Brief About Pulmonary Fibrosis

Pulmonary fibrosis is a lung disease caused by scarring and damage to lung tissue. It’s more difficult for your lungs to work properly because of this thickened, stiff tissue. As your pulmonary fibrosis progresses, you will become increasingly short of breath. A variety of factors can contribute to the scarring associated with pulmonary fibrosis. On the other hand, doctors are frequently unable to determine what is causing the problem. Idiopathic pulmonary fibrosis is a condition that occurs without a known cause. Although the lung damage caused by pulmonary fibrosis cannot be reversed, medications and therapies can help to alleviate symptoms and improve the overall quality of life. A lung transplant may be necessary for some people.

Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is a scarring of the lungs that makes breathing difficult.

It’s unclear what causes it, but it mostly affects people in their 70s and 75s, and it’s uncommon in people under 50. Several treatments can help slow the progression of IPF, but there is currently no treatment that can stop or reverse lung scarring.

Idiopathic pulmonary fibrosis symptoms

IPF symptoms usually appear gradually and progressively worsen over time.

Among the signs and symptoms are:

• Exhaustion, shortness of breath, and a persistent dry cough
• Rounded and swollen fingertips, loss of appetite, and weight loss (clubbed fingers)
• Many people dismiss their shortness of breath at first, blaming it on growing older or being out of shape

However, even simple activities like getting dressed can cause shortness of breath over time.

Causes

1. IPF has no known cause, but it has been shown that certain environmental factors and exposures increase the risk of developing it. 
2. Cigarette smoking is the most well-known and widely accepted risk factor for IPF, with a twofold increase in the risk of developing the disease. 
3. Other environmental and occupational exposures have been linked to an increased risk of IPF, including metal dust, wood dust, coal dust, silica, stone dust, biologic specks of dust such as hay dust, mould spores, or other agricultural products, and farming/livestock occupations. 
4. According to some evidence, viral infections have been linked to idiopathic pulmonary fibrosis and other fibrotic lung diseases.

Diagnosis

An earlier diagnosis of IPF allows for more effective treatment and, potentially, a better long-term clinical outcome of this progressive and fatal disease. When IPF is suspected, diagnosis can be difficult. Still, a multidisciplinary approach involving a pulmonologist, radiologist, and pathologist who are all experts in interstitial lung disease has improved IPF diagnosis accuracy. The American Thoracic Society (ATS) and the European Respiratory Society (ERS) published a Multidisciplinary Consensus Statement on Idiopathic Interstitial Pneumonia in 2000, which proposed specific major and minor criteria for establishing the diagnosis of IPF. The ATS, ERS, the Japanese Respiratory Society (JRS), and the Latin American Thoracic Association published simplified and updated criteria for diagnosing and managing IPF in 2011. (ALAT). A diagnosis of IPF currently necessitates:

Treatment

Treatment for IPF focuses on reducing symptoms, slowing disease progression, preventing acute exacerbations, and extending survival. Every patient should begin preventive care (such as vaccinations) and symptom-based treatment as soon as possible.

1. Treatment with Oxygen: Oxygen therapy, or supplemental oxygen for home use, became a strong recommendation in the 2011 IPF guidelines for use in patients with significantly low oxygen levels at rest. Although there is no evidence that oxygen therapy improves survival in IPF, some evidence suggests that it improves exercise capacity.
2. Rehabilitation of the Lungs: For patients with IPF, fatigue and loss of muscle mass are common and disabling issues. By stabilising and reversing the extrapulmonary features of the disease, pulmonary rehabilitation may alleviate the overt symptoms of IPF and improve functional status. Although few published studies on pulmonary rehabilitation in idiopathic pulmonary fibrosis, most have found significant short-term improvements in functional exercise tolerance, quality of life, and exertional dyspnea. Exercise training, nutritional modification, occupational therapy, education, and psychosocial counselling are common rehabilitation programs.

Conclusion

Idiopathic pulmonary fibrosis (IPF) is a rare, progressive respiratory illness characterized by lung tissue thickening and stiffening and the formation of scar tissue. It’s a chronic scarring lung disease that causes a steady, irreversible decline in lung function. The lung tissue thickens and stiffens, affecting the tissue surrounding the lungs’ air sacs. Shortness of breath and a dry cough are two of the most common symptoms. 

Other signs and symptoms include fatigue and unusually large and dome-shaped fingers and toenails (nail clubbing). Pulmonary hypertension, heart failure, pneumonia, and pulmonary embolism are possible complications.