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DI-076 Pirfenidone: compassionate use in two patients with idiopathic pulmonary fibrosis
  1. E Puerta-Garcia,
  2. C Valencia-Soto,
  3. N Martinez-Casanova,
  4. J Perez-Morales,
  5. A Madrid-Paredes,
  6. J Orantes-Casado de Amezua
  1. Virgen de Las Nieves University Hospital, Pharmacy, Granada, Spain

Abstract

Background Pirfenidone is currently the only agent approved for mild to moderate idiopathic pulmonary fibrosis (IPF) in adults.

Purpose To evaluate the effect of pirfenidone in two patients who met the criteria for use.

Materials and methods Follow-up of two cases of IPF in a Spanish hospital. The most common parameters used when monitoring IPF are functional vital capacity (FVC) and diffusing capacity or Transfer Factor of the Lung for Carbon Monoxide (TLCO).

Results Patient 1 was diagnosed in 2008. Initial treatment: 20 months of prednisone. Changed treatment in 2009 to triple therapy with azathioprine, N-acetylcysteine (NAC) and prednisone. This regimen lasted 11 months, after which NAC was used in monotherapy. In 2012 a new regimen was started: pirfenidone, NAC and prednisone. This lasted until the end of the observation period. During treatment with corticosteroids, TLCO decreased from 83 to 41%, and FVC from 68 to 52%. With the triple therapy, TLCO changed from 41 to 32% and FVC increased slightly from 52 to 57%. During the treatment with NAC in monotherapy, TLCO values remained at 35% and FVC at 58%. Finally, with pirfenidone, TLCO stabilised between 31% and 34% and FVC remained between 50% and 51%.

Patient 2 was diagnosed in 2007. Initial treatment: 19 months with triple therapy after which NAC remained in monotherapy for 24 months. Treatment with pirfenidone, NAC and prednisone began in 2012 and continued until the end of the observation period. With the triple therapy regimen, TLCO decreased from 50 to 44% and FVC remained constant (80%). During the NAC monotherapy period, TLCO values fluctuated around 43 to 42%, with a minimum of 31%. With pirfenidone TLCO ranged from 37 to 39%, and FVC remained at 91%.

Conclusions Although the two patients clearly stabilised in breathing patterns, in both cases this stability had been reached previously with NAC monotherapy. The results indicated that pirfenidone did not provide evident benefits in the treatment of IPF and its use may not be cost effective.

No conflict of interest.

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