محاضرة مبسطة للفرقة الخامسة

1. By
Alaa metwally
Ass prof. Chest department

2. What is the interstitium?
It is the connective tissue space between basement
membrane of alveolar epithelium and capillary
endothelium .
Matrix
Connective tissue

3. Definition of ILDs
ILDs represent a heterogeneous group ofILDs represent a heterogeneous group of
disorders affecting the alveoli, interstitiumdisorders affecting the alveoli, interstitium
and sometimes bronchioles.They have similarand sometimes bronchioles.They have similar
clinical , radiological and functional features.clinical , radiological and functional features.

4. The characteristic of ILDsThe characteristic of ILDs::
Dyspnea on exercise
chest X-ray shows diffuse abnormality of pulmonary
parenchymal,including nodules, linear(reticular)
infiltrates
pulmonary function tests shows restrictive pattern,
reduced diffusing capacity
tissue biopsy shows pulmonary fibrosis and alveolar
inflammation

5. •Early acute stage is alveolitis (injury
with inflammatory cell infiltration)
•Late stage characterised by fibrosis

6. EtiologyEtiology,,
Connective Tissue DiseasesConnective Tissue Diseases
Drug-inducedDrug-induced
Primary UnclassifiedPrimary Unclassified
OccupationalOccupational
Idiopathic DisordersIdiopathic Disorders

7. Etiology , known cause, CTDEtiology , known cause, CTD
Scleroderma
Polymyositis-dermatomyositis
Systemic lupus erythematosus
Rheumatoid arthritis**
Mixed connective tissue disease
Ankylosing spondylitis
Behcet’s
Sjogren’s syndrome

8. Etiology , known cause, DrugsEtiology , known cause, Drugs
Antibiotics-Antibiotics-- nitrofurantoin, sulfasalazine, ethambutol- nitrofurantoin, sulfasalazine, ethambutol
AntiarrhythmicsAntiarrhythmics--amiodarone, propranolol.--amiodarone, propranolol.
Anti-inflammatories-Anti-inflammatories--gold, penicillamine,aspirin-gold, penicillamine,aspirin
AnticonvulsantsAnticonvulsants-carbamazepine-carbamazepine
Chemotherapeutic agents-Chemotherapeutic agents--mitomycin C, bleomycin,-mitomycin C, bleomycin,
busulfan, methotrexate.busulfan, methotrexate.
Therapeutic radiationTherapeutic radiation
Oxygen toxicityOxygen toxicity
Narcotics–Narcotics– Heroin, CocaineHeroin, Cocaine

9. Etiology , known cause,Etiology , known cause, OccupationalOccupational
Silicosis
Asbestosis
Hard-metal pneumoconiosis
Coal worker's pneumoconiosis
Berylliosis
Siderosis
Stannosis (tin)

10. Etiology, unknown causeEtiology, unknown cause
Sarcoidosis
Eosinophilic
granuloma
Amyloidosis
Lymphangitic
carcinomatosis
Broncholaveolar
carcinoma
Pulmonary lymphoma
Neurofibromatosis
Acute respiratory
distress syndrome
AIDS
Bone marrow
transplantation
Postinfectious
Diffuse alveolar
hemorrhage
syndromes

11. How To Approach
the Diagnosis?

12. HistoryHistory
History of working in dusty job, drug
use,
History of connective tissue and/or
autoimmune disease e.g. joint pain in R
arthritis
History of bird breading
History of chemo and/or radiotherapy,

13. History, clinical manifestationsHistory, clinical manifestations
Progressive dyspnea.
Dry cough (without sputum).
Some patients may have fatigue, weight
loss, and other manifestation of the
cause,
e.g. joint pain in R arthritis, drugs, chemotherapy etc.

14. Physical examinationsPhysical examinations
Bilateral basilar, crepitations (velcro-like rale(velcro-like rale
))are found in most patients
wheezing, rhonchi and coarse rales are
occasionally heard
with advanced disease, patients may have
tachypnea and tachycardia
clubbingclubbing of the fingers and toes is common
At last, pulmonary hypertentionpulmonary hypertention and corcor
pulmonalepulmonale may be exist

15. RadiographyRadiography, c, chest x ray, CThest x ray, CT
It is important method to diagnose the ILDs. The majority of ILDsIt is important method to diagnose the ILDs. The majority of ILDs
cause infiltrates in the lower lung zonescause infiltrates in the lower lung zones..
AA diffuse ground glassdiffuse ground glass pattern is seen early inpattern is seen early in
the diseasethe disease
when the disease progresses, a chest radiographywhen the disease progresses, a chest radiography
demonstratesdemonstrates nodules, linear(reticular)nodules, linear(reticular)
infiltrates,infiltrates, or a combination of the twoor a combination of the two
The infiltrates become coarser andThe infiltrates become coarser and lung volumelung volume
is lostis lost
Honeycomb patternHoneycomb pattern may appear at the end ofmay appear at the end of
the diseasethe disease

16. This 50-year-old man presented with end-stage lung fibrosis
PA chest radiograph shows medium to coarse reticular
B: CT scan shows multiple small cysts (honeycombing) involving predominantly the subpleural
peripheral regions of lung. Traction bronchiectasis, another sign of end-stage lung fibrosis.

17. CT scan shows multiple bilateral round
circumscribed pulmonary nodules.

18. Pulmonary fibrosis and rheumatoid arthritis.

19. Pulmonary function testsPulmonary function tests
Pulmonary function tests of ILDs shows
restrictive pattern.restrictive pattern.It includes:
Reduced lung volumes(vital capacity,Reduced lung volumes(vital capacity,
total lung capacity)total lung capacity)
Reduced diffusing capacityReduced diffusing capacity
Static lung compliance is decreasedStatic lung compliance is decreased

20. Broncho-alveolar Lavage FluidBroncho-alveolar Lavage Fluid
examinationexamination
The cell counts in BALF of ILDs are
more than that of normal humans,for
examples:
Increases in polymorphonuclear leukocyte,
eosinophils, activated alveolar
macrophages, lymphocytes, cytokines and
growth factors for fibroblasts,

21. Lung biopsyLung biopsy
 TBLB (transbronchial biopsy),
 Open-lung or thoracoscopic
biopsy are used to diagnose the
ILDs

23. IPF is a chronic interstitial lung disease
of unknown etiologyunknown etiology . Nowadays It has
become a common disease.
The clinical manifestations, and
pulmonary function tests, chest
radiography examinations and lung
biopsy are similar to that of ILDs
introduced before.

24. Presence of all of the following major criteria as well as at least
three of the four minor criteria:
Major Criteria
1.Exclusion of other known causes of ILD, such as certain drug
toxicities, environmental exposures, and connective tissue diseases
2.Abnormal pulmonary function studies that include evidence of
restriction (reduced VC often with an increased FEV1/FVC ratio).
3.Bibasilar reticular abnormalities with minimal ground glass
opacities on HRCT scans.
4.Transbronchial lung biopsy or bronchoalveolar lavage (BAL)
showing no features to support an alternative diagnosis
How to diagnose IPFHow to diagnose IPF

25. Minor Criteria
Age >50 yr
Insidious onset of otherwise unexplained dyspnea
on exertion
Duration of illness>3 mo
Bibasilar, inspiratory crackles (dry or “Velcro” type
in quality)

26. Chest radiographyChest radiography

28. Treatment of IPFTreatment of IPF
CorticosteroidCorticosteroidss are the main therapy
 The initial treatment of choice is prednisone
0.5mg/kg of ideal body weight per day. For 1
month, the dose is gradually tapered over
several months to a maintenance dose of 0.125
mg/kg per day

29. Immunosuppressive agents, including
azathioprine, cyclophosphamide
Anti-fibrotic drugs, e.g. perfinidone
lung transplantation
Heart lung transplantation

30. Treatment
Some common therapies, including
Oxygen therapy,
Antibiotic therapy when pulmonary
infections exist.
Treatment of complications as heart
failure

33. Diseases associated with occupationalDiseases associated with occupational
exposureexposure
Airway diseasesAirway diseases
Asthma (reversible).
Chronic obstructive lung disease (irreversible).
Cancer
Parenchymal diseasesParenchymal diseases
 Hypersensitivity pneumonitis (reversible)
 Diffuse fibrosis (irreversible) eg. silicosis, asbestosis

34. Occupational asthmaOccupational asthma
 Disease characterized by variable airflow
obstruction and/or airway hyperresponsiveness
due to causes and conditions attributable to a
particular working environment and not to
stimuli encountered outside the workplace.
 The causative agentsThe causative agents are:
1. Animal or plant proteins
2. Latex rubber
3. Wood dust
4. Formaldehyde,glutaraldehyde.

35. Recognise the work causal-relationshipRecognise the work causal-relationship
Occupational history
Medical history suggesting work-relatedness
Symptoms started after employment
Improvement of symptoms during weekends and
holidays
Worsening of symptoms on returning to work
Objective testing

36. Objective testing to confirm work-relatednessObjective testing to confirm work-relatedness
• Pre and post-shift measurement of lung
function
• Monitoring of PEF at and off work, each for a
period of 2 weeks .
• Specific inhalation challenges or occupational
type of exposure tests - "gold standard"

37. Occupational exposure and chronicOccupational exposure and chronic
obstructive pulmonary disease (COPDobstructive pulmonary disease (COPD((
Long-term exposure toLong-term exposure to
Inorganic dustInorganic dust
Organic dustOrganic dust
Chemicals - vapors, irritants, fumesChemicals - vapors, irritants, fumes

39. DefinitionDefinition
Fibrosis and scarring of the lungs
secondary to repeated inhalation of
inorganic (mineral dust)mineral dust) associated with
certain occupation/s

40. Clinical types
SilicosisSilicosis
Coal worker pneumoconiosis (Anthracosis)Coal worker pneumoconiosis (Anthracosis)
AsbestosisAsbestosis
ByssinosisByssinosis
BagassosisBagassosis
Farmer’s lungFarmer’s lung

41. Repeated exposure
to dust particles
Inflammation
chronic
Elastic tissue Fibrous tissue
Scarring of lungs

42. COAL WORKER'S PNEUMOCONIOSISCOAL WORKER'S PNEUMOCONIOSIS
Coal worker's pneumoconiosis is a
lung disease that results from
breathing in coal dust over a long
period of time.
It was formally called anthracosis and
anthraco-silicosis when it occurred
together with silicosis.

43. It has 2 phases;
Simple pneumoconiosis: this is
associated with little ventilatory
impairement.
Progressive massive fibrosis or
complicated: this causes
severe respiratory disability and
premature death.

44. CAUSES
CAUSES: Coal (anthracene) dust inhaled alone or as
mixed silica dust.The following factors increase the risk ofThe following factors increase the risk of
coal worker’s pneumoconiosiscoal worker’s pneumoconiosis:
Type of dust
• Age at first exposureAge at first exposure
 Length of time spent undergroundLength of time spent underground
 SmokingSmoking
 Size of dust particlesSize of dust particles
 OCCUPATIONAL EXPOSURE: Coal mining at coalOCCUPATIONAL EXPOSURE: Coal mining at coal
faceface

45. PATHOLOGYPATHOLOGY
This results in inflammationinflammation of the
lungs, which then leads to fibrosisfibrosis
along with nodular lesions in the lungs,
 Finally, the centers of these lesions
may even become necrotic, causing
large size cavitiescavities in the lungs.
It is characterized by blackblack
pigmentationpigmentation of the lung parenchyme.

46. CLINICAL FEATURES
Chronic coughcough
Shortness of breath
Weight loss
Fever
Features of heart failure
Fine basal crepitations
Finger clubbing

47. RADIOLOGICAL FINDINGSRADIOLOGICAL FINDINGS
There are typically rounded opacitiesrounded opacities
of varying sizes in upper zones.
These opacities can be round or
irregular in outline.
They may calcify and coalesce into
large masses in PMFPMF

49. DIAGNOSIS
Occupational history
Signs and symptoms
Decreased forced expiratory
volume
Decreased total lung capacity
X-ray findings

50. COMPLICATIONS
Cor pulmonale
Caplan’s syndrome: is when
complicated coal worker's
pneumoconiosis occurs along with
rheumatoid arthritis

51. CONTROL
Prevention is the best, especially as
stoppage of further exposure in PMF
does not lead to a better prognosis.
Dust control at coal face.
Environmental monitoring and
personal protection
Periodic medical examination

52. SILICOSISSILICOSIS
Among the occupational lung
diseases, it’s the major cause of
permanent disability and mortality.
It was found out that the
incubation period may vary from a

53. CONT’D
CAUSES: Free silica dust or silicon
dioxide inhaled either in crystalline or
amorphous varieties. The commonest
crystalline form is quartz.
OCCUPATIONAL EXPOSURE:
mining, tunneling, sandblasting,mining, tunneling, sandblasting,
quarriesquarries

54. PATHOLOGY
The histologic lesion is the ‘silicotic‘silicotic
nodulenodule’ ranging from 3 to 4 mm in
diameter.
 These nodules are caused by death of
macrophages containing silica particles
with the release of silica and the
intracellular enzymes causing more and
more fibrosis.fibrosis.

55. Silicosis
Simple
Chronic nodular silicosis
Accelerated silicosis
Acute silicosis
Silicotuberculosis

56. CLINICAL FEATURES
May be symptomless initially
Irritant cough
Dyspnoea on exertion
Chest pain
Cyanosis
Pulmonary hypertension

57. RADIOLOGY FINDINGS
Shows egg- shell hilar calcificationegg- shell hilar calcification
and progressive massive fibrosisprogressive massive fibrosis. it also
shows snow-storm appearance in the
lung fields.
Emphysematous bullae are present in
the upper zones then later affect the
lower lobes.

58. Simple silicosis.
A: CT scan with lung windowing shows numerous circumscribed
pulmonary nodules, 2 to 3 mm in diameter (arrows).
B: CT scan with mediastinal windowing shows densely calcified
hilar (solid arrows) and subcarinal (dashed arrow) nodes.

59. Complicated silicosis. PA chest radiograph shows multiple
nodules involving the upper and middle lungs, with coalescence of
nodules in the left upper lobe resulting in early progressive

60. COMPLICATIONS
Tuberculosis (silico-tuberculosis)
Chronic bronchitis
Cor pulmonale

61. CONTROL
Control is by prevention as there is no
treatment.
Rigorous dust control measures E.g
personal protective equipment i.e.
masks or respirator with mechanical
filters or with oxygen substitution,
hydroblasting
Regular physical examination of
workers

62. ASBESTOSISASBESTOSIS
Is defined as fibrosis of the lungs
caused by asbestos dust.
Asbestos dust cause other diseases
which together with asbestosis are
termed asbestos-related disease.

63. CONT’D
 Asbestos are silicates of varying
composition; the silica is combined with
such bases as magnesium, iron, calcium,
sodium and aluminium.
 Asbestos has a unique combination ofAsbestos has a unique combination of
several useful properties such asseveral useful properties such as
 it is heat, acid and fire resistant
It is light, ductile, malleable .
 it can withstand a lot of weight.

64. CONT’D
There are two main types; chrysolite
and amphiboles;
Chrysolite (white asbestos) which is a
pure magnesium silicate
Amphiboles which contain a varying
amount of other minerals such as iron
and calcium.

65. CONT’D
CAUSE: asbestos dust
OCCUPATIONAL EXPOSURE: ship building,OCCUPATIONAL EXPOSURE: ship building,
motor manufacture,fire-resisting materials .motor manufacture,fire-resisting materials .
PATHOLOGY
Nodular areas more in lower lobes of lungs.
 Histologically, alveolitis with mononuclear infiltration. there
is fibrosis and calcification of the pleura.

66. CLINICAL FEATURES
 asymptomatic in mild cases
Increasing dyspnoea
finger clubbing
Cyanosis
Right heart failure
Fine basal crepitations

67. RADIOLOGICAL FINDINGS
There are small irregular and linear
shadows more in the lower zone.
It shows a ground –glass appearance
in the lower two thirds of the lung
fields.

68. Asbestos-Related Disorders
Asbestosis
Pleural plaques
Rounded
atelectasis
Chronic
obstructive airways
disease
Cancer
Lung cancer
Mesothelioma
If any occupational physician in this
room cannot recognize this as
advanced asbestosis, please
recognize that you are in trouble!

69. AsbestosAsbestos
plaquesplaques

70. AsbestosAsbestos
relatedrelated
lunglung
disorderdisorder

71. Rounded AtelectasisRounded Atelectasis

72. COMPLICATIONS
Lung cancer
Mesothelioma of the pleura and
peritoneum
Cor pulmonale
Pleural effusion

73. CONTROL
There is no cure, primary prevention is key
Pre-employment and periodic medical
examination for workers
Dust control measures. Many countries
have adopted a permissible limit of
exposure to airborne concentrations of
asbestos to 2 fibres/cc or even less

74. BYSSINOSISBYSSINOSIS
Byssinosis is due to inhalation of cotton
fibre dust over a long period of time.
 The main hazard occur when handling
the machines used for cleaning those
plant products of their impurities.
OCCUPATIONAL EXPOSURE: textile
workers with cotton, hemp , flax, jute or
kapok

75. CLINICAL FEATURES
Chronic cough which is
unproductive.
Progressive dyspnoea
Cyanosis
haemoptysis
dyspnoea
Chest tightness

76. RADIOLOGICAL FINDINGS
No specific changes
Advanced cases show the
changes characteristic of chroniccharacteristic of chronic
bronchitis and emphysema.bronchitis and emphysema.

77. DIAGNOSIS
History of exposure
Signs and symptoms
X-ray findings
Decreased total lung capacity and vital
capacity
CONTROL
Primary prevention is the key
Dust control programme
Proper dust control by exhaust ventilation

78. BAGASSOSISBAGASSOSIS
It is caused by inhalation of bagasse or sugar-canesugar-cane
dustdust after the sugar water has been pressed out. It was
first reported in Indian in 1955.
It has been shown to be due to a thermophilic
actinomycete for which the name thermoactinomyces
sacchari was suggested.
OCCUPATIONAL EXPOSURE: sugar cane work places
and exposure to bagasse.

79. CLINICAL FEATURES
Acute fever
Breathlessness
Cough
Haemoptysis
X-ray features of bronchitisbronchitis in
acute cases; otherwise normal.

80. DIAGNOSIS
Occupational history
Clinical presentation
Radiological findings
COMPLICATIONS
emphysema
bronchiectasis

81. CONTROL
Early disposal of sugar cane flax
Dust contro: such as wet process, personal
protective equipment i.e. masks or respirator
with mechanical filters or with oxygen.
Medical control: initial medical examination
and periodical medical check up of workers.
Bagasse control: by keeping the moisture
content above 20% and spraying the bagasse
with 2% propionic acid,a widely used fungicide.

82. BERYLLIOSISBERYLLIOSIS
This is due to exposure to beryllium
which is used in nuclear industries andnuclear industries and
in manufacturing of x-ray tubesin manufacturing of x-ray tubes and
aircrafts.
PATHOLOGY
Biopsy of the lesions show changes
similar to sarcoidosis with non caseatingnon caseating
granulomas and interstitial fibrosisgranulomas and interstitial fibrosis

83. CLINICAL FEATURES
Cough
breathlessness
CONTROL
Steroids may help in controlling the progression of
the disease.
Dust control

84. FARMERFARMER’’S LUNGS LUNG
Is due to inhalation of mouldy hay or grain dustmouldy hay or grain dust.
In grain dust or hay with a moisture content of over 30%,
bacteria and fungi grow rapidly causing a rise of
temperature to 40-50degree celsius.
This heat encourages the growth of thermophlilic
actinomycetes of which Micropolyspora faeni is the main
cause of farmer’s lung

85. CLINICAL FEATURES
Acute fever
Cough
Breathlessness
 COMPLICATION

Cor pumonale
Pulmonary damage

87. In interstitial lung diseases, lung function tests
most often show:
a. Reduced FEV1and VC
b. Increased total lung capacity (TLC)
c. Airflow obstruction
d. Elevated arterial PCO2.
In patients with suspected idiopathic pulmonary
fibrosis, the most valuable measure is:
a. Bronchoscopy
b. Sedimentation rate
c. Trial of steroids
d. Open lung biopsy

88. "Egg shell" calcification is seen in :
a-Bronchiolitis
b-Silicosis
c-Bronchogenic carcinoma
d-Pulmonary TB
Honeycombing of lung in chest radiograph is
seen in :
a-pleural effusion
b-Bronchial asthma
c- Bronchial Carcinoma
e-Interstitial lung disease

89. Most common symptom of interstitial Lung
disease is:
a-Hemoptysis
b-Progressive dyspnea
c-Substernal discomfort
d-Wheezing
Which of the following is one form of "interstitial
lung disease".
a-Asthma
b-Bronchiectasis
c-Idiopathic pulmonary fibrosis
d-Pulmonary hypertension

90. the following does not occur with asbestosis
a. Interstitial fibrosis
b. pleural mesothelioma
c. pleural calcification
d. Methhaemoglobinemia
which of the following disease coexists with
silicosis?
a. sarcoidosis
b. tuberculosis
c. lymphoma
d. rheumatoid arthritis

91. Which of the following is NOT a common
radiological feature of interstitial lung disease:
a- Ground glass pattern
b- Nodular infiltrates
c-Honeycombing
d- Generalized hypertranslucency
The main treatment for interstitial lung diseases
is:
a- Inhaled steroid
b- Antibiotic
c- Systemic steroid
d- Anticoagulant

92. -Which of the following is NOT a feature of
idiopathic pulmonary fibrosis?
a- Age of onset greater than 50 years
b- Bilateral apical inspiratory crackles
c- Restrictive pulmonary function test
d- Bilateral basal reticular abnormalities in chest CT
Pneumoconiosis is a group of diseases caused by
inhalation of:
a- smoke
b- Organic dust
c- Mineral dust
d- Pollens