Difference between revisions of "Diffuse lung diseases"

Jump to navigation Jump to search

Diffuse lung diseases (view source)

Revision as of 03:37, 18 April 2016

6,854 bytes removed ,  03:37, 18 April 2016
→‎Respiratory bronchiolitis-interstitial lung disease: split out
 
(80 intermediate revisions by the same user not shown)
Line 1: Line 1:
'''Diffuse lung diseases''', abbreviated '''DLD''', are a group of uncommon pathologies, also known as '''idiopathic interstitial pneumonias''', abbreviated '''IIPs''', and '''interstitial lung disease'''.  
'''Diffuse lung diseases''', abbreviated '''DLD''', are a group of uncommon pathologies, also known as '''idiopathic interstitial [[pneumonia]]s''', abbreviated '''IIPs''', and '''interstitial lung disease''', abbreviated '''ILD'''.  


They are a subgroup of the [[medical lung diseases]].   
They are a subgroup of the [[medical lung diseases]].   


Diffuse lung disease is the preferred term, as some diseases lumped into this group have involvement of the alveoli, i.e. are not interstitial. Also, some DLDs are not considered idiopathic, e.g. [[LIP]] is caused by [[smoking]].
Diffuse lung disease is the preferred term, as some diseases lumped into this group have involvement of the alveoli, i.e. are not interstitial. Also, some DLDs are not considered idiopathic, e.g. [[DIP]] is caused by [[smoking]].


An introduction to pulmonary pathology is found in the ''[[pulmonary pathology]]'' article.
An introduction to pulmonary pathology is found in the ''[[pulmonary pathology]]'' article.


=Overview - histologic classification=
=Overview=
==Specimens==
*Diffuse lung disease is best assessed on an open biopsy.
**These procedure are quite invasive; thus, not infrequently clinicians will try a transbrochial biopsy that ends-up providing in useful information.
 
===Sign out===
<pre>
LUNG, LEFT, TRANSBRONCHIAL BIOPSY:
- SMALL FRAGMENTS OF LUNG PARENCHYMA WITHOUT SIGNIFICANT CHANGES, SEE MICROSCOPIC
  DESCRIPTION AND COMMENT.
 
COMMENT:
The clinical findings are noted. The size and type of biopsy significantly limit the
interpretation.  Suspected diffuse lung disease (interstitial lung disease)
is best assessed on an open lung biopsy.
</pre>
====Micro====
The sections show small fragments of lung parenchyma. A very small amount of anthracotic
pigment is present. Interstitial fibrosis is not apparent. No granulomata are identified.
No nuclear atypia is identified. Air-space macrophages show minimal pigmentation and are
not present in large numbers. No significant airspace or interstitial inflammation is
identified.
 
==Histologic classification==
*Can be complex,<ref name="pmid12405906">{{cite journal |author=Nicholson AG |title=Classification of idiopathic interstitial pneumonias: making sense of the alphabet soup |journal=Histopathology |volume=41 |issue=5 |pages=381-91 |year=2002 |month=November |pmid=12405906 |doi= |url=http://www3.interscience.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0309-0167&date=2002&volume=41&issue=5&spage=381}}</ref> and the combined efforts of [[clinician]]s, [[radiologist]]s, and [[pathologist]]s can help in the generation of a more specific diagnosis.<ref name="pmid15256390">{{cite journal |author=Flaherty KR, King TE, Raghu G, ''et al'' |title=Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis? |journal=Am. J. Respir. Crit. Care Med. |volume=170 |issue=8 |pages=904-10 |year=2004 |month=October |pmid=15256390 |doi=10.1164/rccm.200402-147OC |url=http://ajrccm.atsjournals.org/cgi/pmidlookup?view=long&pmid=15256390}}</ref><ref name="pmid16738191">{{cite journal |author=Kim DS, Collard HR, King TE |title=Classification and natural history of the idiopathic interstitial pneumonias |journal=Proc Am Thorac Soc |volume=3 |issue=4 |pages=285-92 |year=2006 |month=June |pmid=16738191 |doi=10.1513/pats.200601-005TK |url=http://pats.atsjournals.org/cgi/pmidlookup?view=long&pmid=16738191}}</ref>
*Can be complex,<ref name="pmid12405906">{{cite journal |author=Nicholson AG |title=Classification of idiopathic interstitial pneumonias: making sense of the alphabet soup |journal=Histopathology |volume=41 |issue=5 |pages=381-91 |year=2002 |month=November |pmid=12405906 |doi= |url=http://www3.interscience.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0309-0167&date=2002&volume=41&issue=5&spage=381}}</ref> and the combined efforts of [[clinician]]s, [[radiologist]]s, and [[pathologist]]s can help in the generation of a more specific diagnosis.<ref name="pmid15256390">{{cite journal |author=Flaherty KR, King TE, Raghu G, ''et al'' |title=Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis? |journal=Am. J. Respir. Crit. Care Med. |volume=170 |issue=8 |pages=904-10 |year=2004 |month=October |pmid=15256390 |doi=10.1164/rccm.200402-147OC |url=http://ajrccm.atsjournals.org/cgi/pmidlookup?view=long&pmid=15256390}}</ref><ref name="pmid16738191">{{cite journal |author=Kim DS, Collard HR, King TE |title=Classification and natural history of the idiopathic interstitial pneumonias |journal=Proc Am Thorac Soc |volume=3 |issue=4 |pages=285-92 |year=2006 |month=June |pmid=16738191 |doi=10.1513/pats.200601-005TK |url=http://pats.atsjournals.org/cgi/pmidlookup?view=long&pmid=16738191}}</ref>


Line 20: Line 44:
| [[Desquamative interstitial pneumonia]] (DIP)
| [[Desquamative interstitial pneumonia]] (DIP)
| DIP
| DIP
| Smoking
| [[smoking]]
|-
|-
| [[Diffuse alveolar damage]] (DAD)
| [[Diffuse alveolar damage]] (DAD)
Line 32: Line 56:
| [[Respiratory bronchiolitis]]  
| [[Respiratory bronchiolitis]]  
| [[Respiratory bronchiolitis-associated interstitial lung disease|RB-ILD]]
| [[Respiratory bronchiolitis-associated interstitial lung disease|RB-ILD]]
| Smoking
| smoking
|-
|-
| [[Usual interstitial pneumonia]] (UIP)
| [[Usual interstitial pneumonia]] (UIP)
| [[collagen vascular disease|CVD]], [[idiopathic pulmonary fibrosis|IPF]], [[drug toxicity]], [[pneumoconiosis]]
| [[collagen vascular disease|CVD]], [[idiopathic pulmonary fibrosis|IPF]], [[drug toxicity]], [[pneumoconiosis]]
| Allergen (hypersensitivity pneumonitis), idiopathic, autoimmune
| allergen (hypersensitivity pneumonitis), idiopathic, autoimmune
|-
|-
| [[Organizing pneumonia]]
| [[Organizing pneumonia]]
Line 44: Line 68:
| [[Lymphocytic interstitial pneumonia]] (LIP)
| [[Lymphocytic interstitial pneumonia]] (LIP)
| LIP
| LIP
| Viral/autoimmune
| viral/autoimmune
|}
|}
ARDS = adult respiratory distress syndrome, AIP = acute interstitial pneumonia, TRALI = transfusion related acute lung injury, CVD = collagen vascular disease, IPF = idiopathic pulmonary fibrosis.
ARDS = adult respiratory distress syndrome, AIP = acute interstitial pneumonia, TRALI = transfusion related acute lung injury, CVD = [[collagen vascular disease]], IPF = idiopathic pulmonary fibrosis.
 


Notes:
Notes:
*''Usual interstitial pneumonia'' is the most common type of ILD.<ref name="pmid16738196">{{cite journal |author=Visscher DW, Myers JL |title=Histologic spectrum of idiopathic interstitial pneumonias |journal=Proc Am Thorac Soc |volume=3 |issue=4 |pages=322-9 |year=2006 |month=June |pmid=16738196 |doi=10.1513/pats.200602-019TK |url=http://pats.atsjournals.org/cgi/pmidlookup?view=long&pmid=16738196}}</ref>
*''Usual interstitial pneumonia'' is the most common type of ILD.<ref name="pmid16738196">{{cite journal |author=Visscher DW, Myers JL |title=Histologic spectrum of idiopathic interstitial pneumonias |journal=Proc Am Thorac Soc |volume=3 |issue=4 |pages=322-9 |year=2006 |month=June |pmid=16738196 |doi=10.1513/pats.200602-019TK |url=http://pats.atsjournals.org/cgi/pmidlookup?view=long&pmid=16738196}}</ref>
==Gross pathologic DDx by location==
Causes of upper lung fibrosis ''FASSTEN'':<ref name=Ref_TN2005_R13>{{Ref TN2005| R13}}</ref>
*[[Farmer's lung]].
*[[Ankylosing spondylitis]].
*[[Sarcoidosis]].
*[[Silicosis]].
*[[Tuberculosis]] (miliary).
*[[Eosinophilic granuloma]].
*[[Neurofibromatosis]].
Causes of lower lung fibrosis ''BAD RASH'':<ref name=Ref_TN2005_R13>{{Ref TN2005| R13}}</ref>
*[[Bronchiolitis obliterans organizing pneumonia]] (BOOP).
*[[Asbestosis]].
*Drugs (nitrofurantoin, hydralazine, isoniazid (INH), amiodarone).
*[[Rheumatologic disease]].
*[[aspiration pneumonia|Aspiration]].
*[[Scleroderma]].
*Hamman-Rich syndrome (really should be -- ''[[idiopathic pulmonary fibrosis]]'').
Note:
*''Hamman-Rich syndrome'' is another name for [[acute interstitial pneumonia]].<ref name=Ref_WMSP90>{{Ref WMSP|90}}</ref>


=Specific diseases/pattterns=
=Specific diseases/pattterns=
Line 56: Line 101:
==Diffuse alveolar damage==
==Diffuse alveolar damage==
*Abbreviated ''DAD''.
*Abbreviated ''DAD''.
===General===
{{Main|Diffuse alveolar damage}}
DAD is the histologic correlate of:
*Adult respiratory distress syndrome (ARDS).
*Acute interstitial pneumonia (AIP).
*Transfusion related acute lung injury (TRALI).
 
===Microscopic===
Features:<ref name=Ref_Klatt103>{{Ref Klatt|103}}</ref>
*Early:
**Hyaline membrane: debris (pink crap) lines the alveolar spaces.
*Intermediate:
**Macrophage proliferation.
*Late:
**Interstitial inflammation.
**Fibrosis.
 
Images:
*[http://commons.wikimedia.org/wiki/File:Hyaline_membranes_-_intermed_mag.jpg DAD - intermed. mag. (WC)].
*[http://en.wikipedia.org/wiki/File:Hyaline_membranes_-_very_high_mag.jpg DAD - very high mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:ARDS.jpg DAD (WC)].


==Usual interstitial pneumonia==
==Usual interstitial pneumonia==
*Abbreviated ''UIP''.
*Abbreviated ''UIP''.
===General===
{{Main|Usual interstitial pneumonia}}
*It is sometimes used incorrectly as a synoym for ''idiopathic pulmonary fibrosis''.
*Cannot be diagnosed via bronchoscopic or transbronchial biopsy.<ref name=Ref_PPP186>{{Ref PPP|186}}</ref>
 
====Epidemiology====
*Disease of the old - rare in under 50 years old.<ref>AC UBC S.102.</ref>
*Dismal prognosis - mean survival after diagnosis ~ 2.8 years.<ref name=pmid9445300>{{Cite journal  | last1 = Bjoraker | first1 = JA. | last2 = Ryu | first2 = JH. | last3 = Edwin | first3 = MK. | last4 = Myers | first4 = JL. | last5 = Tazelaar | first5 = HD. | last6 = Schroeder | first6 = DR. | last7 = Offord | first7 = KP. | title = Prognostic significance of histopathologic subsets in idiopathic pulmonary fibrosis. | journal = Am J Respir Crit Care Med | volume = 157 | issue = 1 | pages = 199-203 | month = Jan | year = 1998 | doi =  | PMID = 9445300 }}</ref>
 
====Differential diagnosis====
UIP is seen in:<ref name=leslie>{{cite book |author=Wick, Mark R.; Leslie, Kevin |title=Practical pulmonary pathology: a diagnostic approach |publisher=Churchill Livingstone |location=Edinburgh |year=2005 |pages= |isbn=0-443-06631-0 |oclc= 156861539|doi= |accessdate=}}</ref>
*Idiopathic pulmonary fibrosis.
*Asbestosis - one ought to see ''ferruginous bodies''.
*Chronic hypersensitivity pneumonitis (extrinsic allergic alveolitis).
*Collagen vascular disease - includes [[systemic lupus erythematosus]], [[rheumatoid arthritis]], [[scleroderma]].<ref>{{Ref PCPBoD8|374}}</ref>
*Chronic drug toxicity.<ref>{{cite journal |author=Rossi SE, Erasmus JJ, McAdams HP, Sporn TA, Goodman PC |title=Pulmonary drug toxicity: radiologic and pathologic manifestations |journal=Radiographics : a review publication of the Radiological Society of North America, Inc |volume=20 |issue=5 |pages=1245-59 |year=2000 |pmid=10992015 |doi=}}</ref>
 
===Radiology===
*Honeycombing - ''multiple'' defects that obliterate the normal lung architecture - multiple spherical voids in the lung parenchyma; radiologically these are seen as lucencies.<ref>[http://www.medcyclopaedia.com/library/topics/volume_v_1/h/honeycombing.aspx http://www.medcyclopaedia.com/library/topics/volume_v_1/h/honeycombing.aspx]</ref>
**Usually subplural, i.e. peripheral lung.
**Classically lower lobe predominant.
**Associated with interstitial thickening. (???)
 
Note:
*Cysts - have thin walls (think of emphysema, lymphangioleiomyomatosis et cetera).
**Cysts may be isolated/not close to a neighbour.
**Medcyclopaedia defines it as: thin-walled, well-demarcated and >1 cm.<ref>[http://www.medcyclopaedia.com/library/topics/volume_v_1/l/lung_cyst.aspx http://www.medcyclopaedia.com/library/topics/volume_v_1/l/lung_cyst.aspx]</ref>
 
===Microscopic===
Features:<ref name=Ref_PPP186-9>{{Ref PPP|186-9}}</ref>
*Fibroblast foci:
**"Crescent-shaped bulge" of fibroblasts -- a rounded projection of spindle cells into the airspace.
**Location: in the areas of transisition between active inflammation and old inflammation.<ref>[http://www.epler.com/IPFWhat%27sIPFDiseaseInformation2.htm http://www.epler.com/IPFWhat%27sIPFDiseaseInformation2.htm]</ref>
**Note: Technically, ''fibroblast foci'' are composed of myofibroblasts.<ref name=Ref_PPP189>{{Ref PPP|189}}</ref>
*Interstitial inflammation,
*Microscopic honeycombing,
**Typically peripheral - cysts lined by ciliated epithelium.
*Spatial heterogeneity - patchy lesional distribution (areas of abnormal and normal lung may appear beside one another).
*Temporal heterogeneity - lesions of differing age side-by-side.<ref>H. 8 July, 2009.</ref>
 
Notes:
*Disease worse distant from large airways: lower lung field predominance, typically worse at periphery of lobule and lung.<ref>A. Churg. UBC S.103.</ref>
*Heterogeneity of inflammation: airspace macrophages & inflammation minimal in honeycombed foci.


==Asbestosis==
==Asbestosis==
===General===
{{Main|Asbestosis}}
*Important to diagnose... asbestosis = compensation.
 
===Microscopic===
*Histologic appearance as for UIP -- plus ''ferruginous bodies''.
**Segmented twirling batton with long slender fibre within.
 
Image(s):
*[http://commons.wikimedia.org/wiki/File:Ferruginous_body.jpg Ferruginous bodies (WC)].
*[http://commons.wikimedia.org/wiki/File:Asbestosis_high_mag.jpg Asbestosis (WC)].


==Non-specific interstitial pneumonia==
==Non-specific interstitial pneumonia==
*Abbreviated ''NSIP''.
*Abbreviated ''NSIP''.
*Better prognosis than UIP.
{{Main|Non-specific interstitial pneumonia}}
*Some radiologists and pathologists don't believe in this entity.


===Gross/Radiology===
==Organizing pneumonia==
*No honeycombing.
{{Main|Organizing pneumonia}}
*Fibrosis usually lower lung zone.
*Patchy ground glass.
 
===Microscopic===
*Fibrosis:
**May be uniform.
**"Linear fibrosis" has a good prognosis - should be mentioned in the report.
***''Linear fibrosis'' = fibrosis that follows alveolar walls + no architectural distortion.
*+/-Lymphoid nodules - assoc. with collagen vascular disease.
 
Notes:
*Like UIP... also temporally and spatially heterogeneous.
*Inflammation in NSIP usually more prominent than in UIP.
*No honeycombing - key difference between UIP and NSIP.
 
===DDx===
*Collagen vascular disease.
*Drug reaction.
*Hypersensitivity pneumonitis (extrinic allergic alveolitis).


==Hypersensitivity pneumonitis==
==Hypersensitivity pneumonitis==
*AKA ''extrinsic allergic alveolitis''
*AKA ''extrinsic allergic alveolitis''
*Exposure to stuffs... e.g. moldy hay - ''Farmer's lung'', atypical mycobacteria - ''hot tub lung''.
{{Main|Hypersensitivity pneumonitis}}
*Upper lung predominant disease (???).
 
===Microscopic===
Features:
* Lesions have centrilobular prominence - '''important feature'''. <ref name=pmid16061708/>
** Allergens enter lung through airway which has a centrilobular location.
* [[Granulomata]] (not typically seen in UIP) - '''important feature'''.<ref name=pmid16061708>{{Cite journal  | last1 = Ohtani | first1 = Y. | last2 = Saiki | first2 = S. | last3 = Kitaichi | first3 = M. | last4 = Usui | first4 = Y. | last5 = Inase | first5 = N. | last6 = Costabel | first6 = U. | last7 = Yoshizawa | first7 = Y. | title = Chronic bird fancier's lung: histopathological and clinical correlation. An application of the 2002 ATS/ERS consensus classification of the idiopathic interstitial pneumonias. | journal = Thorax | volume = 60 | issue = 8 | pages = 665-71 | month = Aug | year = 2005 | doi = 10.1136/thx.2004.027326 | PMID = 16061708 }}</ref>
* Chronic interstitial inflammation consisting primarily of lymphocytes.
* Interstitial fibrosis.
* Air space involvement (alveolitis).
 
Images:
*[http://commons.wikimedia.org/wiki/File:Hypersensitivity_pneumonitis_intermed_mag.jpg Hypersensitivity pneumonitis - intermed. mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Hypersensitivity_pneumonitis_high_mag.jpg Hypersensitivity pneumonitis - high mag. (WC)].


==Lymphocytic interstitial pneumonia==
==Lymphocytic interstitial pneumonia==
===General===
*Often abbreviated '''''LIP'''''.
*Often abbreviated '''''LIP'''''.
*Associated with autoimmune disorders ([[rheumatoid arthritis]], [[pernicious anemia]], Sjoegren syndrome)<ref>URL: [http://emedicine.medscape.com/article/299643-overview http://emedicine.medscape.com/article/299643-overview]. Accessed on: 2 June 2010.</ref> and immunodeficiency.<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>
{{Main|Lymphocytic interstitial pneumonia}}
*Associated with viral infections ([[HIV]], EBV, human T-cell leukemia virus (HTLV) type 1).
 
===Gross===
*Location: basilar predominance.
*Increased interstitial markings.
 
===Microscopic===
Features:<ref>URL: [http://emedicine.medscape.com/article/299643-diagnosis http://emedicine.medscape.com/article/299643-diagnosis]. Accessed on: 2 June 2010.</ref>
*Small mature lymphocytes (usually B cells).<ref name=afip_vol2_265>AFIP atlas of nontumour pathology. 2002. Vo. 2. P.265. ISBN 1-881041-79-4.</ref>
*Plasma cells.
*+/-Lymphoid follicles.


Negatives:
==Respiratory bronchiolitis-interstitial lung disease==
*No Vasculitis.
*Abbreviated ''RB-ILD'' or ''RBILD''.
*No necrosis.
*[[AKA]] ''respiratory bronchiolitis-associated interstitial lung disease''.
 
{{Main|Respiratory bronchiolitis-interstitial lung disease}}
Image: [http://www.scielo.br/img/revistas/jbpneu/v34n2/en_a02fig01.jpg LIP (scielo.br)].
 
DDx:
*[[Lymphoma]].
*Follicular bronchitis/bronchiolitis.
*Nodular lymphoid hyperplasia.
**This is determined in part by radiology; it has nodules radiographically.
 
==Respiratory bronchiolitis interstitial lung disease==
*Abbreviated ''RBILD''.
**Also written as ''RB-ILD''.
===General===
*May be an early version of ''[[DIP]]''.
 
===Microscopic===
Features:<ref name=Ref_PPP197-8>{{Ref PPP|197-8}}</ref>
*Brown pigmented airspace macrophages - ''smoker's macrophages''.
*Inflammation of the terminal bronchioles.
 
Note:
*The histologic features of RBILD may be present peri-tumoural.


==Desquamative interstitial pneumonia==
==Desquamative interstitial pneumonia==
*Abbreviated ''DIP''.
*Abbreviated ''DIP''.
===General===
{{Main|Desquamative interstitial pneumonia}}
*Thought to be advanced ''[[RBILD]]''.
 
===Microscopic===
Features:
*Brown pigmented airspace macrophages - ''smoker's macrophages''.
*Architecture preserved; "linear fibrosis".
 
Notes:
*Some fields of view may be indistinguishable from RBILD.
*Amiodarone toxicity, fibrotic NSIP - may appear similar.


=See also=
=See also=
Michael
48,460

edits

Retrieved from "https://librepathology.org/wiki/Special:MobileDiff/9498...43763"

Navigation menu