Pulmonary fibrosis (PF) is a part of interstitial lung diseases (ILDs) that is a family of >200 different lung diseases, which are characterized by inflammation, cellular prolifer...
Very insidious and slow onset
Exertional breathlessness and nonproductive cough
Constitutional symptoms are uncommon but can present with weight loss, fever, fatigue, myalgia, and arthr...
Smoking cessation
Pulmonary rehabilitation
Referral to a transplant center
Supplemental oxygen:
Clinical practice guidelines strongly recommend supplemental oxygen.
Goal oxyhemogl...
Pulmonary clinic and, if possible, at an ILD clinic.
Monitoring with serial HRCT and PFT along with 6MWD and exercise oximetry
If on treatment with any of the newly...
Lederer DJ, Martinez FJ. Idiopathic pulmonary fibrosis. N Engl J Med. 2018;378(19):1811–1823. [View Abstract on OvidMedline]
J84.10 Pulmonary fibrosis, unspecified
J84.112 Idiopathic pulmonary fibrosis
515 Postinflammatory pulmonary fibrosis
516.31 Idiopathic pulmonary fibrosis
51615001 Fibrosis of lung (di...
IPF is most common idiopathic ILD and frequently misdiagnosed and mistreated.
HRCT is cornerstone for diagnosis.
Pulmonary hypertension is very common.
Until recently available two newer ...
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