Combined Therapy for IPF Patients (1)
Not approved by the FDA for the treatment of IPF.
Corticosteroid (prednisone or equivalent)
- 0.5 mg/kg LBW*/d orally for 4 wk
- 0.25 mg/kg/d for 8 wk
- Taper to 0.125 mg/kg/d or 0.25 mg/kg on alternate days
- Possible side effects include peptic ulcer disease, posterior capsular cataracts, increased intraocular pressure, hypertension, endocrine and metabolic alterations, musculoskeletal complications, and psychological effects
PLUS
Azathioprine
- 2-3 mg/kg LBW/d orally (up to 150 mg/d)
- Dosing should begin at 25-50 mg/d, increasing by 25 mg increments every 1-2 wk until the maximum dose is achieved
- Possible side effects include hepatotoxicity and hematologic abnormalities
OR
Cyclophosphamide
- 2 mg/kg LBW/d orally (up to 150 mg/d)
- Dosing should begin at 25-50 mg/d, increasing by 25 mg increments every 1-2 wk until the maximum dose is achieved
- Possible side effects include bladder injury, oncogenic potential, and hematologic abnormalities
Therapy should be continued for a minimum of 6 months. Response to treatment should be determined by symptoms and radiologic and physiologic findings. Close monitoring for adverse effects is mandatory. Because of the risk of hepatotoxicity from azathioprine and neutropenia from cyclophosphamide, complete cell counts and liver enzyme levels should be followed bimonthly for the first 2 months, then monthly. Pneumocystis carinii pneumonia (PCP) prophylaxis with BactrimŽ DS (one tablet three times per week) is advised.
*LBW = lean body weight
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