Difference between revisions of "Usual interstitial pneumonia"

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~~#redirect~~ [[~~Diffuse_lung_diseases#Usual_interstitial_pneumonia~~]]

'''Usual interstitial pneumonia''', abbreviated '''UIP''', is common [[diffuse lung disease]]. Overall, it is uncommon.

==General==

*It is sometimes used incorrectly as a synonym for ''idiopathic pulmonary fibrosis''. It is a histomorphologic pattern and has a DDx (see below).

*UIP cannot be diagnosed via bronchoscopic or transbronchial biopsy,<ref name=Ref_PPP186>{{Ref PPP|186}}</ref> as it is peripheral.

===Epidemiology===

*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>{{Cite journal | last1 = Bjoraker | first1 = JA. | last2 = Ryu | first2 = JH. | last3 = Edwin | first3 = MK. | last4 = Myers | first4 = JL. | last5 = Tazelaar | first5 = HD. | last6 = Schroeder | first6 = DR. | last7 = Offord | first7 = KP. | title = Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. | journal = Am J Respir Crit Care Med | volume = 157 | issue = 1 | pages = 199-203 | month = Jan | year = 1998 | doi = | PMID = 9445300 }}</ref>

==Radiology==

*Honeycombing - ''multiple'' defects that obliterate the normal lung architecture - multiple spherical voids in the lung parenchyma; radiologically these are seen as lucencies.<ref>[http://www.medcyclopaedia.com/library/topics/volume_v_1/h/honeycombing.aspx http://www.medcyclopaedia.com/library/topics/volume_v_1/h/honeycombing.aspx]</ref>

**Usually subplural, i.e. peripheral lung.

**Classically lower lobe predominant.

*Traction [[bronchiectasis]].

Note:

*Cysts - have thin walls (think of emphysema, [[lymphangioleiomyomatosis]] et cetera).

**Cysts may be isolated/not close to a neighbour.

**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 name=Ref_PPP186-9>{{Ref PPP|186-9}}</ref>

*Fibroblast foci:

**"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>

**Note: Technically, ''fibroblast foci'' are composed of myofibroblasts.<ref name=Ref_PPP189>{{Ref PPP|189}}</ref>

*Interstitial inflammation.

*Microscopic honeycombing.

**Typically peripheral - cysts lined by ciliated epithelium.

*Spatial heterogeneity - patchy lesional distribution (areas of abnormal and normal lung may appear beside one another).

*Temporal heterogeneity - lesions of differing age side-by-side.<ref name=Ref_WMSP92>{{Ref WMSP|92}}</ref>

Notes:

*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.

DDx of UIP:<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 (UIP not otherwise specified).

*Asbestosis = UIP pattern + ferruginous bodies with asbestos fibers.

*Chronic hypersensitivity pneumonitis ([[AKA]] extrinsic allergic alveolitis) - classically centrilobular predominant +/- granulomas.

*Collagen vascular disease - includes [[systemic lupus erythematosus]], [[rheumatoid arthritis]], [[scleroderma]].<ref name=Ref_PCPBoD8_374>{{Ref PCPBoD8|374}}</ref>

*Chronic drug toxicity.<ref name=pmid10992015>{{cite journal |author=Rossi SE, Erasmus JJ, McAdams HP, Sporn TA, Goodman PC |title=Pulmonary drug toxicity: radiologic and pathologic manifestations |journal=Radiographics : a review publication of the Radiological Society of North America, Inc |volume=20 |issue=5 |pages=1245-59 |year=2000 |pmid=10992015 |doi=}}</ref>

===Images===

Image:UIPlungbiopsy.jpg|UIP - fibrosis - low mag. (WC)

Image:Honeycomb change.jpg|UIP - honeycomb change in UIP - low mag. (WC)

Image:Fibroblast focus.jpg|UIP - fibroblast focus - high mag. (WC)

</gallery>

==See also==

*[[Diffuse lung diseases]].

==References==

[[Category:Diffuse lung diseases]]

[[Category:Diagnosis]]

Difference between revisions of "Usual interstitial pneumonia"

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@@ Line 1: / Line 1: @@
-#redirect [[Diffuse_lung_diseases#Usual_interstitial_pneumonia]]
+'''Usual interstitial pneumonia''', abbreviated '''UIP''', is common [[diffuse lung disease]]. Overall, it is uncommon.
+==General==
+*It is sometimes used incorrectly as a synonym for ''idiopathic pulmonary fibrosis''.  It is a histomorphologic pattern and has a DDx (see below).
+*UIP cannot be diagnosed via bronchoscopic or transbronchial biopsy,<ref name=Ref_PPP186>{{Ref PPP|186}}</ref> as it is peripheral.
+===Epidemiology===
+*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>{{Cite journal  | last1 = Bjoraker | first1 = JA. | last2 = Ryu | first2 = JH. | last3 = Edwin | first3 = MK. | last4 = Myers | first4 = JL. | last5 = Tazelaar | first5 = HD. | last6 = Schroeder | first6 = DR. | last7 = Offord | first7 = KP. | title = Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. | journal = Am J Respir Crit Care Med | volume = 157 | issue = 1 | pages = 199-203 | month = Jan | year = 1998 | doi =  | PMID = 9445300 }}</ref>
+==Radiology==
+*Honeycombing - ''multiple'' defects that obliterate the normal lung architecture - multiple spherical voids in the lung parenchyma; radiologically these are seen as lucencies.<ref>[http://www.medcyclopaedia.com/library/topics/volume_v_1/h/honeycombing.aspx http://www.medcyclopaedia.com/library/topics/volume_v_1/h/honeycombing.aspx]</ref>
+**Usually subplural, i.e. peripheral lung.
+**Classically lower lobe predominant.
+*Traction [[bronchiectasis]].
+Note:
+*Cysts - have thin walls (think of emphysema, [[lymphangioleiomyomatosis]] et cetera).
+**Cysts may be isolated/not close to a neighbour.
+**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 name=Ref_PPP186-9>{{Ref PPP|186-9}}</ref>
+*Fibroblast foci:
+**"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>
+**Note: Technically, ''fibroblast foci'' are composed of myofibroblasts.<ref name=Ref_PPP189>{{Ref PPP|189}}</ref>
+*Interstitial inflammation.
+*Microscopic honeycombing.
+**Typically peripheral - cysts lined by ciliated epithelium.
+*Spatial heterogeneity - patchy lesional distribution (areas of abnormal and normal lung may appear beside one another).
+*Temporal heterogeneity - lesions of differing age side-by-side.<ref name=Ref_WMSP92>{{Ref WMSP|92}}</ref>
+Notes:
+*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.
+DDx of UIP:<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 (UIP not otherwise specified).
+*Asbestosis = UIP pattern + ferruginous bodies with asbestos fibers.
+*Chronic hypersensitivity pneumonitis ([[AKA]] extrinsic allergic alveolitis) - classically centrilobular predominant +/- granulomas.
+*Collagen vascular disease - includes [[systemic lupus erythematosus]], [[rheumatoid arthritis]], [[scleroderma]].<ref name=Ref_PCPBoD8_374>{{Ref PCPBoD8|374}}</ref>
+*Chronic drug toxicity.<ref name=pmid10992015>{{cite journal |author=Rossi SE, Erasmus JJ, McAdams HP, Sporn TA, Goodman PC |title=Pulmonary drug toxicity: radiologic and pathologic manifestations |journal=Radiographics : a review publication of the Radiological Society of North America, Inc |volume=20 |issue=5 |pages=1245-59 |year=2000 |pmid=10992015 |doi=}}</ref>
+===Images===
+<gallery>
+Image:UIPlungbiopsy.jpg|UIP - fibrosis - low mag. (WC)
+Image:Honeycomb change.jpg|UIP - honeycomb change in UIP - low mag. (WC)
+Image:Fibroblast focus.jpg|UIP - fibroblast focus - high mag. (WC)
+</gallery>
+==See also==
+*[[Diffuse lung diseases]].
+==References==
+{{Reflist|2}}
+[[Category:Diffuse lung diseases]]
 [[Category:Diagnosis]]