Eosinophilic Granuloma (Histiocytosis X) Medication

Updated: Mar 29, 2018
  • Author: Eleanor M Summerhill, MD, FACP, FCCP; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Medication

Medication Summary

The mainstays of treatment for pulmonary Langerhans cell histiocytosis X (PLCH) are smoking cessation and supportive therapy. The use of corticosteroids in the treatment of PLCH is controversial. Their efficacy has not been proven in well-designed, prospective, randomized, controlled trials. Some experts recommend a trial of corticosteroids for those patients with persistent symptomatic disease or evidence of progression.

Patients with an obstructive ventilatory defect on pulmonary function testing may benefit from bronchodilator therapy.

Supplemental oxygen therapy is indicated for all patients with either resting or exertional hypoxemia. Oxygen therapy may help to prevent or slow progression of pulmonary hypertension and cor pulmonale, and it provides a mortality benefit in chronic obstructive pulmonary disease.

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Corticosteroids

Class Summary

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.

Prednisone (Deltasone, Meticorten, Orasone, Sterapred)

Prednisone is used as an immunosuppressant to treat autoimmune disorders. By reversing increased capillary permeability and suppressing the activity of polymorphonuclear cells, it may decrease inflammation. Prednisone is an oral corticosteroid with relatively limited mineralocorticoid activity. It is best prescribed in consultation with a pulmonary disease specialist.

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