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.
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: Ganesh Raghu; Harold R Collard; Jim J Egan; Fernando J Martinez; Juergen Behr; Kevin K Brown; Thomas V Colby; Jean-François Cordier; Kevin R Flaherty; Joseph A Lasky; David A Lynch; Jay H Ryu; Jeffrey J Swigris; Athol U Wells; Julio Ancochea; Demosthenes Bouros; Carlos Carvalho; Ulrich Costabel; Masahito Ebina; David M Hansell; Takeshi Johkoh; Dong Soon Kim; Talmadge E King; Yasuhiro Kondoh; Jeffrey Myers; Nestor L Müller; Andrew G Nicholson; Luca Richeldi; Moisés Selman; Rosalind F Dudden; Barbara S Griss; Shandra L Protzko; Holger J Schünemann Journal: Am J Respir Crit Care Med Date: 2011-03-15 Impact factor: 21.405
Authors: Maya M Juarez; Andrew L Chan; Andrew G Norris; Brian M Morrissey; Timothy E Albertson Journal: J Thorac Dis Date: 2015-03 Impact factor: 2.895