Medication Summary
For isolated pulmonary hemosiderosis (IPH), corticosteroids are believed to be useful in the management of the acute alveolar hemorrhage stage. Failure to respond adequately to corticosteroids alone or unacceptable corticosteroid adverse effects may be indications for using other forms of immunosuppression (eg, azathioprine, chloroquine, cyclophosphamide). Published experience with these medications has been very limited and is confined to case reports.[3] Inhaled corticosteroids also have been used, but current reports are insufficient.
Case reports have described the use of other immunosuppressive medications in the long-term management of idiopathic pulmonary hemosiderosis. One report commented on the use of chloroquine in 3 children with idiopathic pulmonary hemosiderosis, with improvement in the course of their disease. Another report mentioned the efficacy of a combination of azathioprine and corticosteroids in abating acute exacerbations of the disease in a child with idiopathic pulmonary hemosiderosis. The long-term efficacy of immunosuppressive therapy is still in doubt.
Immunosuppressive agents
Class Summary
These agents are used for patients with conditions caused by immune dysregulation and autoimmunity. A lack of knowledge about the pathogenetic mechanisms involved in idiopathic pulmonary hemosiderosis makes the theoretical basis of such therapies unclear.[4]
Prednisone (Deltasone, Orasone)
Immunosuppressant for treatment of autoimmune disorders. May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and suppresses lymphocyte and antibody production.
High doses of prednisone or the equivalent dosage of an IV preparation (eg, methylprednisolone) should be used in the management of acute crisis. For IPH, high-dose corticosteroid usage should be continued for at least 7 d after substantial bleeding has subsided, and the dosage should be tapered over several wk. Some children tolerate complete weaning from corticosteroids in this fashion, but other children demonstrate the need for long-term use.
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