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IDIOPATHIC PULMONARY FIBROSIS

Idiopathic pulmonary fibrosis is the progressive parenchymal scarring & loss of pulmonary function due to unidentifiable cause of lung injury.

It is a specific form of chronic fibrosing interstitial pneumonia with the histopathologic characteristics of usual interstitial pneumonia.

Approximately 5% of patients are asymptomatic. Common signs and symptoms are exertional dyspnea, nonproductive cough, finger clubbing and bilateral inspiratory crackles.

Idiopathic%20pulmonary%20fibrosis Management

Management

Follow Up

Follow-up is recommended every 3 months for patients with rapid disease progression or health status deterioration
Six-month intervals for follow-up is recommended for patients with steadily progressing disease
Follow-up every 6 months is recommended for patients with stable disease & annual follow-up for patients with stable disease after 1 year
Patients with ongoing pulmonary rehabilitation should be reassessed at 6- or 12-month intervals
Pulse oximetry measurement at rest & during exertion is recommended during follow-up (3-6 month intervals) to monitor for oxygenation status
- Oxygen desaturation of <88% prompts supplemental oxygenation

Screening for complications (pulmonary hypertension, pulmonary embolism, coronary artery disease, lung cancer) may be done every follow-up

Mavrilimumab improves outcomes in COVID-19 pneumonia with hyperinflammation

Audrey Abella, 03 Aug 2020

Adding the anti-GM-CSF* receptor-α monoclonal antibody mavrilimumab to standard care (SC) improved clinical outcomes in patients with severe coronavirus disease 2019 (COVID-19) pneumonia and systemic hyperinflammation who were not under mechanical ventilation (MV), an Italian study has shown.

Oral as good as IV antibiotics for ridding P aeruginosa in cystic fibrosis

Pearl Toh, 08 Dec 2020

Intravenous (IV) antibiotics are no better than oral antibiotics when it comes to eradicating Pseudomonas (P.) aeruginosa in children and adults with cystic fibrosis, yet are more costly than the latter, reveals the TORPEDO-CF study.