•Idiopathic pulmonary fibrosis is now generally regarded as a consequence of multiple interacting genetic and environmental risk factors, with repetitive local micro-injuries to ageing alveolar epithelium playing a central role. ( Richeldi, Collard & Jones. 2017)

•Environmental factors could include cigarette smoking, metal and wood dusts, farming, viruses etc. 

•The gene MUC5B is the risk variant with the largest genetic effect on development of both familial and sporadic idiopathic pulmonary fibrosis.

• Immunosuppressant therapy has been commonly used treatment . Glass et al, (2022) state the use of prednisone, Azathioprine, and N-acetylcysteine.

• Yet this was show to increase adverse reactions and increase morbidity in IPF patients. 

• Thalidomide has the anti inflammatory properties that make the glutamic acid derivative good for treating IPF.

• Antifibrotics is a approved treatment with Pifenidone and Ninedanib being used .

• Pirfenidone is a modified pyridine small molecule with antifibrotics, anti-inflammatory and antioxidants. With a reduction of collagen it slows the scarring process by suppressing cytokine.(Glass, et al. 2022)

Determining when to transplant is one of the biggest struggles. The survival prediction needs to be high. Antifibrotic medications should be started before transplant is discussed. 

• Understanding the disease 

• Medications such as Pirfenidone and Nintedanib show get response 

• Treating comorbidities  (most common, COPD, Lung cancer, OSA)

• Supportive care (palliative care)

• Identify triggers (cough) 

• Pulmonary rehab ( improve SOB, walking distance, reduce anxiety, Improve quality of life) 

• Psychological support