Restrictive lungdiseases

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Restrictive lungdiseases

  1. 1. Restrictive Diseases Dr.CSBR.Prasad, M.D.
  2. 2. Chronic restrictive diseasesRestrictive diseases due to chest wall disorders Restriction due to interstitial and infiltrative diseases.
  3. 3. Restrictive - chest wall disorder 1. Kyphoscoliosis 2. Ankylosing spondilosis 3. Poliomyelitis 4. Severe obesity 5. Pleural diseases
  4. 4. Interstitial and infiltrative diseases 1. Pneumoconiosis 2. Immunologic lung diseases 3. Collagen vascular diseases 4. Sarcoidosis
  5. 5. Infiltrative - Radiologic term• Chest radiograph - ground glass shadow• Diffuse infiltration by nodules/lines
  6. 6. Clinical features 1. Dyspnoea 2. Tachypnoea 3. Cyanosis 4. No wheezing
  7. 7. Pneumoconiosis• Lung disease caused by inhalation of dust• Synonyms: • Dust diseases • Occupational lung diseases• Dusts –Inert Predispose to TB Predispose to neoplasia
  8. 8. Factors determining - disease1. Size / shape of particles2. Solubility3. Amount of dust retained in lungs4. Effect of other irritants - ex Smoke5. Host factors:  Clearance mechanism  Immune status
  9. 9. Size of dust• Particles larger than 5µm may reach – Terminal airway – Ingestion by alveolar macrophage• Smaller than 1µm may – Reach alveoli – Stimulate macrophage – Result in fibrosis
  10. 10. In the nose, the hair at the externalnares filter out the larger particles,trapping almost all particles larger than5µm.In the trachea and bronchi, 90% of theparticles larger than 3-5µm are caught.Particles between 0.5 and 2µm reachthe alveolar ducts and alveoli.
  11. 11. Solubility and cytotoxicity• Smaller particles: – Pulmonary fluid – Rapid toxic levels – Cause acute lung injury• Larger particles: – Resist dissolution – Persist in lung for years – Evoke fibrosis ex silicosis
  12. 12. Physiochemical reactivity• Quartz particles• Direct injury to tissue and cell membrane• Can trigger proinflammatory / profibrosing reaction
  13. 13. Systemic response• Particles reach LN by macrophages• Initiate immune response / amplifies local reaction• Particles translocate to blood• Evokes systemic inflammation.
  14. 14. Host tissue response1. Fibrous nodules ex coal workers pneumoconiosis, silicosis.2. Interstitial fibrosis ex asbestosis.3. Hypersensitivity ex.berylliosis.
  15. 15. Pneumoconiosis (inorganic dusts) • Simple coal w p• Coal dust • PMF • Caplan’s syndrome• Silica • Silicosis • Caplan’s syndrome• Asbestose • Asbestosis • Pleural disease • Pleural tumors• Berylium • Beryliosis• Iron oxide • Pulmonary siderosis
  16. 16. Pneumoconiosis (biologic dusts)• Mouldy hay • Farmers’ lung• Bagasse • Bagassosis• Cotton, flax, hempdust • Byssinosis• Bird droppings • Bird breeders disease• Mushroom compost dust • Mushroom workers lung• Mouldey barley, malt dust • Malt workers lung• Mouldy maple bark • Maple-bark disease• Silage fermentation • Silo fillers’ disease
  17. 17. One form of hypersensitivity pneumonitis is known as farmers lung - the farmerinhales thermophilic actinomycetes in moldy hay that set off the reaction
  18. 18. "silo fillers disease" which is an acute chemical pneumonitis due to toxicgases released from fermenting silage into the atmosphere inside the silo
  19. 19. Coal workers’s pneumoconiosis1. Asymptomatic2. Simple coal worker’s pneumoconiosis3. Complicated CWP (progressive CWP)
  20. 20. Complicated CWP [PMF]• Confluence of fibrosing reaction in lung that is a complication of any pneumoconiosis• Common in CWP• Can also be seen in silicosis
  21. 21. Anthracosis• Coal - Innocuous• Common in urban dwellers/smokers• Carbon pigment in lung, lymphatics, LN• Autopsy: linear streaks and aggregates in pul. lymphatics, LNs
  22. 22. Simple CWP• Coal macule: 1 to 2 mm, carbon laden macrophages• Coal nodules: larger than macule, contain delicate collagen fibres• Upper lobes, upper zones of lower lobes heavily involved• Located adjacent to resp bronchioles- initial dust deposition site-centrilobular emphysema
  23. 23. Complicated CWP (PMF) In a background of simple CWP Over years Intensely blackened scars  Larger than 2 cms  May be upto 10 cms Multiple,bilateral,involve upper,post region Micro: dense collagen, pigment, necrotic centre,with local ischemia
  24. 24. Caplan syndrome (Rheumatoid pneumoconiosis)• Development of rheumatoid arthritis in CWP, silicosis, asbestosis• Gross; round, firm nodules with central necrosis, cavitation or calcification• Micro: central zone of dust laden fibrinoid necrosis enclosed by palisading fibroblasts and mononuclear cells
  25. 25. Clinical features• Cough with jet black sputum• Dyspnoea• Pul-HT, cor pulmonale• TB and RA are more common in miners• Increased risk for Ca. stomach• Bronchogenic .ca is rare
  26. 26. Figure 15-18 Progressive massivefibrosis superimposed on coalworkers pneumoconiosis. The large,blackened scars are locatedprincipally in the upper lobe. Note theextensions of scars into surroundingparenchyma and retraction ofadjacent pleura.
  27. 27. "coal workers pneumoconiosis"
  28. 28. SilicosisKnife grinders lung
  29. 29. Silicosis knife grinders lung• Silica (silicon dioxide)• Caused by inhalation of crystaline silica• Presents after decades of exposure• Slowly progressing nodular, fibrosing pneumoconiosis
  30. 30. Acute silicosis• Heavy exposure over months to few years• Generalized accumulation of lipoproteinaceous material within alveoli• Morphologically identical to alveolar proteinosis.
  31. 31. Chronic silicosisCharacterized by formation of small collagenous silicotic nodules
  32. 32. Occupations - Silicosis• Miners • Exposure to pencil• Quarry workers • Slate• Tunnelers • Agate grinding• Sand blasters• Grinders• Ceramic workers• Foundry workers• Silica abrasive manufacturers
  33. 33. Silica – Physical formsCrystalline forms Amorphous forms• Quartz • Talc• Crystobalite • Vermiculite• Tridymite • Mica• Are fibrogenic • Less fibrogenic
  34. 34. IARC pointed out that only crystalline form is actually carcinogenic
  35. 35. Pathogenesis - Silicosis• Silica - macrophage ingestion - direct toxic effect• Silica particles - activation and release of mediators by viable macrophages• IL-1, TNF, fibronectin, lipidmediators, oxygen derived free radicles, fibrogenic cytokines• Mixed with other minerals, quartz has less fibrogenic effect. ex: hematite.
  36. 36. Gross pathology• Silicotic lung studded with well circumscribed hard, fibrotic nodules,1 to 5 mm in diameters• Scattered throughout the lung• Simultaneous deposition of coal dust with calcification• Pleura thickened, adherent to chest wall• Similar nodules in LN, pleura
  37. 37. Gross pathology contd…,• X ray nodular lesions - egg shell shadows• Lesion – necrosis, cavitation• Complicated by TB, rheumatoid pneumoconiosis.
  38. 38. Egg shell calcification
  39. 39. FIGURE 15-18Advanced silicosisScarring has contractedthe upper lobe into asmall dark mass (arrow).Note the dense pleuralthickening.
  40. 40. Microscopy of silicosis• Nodular lesions- concentric layers of hyalinised collagen surrounded by dense capsule of more condensed collagen• Polarising microscopy --- birefringent silica particles.
  41. 41. Figure 15-19 Advanced silicosis seen on transection of lung. Scarring hascontracted the upper lobe into a small dark mass (arrow). Note the densepleural thickening.Figure 15-20 Several coalescent collagenous silicotic nodules.
  42. 42. Figure 15-20 Several coalescent collagenous silicotic nodules.
  43. 43. Pneumoconiosis: polarized light microscopy - silica crystals.Here are seen bright white crystals of varying sizes.
  44. 44. Bright white collections of polarizable crystals are seen here, but are diffuse and centeredaround vascular spaces. This is the lung of a patient with a long history of intravenous drug use
  45. 45. Clinical features-silicosis Asymptomatic- routine chest x ray X ray- fine nodularity in upper zones Pulm functions –normal/mildly affected. Slow to kill but impaired pulm fn severely limits activity. Depresses CMI Increased susceptibility to TB Crystalline silica - occupational source of carcinogen.
  46. 46. E N D
  47. 47. Contact:Dr.CSBR.Prasad, M.D.,Associate Professor,Deptt. of Pathology,Sri Devaraj Urs Medical College,Kolar-563101,Karnataka,INDIA.CSBRPRASAD@REDIFFMAIL.COM

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