Idiopathic pulmonary fibrosis (IPF) is a progressive disease with a devastating prognosis. Acute excerebation (AE) is considered to be the worst condition during the clinical course of IPF, as it is unresponsive to most of the conventional therapies such as corticosteroid (CS) and eventually leads to death. New therapeutic approaches need to be explored.
We conducted a retorospective study on autopsied IPF cases developing AE who were then treated with CS combined with Cyclosporin A (CsA) during the period from 1994 to 2004 to evaluate the efficacy of CsA for AE of IPF. The subjects comprised of 11 male individuals with a mean age of 69.9 years. The clinical features of the CsA-treated group were compared to those of the control non-CsA-treated group of 11 patients (11 males with a mean age of 67.0 years).
For AE, CS pulse therapy followed by CS maintenance treatment (0.5-1.0 mg/kg) were conducted in all cases. The patients in the CsA-treated group also received a low dose of CsA (100-150 mg/day). Although 7 out of 11 patients (63.6%) in the CsA-treated group died of AE, 4 (36.4%) recovered from AE. Only two patients died of the first AE, and other nine responded to the initial treatments and survived. However, they experienced repetitive AE and 5 cases eventually died of AE afterwards. In comparison, 7 out of 11 patients (63.4%) in the non-CsA-treated group did at the initial AE, and died other 4 at the following second AE. The mean survival period after the first onset of AE was 285 days in the CsA-treated group and 60 days the in non-CsA-treated group, respectively.
The prognosis in the CsA-treated group was significantly better than that in the group without CsA treatment in AE of IPF.
Administration of CsA combined with CS may be an efficacious approach for AE of IPF.
Susumu Sakamoto, None.