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RESTRICTIVE LUNG DISEASE

Objective :
Describe the etiologies of Restrictive
Lung Disease
Understand the different between
Restrictive and obstrucktive lung
disease
Describe the value of Pulmonary
Function Testing
Background
The lung volumes are reduced either because of:

1. Alteration in lung parenchyma.

2. Diseases of the pleura, chest wall or neuromuscular


apparatus.

Physiologically restrictive lung diseases


are defined by reduced total lung
capacity, vital capacity and functional
residual capacity, but with preserved air
flow.
Restrictive lung diseases may be divided
into the following groups:

Intrinsic lung diseases (diseases of the


lung parenchyma)

Extrinsic disorders (extra-parenchymal


diseases)
Intrinsic Lung Diseases
These diseases cause either:
Inflammation and/or scarring of lung
tissue (interstitial lung disease)
or
Fill the air spaces with exudate and debris
(pneumonitis).

These diseases are classified further


according to the etiological factor.
Extrinsic Disorders
The chest wall, pleura and respiratory
muscles are the components of respiratory
pump.

Disorders of these structures will cause lung


restriction and impair ventilatory function.

These are grouped as:


Non-muscular diseases of the chest wall.
Neuromuscular disorders.
Pathophysiology
Intrinsic lung diseases:
Diffuse parenchymal disorders cause reduction
in all lung volumes.
This is produced by excessive elastic recoil of
the lungs.
Expiratory flows are reduced in proportion to
lung volumes.
Arterial hypoxemia is caused by
ventilation/perfusion mismatch.
Impaired diffusion of oxygen will cause exercise-
induced desaturation.
Hyperventilation at rest secondary to reflex
stimulation.
Extrinsic Disorders

Diseases of the pleura, thoracic cage,


decrease compliance of respiratory system.
There is reduction in lung volumes.
Secondarily, atelectasis occurs leading to
V/Q mismatch hypoxemia.
The thoracic cage and neuromuscular
structures are a part of respiratory system.
Any disease of these structures will cause
restrictive disease and ventilatory
dysfunction.
Diseases of the
Lung Parenchyma
Structure of the Alveolar
Wall
Interstitium
Diffuse Interstitial Pulmonary
Fibrosis
Synonyms: idiopathic pulmonary fibrosis, interstitial
pneumonia, cryptogenic fibrosing alveolitis.

Pathology
Thickening of interstitium.
Initially, infiltration with lymphocytes and plasma
cells.
Later fibroblasts lay down thick collagen bundles.
These changes occur irregularly within the lung.
Eventually alveolar architecture is destroyed
honeycomb lung
Etiology
Unknown, may be immunological reaction.
Clinical Features
Uncommon disease, affects adults in late
middle age.
Progressive exertional dyspnea, later at rest.
Non-productive cough.
Physical examination shows finger clubbing,
fine inspiratory crackles throughout both
lungs.
Patient may develop respiratory failure
terminally.
The disease progresses insidiously, median
survival 4-6 years.
Pulmonary Function
Spirometry reveals a
restrictive pattern.
FVC is reduced, but
FEV1/FVC supernormal.
All lung volumes TLC,
FRC, RV are reduced.
Pressure volume curve
of the lung is displaced
downward and
flattened.
Gas Exchange
Arterial PaO2 and PaCO2 are reduced, pH
normal.
On exercise PaO2 decreases dramatically.
Physiologic dead space and physiologic
shunt and VQ mismatch are increased.
Diffuse impairment contributes to
hypoxemia on exercise.
There is marked reduction in diffusing
capacity due to thickening of blood gas
barrier and VQ mismatch.
Diagnosis
Diagnosis is often suggested by history,
chest radiograph and high resolution CT
scan of the lungs.

If old chest x-rays show classical disease,


absence of other disease processes on
history and no occupational or
environmental exposure clinical
diagnosis can be made.

In other cases a surgical lung biopsy is


obtained.
Treatment
Each patient is individually assessed.
Patients are treated if they have symptoms or
progressive dysfunction on pulmonary function
tests.
Corticosteroids (Prednisone 1 mg/kg) is standard
therapy.
Prednisone dose is lowered over 6-8 weeks and
continued at 15 mg for 1-2 years.
Addition of Imuran may benefit survival.
Cyclophosphamide occasionally used.
Antifibrotics such as colchicine may be used.
Ancillary therapies such as oxygen, rehabilitation,
psychosocial aspects are helpful.
Sarcoidosis
A disease characterized by the presence
of granulomatous tissue.
This is a systemic disease which involves
eyes, brain, heart, lungs, bones and
kidneys, skin, liver and spleen.
On pathology a non-caseating granuloma
composed of histiocytes, giant cells and
lymphocytes.
In advanced lung disease fibrotic changes
are seen.
Etiology
Unknown, likely immunological basis.

Clinical Features
Four stages are identified:
Stage 0: No obvious intrathoracic involvement
Stage 1: Bilateral hilar lymphadenopathy, often
accompanied by arthritis, uveitis and erythema
nodosum.
Stage 2: Pulmonary parenchyma is also
involved, changes in mid and upper zones.
Stage 3: Pulmonary infiltrates and fibrosis
without adenopathy.
Non-caseating granulomas
in Sarcoidosis
Stage I
(bilateral hilar adenopathy)
Stage II
Reticular nodules and BHL
HRCT subpleural nodules
Pulmonary Function
No impairment occurs in stages 0 and 1.
In stages 2 and 3 restrictive changes are
seen.
Treatment and Prognosis
85% of these patients improve
spontaneously, but 15% may develop
progressive fibrosis and respiratory failure.
Treatment is other observation, but in
symptomatic patients or deteriorating PFTs
treatment recommended.
Prednisone 0.5- 1 mg/kg initially, then
tapered and continued for 6 months to 1 year.
Hypersensitivity
Pneumonitis
Also known as extrinsic allergic alveolitis.
Hypersensitivity reaction in the lung
occurs in response to inhaled organic dust.
Example is farmers lung.
The exposure may be occupational or
environmental.
The disease occurs from type III and type
IV hypersensitivity reactions.
Farmers lung is due to thermophilic
actinomyces in moldy hay.
Bird fanciers lung is caused by avian
antigen.
Pathology

There is infiltration of alveolar walls with


lymphocytes, plasma cells and histiocytes.
There are loosely formed granulomas.
Fibrotic changes occur in advanced disease.
Clinical Features
The disease may occur in acute or chronic
forms.

Acute HP
Dyspnea, fever, malaise and cough appear 4-6
hours after exposure.
These symptoms continue for 24-48 hours.
Physical examination shows fine crackles
throughout the lungs.
These patients present with progressive
dyspnea over a period of years.
Chest radiograph may be normal, but may show
reticular nodular infiltration.
HRCT in Acute HP
Chronic HP

These patients present with progressive


dyspnea.
Physical examination shows bilateral
inspiratory crackles.
Chest x-ray shows reticular nodular
infiltration and fibrosis predominantly in
upper lobes.
Pulmonary function tests restrictive
pattern.
Gas exchange shows hypoxemia which
worsens on exercise.
Interstitial Disease Caused by
Drugs, Poisons and Radiation

Various drugs cause acute pulmonary


reaction proceeding to interstitial fibrosis.
These drugs are busulfan, nitrofurantoin,
amiodarone, bleomycin.
High oxygen concentration interstitial
fibrosis.
Radiation exposure acute pneumonitis
fibrosis.
Collagen Vascular Diseases

Several collagen vascular diseases


particularly systemic sclerosis and lupus and
rheumatoid arthritis may lead to systemic
sclerosis.
Dyspnea is often severe.
A definite diagnosis requires surgical lung
biopsy.
Treatment is corticosteroids plus cytotoxic
therapy.
Pleural Diseases

Pneumothorax could be either primary or


secondary.
Pleural effusion can be acute or chronic.
Pleural effusion is divided into exudate and
transudate.
Pleural thickening longstanding pleural
effusion results in fibrotic pleura which
splints the lung and prevents its expansion.
If the disease is bilateral may cause
restrictive lung diease.
Treatment may be decortication.
Diseases of the Chest Wall
Deformity of thoracic cage such as kyphoscoliosis and
ankylosing spondylitis.
Scoliosis lateral curvature of spine, kyphosis
posterior curvature.
Cause is unknown, polio and previous tuberculosis.
Patients develop exertional dyspnea, rapid shallow
breathing.
Hypoxemia, hypercapnia and cor-pulmonale supervene.
Pulmonary function tests show RVP with normal
diffusion.
Cause of death is respiratory failure or intracurrent
pulmonary infection.
Treatment is non-invasive or invasive chronic
ventilation.
Neuromuscular Disorders
Diseases affecting muscles of respiration or their
nerve supply.
Poliomyelitis, Guillain-Barre syndrome, ALS,
myasthenia gravis, muscular dystrophies.
All these lead to dyspnea and respiratory failure.
PFTs show reduced FVC, TLC and FEV 1.
The progress of disease can be monitored by FVC
and blood gases.
Maximal inspiratory and expiratory pressures are
reduced.
Treatment is either treating the underlying cause or
assisted ventilation.
Restrictive Lung Disease
Resume

Any process that


interferes with the
bellows action of the
lungs or chest wall
can cause
restriction.
Reduction in lung
volumes
1. Alteration in lung parenchyma :
Lung Fibrosis :
TB
IPF
Pneumoconiosis
Collagen diseases
Interstitial lung disease
Lung Tumor
Pneumonia
Lung abces
Lung edema
Atelectasis
2. Pleural Disease :
Pleural effusion
Pneumothorax
Schwarte
Pleural tumor

3. Chest wall disorder


Fracture
Pectus excavatus
Scoliosis, Khiposis, Gibbus
Obesity
4. Neuromuscular disorder

5. Mediastinum disorder :
Cardiomegaly
Mediastinum Tumor
Pericardial effusion

6. Diaphragma disorder
Hernia, paralysis
Acites, pregnancy