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.