Pharmacotherapy in idiopathic pulmonary fibrosis

Gaurav Acharya, Sunil Arya, Shivani Badal, Dewesh Kumar, Saurabh Samdariya


Idiopathic pulmonary fibrosis or cryptogenic fibrosing alveolitis is a form of chronic, progressive interstitial lung disease causing scarring of lung tissue and usually affect adults. Treatment is usually aimed at controlling inflammation and thus slowing the process of fibrosis. With only few patients responding to treatment and the disease being ultimately fatal with poor progression, the underlying lesion was considered to be fibrotic rather than inflammatory. Fibrotic foci, deposition of collagen, and lack of inflammatory cells are a predominant finding. Pirfenidone and N-acetyl cysteine are the only effective pharmacotherapy available till date. Interim results of PANTHER Trial clearly indicate more risk with triple therapy. However, in Indian patients, trial of steroid therapy may be tried when there is doubt of chronic hypersensitivity pneumonitis. BIBF 1120 has also shown positive results in Phase II clinical trial and shows a positive response in deteriorating lung function. Supplemental oxygen, education of patient, pulmonary rehabilitation, and Streptococcus pneumoniae and influenza vaccine are the most important supportive care. Pulmonary rehabilitation should be used as a treatment in the majority of patients.


Idiopathic pulmonary fibrosis, Pirfenidone, N-acetyl cysteine, Interstitial lung disease

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Keane MP, Strieter RM, Lynch JP rd, Belperio JA. Inflammation and angiogenesis in fibrotic lung disease. Semin Respir Crit Care Med. 2006;27(6):589-99.

Scotton CJ, Chambers RC. Molecular targets in pulmonary fibrosis: the myofibroblast in focus. Chest. 2007;132(4):1311-21.

Noble PW, Albera C, Bradford WZ, Costabel U, Glassberg MK, Kardatzke D, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet. 2011;377(9779):1760-9.

Richeldi L, Yasothan U, Kirkpatrick P. Pirfenidone. Nat Rev Drug Discov. 2011;10(7):489-90.

Pulmonary-Allergy Drugs Advisory Committee Complete Response on Pirfenidone. Food and Drug Administration Centre for Drug Evaluation and Research, 2010. Available at committees/committees meeting materials/drugs/pulmonary-allergy drugs advisory committee/ucm208806.pdf. Accessed 16 Jun 2014.

Demedts M, Behr J, Buhl R, Costabel U, Dekhuijzen R, Jansen HM, et al. High dose acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med. 2012;366:1968-77.

Idiopathic Pulmonary Fibrosis Clinical Research Network, Raghu G, Anstrom KJ, King TE Jr, Lasky JA, Martinez FJ. Prednisone, azathioprine, and N-acetylcysteine for pulmonary fibrosis. N Engl J Med. 2012;366(21):1968-77.

Richeldi L, Costabel U, Selman M, Kim DS, Hansell DM, Nicholson AG, et al. Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med. 2011;365(12):1079-87.

Idiopathic Pulmonary Fibrosis Clinical Research Network, Zisman DA, Schwarz M, Anstrom KJ, Collard HR, Flaherty KR, et al. A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. N Engl J Med. 2010;363(7):620-8.

Lee JS, Collard HR, Anstrom KJ, Martinez FJ, Noth I, Roberts RS, et al. Anti-acid treatment and disease progression in idiopathic pulmonary fibrosis: an analysis of data from three randomised controlled trials. Lancet Respir Med. 2013;1(5):369-76.

Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824.

Tzouvelekis A, Koliakos G, Ntolios P, Baira I, Bouros E, Oikonomou A, et al. Stem cell therapy for idiopathic pulmonary fibrosis: a protocol proposal. J Transl Med. 2011;9:182.

Orens JB, Estenne M, Arcasoy S, Conte JV, Corris P, Egan JJ, et al. International guidelines for the selection of lung transplant candidates: 2006 update – a consensus report from the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant. 2006;25(7):745-55.