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(move to pulmonary hypertension) |
(Ref PPP) |
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===General=== | ===General=== | ||
*It is sometimes used incorrectly as a synoym for ''idiopathic pulmonary fibrosis''. | *It is sometimes used incorrectly as a synoym for ''idiopathic pulmonary fibrosis''. | ||
*Cannot be diagnosed via bronchoscopic or transbronchial biopsy.<ref>PPP | *Cannot be diagnosed via bronchoscopic or transbronchial biopsy.<ref name=Ref_PPP186>{{Ref PPP|186}}</ref> | ||
===Epidemiology=== | ===Epidemiology=== | ||
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===Histology=== | ===Histology=== | ||
Features:<ref>PPP | Features:<ref>{{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. | ||
**Location: in the areas of transisition between active inflammation and old inflammation.<ref>[http://www.epler.com/IPFWhat%27sIPFDiseaseInformation2.htm http://www.epler.com/IPFWhat%27sIPFDiseaseInformation2.htm]</ref> | **Location: in the areas of transisition between active inflammation and old inflammation.<ref>[http://www.epler.com/IPFWhat%27sIPFDiseaseInformation2.htm http://www.epler.com/IPFWhat%27sIPFDiseaseInformation2.htm]</ref> | ||
**Note: Technically, ''fibroblast foci'' are composed of myofibroblasts.<ref>PPP | **Note: Technically, ''fibroblast foci'' are composed of myofibroblasts.<ref name=Ref_PPP189>{{Ref PPP|189}}</ref> | ||
*Interstitial inflammation, | *Interstitial inflammation, | ||
*Microscopic honeycombing, | *Microscopic honeycombing, | ||
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===Histology=== | ===Histology=== | ||
Features:<ref>PPP | Features:<ref>{{Ref PPP|197-8}}</ref> | ||
*Brown pigmented airspace macrophages - ''smoker's macrophages''. | *Brown pigmented airspace macrophages - ''smoker's macrophages''. | ||
*Inflammation of the terminal bronchioles. | *Inflammation of the terminal bronchioles. | ||
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===General=== | ===General=== | ||
*[[AKA]] eosinophilic granuloma of lung. | *[[AKA]] eosinophilic granuloma of lung. | ||
*Associated with smoking.<ref name= | *Associated with smoking.<ref name=Ref_PPP234>{{Ref PPP|234}}</ref> | ||
*Not assoc. with systemic diseases of Langerhans cells (AKA Hand-Schueller-Christian disease). | *Not assoc. with systemic diseases of Langerhans cells (AKA Hand-Schueller-Christian disease). | ||
Subtypes:<ref name= | Subtypes:<ref name=Ref_PPP234/> | ||
*Cellular form. | *Cellular form. | ||
*Fibrotic form. | *Fibrotic form. | ||
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===Histology=== | ===Histology=== | ||
Features:<ref>PPP | Features:<ref name=Ref_PPP237>{{Ref PPP|237}}</ref> | ||
*Cellular peribronchiolar nodules with: | *Cellular peribronchiolar nodules with: | ||
**Langerhans cells - '''key feature''': | **Langerhans cells - '''key feature''': | ||
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===IHC=== | ===IHC=== | ||
*Langerhans cells: S100+ and CD1a+.<ref>PPP | *Langerhans cells: S100+ and CD1a+.<ref name=Ref_PPP237>{{Ref PPP|237}}</ref> | ||
=Granulomatous lung disease= | =Granulomatous lung disease= |
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