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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}}
Includes:
*[[Acute infectious pneumonia]].
*[[Chronic infectious pneumonia]].
*Others.


==Acute infectious pneumonia==
==Asthma==
This is seem in autopsy from time-to-time.
{{Main|Asthma}}


===Radiologic correlate===
==Emphysema==
*Air space disease.
:''Chronic obstructive pulmonary disease'', abbreviated ''COPD'', is dealt with in the ''emphysema'' article.
{{Main|Emphysema}}


===Gross pathology===
==Chronic bronchitis==
*Consolidation - best appreciated by running a finger over the cut surface from normal region to abnormal region.
===General===
*Often seen together with ''[[emphysema]]'' with which it is lumped together with in the term ''COPD''.
*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.


===Microscopy===
Clinical:<ref name=Ref_PCPBoD8_370>{{Ref PCPBoD8|370}}</ref>
Features:
*Blue bloater (carbon dioxide retainers)
*Alveoli packed with PMNs.
*Develop ''[[cor pulmonale]]''.
*+/-Clusters of bacteria - small dots or rods.
 
===Microscopic===
Features:<ref name=Ref_PCPBoD8_370>{{Ref PCPBoD8|370}}</ref>
*Mucous gland hypertrophy + mucinous secretions in airway.
*Goblet cell metaplasia.
*Bronchiolar inflammation and fibrosis.


==Asthma==
==Pulmonary edema==
===General===
===General===
*The bread and butter of respirology.
*Seen in a number of conditions, e.g. [[congestive heart failure]].
*Associated with atopy.
 
*Mast cells thought to play an important role.
===Gross===
Features - autopsy:
*Bubbles - when squeezed (due to surfactant).
*Heavy.


===Microscopic===
===Microscopic===
Features:<ref>Klatt. AOP P.108.</ref>
Features:<ref name=Ref_Klatt102>{{Ref Klatt|102}}</ref>
*Edema.
*Dilated capillaries.
*Mucous.
*Blood in airspace, focal.
*+/-Smooth muscle hypertrophy.
*Plasma proteins in airspace - light pink acellular junk.
*+/-Inflammation - especially with eosinophils.
*+/-Hemosiderin-laden macrophages (known as ''heart failure cells'' in this context).
*+/-Charcot-Leyden crystals (formed from eosinophilic granules).
**Sharp edge, diamond shaped, intense pink.


Images:  
DDx:
*[http://www.udel.edu/medtech/dlehman/medt372/Ch-lyd.html Charcot-Leyden crystals (udel.edu)].
*[[Pulmonary alveolar proteinosis]].
*[http://www.som.tulane.edu/classware/pathology/medical_pathology/New_for_98/Lung_Review/Lung-26.html Charcot-Leyden crystals (tulane.edu)].
*[[Pulmonary hemorrhage]] - abundant blood.


Notes:
Images:
*Leyden in ''Charcot-Leyden'' is also seen written as ''Leiden''.
*[http://www.flickr.com/photos/pulmonary_pathology/4337007145/in/photostream Pulmonary edema (flickr.com/Yale Rosen)].
*[http://ect.downstate.edu/courseware/histopath_exercises/histo/cardioIIcase2slide11.html Heart failure cells (downstate.edu)].


==Idiopathic interstitial pneumonia==
==Bronchiectasis==
*Often [[abbreviated]] '''IIP''', is a term used for a type of [[diffuse lung disease]].
{{Main|Bronchiectasis}}
**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===
==Pulmonary hemorrhage==
*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>
{{Main|Pulmonary hemorrhage}}
 
[[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>
 
{| class="wikitable"
|-
! Histology
! Clinical Correlates
|-
| [[Desquamative interstitial pneumonia]] (DIP)
| DIP
|-
| [[Diffuse alveolar damage]] (DAD)
| [[ARDS]], [[acute interstitial pneumonia|AIP]], [[Transfusion related acute lung injury|TRALI]]
|-
| [[Nonspecific interstitial pneumonia]] (NSIP)
| NSIP
|-
| [[Respiratory bronchiolitis]]
| [[Respiratory bronchiolitis-associated interstitial lung disease|RB-ILD]]
|-
| [[Usual interstitial pneumonia]] (UIP)
| [[collagen vascular disease|CVD]], [[idiopathic pulmonary fibrosis|IPF]], [[drug toxicity]], [[pneumoconiosis]]
|-
| [[Organizing pneumonia]]
| [[Cryptogenic organizing pneumonia]]
|-
| [[Lymphoid interstitial pneumonia]] (LIP)
| LIP
|}
ARDS = adult respiratory distress syndrome, AIP = acute interstitial pneumonia, TRALI = transfusion related acute lung injury, CVD = collagen vascular disease, IPF = idiopathic pulmonary fibrosis.


==Constrictive bronchiolitis==
*[[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}}


Notes:
==Diffuse lung diseases==
*''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>
{{Main|Diffuse lung diseases}}
These are also known as ''idiopathic interstitial pneumonias''.


==Fibrosis==
==Fibrosis==
Line 97: Line 86:


===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===
Line 125: Line 117:
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]]
|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
|-
|}


===Epidemiology===
==Lymphocytic interstitial pneumonia==
*Disease of the old - rare in under 50 years old.<ref>AC UBC S.102.</ref>
{{Main|Lymphocytic interstitial pneumonia}}
*Dismal prognosis - mean survival after diagnosis ~ 2.8 years.<ref name=pmid9445300/>


===Differential diagnosis===
==Follicular bronchitis/bronchiolitis==
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>
{{Main|Follicular bronchitis/bronchiolitis}}
*Idiopathic pulmonary fibrosis,
*Asbestosis,
*Chronic hypersensitivity pneumonitis (extrinsic allergic alveolitis),
*Collagen vascular disease and
*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===
==Pulmonary nodular lymphoid hyperplasia==
*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>
{{Main|Pulmonary nodular lymphoid hyperplasia}}
**Usually subplural, i.e. peripheral lung.
**Classically lower lobe predominant.
**Assoc. with interstitial thickening ???


Note:
==Lymphoma of the lung==
*Cysts - have thin walls (think of emphysema, lymphangioleiomyomatosis et cetera).
{{Main|Lymphoma of the lung}}
**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===
=[[Smoking]] associated disease=
Features:<ref>PPP P.186-9.</ref>
{{Main|Smoking}}
*Fibroblast foci:
*Respiratory bronchiolitis (RB).
**"Crescent-shaped bulge" of fibroblasts -- a rounded projection of spindle cells into the airspace.
*[[Respiratory bronchiolitis interstitial lung disease]] (RBILD).
**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>
*[[Desquamative interstitial pneumonia]] (DIP).
**Note: Technically, ''fibroblast foci'' are composed of myofibroblasts.<ref>PPP P.189.</ref>
*Eosinophilic granuloma (of lung) - [[AKA]] [[pulmonary langerhans cell histiocytosis]].
*Interstitial inflammation,
*[[Smoking-related interstitial fibrosis]] (SRIF).
*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:
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.
*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.


==Non-specific interstitial pneumonia==
==Respiratory bronchiolitis==
*Abbreviated ''NSIP''.
*Diagnosis is based on clinical criteria.
*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===
===Microscopic===
*Fibrosis:
Features:
**May be uniform.
*Inflammation.
**"Linear fibrosis" has a good prognosis - should be mentioned in the report.
*No interstitial lung disease, i.e. no fibrosis.
***''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===
==Respiratory bronchiolitis interstitial lung disease==
*Collagen vascular disease.
{{Main|Respiratory bronchiolitis interstitial lung disease}}
*Drug reaction.
*Hypersensitivity pneumonitis (extrinic allergic alveolitis).


==Diseases with prominent bronchiolization==
==Desquamative interstitial pneumonia==
There are many.
{{Main|Desquamative interstitial pneumonia}}


==Pulmonary Langerhans cell histiocytosis==
==Pulmonary Langerhans cell histiocytosis==
===General===
*[[AKA]] eosinophilic granuloma of the lung.
*Assoc. with smoking.<ref name=ppp234>PPP P.234.</ref>
{{Main|Pulmonary Langerhans cell histiocytosis}}
*Not assoc. with systemic diseases of Langerhans cells (AKA Hand-Schueller-Christian disease).


Subtypes:<ref name=ppp234/>
=Granulomatous lung disease=
*Cellular form.
:See: ''[[Granulomas]]'' for an introduction to the general topic.
*Fibrotic form.
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>


One form usually predominantes.
Noninfectious causes:<ref name=pmid20441499/>
*Aspiration pneumonia.
*Hypersensitivity pneumonitis.
*Hot tub lung.
*Talc granulomatosis.
*Sarcoidosis.
*[[Granulomatosis with polyangiitis]] (Wegener granulomatosis).


===Histology===
==Sarcoidosis==
Features:<ref>PPP P.237.</ref>
{{Main|Sarcoidosis}}
*Langerhans cells:
===General===
**Pale staining nucleus (H&E) with nuclear infolding - "crumpled tissue paper" appearance.
*Diagnosis of exclusion - infection must be excluded.
*+/-Smoker's macrophages.
*Radiologic differential diagnosis includes carcinomatosis.<ref>URL: [http://www.radiologyassistant.nl/en/46b480a6e4bdc http://www.radiologyassistant.nl/en/46b480a6e4bdc]. Accessed on: 23 May 2010.</ref>
*+/-Eosinophilia.


===IHC===
===Microscopic===
*Langerhans cells: S100+ and CD1a+.<ref>PPP P.237.</ref>
Features:
 
*Granulomata, well-formed, non-necrotizing.
=Smoking assoc. disease=
**Negative for microorganisms with special stains ([[PAS-D]], [[GMS]], [[AFB]]).
*RB = respiratory bronchiolitis.
**Granulomata - interstitial location.
*RBILD = respiratory bronchiolitis interstitial lung disease.
*DIP = desquamative interstitial pneumonia.
*Eosinophilic granuloma (of lung) - AKA pulmonary langerhans cell histiocytosis.
 
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.


==Respiratory bronchiolitis==
Image(s):
*No interstitial lung disease.
*[http://commons.wikimedia.org/wiki/File:Asteroid_body_intermed_mag.jpg Sarcoidosis - lung (WC)].
*[http://commons.wikimedia.org/wiki/File:Asteroid_body_very_high_mag.jpg Granulomata in sarcoidosis with asteroid bodies (WC)].


==RBILD==
==Pulmonary talcosis==
===General===
===General===
*Respiratory bronchiolitis interstitial lung disease.
*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>
*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]].


===Histology===
===Microscopic===
Features:<ref>PPP PP.197-8.</ref>
Features:
*Brown pigmented airspace macrophages - ''smoker's macrophages''.
*Granulomas with foreign material.
*Inflammation of the terminal bronchioles.
**Foreign material often polarizes.


Note:  
====Images====
*The histologic features of RBILD may be present peri-tumoural.
<gallery>
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)].


==DIP==
=Miscellaneous diseases=
*Desquamative interstitial pneumonia.
==Pneumoconioses==
*Thought to be advanced RBILD.
{{Main|Pneumoconioses}}
 
===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.
 
==Eosinophilic granuloma of lung==
*Assoc. with smoking.
 
===Radiology===
*Upper lung zones.
 
===Histology===
*Cellular peribronchiolar nodules with:
**Eosinophils - may be rare.
**Langerhans cells.
**Smoker's macrophages (brown pigmented airspace macrophages).
**Chronic inflammatory cells (lymphocytes).
 
==Hypersensitivity pneumonitis==
*AKA ''extrinsic allergic alveolitis''
*Exposure to stuffs... e.g. moldy hay - ''Farmer's lung'', atypical mycobacteria - ''hot tub lung''.


===IHC===
==Pneumocytoma==
Nodules postive for:
*[[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>
*S-100.
*Previously known as ''sclerosing hemangioma''.
*CD1a.
{{Main|Pneumocytoma}}
 
=Miscellaneous diseases=


==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==
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=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
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==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:
===Severity===
*Passive congestion (PC).
*Heath-Edwards classification - see ''[[pulmonary hypertension]]''.
**Differentiated by fact that PCH has multiple channels in alveolar wall (PC has only one).


==Chronic pulmonary hypertension due to congenital heart disease==
=Eosinophilic pneumonia=
*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>
*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==
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.
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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=
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*[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]]
Retrieved from "https://librepathology.org/wiki/Medical_lung_diseases"