Objective: To describe the clinical, radiological and pathological characteristics of idiopathic 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.