Difference between revisions of "Diffuse lung diseases"

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Diffuse lung diseases (view source)

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==Usual interstitial pneumonia==
==Usual interstitial pneumonia==
*Abbreviated ''UIP''.
*Abbreviated ''UIP''.
===General===
{{Main|Usual interstitial pneumonia}}
*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>


==Asbestosis==
==Asbestosis==
Michael
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