Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
ORIGINAL ARTICLES
Cyclosporin A in the Treatment of Acute Exacerbation of Idiopathic Pulmonary Fibrosis
Susumu SakamotoSakae HommaAtsushi MiyamotoAtsuko KurosakiTakeshi FujiiKunihiko Yoshimura
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JOURNAL OPEN ACCESS

2010 Volume 49 Issue 2 Pages 109-115

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Abstract

Background Acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) is considered to be a nearly fatal condition during the clinical course of IPF, as it is unresponsive to most conventional therapies.
Subjects and Methods To evaluate the efficacy of cyclosporin A (CsA) for AE of IPF, we conducted a retrospective study on autopsied IPF cases who developed AE and were treated with corticosteroids (CS) combined with CsA. The subjects comprised 11 males with a mean age of 69.9 years. The clinical features and prognosis of the CsA-treated group was compared to a group of 11 autopsied IPF cases with a mean age of 68.7 years who developed AE and were treated with CS alone (non-CsA-treated group).
Results CS pulse therapy followed by CS maintenance treatment were conducted in all cases of AE. Patients in the CsA-treated group received in addition a low dosage of CsA (100-150 mg). Although 7 out of 11 patients in the CsA-treated group died of AE per se, 4 patients survived the AE. Only 2 patients died during the first episode of AE. In comparison, 7 out of 11 patients in the non-CsA-treated group died during the first episode of AE. The mean survival period after the first onset of AE was 285 days in the CsA-treated group and 60 days in the non-CsA-treated group. The prognosis of the CsA-treated group therefore was significantly better than that of non-CsA-treated group after AE of IPF.
Conclusion Administration of CsA combined with CS may be efficacious in the treatment of AE of IPF.

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© 2010 by The Japanese Society of Internal Medicine
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