Clinical study of 13 cases of idiopathic pulmonary haemosiderosis

Abstract

Objective To study the clinical features of diopathic pulmonary haemosiderosis. Methods Restrospective review was made on 13 cases with the diagnosis of idiopathic pulmonary haemosiderosis (IPH) in ward. The clinic data of the 13 patients including clinical, physical, laboratory, radiological, pathological and pulmonary function presentations were collected. Results 8 out of 13 cases were childhood-onset(<18 years). Sex distribution appeared to a male predominance. The most important symptom was variable degrees of repetitive haemoptysis (8/13). The signs of CT scan showed from diffused interstitial changes (9/11) to ground-glass opacities (5/11) and micronodular patterns (5/11). The main cellular type of the bronchoalveolar lavage fluid was the alveolar macrophage filled with haemosiderrin (5/5). The pathologic manifestations on light microscopy of lung biopsy were the presence of haemosiderrin-laden macrophages in alveoli (7/8), type two pneumocytes hyperplastic (5/8) and fibroplastic proliferation in interstitium (5/8). Conclusions IPH patients are typically children,often present with haemoptysis. The characteristics of CT scan are diffused interstitial changes,ground-glass opacities and micronodular patterns. The pathologic manifestations are the presence of haemosiderrin-laden macrophages in alveoli, type two pneumocytes hyperplastic and fibroplastic proliferation in interstitium. The exclusion of other diseases is important to the diagnosis. Key words: Idiopathic pulmonary haemosiderosis; Clinical features; Treatment