Medical lung diseases: Difference between revisions

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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. Interstitial lung disease is dealt with in the ''[[diffuse lung diseases]]'' article.
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.


==Acute infectious pneumonia==
==Infectious pneumonia==
This is seen by pathologists in autopsy from time-to-time.
{{Main|Pneumonia}}
 
Includes:
===Radiologic correlate===
*[[Acute infectious pneumonia]].
*Air space disease.
*[[Chronic infectious pneumonia]].
 
*Others.
===Gross pathology===
*Consolidation (the lung parenchyma is firm) - best appreciated by running a finger over the cut surface of the lung with a small-to-moderate amount of pressure.
 
===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.
*May be associated with atopy (allergies), medications (e.g. NSAIDs), occupational exposures.<ref name=Ref_PCPBoD8_370>{{Ref PCPBoD8|370}}</ref>
 
====Pathogenesis====
*T-cell response T<sub>H</sub>2 dominant.
 
Sequence:<ref name=Ref_PCPBoD8_370-2>{{Ref PCPBoD8|370-2}}</ref>
*Antigen T<sub>H</sub>2 lymphocyte -> IgE B-cell -> [[mast cell]] IgE Fc -> leukotrienes + other mediators -> bronchospasm, edema, leukocyte recruitment -> airway remodeling.
 
===Gross===
*Lung over-inflation.
*Mucous plugs.
*Focal resorption atelectasis,<ref name=Ref_PCPBoD8_363>{{Ref PCPBoD8|363}}</ref> i.e. collapse.
 
===Microscopic===
Features:<ref name=Ref_Klatt108>{{Ref Klatt|108}}</ref>
*Edema.
*Mucous (plugs).
*+/-Smooth muscle hypertrophy.
*+/-Inflammation - especially with eosinophils.
*+/-Charcot-Leyden crystals (formed from eosinophil granules -- breakdown product).
**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''.
*[[Pulmonary cytopathology]]: ''Curschmann's spirals'' - spiral-shaped mucous plugs.<ref name=pmid9812228>{{cite journal |author=Cenci M, Giovagnoli MR, Alderisio M, Vecchione A |title=Curschmann's spirals in sputum of subjects exposed daily to urban environmental pollution |journal=Diagn. Cytopathol. |volume=19 |issue=5 |pages=349–51 |year=1998 |month=November |pmid=9812228 |doi= |url=}}</ref>


==Emphysema==
==Emphysema==
===General===
:''Chronic obstructive pulmonary disease'', abbreviated ''COPD'', is dealt with in the ''emphysema'' article.
*Usually due to smoking.
{{Main|Emphysema}}
*May be associated with [[alpha-1 antitrypsin deficiency]].
*Often lumped together with ''[[chronic bronchitis]]'' and called ''chronic obstructive pulmonary disease (COPD)''.<ref name=Ref_PCPBoD8_386>{{Ref PCPBoD8|368}}</ref>
 
====Pathologic classification====
Based on morphology:<ref name=Ref_PCPBoD8_368>{{Ref PCPBoD8|368}}</ref>
#Centriacinar (centrilobular) - associated with heavy smoking.
#Panacinar (panlobular) - associated with alpha-1 antitrypsin deficiency.
#Distal (paraseptal) acinar - associated with spontaneous pneumothorax.
#Irregular - usu. insignificant.
 
*Q. Why does smoking lead to centriacinar emphysema?
*A. The bad stuff from smoking gets enters the acinus at the centre; ergo, this is the location of the most damage.
 
===Gross===
*Holes (blebs, bullae), usually upper lung field predominant.
*Lungs may overlap the heart.<ref name=Ref_PCPBoD8_369>{{Ref PCPBoD8|369}}</ref>
 
Notes:
*Bleb = (small) vesicle.<ref>URL: [http://dictionary.reference.com/browse/bleb http://dictionary.reference.com/browse/bleb]. Accessed on: 3 August 2011.</ref>
*Bulla = large vesicle.<ref>URL: [http://dictionary.reference.com/browse/bulla http://dictionary.reference.com/browse/bulla]. Accessed on: 3 August 2011.</ref>
 
===Microscopic===
Features:<ref name=Ref_PCPBoD8_369>{{Ref PCPBoD8|369}}</ref>
*Large alveoli.
*Thin septa (no interstitial thickening).
 
Image:
*[http://commons.wikimedia.org/wiki/File:Emphysema_low_mag.jpg Emphysema (WC)].


==Chronic bronchitis==
==Chronic bronchitis==
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Clinical:<ref name=Ref_PCPBoD8_370>{{Ref PCPBoD8|370}}</ref>
Clinical:<ref name=Ref_PCPBoD8_370>{{Ref PCPBoD8|370}}</ref>
*Blue bloater (carbon dioxide retainers)
*Blue bloater (carbon dioxide retainers)
*Develop cor pulmonale.
*Develop ''[[cor pulmonale]]''.


===Microscopic===
===Microscopic===
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==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 name=Ref_Klatt102>{{Ref Klatt|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==
DDx:
===General===
*[[Pulmonary alveolar proteinosis]].
*Multiple causes, e.g. transplant rejection, infection.
*[[Pulmonary hemorrhage]] - abundant blood.


Clinical diagnoses:
Images:
*Transplant rejection.
*[http://www.flickr.com/photos/pulmonary_pathology/4337007145/in/photostream Pulmonary edema (flickr.com/Yale Rosen)].
*Cryptogenic organizing pneumonia (COP), [[AKA]] bronchiolitis obliterans organizing pneumonia (BOOP).
*[http://ect.downstate.edu/courseware/histopath_exercises/histo/cardioIIcase2slide11.html Heart failure cells (downstate.edu)].
**Should '''not''' be confused with ''[[constrictive bronchiolitis]]'' (AKA ''[[bronchiolitis obliterans]]'').


===Microscopic===
==Bronchiectasis==
Features:<ref name=Ref_Klatt110>{{Ref Klatt|110}}</ref>
{{Main|Bronchiectasis}}
*Distal airway disease -- airways plugged with organizing exudate ("Masson bodies").
**"Organized exudate" = fluffy light-staining paucicellular regions with stellate cells (fibroblasts & immature connective tissue).


Images:
==Pulmonary hemorrhage==
*www:
{{Main|Pulmonary hemorrhage}}
**[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)].


==Constrictive bronchiolitis==  
==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>
*[[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>
===General===
{{Main|Constrictive bronchiolitis}}
*'''''Not''''' the same as ''[[bronchiolitis obliterans organizing pneumonia]] (BOOP)'' - in short:
**BOOP: clogs the airway, has Masson bodies, treated with steroids - good prognosis.
**Constrictive bronchiolitis: fibrosis around airways, crappy prognosis.
 
*No good treatment.
*Progressive.
 
Etiology/associations:<ref name=pmid16493150/>
*Post-infectious.
**Viral.
**Mycoplasma.
*Post-lung transplant.<ref name=pmid19896545/>
*Post-hematopoietic stem cell transplantation
**Associated with GVHD.<ref name=pmid19896545>{{Cite journal  | last1 = Chien | first1 = JW. | last2 = Duncan | first2 = S. | last3 = Williams | first3 = KM. | last4 = Pavletic | first4 = SZ. | title = Bronchiolitis obliterans syndrome after allogeneic hematopoietic stem cell transplantation-an increasingly recognized manifestation of chronic graft-versus-host disease. | journal = Biol Blood Marrow Transplant | volume = 16 | issue = 1 Suppl | pages = S106-14 | month = Jan | year = 2010 | doi = 10.1016/j.bbmt.2009.11.002 | PMID = 19896545 }}</ref>
*Connective tissue disease, mostly [[rheumatoid arthritis]].
*[[Ulcerative colitis]].
*Drugs - penicillamine.
*Toxins.
*Idiopathic.
 
===Microscopic===
Features:<ref name=pmid16493150>{{Cite journal  | last1 = Visscher | first1 = DW. | last2 = Myers | first2 = JL. | title = Bronchiolitis: the pathologist's perspective. | journal = Proc Am Thorac Soc | volume = 3 | issue = 1 | pages = 41-7 | month =  | year = 2006 | doi = 10.1513/pats.200512-124JH | PMID = 16493150 | url = http://pats.atsjournals.org/cgi/content/full/3/1/41 }}</ref>
#Bronchiolitis - mononuclear and neutrophilic.
#*May be minimal late in the disease.
#Fibrosis - submucosal and peribronchiolar.
#*Can be patchy.
 
Notes:
*Bronchioles = noncartilagenous airways usu. < 2 mm in diameter.<ref name=pmid16493150/>
 
Images:
*[http://pats.atsjournals.org/cgi/content/full/3/1/41/FIG4 Constrictive bronchiolitis (atsjournals.org)].
 
===Stains===
*Elastic trichrome - useful for delineation of obliterated bronchioles.


==Diffuse lung diseases==
==Diffuse lung diseases==
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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>
*[[Bronchiolitis obliterans 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]]'').


Causes of upper lung fibrosis ''FASSTEN'':<ref>TN05 R13.</ref>
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 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]].
Line 224: Line 121:
*[[Asbestosis]].
*[[Asbestosis]].
*[[Hypersensitivity pneumonitis]].
*[[Hypersensitivity pneumonitis]].
==Fibrosing pleuritis==
{{Main|Fibrosing pleuritis}}


=Lymphocytic lesions of the lung=
=Lymphocytic lesions of the lung=


{| class="wikitable"
{| class="wikitable sortable"
|Diagnosis  
!Diagnosis  
|Key histologic feature
!Key histologic feature
|Radiology
!Radiology
|Other diagnostic
!Other diagnostic
|-
|-
|[[Lymphocytic interstitial pneumonia]]  
|[[Lymphocytic interstitial pneumonia]]  
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|
|
|-
|-
|Follicular bronchiolitis/bronchitis
|[[Follicular bronchiolitis/bronchitis]]
|lymphoid cell around bronchioles / bronchus, normal parenchyma
|lymphoid cell around bronchioles / bronchus, normal parenchyma
|interstitial pattern
|interstitial pattern
Line 248: Line 148:
|stains to exclude lymphoma; germinal centres do not exclude [[lymphoma]]
|stains to exclude lymphoma; germinal centres do not exclude [[lymphoma]]
|-
|-
|Lymphoma (BALToma)
|Lymphoma ([[BALToma]])
|abundant lymphoid cells usu. in nodules
|abundant lymphoid cells usu. in nodules
|nodules / interstitial pattern
|nodules / interstitial pattern
Line 259: Line 159:


==Follicular bronchitis/bronchiolitis==
==Follicular bronchitis/bronchiolitis==
===General===
{{Main|Follicular bronchitis/bronchiolitis}}
*Similar to [[lymphocytic interstitial pneumonia]] (LIP).
**Overlaps with LIP.<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 the things seen in LIP;<ref>{{cite journal |author=Aerni MR, Vassallo R, Myers JL, Lindell RM, Ryu JH |title=Follicular bronchiolitis in surgical lung biopsies: clinical implications in 12 patients |journal=Respir Med |volume=102 |issue=2 |pages=307–12 |year=2008 |month=February |pmid=17997299 |doi=10.1016/j.rmed.2007.07.032 |url=}}</ref> however, associations may be less clear in children.<ref name=pmid8404188>{{cite journal |author=Kinane BT, Mansell AL, Zwerdling RG, Lapey A, Shannon DC |title=Follicular bronchitis in the pediatric population |journal=Chest |volume=104 |issue=4 |pages=1183–6 |year=1993 |month=October |pmid=8404188 |doi= |url=}}</ref>
 
===Gross/radiology===
*No distinct nodule or mass.
*Classically: increased reticular marking, i.e. interstitial pattern.
 
Images: [http://pathhsw5m54.ucsf.edu/case31/image317.html Intersitial pattern - radiographs (ucsf.edu)].
===Microscopic===
Features:<ref name=afip_vol2_277>AFIP atlas of nontumour pathology. 2002. Vo. 2. P.277. ISBN 1-881041-79-4.</ref>
*Peribronchiolar/peribronchial lymphoid nodules with:
**Reactive germinal centres.
***Lack of these should raise suspicion for lymphoma.
**Plasma cells.
*+/-Lymphoid nodules in the interlobular septa.
 
Notes:
*Lung parenchyma distant from nodule = normal; no lymphocytic infiltrate.
 
DDx:
*[[Lymphoma]], specifically [[MALToma|MALTomas/BALTomas]].
*[[Lymphocytic interstitial pneumonia]].
*Nodular lymphoid hyperplasia.
**This is determined in part by radiology; it has nodules radiographically.


==Nodular lymphoid hyperplasia==
==Pulmonary nodular lymphoid hyperplasia==
===General===
{{Main|Pulmonary nodular lymphoid hyperplasia}}
*[[AKA]] pseudolymphoma - a term some dislike.<ref name=afip_vol2_277>AFIP atlas of nontumour pathology. 2002. Vo. 2. P.277. ISBN 1-881041-79-4.</ref>
*Definition - reactive lymphoid cells.


===Gross/radiology===
==Lymphoma of the lung==
*Has nodules radiographically.
{{Main|Lymphoma of the lung}}
 
===Microscopic===
Features:<ref name=afip_vol2_277>AFIP atlas of nontumour pathology. 2002. Vo. 2. P.277. ISBN 1-881041-79-4.</ref>
*Reactive lymphoid nodules.
 
Notes:<ref name=afip_vol2_281>AFIP atlas of nontumour pathology. 2002. Vo. 2. P.281. ISBN 1-881041-79-4.</ref>
*Presence of germinal centres do '''not''' exclude lymphoma - may still be a [[BALToma]].
**BALT = bronchial/bronchus associated lymphoid tissue.
 
DDx:
*[[Lymphoma]], specifically [[MALToma|MALTomas/BALTomas]].
*[[Lymphocytic interstitial pneumonia]].
*Follicular bronchiolitis.


=[[Smoking]] associated disease=
=[[Smoking]] associated disease=
{{Main|Smoking}}
{{Main|Smoking}}
*RB = respiratory bronchiolitis.
*Respiratory bronchiolitis (RB).
*RBILD = respiratory bronchiolitis interstitial lung disease.
*[[Respiratory bronchiolitis interstitial lung disease]] (RBILD).
*DIP = desquamative interstitial pneumonia.
*[[Desquamative interstitial pneumonia]] (DIP).
*Eosinophilic granuloma (of lung) - [[AKA]] pulmonary langerhans cell histiocytosis.
*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==
Line 332: Line 193:
==Pulmonary Langerhans cell histiocytosis==
==Pulmonary Langerhans cell histiocytosis==
*[[AKA]] eosinophilic granuloma of the lung.
*[[AKA]] eosinophilic granuloma of the lung.
===General===
{{Main|Pulmonary Langerhans cell histiocytosis}}
*Associated with smoking.<ref name=Ref_PPP234>{{Ref PPP|234}}</ref>
*Not associated with systemic diseases of Langerhans cells ([[AKA]] [[Langerhans cell histiocytosis|Hand-Schueller-Christian disease]]).
 
Subtypes:<ref name=Ref_PPP234/>
*Cellular form.
*Fibrotic form.
 
One form usually predominates.
 
===Radiology===
*Upper lung zones.
 
===Microscopic===
Features:<ref name=Ref_PPP237>{{Ref PPP|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 name=Ref_PPP237>{{Ref PPP|237}}</ref>


=Granulomatous lung disease=
=Granulomatous lung disease=
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*Talc granulomatosis.
*Talc granulomatosis.
*Sarcoidosis.  
*Sarcoidosis.  
*Wegener granulomatosis.
*[[Granulomatosis with polyangiitis]] (Wegener granulomatosis).


==Sarcoidosis==
==Sarcoidosis==
Line 389: 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 396: 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.
 
===Microscopic===
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==
*Abbreviated ''LAM''.
*Abbreviated ''LAM''.
*[[AKA]] lymphangiomyomatosis.
*[[AKA]] lymphangiomyomatosis.
 
{{Main|Lymphangioleiomyomatosis}}
===General===
*Clinical: dyspnea, recurrent pneumothorax.
*May be an indication for lung transplantation.
*Non-neoplastic muscle proliferation vs. tumour that can metastasize.<ref name=pmid20235883>{{Cite journal  | last1 = Taveira-DaSilva | first1 = AM. | last2 = Pacheco-Rodriguez | first2 = G. | last3 = Moss | first3 = J. | title = The natural history of lymphangioleiomyomatosis: markers of severity, rate of progression and prognosis. | journal = Lymphat Res Biol | volume = 8 | issue = 1 | pages = 9-19 | month = Mar | year = 2010 | doi = 10.1089/lrb.2009.0024 | PMID = 20235883 }}</ref>
 
Notes:
*Considered to be a [[PEComa]].
 
===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).
*Usually affects women - primarily in childbearing years; case reports of LAM in men - usu. with [[TSC]].<ref name=pmid17431222>{{Cite journal  | last1 = Schiavina | first1 = M. | last2 = Di Scioscio | first2 = V. | last3 = Contini | first3 = P. | last4 = Cavazza | first4 = A. | last5 = Fabiani | first5 = A. | last6 = Barberis | first6 = M. | last7 = Bini | first7 = A. | last8 = Altimari | first8 = A. | last9 = Cooke | first9 = RM. | title = Pulmonary lymphangioleiomyomatosis in a karyotypically normal man without tuberous sclerosis complex. | journal = Am J Respir Crit Care Med | volume = 176 | issue = 1 | pages = 96-8 | month = Jul | year = 2007 | doi = 10.1164/rccm.200610-1408CR | PMID = 17431222 }}</ref>
*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.
 
===Microscopic===
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.
 
Images:
*[http://www.nature.com/modpathol/journal/v19/n6/fig_tab/3800610f3.html LAM (nature.com)].
*[http://commons.wikimedia.org/wiki/File:Lymphangioleiomyomatosis_-_high_mag.jpg LAM - high mag. (WC)].
 
===IHC===
*HMB-45 +ve.
*ER +ve.
*PR +ve.
*SMA +ve.


==Pulmonary alveolar proteinosis==
==Pulmonary alveolar proteinosis==
*Abbreviated ''PAP''.
*Abbreviated ''PAP''.
===General===
{{Main|Pulmonary alveolar proteinosis}}
*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>


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====
*CXR: airspace disease.
*HRCT: "crazy paving" - see: [http://radiographics.rsnajnls.org/cgi/content/figsonly/23/6/1509 http://radiographics.rsnajnls.org/cgi/content/figsonly/23/6/1509].


===Microscopic===
===Microscopic===
Features:
Features:
*Crap in the alveoli:
*Interstitial cotton candy-like material - see ''[[amyloidosis]]''.
*"Dense bodies" - dead macrophages ("Chatter" in the alveoli).
**Edema - has pink stuff in the alveoli like PAP but no ''dense bodies''.


DDx - may mimic:<ref>{{Ref PPP|248}}</ref>
DDx:
*Edema.
*[[Diffuse lung diseases]].
*Pneumocystis - exudates foamy & vacuolated.
*Pulmonary hemorrhage (acute). (???)


Images:
Images:
*[http://jcp.bmjjournals.com/content/62/5/387/F23.large.jpg PAP (bmjjournals.com)].<ref name=pmid19398592>{{cite journal |author=Leslie KO |title=My approach to interstitial lung disease using clinical, radiological and histopathological patterns |journal=J. Clin. Pathol. |volume=62 |issue=5 |pages=387–401 |year=2009 |month=May |pmid=19398592 |pmc=2668105 |doi=10.1136/jcp.2008.059782 |url=}}</ref>
*[http://pathlabmed.typepad.com/surgical_pathology_and_la/2010/05/digital-case-challenge-pleuropulmonary-amyloidosis.html Pleuropulmonary amyloidosis  - several images (pathlabmed.typepad.com)].
*[http://commons.wikimedia.org/wiki/File:Pulmonary_alveolar_proteinosis_-_2_-_intermed_mag.jpg PAP - intermed. mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Pulmonary_alveolar_proteinosis_-_very_high_mag.jpg PAP - very high mag. (WC)].
 
Images of DDx:
*[http://www.sciencephoto.com/media/98443/enlarge Pulmonary edema (sciencephoto.com)].


==Drug reactions==
==Drug reactions==
Line 551: Line 307:
=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 557: 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==
Line 569: Line 325:
*Abundant eosinophils.
*Abundant eosinophils.
*[[Granuloma]]s.
*[[Granuloma]]s.
=Eosinophilic pleural effusions=
*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>
*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>
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=
=Lung transplant pathology=
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