TY - JOUR
T1 - Usual interstitial pneumonia-pattern fibrosis in surgical lung biopsies. Clinical, radiological and histopathological clues to aetiology
AU - Smith, Maxwell
AU - Dalurzo, Mercedes
AU - Panse, Prasad
AU - Parish, James
AU - Leslie, Kevin
PY - 2013/10
Y1 - 2013/10
N2 - Pulmonary fibrosis in surgical lung biopsies is said to have a 'usual interstitialpneumonia-pattern' (UIP-pattern) of disease when scarring of the parenchyma is present in a patchy, 'temporally heterogeneous' distribution. These biopsies are one of the more common non-neoplastic specimens surgical pathologists encounter and often pose a number of challenges. UIP is the expected histopathological pattern in patients with clinical idiopathic pulmonary fibrosis (IPF), but the UIP-pattern can be seen in other conditions on occasion. Most important among these are the rheumatic interstitial lung diseases (RILD) and chronic hypersensitivity pneumonitis (CHrHP). Because theses entities have different mechanisms of injury, approach to therapy, and expected clinical progression, it is imperative for the surgical pathologist to correctly classify them. Taken in isolation, the UIP-pattern seen in patients with IPF may appear to overlap with that of RILD and CHrHP, at least when using the broadest definition of this term (patchy fibrosis). However, important distinguishing features are nearly always present in our experience, and the addition of a multidisciplinary approach will often resolve the critical differences between these diseases. In this manuscript, we review the distinguishing clinical, radiologic and histopathological features of UIP of IPF, RILD and CHrHP, based, in part, on the existing literature, but also lessons learned from a busy lung biopsy consultation practice.
AB - Pulmonary fibrosis in surgical lung biopsies is said to have a 'usual interstitialpneumonia-pattern' (UIP-pattern) of disease when scarring of the parenchyma is present in a patchy, 'temporally heterogeneous' distribution. These biopsies are one of the more common non-neoplastic specimens surgical pathologists encounter and often pose a number of challenges. UIP is the expected histopathological pattern in patients with clinical idiopathic pulmonary fibrosis (IPF), but the UIP-pattern can be seen in other conditions on occasion. Most important among these are the rheumatic interstitial lung diseases (RILD) and chronic hypersensitivity pneumonitis (CHrHP). Because theses entities have different mechanisms of injury, approach to therapy, and expected clinical progression, it is imperative for the surgical pathologist to correctly classify them. Taken in isolation, the UIP-pattern seen in patients with IPF may appear to overlap with that of RILD and CHrHP, at least when using the broadest definition of this term (patchy fibrosis). However, important distinguishing features are nearly always present in our experience, and the addition of a multidisciplinary approach will often resolve the critical differences between these diseases. In this manuscript, we review the distinguishing clinical, radiologic and histopathological features of UIP of IPF, RILD and CHrHP, based, in part, on the existing literature, but also lessons learned from a busy lung biopsy consultation practice.
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U2 - 10.1136/jclinpath-2013-201442
DO - 10.1136/jclinpath-2013-201442
M3 - Review article
C2 - 23703852
AN - SCOPUS:84884988399
SN - 0021-9746
VL - 66
SP - 896
EP - 903
JO - Journal of clinical pathology
JF - Journal of clinical pathology
IS - 10
ER -