Pulmonary fibrosis (PF) is an interstitial lung disease (ILDs), a family of >200 different lung diseases, characterized by inflammation, cellular proliferation, and/or fibrosis wi...
Exertional breathlessness and nonproductive cough
Constitutional symptoms (weight loss, fever, fatigue, myalgias, arthralgias) are uncommon.
History of exposure (see "Risk ...
Vaccinations: pneumococcus, influenza, SARS-CoV2
Referral to a transplant center
Clinical practice guidelines stron...
Pulmonary clinic and, if possible, at an ILD clinic.
Monitoring with serial HRCT, PFT along with 6MWD
If on antifibrotic medications, monitor LFTs.
J84.10 Pulmonary fibrosis, unspecified
J84.112 Idiopathic pulmonary fibrosis
51615001 Fibrosis of lung (disorder)
700250006 Idiopathic pulmonary fibrosis (disorder)
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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