Difference between revisions of "Diffuse lung diseases"

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'''Diffuse lung diseases''', abbreviated '''DLD''', are a group of uncommon pathologies, also known as '''idiopathic interstitial [[pneumonia]]s''', abbreviated '''IIPs''', and '''interstitial lung disease'''.  
'''Diffuse lung diseases''', abbreviated '''DLD''', are a group of uncommon pathologies, also known as '''idiopathic interstitial [[pneumonia]]s''', abbreviated '''IIPs''', and '''interstitial lung disease''', abbreviated '''ILD'''.  


They are a subgroup of the [[medical lung diseases]].   
They are a subgroup of the [[medical lung diseases]].   


Diffuse lung disease is the preferred term, as some diseases lumped into this group have involvement of the alveoli, i.e. are not interstitial. Also, some DLDs are not considered idiopathic, e.g. [[LIP]] is caused by [[smoking]].
Diffuse lung disease is the preferred term, as some diseases lumped into this group have involvement of the alveoli, i.e. are not interstitial. Also, some DLDs are not considered idiopathic, e.g. [[DIP]] is caused by [[smoking]].


An introduction to pulmonary pathology is found in the ''[[pulmonary pathology]]'' article.
An introduction to pulmonary pathology is found in the ''[[pulmonary pathology]]'' article.


=Overview - histologic classification=
=Overview=
==Specimens==
*Diffuse lung disease is best assessed on an open biopsy.
**These procedure are quite invasive; thus, not infrequently clinicians will try a transbrochial biopsy that ends-up providing in useful information.
 
===Sign out===
<pre>
LUNG, LEFT, TRANSBRONCHIAL BIOPSY:
- SMALL FRAGMENTS OF LUNG PARENCHYMA WITHOUT SIGNIFICANT CHANGES, SEE MICROSCOPIC
  DESCRIPTION AND COMMENT.
 
COMMENT:
The clinical findings are noted. The size and type of biopsy significantly limit the
interpretation.  Suspected diffuse lung disease (interstitial lung disease)
is best assessed on an open lung biopsy.
</pre>
====Micro====
The sections show small fragments of lung parenchyma. A very small amount of anthracotic
pigment is present. Interstitial fibrosis is not apparent. No granulomata are identified.
No nuclear atypia is identified. Air-space macrophages show minimal pigmentation and are
not present in large numbers. No significant airspace or interstitial inflammation is
identified.
 
==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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| [[Desquamative interstitial pneumonia]] (DIP)
| [[Desquamative interstitial pneumonia]] (DIP)
| DIP
| DIP
| Smoking
| [[smoking]]
|-
|-
| [[Diffuse alveolar damage]] (DAD)
| [[Diffuse alveolar damage]] (DAD)
Line 32: Line 56:
| [[Respiratory bronchiolitis]]  
| [[Respiratory bronchiolitis]]  
| [[Respiratory bronchiolitis-associated interstitial lung disease|RB-ILD]]
| [[Respiratory bronchiolitis-associated interstitial lung disease|RB-ILD]]
| Smoking
| smoking
|-
|-
| [[Usual interstitial pneumonia]] (UIP)
| [[Usual interstitial pneumonia]] (UIP)
| [[collagen vascular disease|CVD]], [[idiopathic pulmonary fibrosis|IPF]], [[drug toxicity]], [[pneumoconiosis]]
| [[collagen vascular disease|CVD]], [[idiopathic pulmonary fibrosis|IPF]], [[drug toxicity]], [[pneumoconiosis]]
| Allergen (hypersensitivity pneumonitis), idiopathic, autoimmune
| allergen (hypersensitivity pneumonitis), idiopathic, autoimmune
|-
|-
| [[Organizing pneumonia]]
| [[Organizing pneumonia]]
Line 44: Line 68:
| [[Lymphocytic interstitial pneumonia]] (LIP)
| [[Lymphocytic interstitial pneumonia]] (LIP)
| LIP
| LIP
| Viral/autoimmune
| viral/autoimmune
|}
|}
ARDS = adult respiratory distress syndrome, AIP = acute interstitial pneumonia, TRALI = transfusion related acute lung injury, CVD = [[collagen vascular disease]], IPF = idiopathic pulmonary fibrosis.
ARDS = adult respiratory distress syndrome, AIP = acute interstitial pneumonia, TRALI = transfusion related acute lung injury, CVD = [[collagen vascular disease]], IPF = idiopathic pulmonary fibrosis.


Notes:
Notes:
*''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>
==Gross pathologic DDx by location==
Causes of upper lung fibrosis ''FASSTEN'':<ref name=Ref_TN2005_R13>{{Ref TN2005| R13}}</ref>
*[[Farmer's lung]].
*[[Ankylosing spondylitis]].
*[[Sarcoidosis]].
*[[Silicosis]].
*[[Tuberculosis]] (miliary).
*[[Eosinophilic granuloma]].
*[[Neurofibromatosis]].
Causes of lower lung fibrosis ''BAD RASH'':<ref name=Ref_TN2005_R13>{{Ref TN2005| R13}}</ref>
*[[Bronchiolitis obliterans organizing pneumonia]] (BOOP).
*[[Asbestosis]].
*Drugs (nitrofurantoin, hydralazine, isoniazid (INH), amiodarone).
*[[Rheumatologic disease]].
*[[aspiration pneumonia|Aspiration]].
*[[Scleroderma]].
*Hamman-Rich syndrome (really should be -- ''[[idiopathic pulmonary fibrosis]]'').
Note:
*''Hamman-Rich syndrome'' is another name for [[acute interstitial pneumonia]].<ref name=Ref_WMSP90>{{Ref WMSP|90}}</ref>


=Specific diseases/pattterns=
=Specific diseases/pattterns=
Line 56: Line 101:
==Diffuse alveolar damage==
==Diffuse alveolar damage==
*Abbreviated ''DAD''.
*Abbreviated ''DAD''.
===General===
{{Main|Diffuse alveolar damage}}
Etiology:
*Abrupt hypoxemia with pulmonary infiltrates leading to epithelial cell and endothelial cell death not accompanied by cardiac failure.<ref name=Ref_PCPBoD8_364>{{Ref PCPBoD8|364}}</ref>
 
DAD is the histologic correlate of:
*Acute respiratory distress syndrome (ARDS).
**[[AKA]] adult respiratory distress syndrome (ARDS) to differentiate it from ''[[respiratory distress syndrome]]'' in infants.
*Acute interstitial pneumonia (AIP).
*Transfusion related acute lung injury (TRALI).
 
The DDx is broad:<ref>{{Ref WMSP|91}}</ref>
*Infection/sepsis.
*Toxic (smoke, oxygen).
*Drug (amiodarone, chemotherapy).
*Trauma/shock.
*Inflammatory.
*Idiopathic.
 
===Microscopic===
Features:<ref name=Ref_Klatt103>{{Ref Klatt|103}}</ref><ref name=pmid16766248>{{Cite journal  | last1 = Castro | first1 = CY. | title = ARDS and diffuse alveolar damage: a pathologist's perspective. | journal = Semin Thorac Cardiovasc Surg | volume = 18 | issue = 1 | pages = 13-9 | month =  | year = 2006 | doi = 10.1053/j.semtcvs.2006.02.001 | PMID = 16766248 }}</ref>
#Exudative:
#*Hyaline membranes - '''key feature'''.
#**Debris (pink crap) lines the alveolar spaces.
#Proliferative:
#*Interstitial thickening.
#*Inflammation (lymphocytes).
#*Hobnailing of alveolar lining cells (type 2 pneumocyte hyperplasia<ref>URL: [http://d3jonline.tripod.com/20-Pulmonary_II/Pathology_of_Interstitial_Lung_Diseases.htm http://d3jonline.tripod.com/20-Pulmonary_II/Pathology_of_Interstitial_Lung_Diseases.htm]. Accessed on: 22 February 2012.</ref>).
#*Edema (link pink crap in the alveoli).
#*Masson bodies in the airway.
#*Hyaline material (usu. focal) - '''key feature'''.
#Fibrotic:
#*Interstitial inflammation.
#*Fibrosis.
 
DDx:<ref name=pmid16766248>{{Cite journal  | last1 = Castro | first1 = CY. | title = ARDS and diffuse alveolar damage: a pathologist's perspective. | journal = Semin Thorac Cardiovasc Surg | volume = 18 | issue = 1 | pages = 13-9 | month =  | year = 2006 | doi = 10.1053/j.semtcvs.2006.02.001 | PMID = 16766248 }}</ref>
*[[Cryptogenic organizing pneumonia]] - especially for ''proliferative phase DAD''.
*[[Bronchiolitis obliterans]].
 
Images:
*[[WC]]:
**[http://commons.wikimedia.org/wiki/File:Hyaline_membranes_-_intermed_mag.jpg Exudative phase DAD - intermed. mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Hyaline_membranes_-_very_high_mag.jpg Exudative phase DAD - very high mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:ARDS.jpg Exudative DAD (WC)].
*www:
**[http://www.flickr.com/photos/pulmonary_pathology/4710141110/in/photostream/ Proliferative phase DAD - intermed. mag. (flickr.com/Yale Rosen)].
**[http://www.flickr.com/photos/pulmonary_pathology/4709499629/in/photostream/ Proliferative phase DAD - high mag. (flickr.com/Yale Rosen)].


==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==
===General===
{{Main|Asbestosis}}
Definition:
*Interstitial lung disease due to asbestos exposure.<ref name=Ref_PCPBoD8_375>{{Ref PCPBoD8|375}}</ref>
**Important to diagnose... asbestosis = compensation.
 
Conditions associated with asbestos exposure (mnemonic ''PALM''):<ref name=Ref_PCPBoD8_375>{{Ref PCPBoD8|375}}</ref>
*Pleural plaques.
*Asbestosis.
*[[Lung carcinoma]].
*[[Malignant mesothelioma]].
 
 
Possible association with asbestos exposure:
*[[Gestational trophoblastic disease]].<ref name=pmid19900938>{{Cite journal  | last1 = Reid | first1 = A. | last2 = Heyworth | first2 = J. | last3 = de Klerk | first3 = N. | last4 = Musk | first4 = AW. | title = Asbestos exposure and gestational trophoblastic disease: a hypothesis. | journal = Cancer Epidemiol Biomarkers Prev | volume = 18 | issue = 11 | pages = 2895-8 | month = Nov | year = 2009 | doi = 10.1158/1055-9965.EPI-09-0731 | PMID = 19900938 }}</ref>
 
Diagnosis:
*Rests on morphology with special techniques (e.g. polarization)<ref>{{Cite journal  | last1 = Galateau-Salle | first1 = F. | title = [Anatomopathological tools for screening and medical surveillance of people exposed to asbestos]. | journal = Rev Mal Respir | volume = 16 | issue = 6 Pt 2 | pages = 1244-56 | month = Dec | year = 1999 | doi =  | PMID = 10897845 }}</ref> and includes tunneling [[electron microscopy]] (TEM),<ref name=pmid21479897>{{Cite journal  | last1 = Neumann | first1 = V. | last2 = Löseke | first2 = S. | last3 = Tannapfel | first3 = A. | title = Mesothelioma and analysis of tissue fiber content. | journal = Recent Results Cancer Res | volume = 189 | issue =  | pages = 79-95 | month =  | year = 2011 | doi = 10.1007/978-3-642-10862-4_6 | PMID = 21479897 }}</ref> as done at special centres.
**Tissue is typically digested prior to fibre counting.<ref name=pmid20196674>{{Cite journal  | last1 = Roggli | first1 = VL. | last2 = Gibbs | first2 = AR. | last3 = Attanoos | first3 = R. | last4 = Churg | first4 = A. | last5 = Popper | first5 = H. | last6 = Cagle | first6 = P. | last7 = Corrin | first7 = B. | last8 = Franks | first8 = TJ. | last9 = Galateau-Salle | first9 = F. | title = Pathology of asbestosis- An update of the diagnostic criteria: Report of the asbestosis committee of the college of american pathologists and pulmonary pathology society. | journal = Arch Pathol Lab Med | volume = 134 | issue = 3 | pages = 462-80 | month = Mar | year = 2010 | doi = 10.1043/1543-2165-134.3.462 | PMID = 20196674 }}</ref>
 
===Microscopic===
Features:<ref name=pmid20196674/>
#Interstitial fibrosis - similar to [[usual interstitial pneumonia]] (UIP).
#''Ferruginous bodies'' - '''key feature'''.
#*Segmented twirling baton with long slender fibre within.
 
Images:
*[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==
==Non-specific interstitial pneumonia==
*Abbreviated ''NSIP''.
*Abbreviated ''NSIP''.
 
{{Main|Non-specific interstitial pneumonia}}
===General===
*Better prognosis than [[UIP]].
*Some radiologists and pathologists don't believe in this entity.
 
Associations:<ref name=Ref_WMSP92>{{Ref WMSP|92}}</ref>
*Connective tissue disease.
*[[Rheumatoid arthritis]].
 
===Gross/Radiology===
*No honeycombing.
*Fibrosis usually lower lung zone.
*Patchy ground glass.
 
===Microscopic===
Features:<ref name=Ref_WMSP92>{{Ref WMSP|92}}</ref>
*Diffuse fibrosis:
**Uniform fibrosis (unlike [[UIP]]).
**"Linear fibrosis" has a good prognosis - should be mentioned in the report.
***''Linear fibrosis'' = fibrosis that follows alveolar walls + no architectural distortion.
*+/-Lymphoid nodules - association with collagen vascular disease. (???)
*+/-Focal [[organizing pneumonia]].
 
Notes:
*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).
*[[Lymphocytic interstitial pneumonia]] (LIP) - much more inflammation.


==Organizing pneumonia==
==Organizing pneumonia==
===General===
{{Main|Organizing pneumonia}}
*Multiple causes, e.g. transplant rejection, infection.
 
Clinical diagnoses:<ref name=Ref_WMSP91>{{Ref WMSP|91}}</ref>
*[[Lung transplant pathology|Transplant rejection]].
*Cryptogenic organizing pneumonia (COP), [[AKA]] (idiopathic) bronchiolitis obliterans organizing pneumonia (BOOP).
**Should '''not''' be confused with ''[[constrictive bronchiolitis]]'' (AKA ''[[bronchiolitis obliterans]]'').
*[[Collagen vascular disease]].
*Toxic injury.
*Infection.
*Peri-tumor - in proximity to a space-occupying lesion (abscess, neoplasm).
 
Note:
*BOOP is used as a synonym for ''organizing pneumonia'' which has the long differential diagnosis above.<ref name=Ref_WMSP91>{{Ref WMSP|91}}</ref>
**Confusingly, it may be used to refer to the idiopathic form of organizing pneumonia, now generally known as ''cryptogenic organizing pneumonia'' (COP).
***In other words, strictly speaking, ''BOOP'' is '''not''' the same as ''COP''; idiopathic BOOP ''is'' COP.
 
===Microscopic===
Features:<ref name=Ref_Klatt110>{{Ref Klatt|110}}</ref>
*Distal airway disease -- airways plugged with organizing exudate ("Masson bodies").
**"Organized exudate" = fluffy light-staining paucicellular regions with stellate cells (fibroblasts & immature connective tissue).
*'''No''' hobnailing of pneumocytes.
**Type 2 pneumocytes hyperplasia is absent.
 
DDx:
*[[Diffuse alveolar damage]], proliferative phase - has type 2 pneumoncyte hyperplasia.
*[[Bronchiolitis obliterans]].
 
Images:
*www:
**[http://150.59.224.157/pathology/system/data/image_data/11338411170518.jpg Masson body (150.59.224.157)].<ref>URL: [http://150.59.224.157/pathology/index.php?first_category_id=2&second_category_id=20 http://150.59.224.157/pathology/index.php?first_category_id=2&second_category_id=20]. Accessed on: 4 August 2011.</ref>
**[http://casereports.bmj.com/content/2011/bcr.11.2010.3483.full BOOP (bmj.com)].
**[http://www.flickr.com/photos/pulmonary_pathology/4733384977/ Masson body (flickr.com)].
*[[WC]]:
**[http://commons.wikimedia.org/wiki/File:Masson_body_-_intermed_mag.jpg Masson body - intermed. mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Masson_body_-_very_high_mag.jpg Masson body - very high mag. (WC)].


==Hypersensitivity pneumonitis==
==Hypersensitivity pneumonitis==
*AKA ''extrinsic allergic alveolitis''
*AKA ''extrinsic allergic alveolitis''
 
{{Main|Hypersensitivity pneumonitis}}
===General===
*Exposure to stuffs... e.g. moldy hay - ''Farmer's lung'', atypical mycobacteria - ''hot tub lung''.
*Upper lung predominant disease.
**Mnemonic ''FASSTEN'' - upper lung field: [[Farmer's lung]], [[Ankylosing spondylitis]], [[Sarcoidosis]], [[Silicosis]], [[Tuberculosis]] (miliary), [[Eosinophilic granuloma]], [[Neurofibromatosis]].
*An immune complex-mediated [[hypersensitivity]]<ref name=pmid8279154>{{Cite journal  | last1 = Kaltreider | first1 = HB. | title = Hypersensitivity pneumonitis. | journal = West J Med | volume = 159 | issue = 5 | pages = 570-8 | month = Nov | year = 1993 | doi =  | PMID = 8279154 | url = http://www.ncbi.nlm.nih.gov/pmc/articles/pmid/8279154/?tool=pubmed }}</ref> (Gell-Coombs classification type III).
**If chronic, may develop into a cell-mediated hypersensitivity (Gell-Coombs classification type IV).<ref>URL: [http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/pufrm.html http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/pufrm.html]. Accessed on: 4 December 2011.</ref>
 
===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>{{Cite journal  | last1 = Ohtani | first1 = Y. | last2 = Saiki | first2 = S. | last3 = Kitaichi | first3 = M. | last4 = Usui | first4 = Y. | last5 = Inase | first5 = N. | last6 = Costabel | first6 = U. | last7 = Yoshizawa | first7 = Y. | title = Chronic bird fancier's lung: histopathological and clinical correlation. An application of the 2002 ATS/ERS consensus classification of the idiopathic interstitial pneumonias. | journal = Thorax | volume = 60 | issue = 8 | pages = 665-71 | month = Aug | year = 2005 | doi = 10.1136/thx.2004.027326 | PMID = 16061708 }}</ref>
* Chronic interstitial inflammation consisting primarily of lymphocytes.
* Interstitial fibrosis.
* Air space involvement (alveolitis).
 
DDx:
*[[Usual interstitial pneumonia]].
 
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)].


==Lymphocytic interstitial pneumonia==
==Lymphocytic interstitial pneumonia==
*Often abbreviated '''''LIP'''''.
*Often abbreviated '''''LIP'''''.
===General===
{{Main|Lymphocytic interstitial pneumonia}}
*Associated with autoimmune disorders ([[rheumatoid arthritis]], [[pernicious anemia]], [[Sjoegren syndrome]])<ref>URL: [http://emedicine.medscape.com/article/299643-overview http://emedicine.medscape.com/article/299643-overview]. Accessed on: 2 June 2010.</ref> and immunodeficiency.<ref name=pmid16088689>{{cite journal |author=Nicholson AG |title=Lymphocytic interstitial pneumonia and other lymphoproliferative disorders in the lung |journal=Semin Respir Crit Care Med |volume=22 |issue=4 |pages=409–22 |year=2001 |month=August |pmid=16088689 |doi=10.1055/s-2001-17384 |url=}}</ref>
*Associated with viral infections ([[HIV]], EBV, human T-cell leukemia virus (HTLV) type 1).
**Considered an AIDS-defining diagnosis.<ref>{{Cite journal  | last1 = Turner | first1 = BJ. | last2 = Eppes | first2 = S. | last3 = McKee | first3 = LJ. | last4 = Cosler | first4 = L. | last5 = Markson | first5 = LE. | title = A population-based comparison of the clinical course of children and adults with AIDS. | journal = AIDS | volume = 9 | issue = 1 | pages = 65-72 | month = Jan | year = 1995 | doi =  | PMID = 7893443 }}</ref>
 
===Gross===
*Location: basilar predominance.
*Increased interstitial markings.
 
===Microscopic===
Features:<ref>URL: [http://emedicine.medscape.com/article/299643-diagnosis http://emedicine.medscape.com/article/299643-diagnosis]. Accessed on: 2 June 2010.</ref>
*Small mature lymphocytes (usually B cells)<ref name=afip_vol2_265>AFIP atlas of nontumour pathology. 2002. Vo. 2. P.265. ISBN 1-881041-79-4.</ref> in the interstitium of the lung.
*Plasma cells.
*+/-Lymphoid follicles.
 
Negatives:
*No [[vasculitis]].
*No [[necrosis]].
 
Images:
*[http://www.scielo.br/img/revistas/jbpneu/v34n2/en_a02fig01.jpg LIP (scielo.br)].
*[http://path.upmc.edu/cases/case176/micro.html LIP (upmc.edu)].
 
DDx:
*[[Lymphoma]].
*Follicular bronchitis/bronchiolitis.
*Nodular lymphoid hyperplasia.
**This is determined in part by radiology; it has nodules radiographically.
 
===Stains===
To exclude infection:<ref>URL: [http://path.upmc.edu/cases/case176/dx.html http://path.upmc.edu/cases/case176/dx.html]. Accessed on: 8 January 2012.</ref>
*[[GMS stain]] -ve.
*[[Ziehl-Neelsen stain]] -ve.
 
===IHC===
*May be done to exclude lymphoma.


==Respiratory bronchiolitis-interstitial lung disease==
==Respiratory bronchiolitis-interstitial lung disease==
*Abbreviated ''RB-ILD'' or ''RBILD''.
*Abbreviated ''RB-ILD'' or ''RBILD''.
*[[AKA]] ''respiratory bronchiolitis-associated interstitial lung disease''.
*[[AKA]] ''respiratory bronchiolitis-associated interstitial lung disease''.
 
{{Main|Respiratory bronchiolitis-interstitial lung disease}}
===General===
*May be an early version of ''[[DIP]]''.
*Associated with smoking.
 
===Microscopic===
Features:<ref name=Ref_PPP197-8>{{Ref PPP|197-8}}</ref>
*Brown pigmented airspace macrophages - ''smoker's macrophages'' - '''key feature'''.
*Inflammation of the terminal bronchioles.
 
Note:
*The histologic features of RBILD may be present peri-tumoural.
 
DDx:
*[[Desquamative interstitial pneumonia]].
 
Image:
*[http://www.nature.com/modpathol/journal/v19/n11/fig_tab/3800671f2.html RB-ILD (nature.com)].


==Desquamative interstitial pneumonia==
==Desquamative interstitial pneumonia==
*Abbreviated ''DIP''.
*Abbreviated ''DIP''.
===General===
{{Main|Desquamative interstitial pneumonia}}
*Thought to be advanced ''[[RBILD]]''.
*Strong association with smoking.<ref name=Ref_WMSP93>{{Ref WMSP|93}}</ref>
**Treatment: stop smoking.
 
===Microscopic===
Features:
*Abundant brown pigmented airspace macrophages - ''smoker's macrophages'' - '''key feature'''.
*Architecture preserved; "linear fibrosis".
 
Notes:
*Some fields of view may be indistinguishable from [[RBILD]].
 
DDx:
*Amiodarone toxicity.
*Fibrotic [[NSIP]].
 
Images:
*[http://pathhsw5m54.ucsf.edu/case27/dip27.html DIP (ucsf.edu)].
 
===Stains===
*Macrophages PAS +ve.


=See also=
=See also=

Latest revision as of 03:37, 18 April 2016

Diffuse lung diseases, abbreviated DLD, are a group of uncommon pathologies, also known as idiopathic interstitial pneumonias, abbreviated IIPs, and interstitial lung disease, abbreviated ILD.

They are a subgroup of the medical lung diseases.

Diffuse lung disease is the preferred term, as some diseases lumped into this group have involvement of the alveoli, i.e. are not interstitial. Also, some DLDs are not considered idiopathic, e.g. DIP is caused by smoking.

An introduction to pulmonary pathology is found in the pulmonary pathology article.

Overview

Specimens

  • Diffuse lung disease is best assessed on an open biopsy.
    • These procedure are quite invasive; thus, not infrequently clinicians will try a transbrochial biopsy that ends-up providing in useful information.

Sign out

LUNG, LEFT, TRANSBRONCHIAL BIOPSY:
- SMALL FRAGMENTS OF LUNG PARENCHYMA WITHOUT SIGNIFICANT CHANGES, SEE MICROSCOPIC
  DESCRIPTION AND COMMENT.

COMMENT:
The clinical findings are noted. The size and type of biopsy significantly limit the 
interpretation.  Suspected diffuse lung disease (interstitial lung disease) 
is best assessed on an open lung biopsy.

Micro

The sections show small fragments of lung parenchyma. A very small amount of anthracotic pigment is present. Interstitial fibrosis is not apparent. No granulomata are identified. No nuclear atypia is identified. Air-space macrophages show minimal pigmentation and are not present in large numbers. No significant airspace or interstitial inflammation is identified.

Histologic classification

Idiopathic interstitial pneumonia can be subclassified based on histologic appearance into the following patterns:[4][5]

Histology Clinical Correlates Associations
Desquamative interstitial pneumonia (DIP) DIP smoking
Diffuse alveolar damage (DAD) ARDS, AIP, TRALI ARDS: trauma, infection; TRALI: blood transfusion; AIP: viral (???)
Nonspecific interstitial pneumonia (NSIP) NSIP ???
Respiratory bronchiolitis RB-ILD smoking
Usual interstitial pneumonia (UIP) CVD, IPF, drug toxicity, pneumoconiosis allergen (hypersensitivity pneumonitis), idiopathic, autoimmune
Organizing pneumonia Cryptogenic organizing pneumonia autoimmune (???)
Lymphocytic interstitial pneumonia (LIP) LIP viral/autoimmune

ARDS = adult respiratory distress syndrome, AIP = acute interstitial pneumonia, TRALI = transfusion related acute lung injury, CVD = collagen vascular disease, IPF = idiopathic pulmonary fibrosis.

Notes:

  • Usual interstitial pneumonia is the most common type of ILD.[6]

Gross pathologic DDx by location

Causes of upper lung fibrosis FASSTEN:[7]

Causes of lower lung fibrosis BAD RASH:[7]

Note:

Specific diseases/pattterns

Diffuse alveolar damage

  • Abbreviated DAD.
Main article: Diffuse alveolar damage

Usual interstitial pneumonia

  • Abbreviated UIP.
Main article: Usual interstitial pneumonia

Asbestosis

Main article: Asbestosis

Non-specific interstitial pneumonia

  • Abbreviated NSIP.
Main article: Non-specific interstitial pneumonia

Organizing pneumonia

Main article: Organizing pneumonia

Hypersensitivity pneumonitis

  • AKA extrinsic allergic alveolitis
Main article: Hypersensitivity pneumonitis

Lymphocytic interstitial pneumonia

  • Often abbreviated LIP.
Main article: Lymphocytic interstitial pneumonia

Respiratory bronchiolitis-interstitial lung disease

  • Abbreviated RB-ILD or RBILD.
  • AKA respiratory bronchiolitis-associated interstitial lung disease.
Main article: Respiratory bronchiolitis-interstitial lung disease

Desquamative interstitial pneumonia

  • Abbreviated DIP.
Main article: Desquamative interstitial pneumonia

See also

References

  1. ↑ Nicholson AG (November 2002). "Classification of idiopathic interstitial pneumonias: making sense of the alphabet soup". Histopathology 41 (5): 381-91. PMID 12405906. http://www3.interscience.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0309-0167&date=2002&volume=41&issue=5&spage=381.
  2. ↑ Flaherty KR, King TE, Raghu G, et al (October 2004). "Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis?". Am. J. Respir. Crit. Care Med. 170 (8): 904-10. doi:10.1164/rccm.200402-147OC. PMID 15256390. http://ajrccm.atsjournals.org/cgi/pmidlookup?view=long&pmid=15256390.
  3. ↑ Kim DS, Collard HR, King TE (June 2006). "Classification and natural history of the idiopathic interstitial pneumonias". Proc Am Thorac Soc 3 (4): 285-92. doi:10.1513/pats.200601-005TK. PMID 16738191. http://pats.atsjournals.org/cgi/pmidlookup?view=long&pmid=16738191.
  4. ↑ Leslie KO, Wick MR. Practical Pulmonary Pathology: A Diagnostic Approach. Elsevier Inc. 2005. ISBN 978-0-443-06631-3.
  5. ↑ "American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, June 2001 and by the ERS Executive Committee, June 2001". Am. J. Respir. Crit. Care Med. 165 (2): 277-304. January 2002. PMID 11790668. http://ajrccm.atsjournals.org/cgi/pmidlookup?view=long&pmid=11790668.
  6. ↑ Visscher DW, Myers JL (June 2006). "Histologic spectrum of idiopathic interstitial pneumonias". Proc Am Thorac Soc 3 (4): 322-9. doi:10.1513/pats.200602-019TK. PMID 16738196. http://pats.atsjournals.org/cgi/pmidlookup?view=long&pmid=16738196.
  7. ↑ 7.0 7.1 Yeung, J.C.; Leonard, Blair J. N. (2005). The Toronto Notes 2005 - Review for the MCCQE and Comprehensive Medical Reference (2005 ed.). The Toronto Notes Inc. for Medical Students Inc.. pp. R13. ISBN 978-0968592854.
  8. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 90. ISBN 978-0781765275.
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