In the absence of UIP pattern on HRCT a surgical lung biopsy is required for confident diagnosis.
Histologic specimens for the diagnosis of IPF must be taken at least in three different places and be large enough that the pathologist can comment on the underlying lung architecture.
Small biopsies, such as those obtained via transbronchial lung biopsy (performed during bronchoscopy) are usually not sufficient for this purpose.
Hence larger biopsies obtained surgically via a ‘Thoracotomy’ or ‘Thoracoscopy’ are usually necessary.
Lung tissue from people with IPF usually show a characteristic histopathologic UIP pattern and so is the pathologic counterpart of IPF.
Although a pathologic diagnosis of IPF, a UIP often corresponds to a clinical diagnosis of IPF, a UIP histological pattern can be seen in other diseases as well, and fibrosis of known origin (e.g. Rheumatic diseases).
There are four key features of UIP including interstitial fibrosis in a ‘patch work pattern’ interstitial scarring, honeycomb changes and fibroblast foci.
Fibroblastic foci are dense collections of fibroblasts and scar tissue and together with honeycombing, are the main pathological findings that allow a diagnosis of UIP.
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