Idiopathic pulmonary hemosiderosis: a review of the treatments used during the past 30 years and future directions

This paper reviews the literature on the treatment modalities for idiopathic pulmonary hemosiderosis (IPH) used over the past 30 years, attempting to define treatment options that appear to be efficacious and safe, and in addition presents a treatment algorithm. IPH is an uncommon etiology of diffuse alveolar hemorrhage. IPH is a rare disease in adults and often associated with a significant temporal delay in diagnosis. Patients present with hemoptysis, radiographic chest abnormalities, and iron deficiency anemia. Although several pathogenetic hypotheses have been proposed, IPH appears to be an immunologic disease, possibly with a genetic component. Corticosteroid therapy represents the first line of treatment, including liposome-incorporated dexamethasone palmitate (liposteroid). Additional immunomodulatory/immunosuppressive medications have been used with varying success, especially in the setting of steroid-refractory disease. Cyclophosphamide, azathioprine, hydroxychloroquine, mycophenolate mofetil, and mesenchymal cell transplantation have been attempted to improve outcome and reduce side effects. Controlled studies are needed to assess the optimal combination of medications, which are effective to control the disease.