This PPT includes the information about the interstital lung disease.This PPT is made up from the essentials of cardiopulmonary physical therapy by ellen hillegass.

1. INTERSTITIAL LUNG
DISEASES
-Hitesh Rohit (3rd year B.P.T.)

2. INTRODUCTION :-
• Interstitial lung disease (ILD) is a group of many lung conditions.
All interstitial lung disease affect the interstitium, a part of
the lungs.
• Interstitial lung disease is another term for pulmonary fibrosis, or
“scarring” and “inflammation” of the interstitium (the tissue that
surrounds the lung’s air sacs, blood vessels and airways).
• This scarring makes the lung tissue stiff, which can make breathing
difficult.

3. • These are the interstitial lung disease:-
• 1) Idiopathic pulmonary fibrosis
• 2) Coal worker’s pneumoconiosis
• 3) Silicosis
• 4) Asbestosis
• 5) Bronchopulmonary dysplasia
• 6) Bronchiolitis obliterans
• 7) Atelectasis

4. • Idiopathic pulmonary fibrosis (IPF) is an inflammatory process
involving all of the components of the alveolar wall that progresses
to gross distortion of lung architecture.
• The components of the alveolar wall include the epithelial cells,
the endothelial cells, the cellular and noncellular components of
the interstitium, and the capillary network.
• Other synonyms used for IPF are cryptogenic fibrosing alveolitis,
interstitial pneumonitis, and Hamman–Rich syndrome.
Idiopathic pulmonary fibrosis:-

5. • Etiology:-
• Mainly idiopathic
• Viral
• Genetic
• Immune system disorder
• Combination

6. • It is immunologically mediated disease, set in motion by an
initial acute injury or infection.
• Current evidence points to a number of environmental and
occupational exposures that initiate the injury, including six
major exposures:
• smoking exposure,
• agriculture/farming,
• livestock,
• wood dust,
• metal dust, and stone/sand dust exposure.

7. • The lung involvement in IPF often shows patchy focal lesions
scattered throughout both lungs.
• These lesions first show inflammatory changes and then scar
and become fibrotic, distorting the alveolar septa and the
capillary network.
• The alveolar spaces become irregular in size and shape.
• There can be significant progressive destruction of the capillary
bed.
• These changes combine to cause decreased lung compliance.
• Pathophysiology :-

8. • Decreased lung volumes; increased ventilation–perfusion
mismatching; decreased surface area for gas exchange;
• Decreased diffusing capacity; increased pulmonary arterial
pressure, which increases the work of the right ventricle;
• Increased work of breathing; increased caloric requirements;
and decreased functional capacity.
• The two major pathologic components of IPF are:-
• (1) an inflammatory process in the alveolar wall (sometimes
called an alveolitis) and
(2) a scarring or fibrotic process that is thought to be secondary
to the active inflammation. Both of these pathologic processes
occur simultaneously within the lung.

9. • Clinical menifestation:-
• Low grade fever
• Dyspnea on exertion progresses to dyspnea at rest in late stage
• Fatigue
• Repetitive Nonproductive cough(some have mucus
hypersecretion and expectoration)
• Weight loss
• Decrease in appetite

10. • Diagnosis :-
• Pulmonary function test shows
• Decreased TLC
• Decreased VC
• Decreased FRC
• Decreased RV
• Decresed DLCO
• As disease progresses decreased VT and increased RR

11. • Arterial blood gas analysis:-
• Decreased PaO2
• PaCO2 normal
• Patients hypoxemic with exercise early in disease, hypoxemic at
rest as disease progresses
• Breath sound:-
• Bibasilar end inspiratory dry rales
• Decreaed breath sound

12. • Cardiovascular:-
• One third of IPF patients may develop pulmonary hypertension,
With pulmonary hypertension,right ventricular failure and cor
pulmonale.
• Late in course:- cyanosis and clubbing of digits

14. Diffuse reticulonodular patterns
throughout both lungs
Predominance of abnormal
markings in lower lung field

15. • Management :-
• Corticosteroids(mainly prednisolone)
• Cytotoxic drugs(Cyclophosphamide and azathioprine) is also useful
in the patient who doesn’t show response to corticosteroids.
• Supportive measures:-
• Smoking cessation
• Maintaining adequate oxygenation and ventilation
• Good nutrition
• Aggressive treatment of infection

16. • Final therapeutic intervention is lung transplantation.
• Orthotopic single lung transplantation(SLT) is a viable
therapeutic option for selected IPF patients.

17. COAL WORKERS’ PNEUMOCONIOSIS:-
• Coal workers’ pneumoconiosis (CWP) is an interstitial lung
disease, an occupational pneumoconiosis, caused by the
inhalation of coal dust.
• It is also known as black lung disease or black lung.
• This disease is most commonly divided into simple CWP and
complicated CWP.

18. • Etiology :-
• Inhalation of coal dust over a long period of time; usually, 10 to
12 years of underground work exposure is necessary for the
development of simple CWP.
• Complicated CWP, sometimes called progressive massive
fibrosis, usually occurs only after even longer exposure to coal
dust.
• Anthracite coal is more hazardous than bituminous in the
development of this disease.

19. • The pathologic hallmark of CWP is the coal macule, which is a
focal collection of coal dust with little tissue reaction either in
terms of cellular infiltration or fibrosis.
• These coal macules are often located at the division of
respiratory bronchioles and are often associated with focal
emphysema.
• Lymph nodes are enlarged and homogeneously pigmented and
are firm but not fibrotic. The pleural surface appears black
owing to the deposits of coal dust.
• Simple CWP is a benign disease if complications do not develop.
Less than 5% of cases progress to complicated CWP.
• Pathophysiology :-

20. • The mechanism for the progression of simple CWP to
complicated CWP is unknown. It has been suggested that simple
CWP may progress when it is combined with infection, silicosis,
tuberculosis, or altered immunologic mechanisms.
• Complicated CWP results in large confluent zones of dense
fibrosis that are usually present in apical segments in one or
both lungs.
• These zones are made up of dense, acellular, collagenous, black-
pigmented tissue. The normal lung parenchyma can be
completely replaced, and the blood vessels in the area then show
an obliterative arteritis.
• These fibrous zones can completely replace the entire upper
lobe.

22. • Clinical manifestations:-
• Severe dyspnea and cough
• Copious amount of black sputum
• Barrel chest
• Progressive weight loss

24. • Diagnosis:-
• Pulmonary function test :-
• Decreased VC,RV & DLCO
• Increased RR
• Arterial blood gas analysis:-
• Decreased PaO2

25. • Breath sound :-
• Slightly diminished breath sounds, rhonchi due to concomitant
bronchitis
• Cardiovascular :-
• Fibrotic pulmonary hypertension
• Cor pulmonale

27. • Simple:-
• Small discrete densities more nodular than linear
• Predominantly in upper regions
• Complicated:-
• Coalescent opacities of black fibrous tissue,
• Usually in posterior segments of upper lobes or superior segments of
lower lobes.
• Cavities may be present as a result of superimposed TB or secondary
to ischemic necrosis

28. • Management :-
• Complicated CWP with pulmonary fibrosis is nonreversible;
• There is no cure for it.
• Supportive treatment:-
• Cessation of exposure of coal dust
• Good nutrition
• Adequate oxygenation and ventilation
• Progressive exercise training to maximize remaining lung
function and tolerance to activity.

29. SILICOSIS :-
• Silicosis, one of the occupational pneumoconioses, is a fibrotic
lung disease caused by the inhalation of the inorganic dust
known as free or crystalline silicon dioxide.

30. • Etiology:-
• Repeated inhalation of free or crystalline silicon dioxide, which
is very common and widely distributed in the earth’s crust in a
variety of forms, including quartz, flint, cristobalite, and
tridymite.
• Industries in which silicon dioxide exposure can occur include
mining, tunneling through rock, quarrying, grinding and
polishing rock, sandblasting, ship building, and foundry work.

34. • Pathophysiology :-
• Inhaled silica causes macrophages to enter the area to ingest
these particles. But the macrophages are destroyed by the
cytotoxic effects of the silica.
• This process releases lysosomal enzymes that then induce
progressive formation of collagen,which eventually becomes
fibrotic.
• Another characteristic of silicosis is the formation of acellular
nodules composed of connective tissue called silicotic nodules.

35. • Initially these nodules are small and discrete, but as the disease
progresses they become larger and coalesce.
• Silicosis normally affect the upper lobes of the lung more than
the lower lobes.
• Silicosis also seems to predispose the patient to secondary
infections by mycobacteria including Mycobacterium
tuberculosis.
• Complicated silicosis follows a steadily deteriorating course that
leads to respiratory failure.

36. • Clinical manifestations :-
• Shortness of breath
• Cough(maybe productive)

37. • Pulmonary function test shows
• Decreased TLC
• Decreased VC
• Decreased FEV1
• Decreased pulmonary compliance
• Arterial blood gas analysis :-
• Decreased PaO2 with exercise

38. • Breath sounds :-
• Decreased breath sounds in upper lobe.
• Ronchi maybe present.
• Cardiovascular :-
• No specific changes

39. Small rounded opacities
or nodules that enlarge
over time (more in
upper lung fields)
Hilar lymph nodes
enlarged and calcified

40. • Management :-
• There is no treatment except to avoid further exposure to silica.
• Supportive therapy is used to counteract the patient’s symptoms
and includes measures to provide adequate oxygenation,
ventilation, and nutrition.
• Annual TB screening using PPD skin testing is also
recommended

41. Asbestosis :-
• Asbestosis is a diffuse interstitial pulmonary fibrotic disease
caused by asbestos exposure.
• Occupational asbestos exposure is also associated with an
increased incidence of primary cancer of the larynx,
oropharynx, esophagus, stomach, and colon.

43. • Etiology:-
• The term asbestos is used generically to name a specific group of
naturally occurring fibrous silicates.
• There are four types of commercially significant asbestos.
• Chrysolite accounts for more than 70% of the asbestos used in the
United States and is primarily mined in Canada and the
Commonwealth of Independent States.
• Crocidolite and amosite are mined primarily in South Africa.
Anthophyllite comes from Finland.
• Asbestos is valued because of its resistance to fire;

44. • Those at most risk for are:-
• asbestos miners and millers, construction workers,
shipbuilders, insulation workers, pipe fitters, steamfitters, sheet
metal workers, welders, workers who remove old asbestos
insulation, workers employed in building renovation or
demolition, and auto mechanics who work on brake linings.

45. • Pathophysiology :-
• How asbestos causes a fibrotic reaction is not understood.
• There seems to be a considerable latency period after an initial
exposure, which can extend to 15 to 20 years.
• It is hypothesized that the asbestos fiber causes an alveolitis in the
area of the respiratory bronchioles, which then progresses to
peribronchiolar fibrosis owing to the release of chemical mediators.
• Plaques, which are localized fibrous thickening of the parietal pleura,
are common and are usually seen posteriorly, laterally, or on the
pleural surface of the diaphragm.

46. • Pleural effusions may also occur with asbestosis.
• Also “asbestos bodies” or ferruginous bodies appear in the lungs
and sputum of these patients.
• These rod-shaped bodies with clubbed ends seem to be an
asbestos fiber coated by macrophages with an iron–protein
complex.
• Studies have shown conclusively that cigarette smoking has a
multiplicative effect in the development of primary lung cancer
in persons who have been exposed to asbestos.

47. • Clinical manifestations :-
• Dyspnea on exertion,progresses to dyspnea at rest.
• Chronic cough with or without sputum.
• Recurrent infections
• Decreased appetite
• Weight loss
• Decreased exercise tolerance

48. • Diagnosis :
• Pulmonary function test:-
• Decreased VC
• Decreased FEV1
• Decreased RV
• Decreased DLCO
• Decreased TLC
• Decreased lung volume
• Decreased lung compliance

49. • Arterial blood gas analysis:-
• Decreased PaO2 with exercise;in late case decreased PaO2 at
rest.
• PaCO2 within normal limits.
• Breath sounds :-
• Bibasilar rales and decreased breath sounds
• Percussion dull at bases

50. • Cardiovascular :-
• Pulmonary hypertension develops as pulmonary capillary bed
destroyed: increase work on RV
• Clubbing present;may develop cyanosis and cor pulmonale

52. • Irregularities or linear opacities are distributed throughout lung
fields in lower zones
• Loss of distinct heart and diaphragm borders (shaggy
appearance)
• Diaphragmatic and pericardial calcification
• Late in disease: cyst formation, honeycomb appearance

53. • Management :-
• There is no curative treatment for asbestosis, and the disease
progresses even though exposure to asbestos has ceased.
• Symptomatic support includes cessation of smoking, good
nutrition, exercise conditioning to maximize lung function, and
prompt treatment of recurrent pulmonary infections.

54. Bronchopulmonary dysplasia :-
• Bronchopulmonary dysplasia (BPD) is a chronic pulmonary
syndrome in neonates that occurs in some survivors of RDS who
have been ventilated mechanically and have received high
concentrations of oxygen over a prolonged period of time.
• Other names used for this syndrome are pulmonary fibroplasia
and ventilator lung.
• It is characterized by scarring of lung tissue, thickened
pulmonary arterial walls,and mismatch between lung
ventilation and perfusion.

55. • Etiology :-
• BPD occurs in severely ill infants who have received high levels
of oxygen for long periods of time or who have been on a
ventilator during treatment for respiratory distress syndrome.
• It is more common in infants born early (premature) whose
lungs were not fully developed at birth.

56. • The following risk factors have been identified:
• Premature birth.
• Respiratory Infection.
• Meconium aspiration.
• Congenital heart disease.
• It may also occur as a secondary problem for the neonate
attached to a mechanical ventilator.

57. Pathophysiology:-
• The pathophysiology of BPD is multifactorial and is not yet fully
understood.
• It is believed that a variety of toxic factors contribute to the
formation of BPD by injuring the small airways, eventually
resulting in a reduction of the alveolar surface area.
• This affects gaseous exchange which further compromises blood
oxygenation.

58. Stages
• Even though the exact pathophysiology is still unclear, 4 stages
in the development of BPD have been identified:-
• In stage 1 (1-3 days), the pathologic appearances of BPD are
identical to those of respiratory distress syndrome where there
is not enough surfactant in the lungs.
• Surfactant helps to lower surface tension in the airways and this
helps keep the lung alveoli open.
• It involves the presence of hyaline membranes, atelectasis,
vascular hyperemia, and lymphatic dilatation.

59. • In stage 2 (4-10 days), lung destruction resulting in ischeamic
necrosis of airways occurs due to stretching of the terminal
bronchioles.
• Immediate reparative changes in the lungs as well as bronchiolar
obstruction are also seen in this stage.
• Hyaline membranes can persist into this stage and emphysematous
coalescence of the alveoli is seen.

60. • Stage 3 (11-20 days) involves progressive repair of the lung,
with a decreased number of alveoli.
• There is compensatory hypertrophy of the remaining alveoli,
and hypertrophy of bronchial-wall muscle and glands.
• Regenerating clear cells are seen, along with exudation of
alveolar macrophages and histiocytes into airways.
• Airtrapping, pulmonary hyperinflation, tracheomegaly,
tracheomalacia, interstitial edema, and ciliary dysfunction may
also be present.

61. • In stage 4 (>1 month), emphysematous alveoli are seen.
Chronic lung damage eventually causes Pulmonary
hypertension (caused by thickening of the inner-most lining of
pulmonary arterioles), and results in cor pulmonale.
• Fibrosis, atelectasis, a cobblestone appearance due to uneven
lung aeration, and pleural pseudofissures are often seen.
• Marked hypertrophy of peribronchiolar smooth muscle is
present.

62. • Clinical manifestations :-
• Wheezing
• Shortness of breath
• Cough
• If BPD worsens :-
• Dyspnea
• Chest and abdomen move in opposite direction in every breath
• Nasal flaring- nostrils open wide during each breath
• Rapid RR

63. • Diagnosis:-
• Blood tests:- Blood samples are checked to see whether the baby
has enough oxygen in his or her blood.
• Chest x-rays:- It shows larger areas of air and changes from
inflammation or infection. It also shows areas of the lung that
have collapsed and may help confirm that the lungs aren’t
developing normally.
• Echocardiogram:- The use of sound waves to create a moving
picture of the heart. Echocardiogram is used to rule out
congenital heart defects or pulmonary arterial hypertension as
the cause of the breathing problems.

65. • Management:-
• Doctors begin treatment for respiratory distress at birth and before they
even know whether the baby has BPD.
• Breathing Support
• The baby is usually put on a mechanical ventilator.
• The ventilator, which is connected to a breathing tube that runs through
the baby's mouth or nose into the windpipe, can be set to help a baby
breathe or to completely control a baby's breathing.
• It also is set to give the amount of oxygen the baby requires. With help
breathing, the baby's lungs have a chance to develop.

66. • Surfactant Replacement Therapy
• The baby is given surfactant to open his or her lungs until the
lungs have developed enough to start making their own
surfactant.
• Surfactant is given through a tube that is attached to the
ventilator, which pushes the surfactant directly into the baby's
lungs.

67. • Medication
• Medication is usually prescribed to reduce swelling in the airways
and improve the flow of air in and out of the lungs. These
medications include:
• Bronchodilators – Bronchoconstriction and airway hyper reactivity.
• Diuretics - Pulmonary edema, and removal of extra fluid in the lungs.
• Steroids - To decrease airway inflammation.
• Vasodilators - Cor pulmonale.
• Antibiotics - Control infections

68. • Supportive Therapy
• Treatment in the NICU is designed to limit stress on the baby
and meet his or her basic needs of warmth, nutrition, and
protection.
• Using a radiant warmer or incubator to keep your baby warm
and reduce the chances of infection.
• Ongoing monitoring of blood pressure, heart rate, breathing,
temperature and the amount of oxygen in the baby's blood.
• Monitoring fluid intake to make sure that fluid doesn't build up
in the baby's lungs.

69. Bronchiolitis obliterans
• Bronchiolitis obliterans is a fibrotic lung disease that affects the
smaller airways.
• It can produce restrictive and obstructive lung dysfunction.
• This syndrome has been known and discussed under a variety of
names including bronchiolitis, bronchiolitis obliterans with
organizing pneumonia (BOOP), bronchiolitis fibrosa obliterans,
follicular bronchiolitis, and bronchiolitis obliterans with diffuse
interstitial pneumonia.

70. • Etiology :-
• Bronchiolitis obliterans was first recognized in children,usually
those under the age of 2 years.
• Pediatric bronchiolitis obliterans is often caused by a viral
infection, most commonly by the respiratory syncytial virus,
parainfluenza virus, influenza virus, or adenovirus.
• In the adult, bronchiolitis obliterans may be caused by toxic
fume inhalation (nitrogen dioxide) or by viral, bacterial, or
mycobacterial infectious agents, particularly Mycoplasma
pneumoniae.

71. • It may be associated with connective tissue diseases, such as
rheumatoid arthritis; related to organ transplantation and graft
versus host reactions; or allied with other diseases, such as
idiopathic pulmonary fibrosis.
• It also may be idiopathic, with no known cause.

72. • Pathophysiology:-
• Bronchiolitis obliterans is characterized by necrosis of the
respiratory epithelium in the affected bronchioles.
• This necrosis allows fluid and debris to enter the bronchioles
and alveoli, causing alveolar pulmonary edema and partial or
complete obstruction of these small airways.
• With complete obstruction, the trapped air is absorbed
gradually, and the alveoli then collapse, causing areas of
atelectasis.

73. • When the destruction of the respiratory epithelium is severe or
widespread, it may be followed by a significant inflammatory
response.
• This causes fibrotic changes in the adjacent peribronchial space,
the alveolar walls, and the air spaces.
• The fibrotic changes are patchy and usually occur primarily
within the bronchial tree and alveoli rather than in the
interstitial lung tissue, as happens in IPF.
• All these changes combine to increase ventilation–perfusion
mismatching; decrease lung compliance; impair gas transport;
and, in some patients, cause demonstrable airway obstruction.

74. • Clinical manifestations:-
• Dyspnea,
• Increased RR and hacking,
• Nonproductive cough
• Infants: chest wall retractions
• Cyanosis in some patients, chronic infections in others

75. • Diagnosis :-
• Pulmonary function test :-
• Lung volumes normal or decreased,
• Flow rates normal or decreased
• Decreased DLCO
• Increased RR

76. • Blood gas analysis :-
• Hypoxemia
• PaCO2 Normal or eleavted
• Breath sounds :-
• Rales and expiratory wheezing.
• Some areas of decreased sounds
• Cardiovascular :-
• Tachycardia

78. • Variable depending on cause and extent of BO
• Pediatric BO:
• Hyperinflation and increased bronchial markings with subsegmental
consolidation and collapse
• Some have patchy alveolar infiltrates, some with diffuse nodular or
reticulonodular pattern and interstitial inflammation and scarring
• Adults:
• Pulmonary edema and bilateral patchy alveolar infiltrates.
• Late in course:-
• Nodular pattern with fibrotic changes in bronchi and alveoli

79. • Management :-
• In children, treatment is supportive, usually consisting of hydration
and supplemental oxygen.
• If the child is unable to clear secretions, postural drainage and
suctioning are employed. Mechanical ventilation is rarely needed.
• If respiratory syncytial virus is the causative pathogen, then the
antiviral agent ribavirin may be administered via aerosol.
• Corticosteroids, antibiotics, and bronchodilators are not
recommended in the treatment of pediatric bronchiolitis obliterans.

80. • In adults, supplemental oxygen and proper fluid balance are
also very important.
• Corticosteroids have proved very effective in treating adult
bronchiolitis obliterans that is idiopathic, caused by toxic fume
inhalation, or associated with connective tissue disease.

81. Atelectasis :-
• Atelectasis means incomplete expansion of the lung or loss of volume
of part (or all) of a lung.
• The incomplete expansion can be due to a variety of causes that can
prevent alveoli from fully expanding.
• Primary atelectasis is the failure of the lung to expand at birth.
Compression atelectasis occurs as a result of changes in
transpulmonary distending pressure found in individuals with other
pathologies, including pleural effusion, pneumothorax, or interstitial
or pulmonary edema.
• Atelectasis can also occur as a result of excess secretions and the
failure to get rid of secretions.

82. • Etiology :-
• Atelectasis (obstructive) may result from extrabronchial
compression from tumors or enlarged lymph nodes, or
endobronchial diseases such as bronchial tumors, or an
intrabronchial mass (foreign body or mucous plug).
• Atelectasis (scar and/or postoperative) occurs in the
absence of obstruction and usually as a complication of
either a lobar or segmental pneumonia.

83. • The main contributing factor is the inability or unwillingness
(often because of pain or mental confusion) of the patient to
cough effectively and to take deep breaths throughout the day.
• This type of atelectasis could be prevented with good pulmonary
hygiene.

84. • Pathophysiology/Mechanism:-

85. • Atelectasis (compression) occurs as a result of expanding
volume outside of the alveoli (in pleural space, interstitial space,
etc.) that presses on the alveoli and prevents complete
expansion.
• In all these cases, the alveoli may lose their surfactant and
therefore demonstrate reduced alveolar surface tension, causing
the alveoli to collapse.

86. • Clinical manifestations :-
• Symptoms usually depends upon the cause of the atelectasis:-
such as post operative pain and low grade fever
• If obstruction is the cause, the patient may be extremely
dyspneic and demonstrate increased work of breathing.

87. • Diagnosis:-
• Chest radiograph may show opacification of the atelectatic
segment or lobe, or with significant lung collapse, the
radiograph will show elevation of the hemidiaphragm on
the affected side, shift of the mediastinum toward the
affected side, and a decrease in size of the rib interspaces
over the affected hemithorax.

89. • Management:-
• Acute atelectasis, such as in postoperative or in other hospitalized patients,
can respond to deep breathing or incentive spirometry exercises as well as
coughing.
• Other airway clearance techniques may help with improving atelectasis.
• If simple measures do not improve the lung collapse, fiberoptic
bronchoscopy can be performed to suction secretions that may be causing
the atelectasis.
• Prevention of atelectasis should be the goal for all hospitalized patients
who are unconscious or who may have poor airway clearance techniques,
have postoperative pain preventing adequate inspiration and cough, or
who demonstrate respiratory muscle weakness.