Treatments for IPF (Idiopathic Pulmonary Fibrosis) – 2

Pulmonary Fibrosis

Pulmonary Fibrosis

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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Treatments for IPF (Idiopathic Pulmonary Fibrosis) – 1

Pulmonary Fibrosis

Pulmonary Fibrosis


The goals of treatment in IPF are essentially to reduce the symptoms, stop disease progression, prevent acute exacerbations and prolong survival. Preventive care (e.g. vaccinations) and symptom based treatment should be shared early in every patient.

Pharmacologic Interventions:

A number of treatments have been investigated in the past for IPF, including interferon gamma-1β, bosentan, ambrisentan and anticoagulants, but there are no longer considered effective treatment options.

Many of these studies were based on the hypothesis that IPF is an inflammatory disorder.


Pirfenidone is a small molecule that combines both the anti-inflammatory, anti-oxidant, and anti-fibrotic effects in experimental models of fibrosis.
Pirfenidone marketed under the trade name ‘Esbriet’ is approved in Europe for the treatment of patients with mild-to-moderate IPF.

N-Acetylcysteine and Triple Therapy:

N-Acetylcysteine (NAC) is a precursor to glutathione, an antioxidant.
Treatment with high doses of NAC may repair an oxidant-antioxidant imbalance that occurs in the lung tissue of the patients with IPF.

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Classification of IPF – There are more than 200 lung diseases

Idiopathic Pulmonary Fibrosis

IPF stands Idiopathic Pulmonary Fibrosis.



It is chronic decline in lungs function. Pulmonary Fibrosis means scarring of lung’s tissues & it is a cause of worsening dyspnoea (shortness of breathing).

The type IIP it means that Idiopathic Interstitial Pneumonia. It is type of ILD. It is known as disease Parenchymal Lung Disease.

Different types of diseases : 

1. IPF           2. NSIP             3. RB             4. JLD            5. DIP           6. COP-ATP

Associated Symptoms :

– Hypersensitivity pneumonitis,

– Pulmonary Langerhan’s Cell History cytosis,

– Asbestosis,

– Collagen,

– Vascular Diseases

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