or loss. Among them, hemoptysis is prominent, and the amount of hemoptysis varies, from blood in the sputum to blood in the mouth. Although large mouth hemoptysis is rare, it can be fatal. However, there are a few patients without hemoptysis. In the late stage, shortness of breath is aggravated, pestle finger and hepatosplenomegaly are mostly seen, and heart failure symptoms may appear due to pulmonary hypertension. 3. The course of the disease is inconsistent, with survival times ranging from weeks to years. Some cases may go into spontaneous remission.