Difference between revisions of "Medical lung diseases"

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==Asthma==
==Asthma==
:Cytopathology: ''[[Pulmonary_cytopathology#Asthma|Asthma in pulmonary cytopathology]]''
{{Main|Asthma}}
===General===
*The bread and butter of respirology.
*May be associated with atopy (allergies), medications (e.g. [[NSAID]]s), 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>
**''Creola bodies'' = clusters of (ciliated) bronchial epithelial cells.<ref name=pmid2818212>{{Cite journal  | last1 = Isohima | first1 = K. | last2 = Takahashi | first2 = K. | last3 = Soda | first3 = R. | last4 = Hukasaka | first4 = N. | last5 = Tanabe | first5 = K. | last6 = Ozaki | first6 = K. | last7 = Nakato | first7 = H. | last8 = Araki | first8 = M. | last9 = Tuzi | first9 = M. | title = [The clinical significance of Creola body in the sputum of asthmatic patients]. | journal = Arerugi | volume = 38 | issue = 7 | pages = 542-8 | month = Jul | year = 1989 | doi =  | PMID = 2818212 }}</ref>


==Emphysema==
==Emphysema==
:''Chronic obstructive pulmonary disease'', abbreviated ''COPD'', redirects here.
:''Chronic obstructive pulmonary disease'', abbreviated ''COPD'', is dealt with in the ''emphysema'' article.
===General===
{{Main|Emphysema}}
*Usually due to smoking.
*Often lumped together with ''[[chronic bronchitis]]'' and called ''chronic obstructive pulmonary disease (COPD)''.<ref name=Ref_PCPBoD8_386>{{Ref PCPBoD8|368}}</ref>
 
Causes of emphysema other than smoking:<ref name=pmid12546267>{{Cite journal  | last1 = Lee | first1 = P. | last2 = Gildea | first2 = TR. | last3 = Stoller | first3 = JK. | title = Emphysema in nonsmokers: alpha 1-antitrypsin deficiency and other causes. | journal = Cleve Clin J Med | volume = 69 | issue = 12 | pages = 928-9, 933, 936 passim | month = Dec | year = 2002 | doi =  | PMID = 12546267 | URL = http://www.ccjm.org/cgi/pmidlookup?view=long&pmid=12546267 }}</ref>
*[[Alpha-1 antitrypsin deficiency]]
*[[Connective tissue diseases]].
**[[Marfan syndrome]].
**[[Ehlers-Danlos syndrome]].
*[[IVDU]].
*[[HIV]].
 
====Pathologic classification====
Based on morphology:<ref name=Ref_PCPBoD8_368>{{Ref PCPBoD8|368}}</ref>
#Centriacinar (centrilobular) emphysema - associated with heavy smoking.
#Panacinar (panlobular) emphysema - associated with [[alpha-1 antitrypsin deficiency]].
#Distal (paraseptal) acinar emphysema - associated with spontaneous pneumothorax.
#Irregular emphysema - 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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==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===
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==Bronchiectasis==
==Bronchiectasis==
===General===
{{Main|Bronchiectasis}}
*Benign.
*Uncommon.
*Predisposes for infection.<ref name=Ref_PBoD8_693>{{Ref PBoD8|693}}</ref>
**Usually a mixed flora.
**May be predominantly fungal, e.g. ''allergic bronchopulmonary [[aspergillosis]] (ABPA)''.
*Multitude of causes - including:
**[[Cystic fibrosis]] - typically diffusely involvement, unlike other causes.<ref>URL: [http://library.med.utah.edu/WebPath/LUNGHTML/LUNG053.html http://library.med.utah.edu/WebPath/LUNGHTML/LUNG053.html]. Accessed on: 21 February 2012.</ref>
**[[Primary ciliary dyskinesia]].
 
===Gross===
*Large airways at the periphery of the lung.
*Central airways larger than the adjacent arteries.
*Typically focal.
 
Images:
*[http://library.med.utah.edu/WebPath/LUNGHTML/LUNG053.html Bronchiectasis (utah.edu)].
*[http://commons.wikimedia.org/wiki/File:Bronchiectasis.jpg Bronchiectasis (WC)].
 
Radiologic:
*Central airways larger than the adjacent arteries.
*Airway wall-thickening.<ref>{{Cite journal  | last1 = Stockley | first1 = RA. | title = Commentary: bronchiectasis and inflammatory bowel disease. | journal = Thorax | volume = 53 | issue = 6 | pages = 526-7 | month = Jun | year = 1998 | doi =  | PMID = 9713456 }}</ref>
*"Tree-in-bud" abnormalities.
 
===Microscopic===
Features:
*Dilated airways.
**Airways larger than arteries.
 
Image:
*[http://library.med.utah.edu/WebPath/LUNGHTML/LUNG054.html Bronchiectasis (utah.edu)].


==Pulmonary hemorrhage==
==Pulmonary hemorrhage==
===General===
{{Main|Pulmonary hemorrhage}}
*Many causes.
 
A few causes:
*Trauma.
*Acute [[bronchopneumonia]].
*Lung cancer, e.g. [[lung adenocarcinoma]].
*[[Pulmonary hypertension]].
*Infection, e.g. [[tuberculosis]].
*[[Bronchiectasis]].
 
Pulmonary hemorrhage syndromes:<ref name=Ref_PBoD745>{{Ref PBoD|745}}</ref>
*[[Goodpasture syndrome]].
*Idiopathic pulmonary hemosiderosis.
*Vasculitis-associated hemorrhage (hypersensitivity angiitis, [[Wegener's granulomatosis]]).
*[[Systemic lupus erythematosus]].
 
===Radiology===
*Airspace opacity.
 
===Microscopic===
Features:
*[[Red blood cell]]s in the airspaces.
 
DDx:
*Decomposition.


==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 (idiopathic) ''[[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, e.g. [[Adenovirus]].
**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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==Fibrosing pleuritis==
==Fibrosing pleuritis==
===General===
{{Main|Fibrosing pleuritis}}
*Benign.
 
===Microscopic===
Features:<ref name=ultrapathorg>URL: [http://www.ultrapath.org/oldsite/uscap/uscap05/meso1.html http://www.ultrapath.org/oldsite/uscap/uscap05/meso1.html]. Accessed on: 20 February 2012.</ref>
*Fibrosis.
**Spindle cells.
**Moderate cellularity.
*No [[necrosis]].
 
DDx:<ref name=pmid15559051>{{Cite journal  | last1 = Corson | first1 = JM. | title = Pathology of mesothelioma. | journal = Thorac Surg Clin | volume = 14 | issue = 4 | pages = 447-60 | month = Nov | year = 2004 | doi = 10.1016/j.thorsurg.2004.06.007 | PMID = 15559051 }}</ref>
*[[Mesothelioma]], desmoplastic.
**Mesothelioma should '''not''' be diagnosed on core biopsy.<ref name=ultrapathorg>URL: [http://www.ultrapath.org/oldsite/uscap/uscap05/meso1.html http://www.ultrapath.org/oldsite/uscap/uscap05/meso1.html]. Accessed on: 20 February 2012.</ref>


=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 314: 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
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==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.


==Pulmonary nodular lymphoid hyperplasia==
==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>
{{Main|Pulmonary nodular lymphoid hyperplasia}}
===General===
*Definition - reactive lymphoid cells.
 
===Gross/radiology===
*Has nodules radiographically.
 
===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 of the lung==
*[[Lymphoma]], specifically [[MALToma|MALTomas/BALTomas]].
{{Main|Lymphoma of the lung}}
*[[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==
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==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). (???)
 
Images:
*[http://path.upmc.edu/cases/case299.html Pulmonary LCH & adenocarcinoma - several images (upmc.edu)].
 
===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==
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**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)].
*[http://path.upmc.edu/cases/case698.html Pulmonary talcosis - several images (upmc.edu)].


Line 478: Line 250:
*[[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>
*[[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''.
===General===
{{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).
 
Image:
*[http://moon.ouhsc.edu/kfung/jty1/Com/ComImage/Com309-1-SM2.gif Pneumocytoma (ouhsc.edu)].<ref>URL: [http://moon.ouhsc.edu/kfung/jty1/Com/Com309-1-Diss.htm http://moon.ouhsc.edu/kfung/jty1/Com/Com309-1-Diss.htm]. Accessed on: 14 April 2012.</ref>
 
===IHC===
Features:<ref name=pmid10716159>{{Cite journal  | last1 = Rodriguez-Soto | first1 = J. | last2 = Colby | first2 = TV. | last3 = Rouse | first3 = RV. | title = A critical examination of the immunophenotype of pulmonary sclerosing hemangioma. | journal = Am J Surg Pathol | volume = 24 | issue = 3 | pages = 442-50 | month = Mar | year = 2000 | doi =  | PMID = 10716159 }}</ref>
*EMA +ve.
*PR +ve.<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>
 
Negative stains:<ref name=pmid10716159/>
*SMA -ve.
*CEA -ve.
*CD34 -ve.
*S100 -ve.
*Chromogranin A -ve.
 
Others:<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)].
*[http://path.upmc.edu/cases/case111.html LAM - several images (upmc.edu)].
 
===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:
*GM-CSF (granulocyte-macrophage colony stimulating factor) signaling in macrophages/lack of GM-CSF.
**GM-CSF is required by alveolar macrophages to clear surfactant.
 
Classification:<ref name=pmid14695413/>
#Congenital:
#**Abnormal surfactant.
#**GM-CSF receptor defect.
#Secondary:
#*Infections.
#*Haematologic malignancy.
#Acquired:
#*Dusts - interfere with macrophage function.
 
Clinical:
*Dyspnea & cough - gradual onset.
 
====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===
Features:
*Crap in the alveoli:
*"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>
*[[Pulmonary edema]].
*[[Pneumocystis pneumonia]] - exudates foamy & vacuolated.
*Pulmonary hemorrhage (acute). (???)
 
Images:
*www:
**[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>
*[[WC]]:
**New case:
***[http://commons.wikimedia.org/wiki/File:Pulmonary_alveolar_proteinosis_-3-_low_mag.jpg PAP - low mag. (WC)].
***[http://commons.wikimedia.org/wiki/File:Pulmonary_alveolar_proteinosis_-3-_high_mag.jpg PAP - high mag. (WC)].
**Old case:
***[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)].


==Diffuse panbronchiolitis==
==Diffuse panbronchiolitis==
*Abbreviated ''DPB''.
*Abbreviated ''DPB''.
===General===
{{Main|Diffuse panbronchiolitis}}
*Rare lung disease predominantly found among asians.<ref name=pmid17012632 >{{Cite journal  | last1 = Poletti | first1 = V. | last2 = Casoni | first2 = G. | last3 = Chilosi | first3 = M. | last4 = Zompatori | first4 = M. | title = Diffuse panbronchiolitis. | journal = Eur Respir J | volume = 28 | issue = 4 | pages = 862-71 | month = Oct | year = 2006 | doi = 10.1183/09031936.06.00131805 | PMID = 17012632 | http://erj.ersjournals.com/content/28/4/862.long }}</ref>
*Has been likened to [[cystic fibrosis]] - but doesn't really share any features with it.
===Gross===
Features:<ref name=pmid17012632/>
*[[Bronchiectasis]].
*Hyperinflation.
*Small nodules (~2-3 mm) around the small airways.
*Diffuse involvement of both lungs.
 
===Microscopic===
Features:<ref name=pmid17012632/>
*Transmural inflammation of bronchioles - esp. respiratory bronchioles.
**Lymphocytes, plasma cells, histiocytes.
*Neutrophils within the airway.
 
Images:
*[http://erj.ersjournals.com/content/28/4/862/F1.expansion.html DPB (ersjournals.com)].


==Pulmonary amyloidosis==
==Pulmonary amyloidosis==
Line 683: 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 689: 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==

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

Includes:

Asthma

Emphysema

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

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

Pulmonary hemorrhage

Constrictive bronchiolitis

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

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

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

Follicular bronchitis/bronchiolitis

Pulmonary nodular lymphoid hyperplasia

Lymphoma of the lung

Smoking associated disease

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

Desquamative interstitial pneumonia

Pulmonary Langerhans cell histiocytosis

  • AKA eosinophilic granuloma of the lung.

Granulomatous lung disease

See: Granulomas for an introduction to the general topic.

Most common:

  • Infectious - mycobacterial and fungal.[11]

Noninfectious causes:[11]

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

www:

Miscellaneous diseases

Pneumoconioses

Pneumocytoma

  • AKA benign sclerosing pneumocytoma.[15]
  • Previously known as sclerosing hemangioma.

Lymphangioleiomyomatosis

  • Abbreviated LAM.
  • AKA lymphangiomyomatosis.

Pulmonary alveolar proteinosis

  • Abbreviated PAP.

Diffuse panbronchiolitis

  • Abbreviated DPB.

Pulmonary 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

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

Causes:[17]

Severity

Eosinophilic pneumonia

Specific entities:[18]

Entities which may have eosinophilia as prominent feature:

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

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