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*Cannot be diagnosed via bronchoscopic or transbronchial biopsy.<ref name=Ref_PPP186>{{Ref PPP|186}}</ref> | *Cannot be diagnosed via bronchoscopic or transbronchial biopsy.<ref name=Ref_PPP186>{{Ref PPP|186}}</ref> | ||
===Epidemiology=== | ====Epidemiology==== | ||
*Disease of the old - rare in under 50 years old.<ref>AC UBC S.102.</ref> | *Disease of the old - rare in under 50 years old.<ref>AC UBC S.102.</ref> | ||
*Dismal prognosis - mean survival after diagnosis ~ 2.8 years.<ref name=pmid9445300/> | *Dismal prognosis - mean survival after diagnosis ~ 2.8 years.<ref name=pmid9445300/> | ||
===Differential diagnosis=== | ====Differential diagnosis==== | ||
UIP is seen in:<ref name=leslie>{{cite book |author=Wick, Mark R.; Leslie, Kevin |title=Practical pulmonary pathology: a diagnostic approach |publisher=Churchill Livingstone |location=Edinburgh |year=2005 |pages= |isbn=0-443-06631-0 |oclc= 156861539|doi= |accessdate=}}</ref> | UIP is seen in:<ref name=leslie>{{cite book |author=Wick, Mark R.; Leslie, Kevin |title=Practical pulmonary pathology: a diagnostic approach |publisher=Churchill Livingstone |location=Edinburgh |year=2005 |pages= |isbn=0-443-06631-0 |oclc= 156861539|doi= |accessdate=}}</ref> | ||
*Idiopathic pulmonary fibrosis. | *Idiopathic pulmonary fibrosis. | ||
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**Medcyclopaedia defines it as: thin-walled, well-demarcated and >1 cm.<ref>[http://www.medcyclopaedia.com/library/topics/volume_v_1/l/lung_cyst.aspx http://www.medcyclopaedia.com/library/topics/volume_v_1/l/lung_cyst.aspx]</ref> | **Medcyclopaedia defines it as: thin-walled, well-demarcated and >1 cm.<ref>[http://www.medcyclopaedia.com/library/topics/volume_v_1/l/lung_cyst.aspx http://www.medcyclopaedia.com/library/topics/volume_v_1/l/lung_cyst.aspx]</ref> | ||
=== | ===Microscopic=== | ||
Features:<ref>{{Ref PPP|186-9}}</ref> | Features:<ref name=Ref_PPP186-9>{{Ref PPP|186-9}}</ref> | ||
*Fibroblast foci: | *Fibroblast foci: | ||
**"Crescent-shaped bulge" of fibroblasts -- a rounded projection of spindle cells into the airspace. | **"Crescent-shaped bulge" of fibroblasts -- a rounded projection of spindle cells into the airspace. | ||
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Notes: | Notes: | ||
*Disease worse distant from large airways: lower lung field predominance, typically worse at periphery of lobule and lung.<ref> | *Disease worse distant from large airways: lower lung field predominance, typically worse at periphery of lobule and lung.<ref>A. Churg. UBC S.103.</ref> | ||
*Heterogeneity of inflammation: airspace macrophages & inflammation minimal in honeycombed foci. | *Heterogeneity of inflammation: airspace macrophages & inflammation minimal in honeycombed foci. | ||
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