Cyclosporin A in the treatment of acute exacerbation of idiopathic pulmonary fibrosis.

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.

• Authors: Susumu Sakamoto, Sakae Homma, Atsushi Miyamoto, Atsuko Kurosaki, Takeshi Fujii, Kunihiko Yoshimura
• Journal: Internal medicine (Tokyo, Japan) (Intern Med) Vol. 49 Issue 2 Pg. 109-15 ( 2010) ISSN: 1349-7235 [Electronic] Japan
• PMID: 20075573 (Publication Type: Comparative Study, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Cyclosporine

Topics

• Acute Disease
• Aged
• Aged, 80 and over
• Cyclosporine (therapeutic use)
• Disease Progression
• Female
• Humans
• Idiopathic Pulmonary Fibrosis (drug therapy, mortality, pathology)
• Male
• Middle Aged
• Retrospective Studies
• Survival Rate (trends)
• Treatment Outcome