Guidelines |
A. Significant new airflow obstruction with a % predicted FEV1 ≥70% - Initiate inhaled glucocorticoid therapy. Add inhaled long-acting beta agonist, if patient symptomatic. Treatment should continue until either FEV1 becomes <70% (see B below) or until GVHD resolves (ie, resolution of all reversible manifestations of GVHD without exacerbation for at least 6 months after discontinuation of all systemic immunosuppressive treatment)
- Other immunosuppressive treatment as indicated to control GVHD in other organs
- Treatment should continue until either FEV1 becomes <70% (see B below), or until GVHD resolves (ie, resolution of all reversible manifestation of GVHD without exacerbation for at least 12 months after discontinuation of all systemic treatments)
- Monitor PFTs or spirometry monthly for at least 3 months
- If FEV1 stabilizes, obtain PFTs or spirometry every 3 months for 1 year, then continue at 6 month intervals for 1 year and at 6 to 12 month intervals thereafter
- If FEV1 continues to decrease, go to B below
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B. Significant airflow obstruction with a FEV1 <70% with/without significant airtrapping by high resolution chest CT - Consider bronchoscopy to rule out an undetected infectious etiology for airflow obstruction, even if no radiographic opacity is apparent
- After infection has been ruled out, evaluate the patient's eligibility for clinical trial for treatment of BOS and initiate (or increase) prednisone dose to 1 mg/kg per day
- Start standard chronic GVHD taper at 2 weeks
- Consider continuing inhaled glucocorticoids throughout prednisone therapy
- If FEV1 decreases further to <70% during treatment, discuss changes of immunosuppressive treatment with transplant physician
- CMV monitoring in blood per standard practice
- Monitor PFTs or spirometry monthly for at least 3 months
- If FEV1 stabilizes, continue PFTs or spirometry every 3 months for 1 year
- If FEV1 continues to decrease, go to C below
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C. Glucocorticoid-resistant airflow obstruction defined as progressive decline of FEV1 by ≥10% despite treatment with 1 mg/kg per day of prednisone (or similar glucocorticoids) - May consider increasing the dose of prednisone to 2 mg/kg per day for a maximum of 2 weeks, followed by a taper to reach a dose of 1 mg/kg per day by 2 to 4 weeks
- Another treatment must be considered and discussed with the transplant team
- Monitor CMV in blood per standard practice
- Monitor PFTs monthly for at least 3 months
- If FEV1 stabilizes, monitor PFTs every 3 months for 1 year
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D. Additional considerations - Consider changing prophylaxis for encapsulated bacterial infection to azithromycin 250 mg on Mondays-Wednesdays-Fridays
- Assure patient is receiving adequate prophylaxis for Pneumocystis, varicella virus, and herpes simplex virus infections
- Fungal prophylaxis per standard practice
- Monitor CMV in blood per standard practice
- May continue inhaled glucocorticoids throughout prednisone therapy
- Discontinuation of inhaled glucocorticoid treatment can be considered 12 months after treatment with prednisone has been discontinued
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