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Case 96: When it Runs in the Family
FPF is not uncommon with almost 1/6th of first-degree relatives of sporadic and familial PF showing ILA/ILD
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A 52-years old man presented with a probable UIP pattern in 2018.
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At this age, this is indistinguisable from a fibrotic NSIP pattern. He was a non-smoker and had no etiology including connective tissue disease (CTD).
He progressed and had a successful lung transplant.
8 years later, his elder brother presented with mild breathlessness and a CT scan shows a typical UIP pattern.
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The video runs through both cases and then discusses the findings in a third brother with a detailed discussion of the current concepts related to familial pulmonary fibrosis (FPF).
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