Difference between revisions of "Medical lung diseases"

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(→‎Asthma: split out)
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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 [[eosinophil]]s.
*+/-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==

Revision as of 05:15, 13 March 2015

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

  • Seen in a number of conditions, e.g. congestive heart failure.

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

General

Gross

  • Large airways at the periphery of the lung.
  • Central airways larger than the adjacent arteries.
  • Typically focal.

Radiologic:

  • Central airways larger than the adjacent arteries.
  • Airway wall-thickening.[5]
  • "Tree-in-bud" abnormalities.

Images

www:

Microscopic

Features:

  • Dilated airways.
    • Airways larger than arteries.

Image

www:

Pulmonary hemorrhage

General

  • Many causes.

A few causes:

Pulmonary hemorrhage syndromes:[6]

Radiology

  • Airspace opacity.

Microscopic

Features:

DDx:

  • Decomposition.

Constrictive bronchiolitis

  • AKA bronchiolitis obliterans, AKA obliterative broncholitis,[7] AKA bronchiolitis obliterans syndrome (BOS).[8]

General

  • 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:[7]

Microscopic

Features:[7]

  1. Bronchiolitis - mononuclear and neutrophilic.
    • May be minimal late in the disease.
  2. Fibrosis - submucosal and peribronchiolar.
    • Can be patchy.

Notes:

  • Bronchioles = noncartilagenous airways usu. < 2 mm in diameter.[7]

Images:

Stains

  • Elastic trichrome - useful for delineation of obliterated bronchioles.

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.[10]
    • Reticular = net-like.[11]
  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:[12]

Note:

Causes of upper lung fibrosis FASSTEN:[12]

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.).[14][15]

Patterns of fibrosis:

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

Disease with fibrosis

There are many of 'em.

Fibrosing pleuritis

General

  • Benign.

Microscopic

Features:[16]

  • Fibrosis.
    • Spindle cells.
    • Moderate cellularity.
  • No necrosis.

DDx:[17]

  • Mesothelioma, desmoplastic.
    • Mesothelioma should not be diagnosed on core biopsy.[16]

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

General

Gross/radiology

  • No distinct nodule or mass.
  • Classically: increased reticular marking, i.e. interstitial pattern.

Images: Intersitial pattern - radiographs (ucsf.edu).

Microscopic

Features:[21]

  • 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:

Pulmonary nodular lymphoid hyperplasia

  • AKA pseudolymphoma - a term some dislike.[21]

General

  • Definition - reactive lymphoid cells.

Gross/radiology

  • Has nodules radiographically.

Microscopic

Features:[21]

  • Reactive lymphoid nodules.

Notes:[22]

  • Presence of germinal centres do not exclude lymphoma - may still be a BALToma.
    • BALT = bronchial/bronchus associated lymphoid tissue.

DDx:

Smoking associated disease

  • RB = respiratory bronchiolitis.
  • RBILD = respiratory bronchiolitis interstitial lung disease.
  • DIP = desquamative interstitial pneumonia.
  • Eosinophilic granuloma (of lung) - AKA pulmonary langerhans cell histiocytosis.

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

Respiratory bronchiolitis

  • 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.

General

Subtypes:[23]

  • Cellular form.
  • Fibrotic form.

One form usually predominates.

Radiology

  • Upper lung zones.

Microscopic

Features:[24]

  • 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:

IHC

  • Langerhans cells: S100+ and CD1a+.[24]

Granulomatous lung disease

See: Granulomas for an introduction to the general topic.

Most common:

  • Infectious - mycobacterial and fungal.[25]

Noninfectious causes:[25]

  • Aspiration pneumonia.
  • Hypersensitivity pneumonitis.
  • Hot tub lung.
  • Talc granulomatosis.
  • Sarcoidosis.
  • Wegener granulomatosis.

Sarcoidosis

General

  • Diagnosis of exclusion - infection must be excluded.
  • Radiologic differential diagnosis includes carcinomatosis.[26]

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.[29]
  • Previously known as sclerosing hemangioma.

General

  • Derived from type 2 pneumocyte.[30]
  • Progesterone-receptor positive stromal cells.[31]

Epidemiology

  • Female in 40s.[32]
  • Considered benign; excision is curative.

Gross

  • Peripheral, solitary.
  • Well-circumscribed.

Microscopic

Features:[32]

  • Mixed cell population.
  • Variable architecture:
    • Papillary.
    • Sclerotic.
    • Solid.
    • Hemorrhagic.
  • +/-Granulomas.

DDx:[33]

Image:

IHC

Features:[35]

  • EMA +ve.
  • PR +ve.[31]

Negative stains:[35]

  • SMA -ve.
  • CEA -ve.
  • CD34 -ve.
  • S100 -ve.
  • Chromogranin A -ve.

Others:[30]

  • TTF-1 +ve.
  • HNF-3 alpha +ve.
  • HNF-3 beta +ve.

Lymphangioleiomyomatosis

  • Abbreviated LAM.
  • AKA lymphangiomyomatosis.

Pulmonary alveolar proteinosis

  • Abbreviated PAP.

Diffuse panbronchiolitis

  • Abbreviated DPB.

General

  • Rare lung disease predominantly found among asians.[36]
  • Has been likened to cystic fibrosis - but doesn't really share any features with it.

Gross

Features:[36]

  • Bronchiectasis.
  • Hyperinflation.
  • Small nodules (~2-3 mm) around the small airways.
  • Diffuse involvement of both lungs.

Microscopic

Features:[36]

  • Transmural inflammation of bronchioles - esp. respiratory bronchioles.
    • Lymphocytes, plasma cells, histiocytes.
  • Neutrophils within the airway.

Images:

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:[38]

Severity

Eosinophilic pneumonia

Specific entities:[39]

  • Churg-Strauss syndrome.
  • Acute eosinophilic pneumonia.
  • Chronic eosinophilic pneumonia.
  • Eosinophilic granuloma (pulmonary histiocytosis X, Langerhans cell granulomatosis).

Entities which may have eosinophilia as prominent feature:

Churg-Strauss syndrome

Microscopic

Features:

Eosinophilic pleural effusions

  • Definition: 10%+ eosinophils.[40]
  • Uncommon 5-16% of effusions.[41]

Causes - mnemonic I'M PAID:[41]

  • 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. Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 693. ISBN 978-1416031215.
  4. URL: http://library.med.utah.edu/WebPath/LUNGHTML/LUNG053.html. Accessed on: 21 February 2012.
  5. Stockley, RA. (Jun 1998). "Commentary: bronchiectasis and inflammatory bowel disease.". Thorax 53 (6): 526-7. PMID 9713456.
  6. Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 745. ISBN 0-7216-0187-1.
  7. 7.0 7.1 7.2 7.3 Visscher, DW.; Myers, JL. (2006). "Bronchiolitis: the pathologist's perspective.". Proc Am Thorac Soc 3 (1): 41-7. doi:10.1513/pats.200512-124JH. PMID 16493150. http://pats.atsjournals.org/cgi/content/full/3/1/41.
  8. 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.
  9. 9.0 9.1 Chien, JW.; Duncan, S.; Williams, KM.; Pavletic, SZ. (Jan 2010). "Bronchiolitis obliterans syndrome after allogeneic hematopoietic stem cell transplantation-an increasingly recognized manifestation of chronic graft-versus-host disease.". Biol Blood Marrow Transplant 16 (1 Suppl): S106-14. doi:10.1016/j.bbmt.2009.11.002. PMID 19896545.
  10. http://www.rsna.org/Publications/rsnanews/may06/jrnl_may06.cfm
  11. http://dictionary.reference.com/browse/reticular
  12. 12.0 12.1 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.
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  15. AC UBC S.425.
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  18. Nicholson AG (August 2001). "Lymphocytic interstitial pneumonia and other lymphoproliferative disorders in the lung". Semin Respir Crit Care Med 22 (4): 409–22. doi:10.1055/s-2001-17384. PMID 16088689.
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  37. Hagmeyer, L.; Stieglitz, S.; Röcken, C.; Randerath, W. (Jun 2012). "[Amyloidosis in Pneumology.]". Pneumologie. doi:10.1055/s-0032-1309811. PMID 22692971.
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  39. http://emedicine.medscape.com/article/301070-overview
  40. 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.
  41. 41.0 41.1 Kalomenidis, I.; Light, RW. (Jul 2004). "Pathogenesis of the eosinophilic pleural effusions.". Curr Opin Pulm Med 10 (4): 289-93. PMID 15220754.

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