Background: Idiopathic pulmonary hemosiderosis (IPH) is a rare disorder of unknown etiology which can cause diffuse alveolar hemorrhage. IPH is found primarily in children. In adults, however, it is extremely rare. A systematic review was applied to identify the details of IPH in adults.
Methods: Articles of English or Chinese language published between 2000 and 2015 were included. Data were extracted on the clinical features, examinations, treatments and clinical outcome.
Results: A total of 37 cases of adult-onset IPH were included (13 females and 24 males). IPH combined with coeliac disease was found in five patients, three of whom received gluten-free diet (GFD) only and got full remission. Upon diagnosis, median age was 34 years. The main manifestations were: hemoptysis (n = 30, 81%), dyspnea (n = 23, 64%), anemia (n = 20, 54%). Most patients were treated by corticosteroids initially. The mortality rage was 14% on acute phase.
Conclusion: The adult patients in this study showed some differences from the previously characterized IPH. It is characterized by immunologically mediated, middle-age onset, male dominance, absence of anemia, high mortality on acute phase.
Keywords: Idiopathic pulmonary hemosiderosis; adult-onset; autoimmune; diffuse alveolar hemorrhage.
© 2016 John Wiley & Sons Ltd.