Treatments for IPF (Idiopathic Pulmonary Fibrosis) – 2

Pulmonary Fibrosis

Pulmonary Fibrosis

Nintedanib:
Nintedanibis an investigational orally-administered triple angiokinase inhibitor that targets receptor tyrosine.
Kinases involved in the regulations of angiogenesis -fibroblasts growth factor receptor (FGFR), platelet derived growth factor receptor (PDGFR) which have also been implicates.
Nintendanib, like Pirfenidone was approved for the treatment of IPF by the U.S. FDA in October 2014.

Future Therapeutic Options:
Many agents are investigated I Phase II clinical trials for IPF, including the ‘monoclonal antibodies’ simtuzumab tralokimob, lebrikizumab and FG-2019, a lysophoshpatidic acid receptor antagonist (BMS=986020).

A phase II study of STX-100 is also ongoing.

These molecules are directed against several growth factors and cytokines that are known to play a role in the proliferation, activation, differentiation or inappropriate survival of fibroblasts.

Non Pharmacological Interventions:

Lung Transplantation:
Lung Transplantation may be suitable for those patients, physically eligible to undergo a major transplant operation.

In IPF patients, lung transplantation reduces the risk of death by 75% as compared with patients who remain on the waiting list.

Since the introduction of the lung allocation score (LAS), which prioritizes transplant candidates based on survival for lung transplantation in the USA.

Symptomatic patients with IPF younger than 65 years of age and with a body mass index (BMI) ≤ 26kg/m² should be referred for lung transplantation, but there are no clear data to guide the precise timing for LT.

Bilateral lung transplantation is superior to single lung transplantation in patients with IPF.
Five-year survival rates after lung transplantation IPF are estimated at between 50-56%.

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