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, pink on [[H&E]].
 
Images:
*www:
**[http://www.udel.edu/medtech/dlehman/medt372/Ch-lyd.html Charcot-Leyden crystals (udel.edu)].
**[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3168024/figure/F1/ Charot-Leyden crystals (nih.gov)].
*[[WC]]:
**[http://commons.wikimedia.org/wiki/File:Charcot-Leyden_crystals,_HE_3.jpg Charcot-Leyden crystals (WC/Patho)].
**[http://commons.wikimedia.org/wiki/File:Charcot-Leyden_crystals,_HE_2.jpg Charcot-Leyden crystals (WC/Patho)].
 
Notes:
*''Leyden'' in ''Charcot-Leyden'' is also seen written as ''Leiden''.
**Finding associated with hypereosinophilia<ref name=pmid6162378>{{Cite journal  | last1 = Dincsoy | first1 = HP. | last2 = Burton | first2 = TJ. | last3 = van der Bel-Kahn | first3 = JM. | title = Circulating Charcot-Leyden crystals in the hypereosinophilic syndrome. | journal = Am J Clin Pathol | volume = 75 | issue = 2 | pages = 236-43 | month = Feb | year = 1981 | doi =  | PMID = 6162378 }}</ref> - ''not'' pathognomonic for asthma.
*[[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>
 
DDx:
*Infection - fungal.
 
===Stains===
Features - crystals:
*PAS-D -ve.
*GMS -ve.
 
===Sign out===
<pre>
BRONCHIAL CAST, RIGHT LOWER LOBE, REMOVAL:
- CHARCOT-LEYDEN CRYSTALS.
- CELLULAR DEBRIS WITH NEUTROPHILS AND ABUNDANT EOSINOPHILS.
- RESPIRATORY MUCOSA WITH PROMINENT SMOOTH MUSCLE.
- NEGATIVE FOR MALIGNANCY.
 
COMMENT:
GMS and PAS-D stains are negative for fungal organisms. The findings are non-specific;
however, they would be compatible with asthma. Clinical and microbiological correlation
is required.
</pre>


==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===
===Gross===
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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=
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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 346: 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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*[[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====
<gallery>
Image:Lymphangioleiomyomatosis - very low mag.jpg| LAM - very low mag. (WC)
Image:Lymphangioleiomyomatosis - low mag.jpg| LAM - low mag. (WC)
Image:Lymphangioleiomyomatosis - intermed mag.jpg| LAM - intermed. mag. (WC)
Image:Lymphangioleiomyomatosis - very high mag.jpg| LAM - very high mag. (WC)
</gallery>
www:
*[http://www.nature.com/modpathol/journal/v19/n6/fig_tab/3800610f3.html LAM (nature.com)].
*[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>
<gallery>
Image:Pulmonary_alveolar_proteinosis_-3-_low_mag.jpg | PAP - low mag. (WC)
Image:Pulmonary_alveolar_proteinosis_-3-_high_mag.jpg | PAP - high mag. (WC)
</gallery>
<gallery>
Image:Pulmonary_alveolar_proteinosis_-_2_-_intermed_mag.jpg | PAP - intermed. mag. (WC)
Image:Pulmonary_alveolar_proteinosis_-_very_high_mag.jpg | PAP - very high mag. (WC)
</gallery>
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 724: 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.

Latest revision as of 14:54, 23 March 2017

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

Infectious pneumonia

Main article: Pneumonia

Includes:

Asthma

Main article: Asthma

Emphysema

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

Chronic bronchitis

General

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

Clinical:[1]

Microscopic

Features:[1]

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

Pulmonary edema

General

Gross

Features - autopsy:

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

Microscopic

Features:[2]

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

DDx:

Images:

Bronchiectasis

Main article: Bronchiectasis

Pulmonary hemorrhage

Main article: Pulmonary hemorrhage

Constrictive bronchiolitis

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

Diffuse lung diseases

Main article: Diffuse lung diseases

These are also known as idiopathic interstitial pneumonias.

Fibrosis

Histomorphological classification

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

Radiologic/gross pathologic DDx by location

Causes of lower lung fibrosis BAD RASH:[7]

Note:

Causes of upper lung fibrosis FASSTEN:[7]

Prognosis

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

Patterns of fibrosis:

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

Disease with fibrosis

There are many of 'em.

Fibrosing pleuritis

Main article: Fibrosing pleuritis

Lymphocytic lesions of the lung

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

Lymphocytic interstitial pneumonia

Main article: Lymphocytic interstitial pneumonia

Follicular bronchitis/bronchiolitis

Main article: Follicular bronchitis/bronchiolitis

Pulmonary nodular lymphoid hyperplasia

Main article: Pulmonary nodular lymphoid hyperplasia

Lymphoma of the lung

Main article: Lymphoma of the lung

Smoking associated disease

Main article: Smoking

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

Respiratory bronchiolitis

  • Diagnosis is based on clinical criteria.

Microscopic

Features:

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

Respiratory bronchiolitis interstitial lung disease

Main article: Respiratory bronchiolitis interstitial lung disease

Desquamative interstitial pneumonia

Main article: Desquamative interstitial pneumonia

Pulmonary Langerhans cell histiocytosis

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

Granulomatous lung disease

See: Granulomas for an introduction to the general topic.

Most common:

  • Infectious - mycobacterial and fungal.[11]

Noninfectious causes:[11]

Sarcoidosis

Main article: Sarcoidosis

General

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

Microscopic

Features:

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

Image(s):

Pulmonary talcosis

General

Microscopic

Features:

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

Images

  • Pulmonary talcosis - low mag. cropped (WC)

  • Pulmonary talcosis - low mag. (WC)

www:

Miscellaneous diseases

Pneumoconioses

Main article: Pneumoconioses

Pneumocytoma

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

Lymphangioleiomyomatosis

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

Pulmonary alveolar proteinosis

  • Abbreviated PAP.
Main article: Pulmonary alveolar proteinosis

Diffuse panbronchiolitis

  • Abbreviated DPB.
Main article: Diffuse panbronchiolitis

Pulmonary amyloidosis

Main article: Amyloidosis

General

Microscopic

Features:

  • Interstitial cotton candy-like material - see amyloidosis.

DDx:

Images:

Drug reactions

  • Effects are often non-specific.

Website: http://www.pneumotox.com

Pulmonary hypertension

Main article: Pulmonary hypertension

General classification:

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

Non-secondary pulmonary hypertension

Main article: Pulmonary hypertension

Causes:[17]

Severity

Eosinophilic pneumonia

Specific entities:[18]

Entities which may have eosinophilia as prominent feature:

Churg-Strauss syndrome

Main article: Churg-Strauss syndrome

Microscopic

Features:

Eosinophilic pleural effusions

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

Causes - mnemonic I'M PAID:[20]

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

Lung transplant pathology

Main article: Lung transplant pathology

This subspecialty is dealt with in its own article.

See also

References

  1. ↑ 1.0 1.1 1.2 Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 370. ISBN 978-1416054542.
  2. ↑ Klatt, Edward C. (2006). Robbins and Cotran Atlas of Pathology (1st ed.). Saunders. pp. 102. ISBN 978-1416002741.
  3. ↑ Cite error: Invalid <ref> tag; no text was provided for refs named pmid16493150
  4. ↑ Sato, M.; Keshavjee, S. (2008). "Bronchiolitis obliterans syndrome: alloimmune-dependent and -independent injury with aberrant tissue remodeling.". Semin Thorac Cardiovasc Surg 20 (2): 173-82. doi:10.1053/j.semtcvs.2008.05.002. PMID 18707652.
  5. ↑ http://www.rsna.org/Publications/rsnanews/may06/jrnl_may06.cfm
  6. ↑ http://dictionary.reference.com/browse/reticular
  7. ↑ 7.0 7.1 Yeung, J.C.; Leonard, Blair J. N. (2005). The Toronto Notes 2005 - Review for the MCCQE and Comprehensive Medical Reference (2005 ed.). The Toronto Notes Inc. for Medical Students Inc.. pp. R13. ISBN 978-0968592854.
  8. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 90. ISBN 978-0781765275.
  9. ↑ Bjoraker JA, Ryu JH, Edwin MK, et al. (January 1998). "Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis". Am. J. Respir. Crit. Care Med. 157 (1): 199-203. PMID 9445300. http://ajrccm.atsjournals.org/cgi/content/full/157/1/199.
  10. ↑ AC UBC S.425.
  11. ↑ 11.0 11.1 Mukhopadhyay S, Gal AA (May 2010). "Granulomatous lung disease: an approach to the differential diagnosis". Arch. Pathol. Lab. Med. 134 (5): 667–90. PMID 20441499.
  12. ↑ URL: http://www.radiologyassistant.nl/en/46b480a6e4bdc. Accessed on: 23 May 2010.
  13. ↑ Davis, LL. (Dec 1983). "Pulmonary "mainline" granulomatosis: talcosis secondary to intravenous heroin abuse with characteristic x-ray findings of asbestosis.". J Natl Med Assoc 75 (12): 1225–8. PMC 2561715. PMID 6655726. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2561715/.
  14. ↑ Marchiori, E.; Lourenço, S.; Gasparetto, TD.; Zanetti, G.; Mano, CM.; Nobre, LF. (Apr 2010). "Pulmonary talcosis: imaging findings.". Lung 188 (2): 165-71. doi:10.1007/s00408-010-9230-y. PMID 20155272.
  15. ↑ Chan, KW.; Gibbs, AR.; Lo, WS.; Newman, GR. (Jun 1982). "Benign sclerosing pneumocytoma of lung (sclerosing haemangioma).". Thorax 37 (6): 404-12. PMID 6291188.
  16. ↑ Hagmeyer, L.; Stieglitz, S.; Röcken, C.; Randerath, W. (Jun 2012). "[Amyloidosis in Pneumology.]". Pneumologie. doi:10.1055/s-0032-1309811. PMID 22692971.
  17. ↑ Bush A (December 2000). "Pulmonary hypertensive diseases". Paediatr Respir Rev 1 (4): 361-7. doi:10.1053/prrv.2000.0077. PMID 16263465.
  18. ↑ http://emedicine.medscape.com/article/301070-overview
  19. ↑ Matthai, SM.; Kini, U. (Feb 2003). "Diagnostic value of eosinophils in pleural effusion: a prospective study of 26 cases.". Diagn Cytopathol 28 (2): 96-9. doi:10.1002/dc.10227. PMID 12561030.
  20. ↑ 20.0 20.1 Kalomenidis, I.; Light, RW. (Jul 2004). "Pathogenesis of the eosinophilic pleural effusions.". Curr Opin Pulm Med 10 (4): 289-93. PMID 15220754.

External links

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