Introduction
Idiopathic pulmonary hemosiderosis (IPH) is one of the uncommon reasons for diffuse alveolar hemorrhage, with an estimated incidence of 0.24-1.23 per million.1-3 The definition of IPH is used in cases where the underlying cause of the pulmonary bleeding cannot be identified with extensive investigations.3,4The disease is manifested by pulmonary hemorrhage and characterized by the triad of iron deficiency anemia, hemoptysis, and pulmonary infiltrates on chest imaging 5-9. Although the pathophysiology of the disease unknown, different hypotheses have been proposed regarding the underlying mechanism of the disorder such as environmental, auto-immune, allergic, genetic.10,11The course of the disease is highly variable from single to recurrent exacerbations. Some patients recover fully, whereas others develop pulmonary fibrosis and respiratory insufficiency.12-14
Currently, there is a lack of consensus regarding the management of IPH.12,15 There is limited information about factors that can help in predicting patients with recurrent exacerbations at the time of diagnosis. There is also no consensus regarding the definition of pulmonary exacerbations for children with IPH.
The purpose of this study was to investigate the clinical characteristics and prognosis of patients with IPH, and to evaluate the incidence and risk factors of recurrent pulmonary exacerbations. Also, we tried to investigate factors that contributed to treatment responses.