Difference between revisions of "Medical lung diseases"

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The '''medical lung diseases''' are a huge topic.  Most pathologists have little to do with 'em.  They are the domain of respirology.  An introduction to lung pathology is in the ''[[lung]]'' article, along with a general approach.
The '''medical [[lung]] diseases''' are a huge topic.  Most pathologists have little to do with 'em.  They are the domain of respirology.  An introduction to lung pathology is in the ''[[lung]]'' article, along with a general approach. Interstitial lung disease is dealt with in the ''[[diffuse lung diseases]]'' article.


This article includes a discussion about pulmonary hypertension, which may arise due to [[congenital heart disease]].
==Infectious pneumonia==
 
{{Main|Pneumonia}}
==Acute infectious pneumonia==
Includes:
This is seen by pathologists in autopsy from time-to-time.
*[[Acute infectious pneumonia]].
 
*[[Chronic infectious pneumonia]].
===Radiologic correlate===
*Others.
*Air space disease.
 
===Gross pathology===
*Consolidation (the lung parenchyma is firm) - best appreciated by running a finger over the cut surface from normal region to abnormal region.
 
===Microscopy===
Features:
*Alveoli packed with [[PMN]]s.
*+/-Clusters of bacteria - small dots or rods.
 
Image: [http://commons.wikimedia.org/wiki/File:Pneumonia_alveolus.jpg Normal alveoli & pneumonia (WC)].


==Asthma==
==Asthma==
===General===
{{Main|Asthma}}
*The bread and butter of respirology.
*Associated with atopy.
*Mast cells thought to play an important role.


===Microscopic===
==Emphysema==
Features:<ref>Klatt. AOP P.108.</ref>
:''Chronic obstructive pulmonary disease'', abbreviated ''COPD'', is dealt with in the ''emphysema'' article.
*Edema.
{{Main|Emphysema}}
*Mucous.
*+/-Smooth muscle hypertrophy.
*+/-Inflammation - especially with eosinophils.
*+/-Charcot-Leyden crystals (formed from eosinophilic granules).
**Sharp edge, diamond shaped, intense pink.
 
Images:
*[http://www.udel.edu/medtech/dlehman/medt372/Ch-lyd.html Charcot-Leyden crystals (udel.edu)].
*[http://www.som.tulane.edu/classware/pathology/medical_pathology/New_for_98/Lung_Review/Lung-26.html Charcot-Leyden crystals (tulane.edu)].
 
Notes:
*''Leyden'' in ''Charcot-Leyden'' is also seen written as ''Leiden''.


==Emphysema==
==Chronic bronchitis==
===General===
===General===
*Usually due to smoking.
*Often seen together with ''[[emphysema]]'' with which it is lumped together with in the term ''COPD''.
*May be associated with alpha-1 antitrypsin deficiency.
*It's a clinical diagnosis - criteria:<ref name=Ref_PCPBoD8_370>{{Ref PCPBoD8|370}}</ref>
**Cough with sputum for thee months in at least two consecutive years.
**No other cause identified.


===Gross===
Clinical:<ref name=Ref_PCPBoD8_370>{{Ref PCPBoD8|370}}</ref>
*Holes, usually upper lung field predominant.
*Blue bloater (carbon dioxide retainers)
*Develop ''[[cor pulmonale]]''.


===Microscopic===
===Microscopic===
Features:
Features:<ref name=Ref_PCPBoD8_370>{{Ref PCPBoD8|370}}</ref>
*Large alveoli.
*Mucous gland hypertrophy + mucinous secretions in airway.
*No interstitial thickening.
*Goblet cell metaplasia.
 
*Bronchiolar inflammation and fibrosis.
Image: [http://commons.wikimedia.org/wiki/File:Emphysema_low_mag.jpg Emphysema (WC)].


==Pulmonary edema==
==Pulmonary edema==
===General===
===General===
*Seen in a number of conditions, e.g. congestive heart failure.
*Seen in a number of conditions, e.g. [[congestive heart failure]].
 
===Gross===
Features - autopsy:
*Bubbles - when squeezed (due to surfactant).
*Heavy.


===Microscopic===
===Microscopic===
Features:<ref>Klatt. AOP P.102.</ref>
Features:<ref name=Ref_Klatt102>{{Ref Klatt|102}}</ref>
*Dilated capillaries.
*Dilated capillaries.
*Blood in airspace.
*Blood in airspace, focal.
*Plasma proteins in airspace - light pink acellular junk.
*Plasma proteins in airspace - light pink acellular junk.
*+/-Hemosiderin-laden macrophages (heart failure cells).
*+/-Hemosiderin-laden macrophages (known as ''heart failure cells'' in this context).
 
==Organizing pneumonia==
===General===
*Multiple causes, e.g. transplant rejection, infection.


Clinical diagnoses:
DDx:
*Transplant rejection.
*[[Pulmonary alveolar proteinosis]].
*Cryptogenic organizing pneumonia (COP).
*[[Pulmonary hemorrhage]] - abundant blood.
**[[AKA]] bronchiolitis obliterans organizing pneumonia (BOOP).


===Microscopic===
Images:
Features:<ref>Klatt. AOP P.110.</ref>
*[http://www.flickr.com/photos/pulmonary_pathology/4337007145/in/photostream Pulmonary edema (flickr.com/Yale Rosen)].
*Distal airway disease -- airways plugged with organizing exudate.
*[http://ect.downstate.edu/courseware/histopath_exercises/histo/cardioIIcase2slide11.html Heart failure cells (downstate.edu)].
**"Organized exudate" = fluffy light-staining paucicellular regions with stellate cells (fibroblasts?).


==Obliterative broncholitis==  
==Bronchiectasis==
===General===
{{Main|Bronchiectasis}}
*[[AKA]] ''bronchiolitis obliterans''.
*Not the same as ''Bronchiolitis obliterans organizing pneumonia (BOOP)''.


==Idiopathic interstitial pneumonia==
==Pulmonary hemorrhage==
*Often [[abbreviated]] '''IIP''', is a term used for a type of [[diffuse lung disease]].
{{Main|Pulmonary hemorrhage}}
**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.


===Histologic classification of IIP===
==Constrictive bronchiolitis==  
*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>
*[[AKA]] ''bronchiolitis obliterans'', [[AKA]] ''obliterative broncholitis'',<ref name=pmid16493150/> [[AKA]] bronchiolitis obliterans syndrome (BOS).<ref name=pmid18707652>{{Cite journal | last1 = Sato | first1 = M. | last2 = Keshavjee | first2 = S. | title = Bronchiolitis obliterans syndrome: alloimmune-dependent and -independent injury with aberrant tissue remodeling. | journal = Semin Thorac Cardiovasc Surg | volume = 20 | issue = 2 | pages = 173-82 | month = | year = 2008 | doi = 10.1053/j.semtcvs.2008.05.002 | PMID = 18707652 }}</ref>
{{Main|Constrictive bronchiolitis}}


[[Idiopathic]] interstitial pneumonia can be subclassified based on [[histologic]] appearance into the following patterns:<ref>Leslie KO, Wick MR. Practical Pulmonary Pathology: A Diagnostic Approach. Elsevier Inc. 2005. ISBN 978-0-443-06631-3.</ref><ref name=atsers>{{cite journal |author= |title=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 |journal=Am. J. Respir. Crit. Care Med. |volume=165 |issue=2 |pages=277-304 |year=2002 |month=January |pmid=11790668 |doi= |url=http://ajrccm.atsjournals.org/cgi/pmidlookup?view=long&pmid=11790668}}</ref>
==Diffuse lung diseases==
 
{{Main|Diffuse lung diseases}}
{| class="wikitable"
These are also known as ''idiopathic interstitial pneumonias''.
|-
! Histology
! Clinical Correlates
! Associations
|-
| [[Desquamative interstitial pneumonia]] (DIP)
| DIP
| Smoking
|-
| [[Diffuse alveolar damage]] (DAD)
| [[ARDS]], [[acute interstitial pneumonia|AIP]], [[Transfusion related acute lung injury|TRALI]]
| ARDS: trauma, infection; TRALI: blood transfusion; AIP: ???
|-
| [[Nonspecific interstitial pneumonia]] (NSIP)
| NSIP
| ???
|-
| [[Respiratory bronchiolitis]]
| [[Respiratory bronchiolitis-associated interstitial lung disease|RB-ILD]]
| Smoking
|-
| [[Usual interstitial pneumonia]] (UIP)
| [[collagen vascular disease|CVD]], [[idiopathic pulmonary fibrosis|IPF]], [[drug toxicity]], [[pneumoconiosis]]
| Allergen (hypersensitivity pneumonitis), idiopathic, autoimmune
|-
| [[Organizing pneumonia]]
| [[Cryptogenic organizing pneumonia]]
| autoimmune (???)
|-
| [[Lymphoid 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.<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>


==Fibrosis==
==Fibrosis==
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===Radiologic/gross pathologic DDx by location===
===Radiologic/gross pathologic DDx by location===
Causes of lower lung fibrosis ''BAD RASH'':<ref>TN05 R13.</ref>
Causes of lower lung fibrosis ''BAD RASH'':<ref name=Ref_TN2005_R13>{{Ref TN2005| R13}}</ref>
*Brochiolitis obliterans with organizing pneumonia (BOOP).  
*[[Bronchiolitis obliterans organizing pneumonia]] (BOOP).  
*Asbestosis.  
*[[Asbestosis]].  
*Drugs (nitrofurantoin, hydralazine, isoniazid (INH), amiodarone).  
*Drugs (nitrofurantoin, hydralazine, isoniazid (INH), amiodarone).  
*Rheumatologic disease.
*[[Rheumatologic disease]].
*Aspiration.
*[[aspiration pneumonia|Aspiration]].
*Scleroderma.  
*[[Scleroderma]].  
*Hamman-Rich syndrome (really should be -- ''interstital pulmonary fibrosis'').
*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>


Causes of upper lung fibrosis ''FASSTEN'':<ref>TN05 R13.</ref>
Causes of upper lung fibrosis ''FASSTEN'':<ref name=Ref_TN2005_R13>{{Ref TN2005| R13}}</ref>
*Farmer's lung.  
*[[Farmer's lung]].  
*Ankylosing spondylitis.
*[[Ankylosing spondylitis]].
*Sarcoidosis.
*[[Sarcoidosis]].
*Silicosis.
*[[Silicosis]].
*Tuberculosis (miliary).  
*[[Tuberculosis]] (miliary).  
*Eosinophilic granuloma.  
*[[Eosinophilic granuloma]].  
*Neurofibromatosis.
*[[Neurofibromatosis]].


===Prognosis===
===Prognosis===
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There are many of 'em.
There are many of 'em.


==Diffuse alveolar damage==
*[[Diffuse alveolar damage]].
===General===
*[[Usual interstitial pneumonia]].
*Abbreviated ''DAD''.
*[[Asbestosis]].
*[[Hypersensitivity pneumonitis]].


DAD is the histologic correlate of:
==Fibrosing pleuritis==
*Adult respiratory distress syndrome (ARDS).
{{Main|Fibrosing pleuritis}}
*Acute interstitial pneumonia (AIP).
*Transfusion related acute lung injury (TRALI).


===Microscopic===
=Lymphocytic lesions of the lung=
Features:<ref>Klatt. AOP P.103.</ref>
*Early:
**Hyaline membrane: debris (pink crap) lines the alveolar spaces.
*Intermediate:
**Macrophage proliferation.
*Late:
**Interstitial inflammation.
**Fibrosis.


Image: [http://commons.wikimedia.org/wiki/File:ARDS.jpg Diffuse alveolar damage (WC)].
{| class="wikitable sortable"
 
!Diagnosis
==Usual interstitial pneumonia==
!Key histologic feature
===General===
!Radiology
*It is sometimes used incorrectly as a synoym for ''idiopathic pulmonary fibrosis''.
!Other diagnostic
*Cannot be diagnosed via bronchoscopic or transbronchial biopsy.<ref>PPP P.186.</ref>
|-
 
|[[Lymphocytic interstitial pneumonia]]
===Epidemiology===
|interstitial lymphoid cells, usu. no nodules
*Disease of the old - rare in under 50 years old.<ref>AC UBC S.102.</ref>
|interstitial pattern
*Dismal prognosis - mean survival after diagnosis ~ 2.8 years.<ref name=pmid9445300/>
|
 
|-
===Differential diagnosis===
|[[Follicular bronchiolitis/bronchitis]]
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>
|lymphoid cell around bronchioles / bronchus, normal parenchyma
*Idiopathic pulmonary fibrosis.
|interstitial pattern
*Asbestosis - one ought to see ''ferruginous bodies''.
|
*Chronic hypersensitivity pneumonitis (extrinsic allergic alveolitis).
|-
*Collagen vascular disease.
|[[Nodular lymphoid hyperplasia]]
*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>
|abundant lymphoid cells in nodules
 
|nodules /interstitial pattern
===Radiologic===
|stains to exclude lymphoma; germinal centres do not exclude [[lymphoma]]
*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.
|Lymphoma ([[BALToma]])
**Classically lower lobe predominant.
|abundant lymphoid cells usu. in nodules
**Associated with interstitial thickening. (???)
|nodules / interstitial pattern
 
|may require stains to prove, germinal centres may be present
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>
 
===Histology===
Features:<ref>PPP P.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>PPP P.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>AC 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)].


==Lymphocytic interstitial pneumonia==
==Lymphocytic interstitial pneumonia==
===General===
{{Main|Lymphocytic interstitial pneumonia}}
*Often abbreviated '''''LIP'''''.
*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>
*Associated with viral infections (HIV, EBV, human T-cell leukemia virus (HTLV) type 1).
 
===Gross===
*Basilar predominance.


===Microscopic===
==Follicular bronchitis/bronchiolitis==
Features:<ref>URL: [http://emedicine.medscape.com/article/299643-diagnosis http://emedicine.medscape.com/article/299643-diagnosis]. Accessed on: 2 June 2010.</ref>
{{Main|Follicular bronchitis/bronchiolitis}}
*Small mature lymphocytes.
*Plasma cells.
*+/-Lymphoid follicles.


Negatives:
==Pulmonary nodular lymphoid hyperplasia==
*No Vasculitis.
{{Main|Pulmonary nodular lymphoid hyperplasia}}
*No necrosis.


Image: [http://www.scielo.br/img/revistas/jbpneu/v34n2/en_a02fig01.jpg LIP (scielo.br)].
==Lymphoma of the lung==
{{Main|Lymphoma of the lung}}


=Smoking assoc. disease=
=[[Smoking]] associated disease=
*RB = respiratory bronchiolitis.
{{Main|Smoking}}
*RBILD = respiratory bronchiolitis interstitial lung disease.
*Respiratory bronchiolitis (RB).
*DIP = desquamative interstitial pneumonia.
*[[Respiratory bronchiolitis interstitial lung disease]] (RBILD).
*Eosinophilic granuloma (of lung) - [[AKA]] pulmonary langerhans cell histiocytosis.
*[[Desquamative interstitial pneumonia]] (DIP).
*Eosinophilic granuloma (of lung) - [[AKA]] [[pulmonary langerhans cell histiocytosis]].
*[[Smoking-related interstitial fibrosis]] (SRIF).


All of the above are assoc. with smoking.  RBILD & DIP are considered by many to be on a continuum, i.e. RBILD is early DIP.
All of the above are associated with smoking.  RBILD & DIP are considered by many to be on a continuum, i.e. RBILD is early DIP.


==Respiratory bronchiolitis==
==Respiratory bronchiolitis==
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*No interstitial lung disease, i.e. no fibrosis.
*No interstitial lung disease, i.e. no fibrosis.


==RBILD==
==Respiratory bronchiolitis interstitial lung disease==
===General===
{{Main|Respiratory bronchiolitis interstitial lung disease}}
*Respiratory bronchiolitis interstitial lung disease.
 
===Histology===
Features:<ref>PPP PP.197-8.</ref>
*Brown pigmented airspace macrophages - ''smoker's macrophages''.
*Inflammation of the terminal bronchioles.


Note:
==Desquamative interstitial pneumonia==
*The histologic features of RBILD may be present peri-tumoural.
{{Main|Desquamative interstitial pneumonia}}
 
==DIP==
*Desquamative interstitial pneumonia.
*Thought to be advanced RBILD.
 
===Histology===
*Brown pigmented airspace macrophages - ''smoker's macrophages''.
*Architecture preserved; "linear fibrosis".
 
Notes:
*Some fields of view may be indistinguishable from RBILD.
*Amiodarone toxicity, fibrotic NSIP - may appear similar.


==Pulmonary Langerhans cell histiocytosis==
==Pulmonary Langerhans cell histiocytosis==
===General===
*[[AKA]] eosinophilic granuloma of the lung.
*[[AKA]] eosinophilic granuloma of lung.
{{Main|Pulmonary Langerhans cell histiocytosis}}
*Associated with smoking.<ref name=ppp234>PPP P.234.</ref>
*Not assoc. with systemic diseases of Langerhans cells (AKA Hand-Schueller-Christian disease).
 
Subtypes:<ref name=ppp234/>
*Cellular form.
*Fibrotic form.
 
One form usually predominates.
 
===Radiology===
*Upper lung zones.
 
===Histology===
Features:<ref>PPP P.237.</ref>
*Cellular peribronchiolar nodules with:
**Langerhans cells - '''key feature''':
***Pale staining nucleus (H&E) with nuclear infolding - "crumpled tissue paper" appearance.
**+/-Smoker's macrophages (brown pigmented airspace macrophages).
**+/-Eosinophilia (may be rare) - '''significantly narrow DDx'''.
**Chronic inflammatory cells (lymphocytes). (???)
 
===IHC===
*Langerhans cells: S100+ and CD1a+.<ref>PPP P.237.</ref>


=Granulomatous lung disease=
=Granulomatous lung disease=
:See: ''[[Granulomas]]'' for an introduction to the general topic.
Most common:
Most common:
*Infectious - mycobacterial and fungal.<ref name=pmid20441499>{{cite journal |author=Mukhopadhyay S, Gal AA |title=Granulomatous lung disease: an approach to the differential diagnosis |journal=Arch. Pathol. Lab. Med. |volume=134 |issue=5 |pages=667–90 |year=2010 |month=May |pmid=20441499 |doi= |url=}}</ref>
*Infectious - mycobacterial and fungal.<ref name=pmid20441499>{{cite journal |author=Mukhopadhyay S, Gal AA |title=Granulomatous lung disease: an approach to the differential diagnosis |journal=Arch. Pathol. Lab. Med. |volume=134 |issue=5 |pages=667–90 |year=2010 |month=May |pmid=20441499 |doi= |url=}}</ref>
Line 400: Line 206:
*Talc granulomatosis.
*Talc granulomatosis.
*Sarcoidosis.  
*Sarcoidosis.  
*Wegener granulomatosis.
*[[Granulomatosis with polyangiitis]] (Wegener granulomatosis).


==Sarcoidosis==
==Sarcoidosis==
{{Main|Sarcoidosis}}
===General===
===General===
*Diagnosis of exclusion - infection must be excluded.
*Diagnosis of exclusion - infection must be excluded.
Line 410: Line 217:
Features:
Features:
*Granulomata, well-formed, non-necrotizing.
*Granulomata, well-formed, non-necrotizing.
**Negative for microorganisms with special stains (PAS-D, GMS, AFB).
**Negative for microorganisms with special stains ([[PAS-D]], [[GMS]], [[AFB]]).
**Granulomata - interstitial location.
**Granulomata - interstitial location.


Line 420: Line 227:
===General===
===General===
*Associated with herion use.<ref name=pmid6655726>{{Cite journal  | last1 = Davis | first1 = LL. | title = Pulmonary "mainline" granulomatosis: talcosis secondary to intravenous heroin abuse with characteristic x-ray findings of asbestosis. | journal = J Natl Med Assoc | volume = 75 | issue = 12 | pages = 1225–8 | month = Dec | year = 1983 | doi =  | PMID = 6655726 | PMC=2561715 | url=http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2561715/ }}</ref>
*Associated with herion use.<ref name=pmid6655726>{{Cite journal  | last1 = Davis | first1 = LL. | title = Pulmonary "mainline" granulomatosis: talcosis secondary to intravenous heroin abuse with characteristic x-ray findings of asbestosis. | journal = J Natl Med Assoc | volume = 75 | issue = 12 | pages = 1225–8 | month = Dec | year = 1983 | doi =  | PMID = 6655726 | PMC=2561715 | url=http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2561715/ }}</ref>
*X-ray findings similar to asbestosis.
*Seen in drug users that [[IVDU|intravenously inject crushed pills]] intended to be taken PO.<ref name=pmid20155272>{{Cite journal  | last1 = Marchiori | first1 = E. | last2 = Lourenço | first2 = S. | last3 = Gasparetto | first3 = TD. | last4 = Zanetti | first4 = G. | last5 = Mano | first5 = CM. | last6 = Nobre | first6 = LF. | title = Pulmonary talcosis: imaging findings. | journal = Lung | volume = 188 | issue = 2 | pages = 165-71 | month = Apr | year = 2010 | doi = 10.1007/s00408-010-9230-y | PMID = 20155272 }}</ref>
*X-ray findings similar to [[asbestosis]].


===Microscopic===
===Microscopic===
Line 427: Line 235:
**Foreign material often polarizes.
**Foreign material often polarizes.


Images:
====Images====
*[http://commons.wikimedia.org/wiki/File:Pulmonary_talcosis_low_mag_cropped.jpg Pulmonary talcosis - low mag. cropped (WC)].
<gallery>
*[http://commons.wikimedia.org/wiki/File:Pulmonary_talcosis_low_mag.jpg Pulmonary talcosis - low mag. (WC)]
Image:Pulmonary_talcosis_low_mag_cropped.jpg | Pulmonary talcosis - low mag. cropped (WC)
Image:Pulmonary_talcosis_low_mag.jpg | Pulmonary talcosis - low mag. (WC)
</gallery>
www:
*[http://path.upmc.edu/cases/case698.html Pulmonary talcosis - several images (upmc.edu)].


=Miscellaneous diseases=
=Miscellaneous diseases=
==Pneumoconioses==
{{Main|Pneumoconioses}}
==Pneumocytoma==
==Pneumocytoma==
===General===
*[[AKA]] ''benign sclerosing pneumocytoma''.<ref name=pmid6291188>{{Cite journal  | last1 = Chan | first1 = KW. | last2 = Gibbs | first2 = AR. | last3 = Lo | first3 = WS. | last4 = Newman | first4 = GR. | title = Benign sclerosing pneumocytoma of lung (sclerosing haemangioma). | journal = Thorax | volume = 37 | issue = 6 | pages = 404-12 | month = Jun | year = 1982 | doi =  | PMID = 6291188 }}</ref>
*Previously known as ''sclerosing hemangioma''.
*Previously known as ''sclerosing hemangioma''.
*[[AKA]] ''sclerosing hemangioma''.
{{Main|Pneumocytoma}}
*Derived from type 2 pneumocyte.<ref name=pmid15138814>{{Cite journal  | last1 = Yamazaki | first1 = K. | title = Type-II pneumocyte differentiation in pulmonary sclerosing hemangioma: ultrastructural differentiation and immunohistochemical distribution of lineage-specific transcription factors (TTF-1, HNF-3 alpha, and HNF-3 beta) and surfactant proteins. | journal = Virchows Arch | volume = 445 | issue = 1 | pages = 45-53 | month = Jul | year = 2004 | doi = 10.1007/s00428-004-1023-3 | PMID = 15138814 }}</ref>
*Progesterone-receptor positive stromal cells.<ref name=pmid15731902>{{Cite journal  | last1 = Einsfelder | first1 = BM. | last2 = Müller | first2 = KM. | title = ["Pneumocytoma" or "sclerosing hemangioma": histogenetic aspects of a rare tumor of the lung] | journal = Pathologe | volume = 26 | issue = 5 | pages = 367-77 | month = Sep | year = 2005 | doi = 10.1007/s00292-005-0751-8 | PMID = 15731902 }}</ref>
 
===Epidemiology===
*Female in 40s.<ref name=pmid19415961>{{Cite journal  | last1 = Keylock | first1 = JB. | last2 = Galvin | first2 = JR. | last3 = Franks | first3 = TJ. | title = Sclerosing hemangioma of the lung. | journal = Arch Pathol Lab Med | volume = 133 | issue = 5 | pages = 820-5 | month = May | year = 2009 | doi =  | PMID = 19415961 }}</ref>
*Considered benign; excision is curative.
**Rare case reports of metastases.
 
===Gross===
*Peripheral, solitary.
*Well-circumscribed.
 
===Microscopy===
Features:<ref name=pmid19415961>{{Cite journal  | last1 = Keylock | first1 = JB. | last2 = Galvin | first2 = JR. | last3 = Franks | first3 = TJ. | title = Sclerosing hemangioma of the lung. | journal = Arch Pathol Lab Med | volume = 133 | issue = 5 | pages = 820-5 | month = May | year = 2009 | doi =  | PMID = 19415961 }}</ref>
*Mixed cell population.
*Variable architecture:
**Papillary.
**Sclerotic.
**Solid.
**Hemorrhagic.
* +/-Granulomas.
 
DDx:<reF>URL: [http://www.med.muni.cz/biomedjournal/pdf/2004/01/37_42.pdf http://www.med.muni.cz/biomedjournal/pdf/2004/01/37_42.pdf]. Accessed on: 17 June 2010.</ref>
*Papillary adenoma.
*Neuroendocrine tumour (carcinoid).
 
===IHC===
Features:<ref name=pmid15138814/>
*TTF-1 +ve.
*HNF-3 alpha +ve.
*HNF-3 beta +ve.


==Lymphangioleiomyomatosis==
==Lymphangioleiomyomatosis==
===General===
*Abbreviated ''LAM''.
*Abbreviated ''LAM''.
*Clinical: dyspnea, recurrent pneumothorax.
*[[AKA]] lymphangiomyomatosis.
*May be an indication for lung transplantation.
{{Main|Lymphangioleiomyomatosis}}
 
===Epidemiology===
*Associated with [[angiomyolipoma]]s.<ref name=emedicine299545>[http://emedicine.medscape.com/article/299545-overview http://emedicine.medscape.com/article/299545-overview]</ref>
*Associated with [[tuberous sclerosis]]<ref name=emedicine299545/> - abnormality in same gene (TSC2).
*Only affects women - primarily in childbearing years.
*Rare.
 
===Radiology===
*Bullae/thin walled cysts - distributed in all lung fields.
*Lymphadenopathy.
 
Radiologic DDx (of cysts):
*Eosinophilic granuloma (assoc. with smoking).
*Interstitial pulmonary fibrosis (UIP).
*Emphysema.
 
===Histology===
Features:<ref>[http://emedicine.medscape.com/article/299545-diagnosis http://emedicine.medscape.com/article/299545-diagnosis]</ref>
*Spindle cells with small nuclei + larger epithelioid cells with clear cytoplasm and round nuclei.
*Cyst formation.
*Thick arterial walls.
 
IHC:
*HMB-45 +ve
*ER +ve
*PR +ve
*SMA +ve


==Pulmonary alveolar proteinosis==
==Pulmonary alveolar proteinosis==
*Abbreviated ''PAP''.
*Abbreviated ''PAP''.
*Associated with smoking - particularily in men.<ref name=pmid14695413>{{cite journal |author=Trapnell BC, Whitsett JA, Nakata K |title=Pulmonary alveolar proteinosis |journal=N. Engl. J. Med. |volume=349 |issue=26 |pages=2527-39 |year=2003 |month=December |pmid=14695413 |doi=10.1056/NEJMra023226 |url=http://content.nejm.org/cgi/content/extract/349/26/2527}}</ref>
{{Main|Pulmonary alveolar proteinosis}}


Pathophysiology:
==Diffuse panbronchiolitis==
*GM-CSF (granulocyte-macrophage colony stimulating factor) signaling in macrophages/lack of GM-CSF.
*Abbreviated ''DPB''.
**GM-CSF is required by alveolar macrophages to clear surfactant.
{{Main|Diffuse panbronchiolitis}}


Classification:<ref name=pmid14695413/>
==Pulmonary amyloidosis==
#Congenital:
{{Main|Amyloidosis}}
#**Abnormal surfactant.
#**GM-CSF receptor defect.
#Secondary:
#*Infections.
#*Haematologic malignancy.
#Acquired:
#*Dusts - interfere with macrophage function.


Clinical:
===General===
*Dyspnea & cough - gradual onset.
*Rare.<ref name=pmid22692971>{{Cite journal  | last1 = Hagmeyer | first1 = L. | last2 = Stieglitz | first2 = S. | last3 = Röcken | first3 = C. | last4 = Randerath | first4 = W. | title = [Amyloidosis in Pneumology.] | journal = Pneumologie | volume =  | issue =  | pages =  | month = Jun | year = 2012 | doi = 10.1055/s-0032-1309811 | PMID = 22692971 }}</ref>


===Radiology===
===Microscopic===
*CXR: airspace disease.
Features:
*HRCT: "crazy paving" - see: [http://radiographics.rsnajnls.org/cgi/content/figsonly/23/6/1509 http://radiographics.rsnajnls.org/cgi/content/figsonly/23/6/1509].
*Interstitial cotton candy-like material - see ''[[amyloidosis]]''.


===Histology===
DDx:
*Crap in alveoli.
*[[Diffuse lung diseases]].
*"Dense bodies" - dead macrophages ("Chatter" in the alveoli).
**Edema - has pink stuff in the alveoli like PAP but no ''dense bodies''.


DDx - may mimic:
Images:
*Edema.
*[http://pathlabmed.typepad.com/surgical_pathology_and_la/2010/05/digital-case-challenge-pleuropulmonary-amyloidosis.html Pleuropulmonary amyloidosis  - several images (pathlabmed.typepad.com)].
*Pneumocystis.


==Drug reactions==
==Drug reactions==
Line 541: Line 287:


=Pulmonary hypertension=
=Pulmonary hypertension=
{{Main|Pulmonary hypertension}}
General classification:
General classification:
*Primary, i.e. ''primary pulmonary hypertension'', or
*Primary, i.e. ''primary pulmonary hypertension'', or
Line 546: Line 294:


==Non-secondary pulmonary hypertension==
==Non-secondary pulmonary hypertension==
{{Main|Pulmonary hypertension}}
Causes:<ref name=pmid16263465>{{cite journal |author=Bush A |title=Pulmonary hypertensive diseases |journal=Paediatr Respir Rev |volume=1 |issue=4 |pages=361-7 |year=2000 |month=December |pmid=16263465 |doi=10.1053/prrv.2000.0077 |url=}}</ref>
Causes:<ref name=pmid16263465>{{cite journal |author=Bush A |title=Pulmonary hypertensive diseases |journal=Paediatr Respir Rev |volume=1 |issue=4 |pages=361-7 |year=2000 |month=December |pmid=16263465 |doi=10.1053/prrv.2000.0077 |url=}}</ref>
*Primary pulmonary hypertension.
*Primary pulmonary hypertension.
*Pulmonary embolic disease (thromboembolism, and non-thrombotic embolism).
*[[Pulmonary embolism|Pulmonary embolic disease]] (thromboembolism, and non-thrombotic embolism).
*Pulmonary capillary haemangiomatosis (PCH).
*[[Pulmonary capillary haemangiomatosis]] (PCH).
*Pulmonary veno-occlusive disease (PVOD).
*[[Pulmonary veno-occlusive disease]] (PVOD).  
 
Notes:
*Some people consider PCH and PVOD to the be same thing.<ref name=pmid16819327>{{cite journal |author=Lantuéjoul S, Sheppard MN, Corrin B, Burke MM, Nicholson AG |title=Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis: a clinicopathologic study of 35 cases |journal=Am. J. Surg. Pathol. |volume=30 |issue=7 |pages=850-7 |year=2006 |month=July |pmid=16819327 |doi=10.1097/01.pas.0000209834.69972.e5 |url=}}</ref>
**Both have a poor prognosis.
**Clinically they present the same way.
*PVOD is based on case reports - it is extremely rare.<ref name=pmid3342678>{{cite journal |author=Vevaina JR, Mark EJ |title=Thoracic hemangiomatosis masquerading as interstitial lung disease |journal=Chest |volume=93 |issue=3 |pages=657-9 |year=1988 |month=March |pmid=3342678 |doi= |url=}}</ref>
 
===Primary pulmonary hypertension===
*AKA ''pulmonary plexogenic arteriopathy''.<ref name=dccpad>Lie JT, Silver MD. Diagnostic criteria of cardiovascular pathology: acquired diseases. ISBN 0-397-51630-4. PP.208-9.</ref>
*Like chronic pulmonary hypertension due to congenital heart disease but ''without'' the congenital heart disease.<ref name=dccpad/>
**Classified by ''Heath-Edwards classification'' (see below) into six grades.
 
===Pulmonary veno-occlusive disease (PVOD)===
Features:<ref>PPP PP.393-6.</ref>
*Clinical - gradual dyspnea +/- non-productive cough, +/- clubbing.
*Thrombosis - small veins & venules, particularily at the interlobular septae.
*Associated with mild homogenous peripheral interstitial fibrosis.
 
DDx: chronic interstitial pneumonia.
 
===Pulmonary capillary hemangiomatosis (PCH)===
General:
*First reported in 1978 by Wagenvoort et al..<ref name=pmid730121>{{cite journal |author=Wagenvoort CA, Beetstra A, Spijker J |title=Capillary haemangiomatosis of the lungs |journal=Histopathology |volume=2 |issue=6 |pages=401-6 |year=1978 |month=November |pmid=730121 |doi= |url=}}</ref>
 
Features:
*Proliferating and invasive capillaries.<ref name=pmid3770733>{{cite journal |author=Tron V, Magee F, Wright JL, Colby T, Churg A |title=Pulmonary capillary hemangiomatosis |journal=Hum. Pathol. |volume=17 |issue=11 |pages=1144-50 |year=1986 |month=November |pmid=3770733 |doi= |url=}}</ref>
*Demonstrated by CD34 immunostaining.<ref name=pmid16819327/>
*'''Dilated capillaries'''<ref>MC August 2009.</ref><ref>PPP PP.396-7.</ref> - key feature.
 
DDx:
*Passive congestion (PC).
**Differentiated by fact that PCH has multiple channels in alveolar wall (PC has only one).


==Chronic pulmonary hypertension due to congenital heart disease==
===Severity===
*Graded using the ''Heath-Edwards system''.<ref name=pmid13573570>{{cite journal |author=HEATH D, EDWARDS JE |title=The pathology of hypertensive pulmonary vascular disease; a description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects |journal=Circulation |volume=18 |issue=4 Part 1 |pages=533-47 |year=1958 |month=October |pmid=13573570 |doi= |url=}}</ref>
*Heath-Edwards classification - see ''[[pulmonary hypertension]]''.
*A reason for open lung biopsy in children.<ref name=pmid11426747>{{cite journal |author=Jaklitsch MT, Linden BC, Braunlin EA, Bolman RM, Foker JE |title=Open-lung biopsy guides therapy in children |journal=Ann. Thorac. Surg. |volume=71 |issue=6 |pages=1779-85 |year=2001 |month=June |pmid=11426747 |doi= |url=}}</ref>
 
===Heath-Edwards classification===
Definition:<ref name=pmid13573570>{{cite journal |author=HEATH D, EDWARDS JE |title=The pathology of hypertensive pulmonary vascular disease; a description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects |journal=Circulation |volume=18 |issue=4 Part 1 |pages=533-47 |year=1958 |month=October |pmid=13573570 |doi= |url=}}</ref>
*Six grades - based on ''intimal reaction'' and ''media of arteries and arterioles'':
**Grade 1:
***Intima: no intimal reaction.
***Media: hypertrophied.
**Grade 2:
***Intima: '''cellular intimal reaction'''.
***Media: hypertrophied.
**Grade 3:
***Intima: '''fibrous & fibroelastic reaction''' + cellular intimal reaction.
***Media: hypertrophy +/- generalized dilation.
**Grade 4:
***Intima: '''"plexiform lesions"''' + fibrous & fibroelastic reaction, + cellular intimal reaction.
*****Plexiform lesions = multiple channels that are dilated, assoc. with loss of elastic laminae; thought to arise at branch points due to aberant WSS.<ref>[http://pathhsw5m54.ucsf.edu/overview/vessels.html http://pathhsw5m54.ucsf.edu/overview/vessels.html]</ref>
***Media: generalized dilation +/- '''local "dilation lesions"'''.
***Micrographs: [http://pathhsw5m54.ucsf.edu/overview/vessels.html Plexiform lesions (ucsf.edu)], [http://www.pvrireview.org/viewimage.asp?img=PVRIReview_2009_1_1_34_44882_u6.jpg Plexiform lesions (pvrireview.org)].
**Grade 5:
***Intima: as in Grade 4.
***Media: generalized dilation + local "dilation lesions" + '''pulmonary hemosiderosis'''.
**Grade 6:
***Intima: as in Grade 4.
***Media: generalized dilation + local "dilation lesions" + pulmonary hemosiderosis + '''necrotizing arteritis'''.
 
Notes:
*'''Bolded''' text - defining feature.
*It is discussed here: [http://www.pathology.or.kr/studygroup/cardiopulmonary/lecture/lenote/hka.htm http://www.pathology.or.kr/studygroup/cardiopulmonary/lecture/lenote/hka.htm].


=Eosinophilic pneumonia=
=Eosinophilic pneumonia=
Specific entities:<ref name=emedicine301070>[http://emedicine.medscape.com/article/301070-overview http://emedicine.medscape.com/article/301070-overview]</ref>
Specific entities:<ref name=emedicine301070>[http://emedicine.medscape.com/article/301070-overview http://emedicine.medscape.com/article/301070-overview]</ref>
*Churg-Strauss syndrome.
*[[Eosinophilic granulomatosis with polyangiitis]] (Churg-Strauss syndrome).
*Acute eosinophilic pneumonia.
*Acute eosinophilic pneumonia.
*Chronic eosinophilic pneumonia.
*Chronic eosinophilic pneumonia.
Line 624: Line 313:


Entities which may have eosinophilia as prominent feature:
Entities which may have eosinophilia as prominent feature:
*AIDS.
*[[AIDS]].
*Lymphoma.
*Lymphoma.
*Collagen vascular disease.
*[[Collagen vascular disease]].


==Churg-Strauss syndrome==
==Churg-Strauss syndrome==
Features ''GAFE'':
{{Main|Churg-Strauss syndrome}}
*'''G'''ranulomata.
 
*'''A'''sthma.
===Microscopic===
*'''F'''ever.
Features:
*'''E'''osinophilia.
*Small vessel [[vasculitis]].
*Abundant eosinophils.
*[[Granuloma]]s.


===General===
=Eosinophilic pleural effusions=
*AKA ''allergic granulomatous angiitis''.<ref name=emedicine333492>[http://emedicine.medscape.com/article/333492-overview http://emedicine.medscape.com/article/333492-overview]</ref>
*Definition: 10%+ eosinophils.<ref name=pmid12561030 >{{Cite journal  | last1 = Matthai | first1 = SM. | last2 = Kini | first2 = U. | title = Diagnostic value of eosinophils in pleural effusion: a prospective study of 26 cases. | journal = Diagn Cytopathol | volume = 28 | issue = 2 | pages = 96-9 | month = Feb | year = 2003 | doi = 10.1002/dc.10227 | PMID = 12561030 }}</ref>
*Small vessel vasculitis.
*Uncommon  5-16% of effusions.<ref name=pmid15220754>{{Cite journal  | last1 = Kalomenidis | first1 = I. | last2 = Light | first2 = RW. | title = Pathogenesis of the eosinophilic pleural effusions. | journal = Curr Opin Pulm Med | volume = 10 | issue = 4 | pages = 289-93 | month = Jul | year = 2004 | doi =  | PMID = 15220754 }}</ref>
*Similar to ''Wegener's granulomatosis'' (classically c-ANCA +ve) and ''microscopic polyangiitis'' (a form of ''polyarteritis nodosa'').<ref>[http://emedicine.medscape.com/article/334024-overview http://emedicine.medscape.com/article/334024-overview]</ref>
 
Causes - mnemonic ''I'M PAID'':<ref name=pmid15220754/>
*Infection, e.g. [[tuberculosis]].
*Malignancy - uncommon.
*Pulmonary emboli.
*Asbestos exposure.
*Inflammatory diseases.
*Drug reactions.
 
=Lung transplant pathology=
{{Main|Lung transplant pathology}}
This subspecialty is dealt with in its own article.


=See also=
=See also=
Line 651: Line 353:
*[http://www.pilotforipf.org/image_library.php Pulmonary Fibrosis Idenification Lessons for Optimizing Treatment (pilotforipf.org)].
*[http://www.pilotforipf.org/image_library.php Pulmonary Fibrosis Idenification Lessons for Optimizing Treatment (pilotforipf.org)].


[[Category:Lung pathology]]
[[Category:Pulmonary pathology]]

Latest revision as of 14:54, 23 March 2017

The medical lung diseases are a huge topic. Most pathologists have little to do with 'em. They are the domain of respirology. An introduction to lung pathology is in the lung article, along with a general approach. Interstitial lung disease is dealt with in the diffuse lung diseases article.

Infectious pneumonia

Main article: Pneumonia

Includes:

Asthma

Main article: Asthma

Emphysema

Chronic obstructive pulmonary disease, abbreviated COPD, is dealt with in the emphysema article.
Main article: Emphysema

Chronic bronchitis

General

  • Often seen together with emphysema with which it is lumped together with in the term COPD.
  • It's a clinical diagnosis - criteria:[1]
    • Cough with sputum for thee months in at least two consecutive years.
    • No other cause identified.

Clinical:[1]

Microscopic

Features:[1]

  • Mucous gland hypertrophy + mucinous secretions in airway.
  • Goblet cell metaplasia.
  • Bronchiolar inflammation and fibrosis.

Pulmonary edema

General

Gross

Features - autopsy:

  • Bubbles - when squeezed (due to surfactant).
  • Heavy.

Microscopic

Features:[2]

  • Dilated capillaries.
  • Blood in airspace, focal.
  • Plasma proteins in airspace - light pink acellular junk.
  • +/-Hemosiderin-laden macrophages (known as heart failure cells in this context).

DDx:

Images:

Bronchiectasis

Main article: Bronchiectasis

Pulmonary hemorrhage

Main article: Pulmonary hemorrhage

Constrictive bronchiolitis

  • AKA bronchiolitis obliterans, AKA obliterative broncholitis,[3] AKA bronchiolitis obliterans syndrome (BOS).[4]
Main article: Constrictive bronchiolitis

Diffuse lung diseases

Main article: Diffuse lung diseases

These are also known as idiopathic interstitial pneumonias.

Fibrosis

Histomorphological classification

  1. Hyaline membranes - glassy pink material lining airways & alveoli.
  2. Microscopic honeycombing - "holes" in the lung.
  3. Bronchiolization - ciliated (respiratory) epithelium in distal airway.
  4. Uniform alveolar septal thickening - septae look similar at low power.
  5. Peripheral lobular fibrosis - septae thickening peripheral, HRCT shows: irregular peripheral reticular opacities.[5]
    • Reticular = net-like.[6]
  6. Siderophages in alveoli - macrophages with hemosiderin the alveoli.
  7. Fibrinous pleuritis - peripheral only (based on imaging).
  8. Granulomata, non-necrotizing.
  9. Abundance of vacuolated cells.
  10. Chronic inflammation.
  11. Bronchiolocentric scarring - fibrosis concentrated around airway/assoc. with airway.

Radiologic/gross pathologic DDx by location

Causes of lower lung fibrosis BAD RASH:[7]

Note:

Causes of upper lung fibrosis FASSTEN:[7]

Prognosis

  • The pattern and severity of fibrosis seems to be the most important factors prognostically - more important than the underlying cause (ILD, CVD, drug reaction etc.).[9][10]

Patterns of fibrosis:

  • "Linear" - follows alveolar walls, no architectural distortion.
  • UIP-like (honeycombing).

Disease with fibrosis

There are many of 'em.

Fibrosing pleuritis

Main article: Fibrosing pleuritis

Lymphocytic lesions of the lung

Diagnosis Key histologic feature Radiology Other diagnostic
Lymphocytic interstitial pneumonia interstitial lymphoid cells, usu. no nodules interstitial pattern
Follicular bronchiolitis/bronchitis lymphoid cell around bronchioles / bronchus, normal parenchyma interstitial pattern
Nodular lymphoid hyperplasia abundant lymphoid cells in nodules nodules /interstitial pattern stains to exclude lymphoma; germinal centres do not exclude lymphoma
Lymphoma (BALToma) abundant lymphoid cells usu. in nodules nodules / interstitial pattern may require stains to prove, germinal centres may be present

Lymphocytic interstitial pneumonia

Main article: Lymphocytic interstitial pneumonia

Follicular bronchitis/bronchiolitis

Main article: Follicular bronchitis/bronchiolitis

Pulmonary nodular lymphoid hyperplasia

Main article: Pulmonary nodular lymphoid hyperplasia

Lymphoma of the lung

Main article: Lymphoma of the lung

Smoking associated disease

Main article: Smoking

All of the above are associated with smoking. RBILD & DIP are considered by many to be on a continuum, i.e. RBILD is early DIP.

Respiratory bronchiolitis

  • Diagnosis is based on clinical criteria.

Microscopic

Features:

  • Inflammation.
  • No interstitial lung disease, i.e. no fibrosis.

Respiratory bronchiolitis interstitial lung disease

Main article: Respiratory bronchiolitis interstitial lung disease

Desquamative interstitial pneumonia

Main article: Desquamative interstitial pneumonia

Pulmonary Langerhans cell histiocytosis

  • AKA eosinophilic granuloma of the lung.
Main article: Pulmonary Langerhans cell histiocytosis

Granulomatous lung disease

See: Granulomas for an introduction to the general topic.

Most common:

  • Infectious - mycobacterial and fungal.[11]

Noninfectious causes:[11]

Sarcoidosis

Main article: Sarcoidosis

General

  • Diagnosis of exclusion - infection must be excluded.
  • Radiologic differential diagnosis includes carcinomatosis.[12]

Microscopic

Features:

  • Granulomata, well-formed, non-necrotizing.
    • Negative for microorganisms with special stains (PAS-D, GMS, AFB).
    • Granulomata - interstitial location.

Image(s):

Pulmonary talcosis

General

Microscopic

Features:

  • Granulomas with foreign material.
    • Foreign material often polarizes.

Images

  • Pulmonary talcosis - low mag. cropped (WC)

  • Pulmonary talcosis - low mag. (WC)

www:

Miscellaneous diseases

Pneumoconioses

Main article: Pneumoconioses

Pneumocytoma

  • AKA benign sclerosing pneumocytoma.[15]
  • Previously known as sclerosing hemangioma.
Main article: Pneumocytoma

Lymphangioleiomyomatosis

  • Abbreviated LAM.
  • AKA lymphangiomyomatosis.
Main article: Lymphangioleiomyomatosis

Pulmonary alveolar proteinosis

  • Abbreviated PAP.
Main article: Pulmonary alveolar proteinosis

Diffuse panbronchiolitis

  • Abbreviated DPB.
Main article: Diffuse panbronchiolitis

Pulmonary amyloidosis

Main article: Amyloidosis

General

Microscopic

Features:

  • Interstitial cotton candy-like material - see amyloidosis.

DDx:

Images:

Drug reactions

  • Effects are often non-specific.

Website: http://www.pneumotox.com

Pulmonary hypertension

Main article: Pulmonary hypertension

General classification:

  • Primary, i.e. primary pulmonary hypertension, or
  • Secondary, e.g. due to congenital heart disease (like ventricular septal defect), interstitial pulmonary fibrosis.

Non-secondary pulmonary hypertension

Main article: Pulmonary hypertension

Causes:[17]

Severity

Eosinophilic pneumonia

Specific entities:[18]

Entities which may have eosinophilia as prominent feature:

Churg-Strauss syndrome

Main article: Churg-Strauss syndrome

Microscopic

Features:

Eosinophilic pleural effusions

  • Definition: 10%+ eosinophils.[19]
  • Uncommon 5-16% of effusions.[20]

Causes - mnemonic I'M PAID:[20]

  • Infection, e.g. tuberculosis.
  • Malignancy - uncommon.
  • Pulmonary emboli.
  • Asbestos exposure.
  • Inflammatory diseases.
  • Drug reactions.

Lung transplant pathology

Main article: Lung transplant pathology

This subspecialty is dealt with in its own article.

See also

References

  1. ↑ 1.0 1.1 1.2 Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 370. ISBN 978-1416054542.
  2. ↑ Klatt, Edward C. (2006). Robbins and Cotran Atlas of Pathology (1st ed.). Saunders. pp. 102. ISBN 978-1416002741.
  3. ↑ Cite error: Invalid <ref> tag; no text was provided for refs named pmid16493150
  4. ↑ Sato, M.; Keshavjee, S. (2008). "Bronchiolitis obliterans syndrome: alloimmune-dependent and -independent injury with aberrant tissue remodeling.". Semin Thorac Cardiovasc Surg 20 (2): 173-82. doi:10.1053/j.semtcvs.2008.05.002. PMID 18707652.
  5. ↑ http://www.rsna.org/Publications/rsnanews/may06/jrnl_may06.cfm
  6. ↑ http://dictionary.reference.com/browse/reticular
  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.
  9. ↑ Bjoraker JA, Ryu JH, Edwin MK, et al. (January 1998). "Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis". Am. J. Respir. Crit. Care Med. 157 (1): 199-203. PMID 9445300. http://ajrccm.atsjournals.org/cgi/content/full/157/1/199.
  10. ↑ AC UBC S.425.
  11. ↑ 11.0 11.1 Mukhopadhyay S, Gal AA (May 2010). "Granulomatous lung disease: an approach to the differential diagnosis". Arch. Pathol. Lab. Med. 134 (5): 667–90. PMID 20441499.
  12. ↑ URL: http://www.radiologyassistant.nl/en/46b480a6e4bdc. Accessed on: 23 May 2010.
  13. ↑ Davis, LL. (Dec 1983). "Pulmonary "mainline" granulomatosis: talcosis secondary to intravenous heroin abuse with characteristic x-ray findings of asbestosis.". J Natl Med Assoc 75 (12): 1225–8. PMC 2561715. PMID 6655726. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2561715/.
  14. ↑ Marchiori, E.; Lourenço, S.; Gasparetto, TD.; Zanetti, G.; Mano, CM.; Nobre, LF. (Apr 2010). "Pulmonary talcosis: imaging findings.". Lung 188 (2): 165-71. doi:10.1007/s00408-010-9230-y. PMID 20155272.
  15. ↑ Chan, KW.; Gibbs, AR.; Lo, WS.; Newman, GR. (Jun 1982). "Benign sclerosing pneumocytoma of lung (sclerosing haemangioma).". Thorax 37 (6): 404-12. PMID 6291188.
  16. ↑ Hagmeyer, L.; Stieglitz, S.; Röcken, C.; Randerath, W. (Jun 2012). "[Amyloidosis in Pneumology.]". Pneumologie. doi:10.1055/s-0032-1309811. PMID 22692971.
  17. ↑ Bush A (December 2000). "Pulmonary hypertensive diseases". Paediatr Respir Rev 1 (4): 361-7. doi:10.1053/prrv.2000.0077. PMID 16263465.
  18. ↑ http://emedicine.medscape.com/article/301070-overview
  19. ↑ Matthai, SM.; Kini, U. (Feb 2003). "Diagnostic value of eosinophils in pleural effusion: a prospective study of 26 cases.". Diagn Cytopathol 28 (2): 96-9. doi:10.1002/dc.10227. PMID 12561030.
  20. ↑ 20.0 20.1 Kalomenidis, I.; Light, RW. (Jul 2004). "Pathogenesis of the eosinophilic pleural effusions.". Curr Opin Pulm Med 10 (4): 289-93. PMID 15220754.

External links

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