| Objective:To analyze the characteristic of a cases of adult idiopathic pulmonary hemosiderosis combining with pulmonary hypertension, in order to improve clinicians awareness of the disease and reduce misdiagnosis, causing clinical attention.Method:A case of adult idiopathic pulmonary hemosiderosis (IPH) combining with pulmonary hypertension diagnosed in the respiratory medicine department of the First Affiliated Hospital of Guangxi Medical University in 2014 was reported. We retrospectively analyzed the case related clinical symptoms, laboratory examination, imaging manifestations, bronchial alveolar irrigation lavage fluid (BALF), transbronchial lung biopsy (TBLB), diagnosis and treatment, and summarized the literature at domestic and overseas in order to explore the clinical features and prognosis of the disease.Results:(1) 32-year-old female, she did not have the previous history of hemoptysis and anemia, and has no history of drug and food (such as milk) allergy, and has no history of parasitic contacting. She has not a history of miners or heart disease and denied the long-term history of dust exposure. The family members had no history of similar disease. She mainly complained for cough, expectoration, fever, shortness of breath and slight haemoptysis. Three mouths ago, the patient experienced sudden dyspnea, syncope and she was admitted to the ICU on local hospital. The blood clotting function and blood platelet were normal. The urine routines was normal.There were no renal damage, such as no change of blood creatinine and urea nitrogen. Tests of ASO, RF, ANA, ds-DNA, ANCA, anti-GBM were negative. The chest CT revealed diffuse ground glass opacity in both lungs.The BALF found numerous hemosiderin-laden macrophages. Echocardiography revealed no change of heart organic disease, but the pulmonary artery pressure is moderate and severe increasing. The TBLB revealed numerous hemosiderin-laden macrophages in the alveoli and had no phenomenon of vasculitis. Glucocorticoid therapy had good effects.The disease had no progress in clinical observation for one and a half years. (2) Results of literature review:35 cases of IPH were reported from PubMed since 1957, among 18 cases were reported in the last 10 years; 36 cases of IPH were reported in domestic journals since 1990. In these cases, men were more than women, mean age of onset was older than 30 years. The main clinical manifestations were hemoptysis, dyspnea, fatigue, fever, cough and chest tightness. Laboratory examination revealed anemia, autoimmune antibody negative. Imaging manifestations revealed grinding glass, real change shadow, pneumothorax, lymph node enlargement, pleural effusion, pulmonary interstitial fibrosis, pulmonary artery expansion, right heart shadow increased. Before diagnosis those patients had been misdiagnosed pulmonary miliary tuberculosis, silicosis, interstitial lung disease, exogenous allergic alveolitis, or pulmonary carcinoma disease. Time from the onset to the final diagnosis was 0-2 years, and the average time was 7 months. Corticosteroid therapy was performed in all cases.5 cases were experienced mechanical ventilation due to respiratory failure,finally 2 cases was dead. After discharge, the patients were followed up for 1 months to 6 years, and all of them were alive. (3) In 71 cases of adult IPH, only 6 cases were examined by echocardiography,3 cases of pulmonary hypertension were found by chest imaging,3 cases had respiratory failure,1 cases were improved after treatment, and 2 cases died.Conclusion:(1)Children IPH is more common, but adult IPH is rare. The cause of the disease is not yet fully understood. (2) Different from the children, the clinical manifestations of adult IPH patients are not typical. Onset of adult IPH is dormant and invasive. The response to treatment and prognosis in adult may be better than those in children. Natural course and prognosis are very different. (3)Diagnosis depend on ruling out other diseases. HRCT is necessary. The diagnosis depends on a lung biopsy. (4) Adult IPH with pulmonary hypertension may indicate the severity and prognosis of disease, and clinicians should pay more attention to pulmonary hypertension complications. |
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