Medical lung diseases

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

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

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

  • Bronchiectasis. (WC)

www:

Microscopic

Features:

  • Dilated airways.
    • Airways larger than arteries.

Image

www:

Pulmonary hemorrhage

Main article: Pulmonary hemorrhage

Constrictive bronchiolitis

  • AKA bronchiolitis obliterans, AKA obliterative broncholitis,[6] AKA bronchiolitis obliterans syndrome (BOS).[7]

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:[6]

Microscopic

Features:[6]

  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.[6]

Images:

Stains

  • Elastic trichrome - useful for delineation of obliterated bronchioles.

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.[9]
    • Reticular = net-like.[10]
  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:[11]

Note:

Causes of upper lung fibrosis FASSTEN:[11]

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.).[13][14]

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

  • AKA pseudolymphoma - a term some dislike.[15]

General

  • Definition - reactive lymphoid cells.

Gross/radiology

  • Has nodules radiographically.

Microscopic

Features:[15]

  • Reactive lymphoid nodules.

Notes:[16]

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

DDx:

Smoking associated disease

Main article: Smoking
  • 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

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.[17]

Noninfectious causes:[17]

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

Sarcoidosis

Main article: Sarcoidosis

General

  • Diagnosis of exclusion - infection must be excluded.
  • Radiologic differential diagnosis includes carcinomatosis.[18]

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.[21]
  • 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:[23]

Severity

Eosinophilic pneumonia

Specific entities:[24]

  • 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

Main article: Churg-Strauss syndrome

Microscopic

Features:

Eosinophilic pleural effusions

  • Definition: 10%+ eosinophils.[25]
  • Uncommon 5-16% of effusions.[26]

Causes - mnemonic I'M PAID:[26]

  • 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. ↑ 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. ↑ 6.0 6.1 6.2 6.3 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.
  7. ↑ 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.
  8. ↑ 8.0 8.1 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.
  9. ↑ http://www.rsna.org/Publications/rsnanews/may06/jrnl_may06.cfm
  10. ↑ http://dictionary.reference.com/browse/reticular
  11. ↑ 11.0 11.1 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.
  12. ↑ 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.
  13. ↑ 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.
  14. ↑ AC UBC S.425.
  15. ↑ 15.0 15.1 AFIP atlas of nontumour pathology. 2002. Vo. 2. P.277. ISBN 1-881041-79-4.
  16. ↑ AFIP atlas of nontumour pathology. 2002. Vo. 2. P.281. ISBN 1-881041-79-4.
  17. ↑ 17.0 17.1 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.
  18. ↑ URL: http://www.radiologyassistant.nl/en/46b480a6e4bdc. Accessed on: 23 May 2010.
  19. ↑ 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/.
  20. ↑ 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.
  21. ↑ Chan, KW.; Gibbs, AR.; Lo, WS.; Newman, GR. (Jun 1982). "Benign sclerosing pneumocytoma of lung (sclerosing haemangioma).". Thorax 37 (6): 404-12. PMID 6291188.
  22. ↑ Hagmeyer, L.; Stieglitz, S.; Röcken, C.; Randerath, W. (Jun 2012). "[Amyloidosis in Pneumology.]". Pneumologie. doi:10.1055/s-0032-1309811. PMID 22692971.
  23. ↑ Bush A (December 2000). "Pulmonary hypertensive diseases". Paediatr Respir Rev 1 (4): 361-7. doi:10.1053/prrv.2000.0077. PMID 16263465.
  24. ↑ http://emedicine.medscape.com/article/301070-overview
  25. ↑ 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.
  26. ↑ 26.0 26.1 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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