Introduction Idiopathic pulmonary fibrosis is a chronic, progressive condition. BTS guidelines indicate that the ‘triple therapy’ - Prednisolone, Azathioprine, N-acetylcysteine (NAC) – can be considered in the management of UIP (usual interstitial pneumonitis) pattern disease based on the outcome of the IFIGENIA study (Demedts NEJM 2005). Recent data from the PANTHER study (Raghu NEJM 2012) has led to reversal of this recommendation due to concerns of increased adverse events, deaths and hospitalisation in the triple therapy arm as compared with placebo controls and those receiving NAC alone. This retrospective review examines the survival outcome of patients with UIP and UIP/COPD in a single centre (Sheffield Teaching Hospitals NHS Foundation Trust) where triple therapy was used over a five year period.
Methodology All patients with a UIP or UIP/COPD diagnosis managed within the interstitial lung disease clinic were identified through review of clinical notes and correspondence. Therapy, survival and demographic details were collected. Cox regression analysis was conducted using covariates of age at diagnosis, gender, occupational risk factors, COPD comorbidity and life limiting comorbidities.
Results 73 patients with UIP alone were identified; 16 patients had a UIP/COPD diagnosis. 8 patients received prednisolone alone, 2 NAC alone, 7 Pred/NAC, 3 Pred/Aza, 14 triple therapy and 11 received other immunosuppressive regimens (due to Azathioprine intolerance). 44 patients received no immunosuppressive therapy. Median survival, as derived from Kaplan-Meier curves, for those on no active therapy was 632 days (IQ range 485), 555 (IQ range 723) days for those on Prednisolone alone, 873 (IQ range 577)days for those on Pred/Aza and 869 (IQ range 918) days for those on triple therapy. No increase in hospitalisation was noted in the triple therapy group as compared with ‘untreated’ patients.
Conclusions In a cohort of 89 patients treated over a 5 year period triple therapy has been associated with an improved survival as compared to any other treatment regimen. No increase in hospitalisation has been identified and serious untoward events including blood abnormalities have been rare and manageable. Further Cox analysis using the covariates discussed above allied with smoking history, FVC and TLCO at diagnosis and rate of decline in FVC and TLCO at ‘decision to treat’ as covariates is awaited. We hope this data may enlighten practitioners to the progression of UIP over time, the reasons adopted by clinicians in selection of various (often unproven) therapies and the safety and utility of these treatments in a real life population.