Intravenous monthly pulse methylprednisolone treatment for ABPA in patients with cystic fibrosis

Journal of Cystic FibrosisVolume 8, Issue 4, July 2009, Pages 253-257


Intravenous monthly pulse methylprednisolone treatment for ABPA in patients with cystic fibrosis


Malena Cohen-Cymberknoha, Hannah Blaub, David Shoseyova, Meir Mei-Zahavb, Ori Efratic, Shoshana Armonia and Eitan Kerema, ,
aCF Centers of Hadassah Medical Center, Mount Scopus, Jerusalem, Israel
bSchneider Children's Medical Center, Petah Tikvah, Israel
cSheba Medical Center, Tel-Hashomer, Israel
Received 3 February 2009;
revised 7 April 2009;
accepted 16 April 2009.
Available online 15 May 2009.

Abstract

Background

Allergic bronchopulmonary aspergillosis (ABPA) in patients with CF is associated with frequent exacerbations and deterioration of lung function. Oral corticosteroids are standard therapy for ABPA and are associated with severe side effects. Monthly pulses of high-dose intravenous methylprednisolone (HDIVPM) are an effective therapy for autoimmune diseases with fewer side effects compared to oral prednisone, implicating its use for patients with CF who suffer from ABPA.

Methods
9 patients with CF and ABPA (4 male, 5 female, ages 7–36 years) received HDIVPM (10–15 mg/kg/d), for 3 days per month, and itraconazole, until clinical and laboratory resolution of ABPA.

Results

All patients showed clinical and laboratory improvement (FEV1 increase, serum IgE levels and total eosinophil counts decrease) and treatment was discontinued after 6–10 pulses. Adverse effects were minor and disappeared shortly after each IV pulse therapy.

Conclusion
High-dose IV-pulse methylprednisolone is an effective treatment for ABPA in CF with minor side effects.