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An introduction to pulmonary pathology is found in the ''[[pulmonary pathology]]'' article. | An introduction to pulmonary pathology is found in the ''[[pulmonary pathology]]'' article. | ||
*Often [[abbreviated]] '''IIP''', is a term used for a type of | *Often [[abbreviated]] '''IIP''', is a term used for a type of diffuse lung disease. | ||
**Diffuse lung disease is also known as ''interstitial lung disease''. | **Diffuse lung disease is also known as ''interstitial lung disease''. | ||
***Diffuse lung disease is probably a better term... as some diseases lumped into this category have involvement of the alveoli, i.e. are not interstitial. | ***Diffuse lung disease is probably a better term... as some diseases lumped into this category have involvement of the alveoli, i.e. are not interstitial. | ||
= | =Overview - histologic classification= | ||
*Can be complex,<ref name="pmid12405906">{{cite journal |author=Nicholson AG |title=Classification of idiopathic interstitial pneumonias: making sense of the alphabet soup |journal=Histopathology |volume=41 |issue=5 |pages=381-91 |year=2002 |month=November |pmid=12405906 |doi= |url=http://www3.interscience.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0309-0167&date=2002&volume=41&issue=5&spage=381}}</ref> and the combined efforts of [[clinician]]s, [[radiologist]]s, and [[pathologist]]s can help in the generation of a more specific diagnosis.<ref name="pmid15256390">{{cite journal |author=Flaherty KR, King TE, Raghu G, ''et al'' |title=Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis? |journal=Am. J. Respir. Crit. Care Med. |volume=170 |issue=8 |pages=904-10 |year=2004 |month=October |pmid=15256390 |doi=10.1164/rccm.200402-147OC |url=http://ajrccm.atsjournals.org/cgi/pmidlookup?view=long&pmid=15256390}}</ref><ref name="pmid16738191">{{cite journal |author=Kim DS, Collard HR, King TE |title=Classification and natural history of the idiopathic interstitial pneumonias |journal=Proc Am Thorac Soc |volume=3 |issue=4 |pages=285-92 |year=2006 |month=June |pmid=16738191 |doi=10.1513/pats.200601-005TK |url=http://pats.atsjournals.org/cgi/pmidlookup?view=long&pmid=16738191}}</ref> | *Can be complex,<ref name="pmid12405906">{{cite journal |author=Nicholson AG |title=Classification of idiopathic interstitial pneumonias: making sense of the alphabet soup |journal=Histopathology |volume=41 |issue=5 |pages=381-91 |year=2002 |month=November |pmid=12405906 |doi= |url=http://www3.interscience.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0309-0167&date=2002&volume=41&issue=5&spage=381}}</ref> and the combined efforts of [[clinician]]s, [[radiologist]]s, and [[pathologist]]s can help in the generation of a more specific diagnosis.<ref name="pmid15256390">{{cite journal |author=Flaherty KR, King TE, Raghu G, ''et al'' |title=Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis? |journal=Am. J. Respir. Crit. Care Med. |volume=170 |issue=8 |pages=904-10 |year=2004 |month=October |pmid=15256390 |doi=10.1164/rccm.200402-147OC |url=http://ajrccm.atsjournals.org/cgi/pmidlookup?view=long&pmid=15256390}}</ref><ref name="pmid16738191">{{cite journal |author=Kim DS, Collard HR, King TE |title=Classification and natural history of the idiopathic interstitial pneumonias |journal=Proc Am Thorac Soc |volume=3 |issue=4 |pages=285-92 |year=2006 |month=June |pmid=16738191 |doi=10.1513/pats.200601-005TK |url=http://pats.atsjournals.org/cgi/pmidlookup?view=long&pmid=16738191}}</ref> | ||
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*''Usual interstitial pneumonia'' is the most common type of ILD.<ref name="pmid16738196">{{cite journal |author=Visscher DW, Myers JL |title=Histologic spectrum of idiopathic interstitial pneumonias |journal=Proc Am Thorac Soc |volume=3 |issue=4 |pages=322-9 |year=2006 |month=June |pmid=16738196 |doi=10.1513/pats.200602-019TK |url=http://pats.atsjournals.org/cgi/pmidlookup?view=long&pmid=16738196}}</ref> | *''Usual interstitial pneumonia'' is the most common type of ILD.<ref name="pmid16738196">{{cite journal |author=Visscher DW, Myers JL |title=Histologic spectrum of idiopathic interstitial pneumonias |journal=Proc Am Thorac Soc |volume=3 |issue=4 |pages=322-9 |year=2006 |month=June |pmid=16738196 |doi=10.1513/pats.200602-019TK |url=http://pats.atsjournals.org/cgi/pmidlookup?view=long&pmid=16738196}}</ref> | ||
=Specific diseases/pattterns= | |||
==Diffuse alveolar damage== | |||
===General=== | |||
*Abbreviated ''DAD''. | |||
==See also | DAD is the histologic correlate of: | ||
*Adult respiratory distress syndrome (ARDS). | |||
*Acute interstitial pneumonia (AIP). | |||
*Transfusion related acute lung injury (TRALI). | |||
===Microscopic=== | |||
Features:<ref name=Ref_Klatt103>{{Ref Klatt|103}}</ref> | |||
*Early: | |||
**Hyaline membrane: debris (pink crap) lines the alveolar spaces. | |||
*Intermediate: | |||
**Macrophage proliferation. | |||
*Late: | |||
**Interstitial inflammation. | |||
**Fibrosis. | |||
Images: | |||
*[http://commons.wikimedia.org/wiki/File:Hyaline_membranes_-_intermed_mag.jpg DAD - intermed. mag. (WC)]. | |||
*[http://en.wikipedia.org/wiki/File:Hyaline_membranes_-_very_high_mag.jpg DAD - very high mag. (WC)]. | |||
*[http://commons.wikimedia.org/wiki/File:ARDS.jpg DAD (WC)]. | |||
==Usual interstitial pneumonia== | |||
===General=== | |||
*It is sometimes used incorrectly as a synoym for ''idiopathic pulmonary fibrosis''. | |||
*Cannot be diagnosed via bronchoscopic or transbronchial biopsy.<ref name=Ref_PPP186>{{Ref PPP|186}}</ref> | |||
====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/> | |||
====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> | |||
*Idiopathic pulmonary fibrosis. | |||
*Asbestosis - one ought to see ''ferruginous bodies''. | |||
*Chronic hypersensitivity pneumonitis (extrinsic allergic alveolitis). | |||
*Collagen vascular disease - includes [[systemic lupus erythematousus]], [[rheumatoid arthritis]], [[scleroderma]].<ref>{{Ref PCPBoD8|374}}</ref> | |||
*Chronic drug toxicity.<ref>{{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> | |||
===Radiologic=== | |||
*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. | |||
**Associated with interstitial thickening. (???) | |||
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>H. 8 July, 2009.</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. | |||
==Asbestosis== | |||
===General=== | |||
*Important to diagnose... asbestosis = compensation. | |||
===Microscopic=== | |||
*Histologic appearance as for UIP -- plus ''ferruginous bodies''. | |||
**Segmented twirling batton with long slender fibre within. | |||
Image(s): | |||
*[http://commons.wikimedia.org/wiki/File:Ferruginous_body.jpg Ferruginous bodies (WC)]. | |||
*[http://commons.wikimedia.org/wiki/File:Asbestosis_high_mag.jpg Asbestosis (WC)]. | |||
==Non-specific interstitial pneumonia== | |||
*Abbreviated ''NSIP''. | |||
*Better prognosis than UIP. | |||
*Some radiologists and pathologists don't believe in this entity. | |||
===Gross/Radiology=== | |||
*No honeycombing. | |||
*Fibrosis usually lower lung zone. | |||
*Patchy ground glass. | |||
===Microscopic=== | |||
*Fibrosis: | |||
**May be uniform. | |||
**"Linear fibrosis" has a good prognosis - should be mentioned in the report. | |||
***''Linear fibrosis'' = fibrosis that follows alveolar walls + no architectural distortion. | |||
*+/-Lymphoid nodules - assoc. with collagen vascular disease. | |||
Notes: | |||
*Like UIP... also temporally and spatially heterogeneous. | |||
*Inflammation in NSIP usually more prominent than in UIP. | |||
*No honeycombing - key difference between UIP and NSIP. | |||
===DDx=== | |||
*Collagen vascular disease. | |||
*Drug reaction. | |||
*Hypersensitivity pneumonitis (extrinic allergic alveolitis). | |||
==Hypersensitivity pneumonitis== | |||
*AKA ''extrinsic allergic alveolitis'' | |||
*Exposure to stuffs... e.g. moldy hay - ''Farmer's lung'', atypical mycobacteria - ''hot tub lung''. | |||
*Upper lung predominant disease (???). | |||
===Microscopic=== | |||
Features: | |||
* Lesions have centrilobular prominence - '''important feature'''. <ref name=pmid16061708/> | |||
** Allergens enter lung through airway which has a centrilobular location. | |||
* [[Granulomata]] (not typically seen in UIP) - '''important feature'''.<ref name=pmid16061708>PMID 16061708.</ref> | |||
* Chronic interstitial inflammation consisting primarily of lymphocytes. | |||
* Interstitial fibrosis. | |||
* Air space involvement (alveolitis). | |||
Images: | |||
*[http://commons.wikimedia.org/wiki/File:Hypersensitivity_pneumonitis_intermed_mag.jpg Hypersensitivity pneumonitis - intermed. mag. (WC)]. | |||
*[http://commons.wikimedia.org/wiki/File:Hypersensitivity_pneumonitis_high_mag.jpg Hypersensitivity pneumonitis - high mag. (WC)]. | |||
=See also= | |||
*[[Medical lung diseases]]. | *[[Medical lung diseases]]. | ||
*[[Pulmonary pathology]]. | *[[Pulmonary pathology]]. | ||
=References= | |||
{{Reflist|2}} | {{Reflist|2}} | ||
[[Category: Pulmonary pathology]] | [[Category: Pulmonary pathology]] |
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