Pulmonary fibrosis (PF) is an interstitial lung disease (ILD), a family of >200 different lung diseases, characterized by inflammation, cellular proliferation, and fibrosis within...
Gradual onset
Exertional breathlessness and nonproductive cough
Constitutional symptoms (weight loss, fever, fatigue, myalgias, arthralgias) are uncommon.
History of exposure (see "Risk ...
Smoking cessation
Vaccinations: pneumococcus, influenza, SARS-CoV2
Pulmonary rehabilitation
Referral to a transplant center
Supplemental oxygen: Clinical practice guidelines stro...
Pulmonary clinic and, if possible, at an ILD clinic.
Monitoring with serial HRCT (at least yearly), PFT (at least every 6 months)
If on antifibrotic medications, mo...
J84.10 Pulmonary fibrosis, unspecified
J84.112 Idiopathic pulmonary fibrosis
51615001 Fibrosis of lung (disorder)
700250006 Idiopathic pulmonary fibrosis (disorder)
266368002 Post-inflamm...
IPF is the most common idiopathic ILD and frequently misdiagnosed or has delayed diagnosis.
High-resolution CT scan is the cornerstone for diagnosis.
Two anti-fibrotic drugs are the only...
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