Thanks to Anne and Vincent for presenting the case of a middle-aged man with IPF who came in with acutely worsening shortness of breath after VATS lung biopsy! We talked about workup and management of IPF, and emphasized that IPF flare is a diagnosis of exclusion when an IPF patient comes in with acute worsening dyspnea.
UIP is both a histopathologic and radiographic pattern seen in IPF.
Typical onset 50s-60s, earlier if familial, M>F, smokers>non.
Gradual onset of dyspnea over months, non-productive cough. Fever is rare.
HRCT shows subpleural bibasilar reticular opacities, honeycombing and traction bronchiectasis. Ground glass opacities are uncommon.
BAL has no role if HRCT suggests IPF.
Lung biopsy is indicated if diagnosis is otherwise unclear. Surgical VATS biopsy is preferred over transbronchial since large sample size is important.
– Exclusion of other causes of ILD (IPF is an idiopathic interstitial pneumonia)
– Definite UIP on HRCT or combination of HRCT and lung biopsy with UIP features
– Supportive care (O2, pulm rehab, flu and pneumococcal vaccines)
– Antifibrotics (pirfenidone, nintedanib)
– No efficacy and possible harm: glucocorticoids, azathioprine, N-acetylcysteine
– Early referral for lung transplant
Acute IPF exacerbation:
– High dose glucocorticoids (anecdotal, not evidence-based)
Breakdown of ILD from Uptodate (I like this one, but there are other ways to classify):