Idiopathic Pulmonary Hemosiderosis: Diagnostic and Therapeutic Challenge

Background: Idiopathic pulmonary hemosiderosis is a rare cause of alveolar hemorrhage characterized by iron deficiency anemia, hemoptysis and pulmonary infiltrates. The diagnosis implicates the exclusion of other disorders causing diffuse alveolar hemorrhage. Treatment is not well established and consists on glucocorticoids and other immunosuppressant agents.

Case Report: Nine-year-old child with type 1 diabetes mellitus diagnosed by the age of three and idiopathic pulmonary hemosiderosis diagnosed at five. At diagnosis, he presented with severe anemia (Hb 4.9 g/dL) and after exclusion of infectious, gastrointestinal and hemolytic causes, the study revealed diffuse pulmonary infiltrates. Bronchoalveolar lavage and lung biopsy revealed hemosiderin-laden macrophages. Other causes of pulmonary hemosiderosis were excluded. Autoimmune study was negative. Initially treated with intravenous corticosteroids (methylprednisolone, monthly pulses), prednisolone and hydroxychloroquine. After three months, a pulmonary computerized scan revealed no evidence of pulmonary hemorrhage. He completed six methylprednisolone pulses, eight months of prednisolone and 13 months of hydroxychloroquine. Two months later, he had a relapse of pulmonary hemorrhage and reinitiated corticosteroids and hydroxychloroquine. He repeated methylprednisolone pulses, maintained prednisolone for 20 months and maintained hydroxychloroquine until nowadays. Eight months after suspending prednisolone, and while on treatment with hydroxychloroquine, he had a new relapse of pulmonary hemorrhage, reinitiating prednisolone. While on corticosteroids, glycemic control required insulin adjustment.

Conclusions: The treatment of this condition on a child with type 1 diabetes mellitus is challenging, since glycemic control is very difficult and demands high insulin doses. The coexistence of type 1 diabetes mellitus rises questions on the idiopathic etiology, since autoimmunity is one of the etiologies proposed for this disorder. During follow-up, we understood that the suspension of corticosteroids, even maintaining hydroxychloroquine, lead to the relapse of pulmonary hemorrhage. It is important to manage the balance between pulmonary hemorrhage control and glycemic control in order to minimize the sequels from both disorders.