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Abstract :
[en] Canine Idiopathic Pulmonary Fibrosis (CIPF) is a progressive fibrotic interstitial lung disease of unknown aetiology, affecting predominantly the West Highland White Terrier (WHWT) breed. Currently, there is no curative treatment available. Fibroblast Activation Protein (FAP) is a cell surface protease usually absent from normal tissue but specifically expressed in areas of active tissue remodelling such as in fibroblast foci in human idiopathic pulmonary fibrosis, which shares features with CIPF. The aim of this study was to assess the expression and localization of FAP in the lungs of WHWTs affected with CIPF in comparison with healthy lungs.
Post-mortem formalin-fixed lung biopsies prepared from WHWTs with CIPF (n=17, age from 10 to 15y), control WHWTs exempt from lung disease (n=4, age from 11 to 15y) and dogs from various breeds with healthy lungs (n=11, age from 7 to 17y) were retrospectively used. Hematoxyllin and eosin-stained slides were reviewed for histopathology. Immunohistochemistry (IHC) was performed using a rabbit anti-human FAP monoclonal antibody (#ab207178). An IHC staining index (absent, low, moderate or high) was attributed according to the percentage of positive cells combined with the staining intensity.
FAP was identified in the lungs of 16 out of 17 (94%) WHWTs with CIPF (IHC index high, moderate, or low in respectively 10, 4 and 2 dogs) and absent from the interstitium of healthy lungs. Results showed that FAP is moderately to markedly expressed in lungs in most dogs affected with CIPF. Accordingly, FAP should be considered as an interesting potential therapeutic target in future studies.