Idiopathic pulmonary hemosiderosis in adults:report of two cases and literatu rereview / 北京大学学报(医学版)

ABSTRACT

Objective:To describe the clinical,radiological and pathological characteristics of idiopa-thic pulmonary haemosiderosis(IPH) in adults and to evaluate the methods of diagnosis and treatment.Methods:Two patients were successfully diagnosed and treated in our hospital and the literature on the subject was reviewed.Results:Two adult patients(19 and 34 years old) diagnosed in our hospital had 5 and 10 years of history of hemoptysis respectively,and chest CT showed bilateral diffuse alveolar opacities over mid and lower zones.Tests of antinuclear antibodies(ANAs),rheumatoid factor(RF),antineutrophilic cytopasmic antibodies(ANCA) and Anti-glomerular basement membrane(anti-GBM) antibody were negative.Haemosiderin-laden macrophages were found in bronchoalveolar lavage fluid(BALF) whose color was yellow.Microscopic examination of the lung tissue specimens obtained by transbronchial lung biopsy(TBLB) revealed hemorrhage and numerous hemosiderin-laden macrophages in the alveoli and no vasculitis or capillaritis were seen.These findings were consistent with a diagnosis of IPH.Steroid therapy had good effects.Conclusion:IPH is a diagnosis of exclusion of other causes of diffuse alveolar hemorrhage(DAH).IPH adults have relatively good drug responses and relatively good prognoses.