This ppt is mainly designed for UG pathology teaching.

1. Restrictive Diseases
Dr.CSBR.Prasad, M.D.

2. Chronic restrictive diseases
Restrictive diseases due to chest wall disorders
Restriction due to interstitial and infiltrative
diseases.

3. Restrictive - chest wall disorder
1. Kyphoscoliosis
2. Ankylosing spondilosis
3. Poliomyelitis
4. Severe obesity
5. Pleural diseases

4. Interstitial and infiltrative diseases
1. Pneumoconiosis
2. Immunologic lung diseases
3. Collagen vascular diseases
4. Sarcoidosis

5. Infiltrative - Radiologic term
• Chest radiograph - ground glass shadow
• Diffuse infiltration by nodules/lines

8. Clinical features
1. Dyspnoea
2. Tachypnoea
3. Cyanosis
4. No wheezing

9. Pneumoconiosis
• Lung disease caused by inhalation of dust
• Synonyms:
• Dust diseases
• Occupational lung diseases
• Dusts –Inert
Predispose to TB
Predispose to neoplasia

10. Factors determining - disease
1. Size / shape of particles
2. Solubility
3. Amount of dust retained in lungs
4. Effect of other irritants - ex Smoke
5. Host factors:
 Clearance mechanism
 Immune status

11. 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

12. In the nose, the hair at the external
nares filter out the larger particles,
trapping almost all particles larger than
5µm.
In the trachea and bronchi, 90% of the
particles larger than 3-5µm are caught.
Particles between 0.5 and 2µm reach
the alveolar ducts and alveoli.

13. 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

14. Physiochemical reactivity
• Quartz particles
• Direct injury to tissue and cell membrane
• Can trigger proinflammatory / profibrosing
reaction

15. Systemic response
• Particles reach LN by macrophages
• Initiate immune response / amplifies local
reaction
• Particles translocate to blood
• Evokes systemic inflammation.

16. Host tissue response
1. Fibrous nodules ex coal workers
pneumoconiosis, silicosis.
2. Interstitial fibrosis ex asbestosis.
3. Hypersensitivity ex.berylliosis.

17. 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

18. 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

19. One form of hypersensitivity pneumonitis is known as farmer's lung - the farmer
inhales thermophilic actinomycetes in moldy hay that set off the reaction

20. "silo filler's disease" which is an acute chemical pneumonitis due to toxic
gases released from fermenting silage into the atmosphere inside the silo

21. Coal workers’s pneumoconiosis
1. Asymptomatic
2. Simple coal worker’s pneumoconiosis
3. Complicated CWP (progressive CWP)

22. 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

23. Anthracosis
• Coal - Innocuous
• Common in urban dwellers/smokers
• Carbon pigment in lung, lymphatics, LN
• Autopsy: linear streaks and aggregates in
pul. lymphatics, LNs

24. 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

25. 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

26. 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

27. 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

28. Figure 15-18 Progressive massive
fibrosis superimposed on coal
workers' pneumoconiosis. The large,
blackened scars are located
principally in the upper lobe. Note the
extensions of scars into surrounding
parenchyma and retraction of
adjacent pleura.

29. "coal worker's pneumoconiosis"

30. Silicosis
Knife grinders lung

31. Silicosis
knife grinders lung
• Silica (silicon dioxide)
• Caused by inhalation of crystaline silica
• Presents after decades of exposure
• Slowly progressing nodular, fibrosing
pneumoconiosis

32. Acute silicosis
• Heavy exposure over months to few years
• Generalized accumulation of
lipoproteinaceous material within alveoli
• Morphologically identical to alveolar
proteinosis.

33. Chronic silicosis
Characterized by formation of small
collagenous silicotic nodules

34. Occupations - Silicosis
• Miners • Exposure to pencil
• Quarry workers • Slate
• Tunnelers • Agate grinding
• Sand blasters
• Grinders
• Ceramic workers
• Foundry workers
• Silica abrasive
manufacturers

38. Silica – Physical forms
Crystalline forms Amorphous forms
• Quartz • Talc
• Crystobalite • Vermiculite
• Tridymite • Mica
• Are fibrogenic • Less fibrogenic

39. IARC pointed out that only crystalline form is actually carcinogenic

40. 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.

41. 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

42. Gross pathology contd…,
• X ray nodular lesions - egg shell shadows
• Lesion – necrosis, cavitation
• Complicated by TB, rheumatoid
pneumoconiosis.

44. Egg shell calcification

45. FIGURE 15-18
Advanced silicosis
Scarring has contracted
the upper lobe into a
small dark mass (arrow).
Note the dense pleural
thickening.

46. Microscopy of silicosis
• Nodular lesions- concentric layers of
hyalinised collagen surrounded by dense
capsule of more condensed collagen
• Polarising microscopy --- birefringent silica
particles.

47. Figure 15-19 Advanced silicosis seen on transection of lung. Scarring has
contracted the upper lobe into a small dark mass (arrow). Note the dense
pleural thickening.
Figure 15-20 Several coalescent collagenous silicotic nodules.

48. Figure 15-20 Several coalescent collagenous silicotic nodules.

50. Pneumoconiosis: polarized light microscopy - silica crystals.
Here are seen bright white crystals of varying sizes.

51. Bright white collections of polarizable crystals are seen here, but are diffuse and centered
around vascular spaces. This is the lung of a patient with a long history of intravenous drug use

52. 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.

53. E N D

54. Contact:
Dr.CSBR.Prasad, M.D.,
Associate Professor,
Deptt. of Pathology,
Sri Devaraj Urs Medical College,
Kolar-563101,
Karnataka,
INDIA.
CSBRPRASAD@REDIFFMAIL.COM