Difference between revisions of "Diffuse lung diseases"

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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 [[diffuse lung disease]].
*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.


==Overivew - histologic classification===
=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==
=References=
{{Reflist|2}}
{{Reflist|2}}


[[Category: Pulmonary pathology]]
[[Category: Pulmonary pathology]]
Michael
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