Idiopathic pulmonary hemosiderosis in adults: review of cases reported in the latest 15 years.

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.