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


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.


CONCLUSIONS
Administration of CsA combined with CS may be efficacious in the treatment of AE of IPF.
Sakamoto
Susumu
S
Division of Respiratory Medicine, Toho University School of Medicine, Department of Respiratory Medicine, Toranomon Hospital, Tokyo. susumu1029@fiberbit.net
Homma
Sakae
S
Miyamoto
Atsushi
A
Kurosaki
Atsuko
A
Fujii
Takeshi
T
Yoshimura
Kunihiko
K
D000208:Acute Disease; D000368:Aged; D000369:Aged, 80 and over; D016572:Cyclosporine / Q000627:therapeutic use*; D018450:Disease Progression; D005260:Female; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy* / Q000401:mortality / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D016896:Treatment Outcome
D016572:Cyclosporine