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RESTRICTIVE LUNG DISEASE

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Definition:Restrictive lung disease is actually group of

disease with differing etiologies Thecommon link among these disorders isdifficulty in expanding the lung and the

reduction in lung volume .

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Characteristic :

All of lung volumes decreased VC < 80%FEV1/FVC still > 75%Loop volume flow is smaller thannormal but in the similar shape.

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Lung respiration process

Ventilation : Obstructive & Restrictive

Diffusion : Alveole wall, Interstitial,Endothel, Plasma, ErithrocytePerfussion : Lung Emboly, < Hb

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Restictive

1. Parenchymal disorder :Lung tumor

PneumoniaLung abcessLung edema

AtelectasisLung fibrosis : TB, Pneumoconiosis,fibrosis lung diss., collagen diss.,interstitial lung diss .

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2. Pleural disseases :EffusionPneumothoraxPleuritis sicca

Pleural tumor

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3. Chest wall/bone disorder :Broken rib, obesity, pectus excavatus,scoliosis, khiposis, gibbus

4. Neuromuscular dissease :Myasthenia gravis

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Normal vs. Obstructive vs.Restrictive

(Hyatt,2003)

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Treatment of Restrictive lung dysfunction

Goals Methods of treatment

Maintain adequate ventilation andRe-expand the lung tissues to

Prevent atelactasis

Deep diaphragmatic breathing

Exercise-Incentive Spirometery toImprove inspiratory capacity

Assist in the removal and

Mobilizing the secretion

Deep effective coughingModified postural drainage with

Percussion and vibration to

Specific area

Maintain adequate circulation inThe lower limbs to prevent

thrombosis

Active exercise especially anklemovements

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Treatment of Restrictive lung dysfunction

Goals Methods of treatment

Maintain range of motion inShoulders and trunk to prevent

Postural defect

Begin relaxation exercise to

Shoulder and trunk

Restore exercise toleranceBegin a progressive and graded

Ambulation programs

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Background

The lung volumes are reduced either because of:

1. Alteration in lung parenchyma.

2. Diseases of the pleura, chest wall or neuromuscularapparatus.

Physiologically restrictive lung diseases aredefined by reduced total lung capacity, vitalcapacity and functional residual capacity, butwith preserved air flow.

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Restrictive lung diseases may be divided into thefollowing groups:

Intrinsic lung diseases (diseases of the lung

parenchyma)

Extrinsic disorders (extra-parenchymaldiseases)

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Intrinsic Lung Diseases

These diseases cause either:Inflammation and/or scarring of lung tissue(interstitial lung disease)

orFill the air spaces with exudate and debris(pneumonitis).

These diseases are classified further accordingto the etiological factor.

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Extrinsic DisordersThe chest wall, pleura and respiratory muscles

are the components of respiratory pump.

Disorders of these structures will cause lungrestriction and impair ventilatory function.

These are grouped as:Non-muscular diseases of the chest wall.Neuromuscular disorders.

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PathophysiologyIntrinsic lung diseases:

Diffuse parenchymal disorders cause reduction in alllung volumes.This is produced by excessive elastic recoil of thelungs.Expiratory flows are reduced in proportion to lungvolumes.

Arterial hypoxemia is caused by ventilation/perfusion

mismatch.Impaired diffusion of oxygen will cause exercise-induced desaturation.Hyperventilation at rest secondary to reflexstimulation.

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Extrinsic Disorders

Diseases of the pleura, thoracic cage, decreasecompliance of respiratory system.There is reduction in lung volumes.Secondarily, atelectasis occurs leading to V/Qmismatch hypoxemia.The thoracic cage and neuromuscularstructures are a part of respiratory system.

Any disease of these structures will causerestrictive disease and ventilatory dysfunction.

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Diseases of the LungParenchyma

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Structure of the Alveolar Wall

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Diffuse Interstitial PulmonaryFibrosis

Synonyms: idiopathic pulmonary fibrosis, interstitialpneumonia, cryptogenic fibrosing alveolitis.

PathologyThickening 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

l

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EtiologyUnknown, may be immunological reaction.

Clinical FeaturesUncommon disease, affects adults in latemiddle 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 failureterminally.The disease progresses insidiously, median

survival 4-6 years.

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Pulmonary Function

Spirometry reveals arestrictive pattern. FVC isreduced, but FEV 1 /FVC

supernormal. All lung volumes TLC,FRC, RV are reduced.

Pressure volume curve ofthe lung is displaceddownward and flattened.

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Gas Exchange

Arterial PaO 2 and PaCO 2 are reduced, pHnormal.On exercise PaO 2 decreases dramatically.

Physiologic dead space and physiologic shuntand VQ mismatch are increased.Diffuse impairment contributes to hypoxemiaon exercise.There is marked reduction in diffusing capacitydue to thickening of blood gas barrier and VQmismatch.

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DiagnosisDiagnosis is often suggested by history, chestradiograph and high resolution CT scan of thelungs.

If old chest x-rays show classical disease,absence of other disease processes on historyand no occupational or environmental

exposure clinical diagnosis can be made.

In other cases a surgical lung biopsy isobtained.

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TreatmentEach patient is individually assessed.

Patients are treated if they have symptoms orprogressive dysfunction on pulmonary function tests.Corticosteroids (Prednisone 1 mg/kg) is standardtherapy.Prednisone dose is lowered over 6-8 weeks andcontinued 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.

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Sarcoidosis

A disease characterized by the presence ofgranulomatous 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 andlymphocytes.In advanced lung disease fibrotic changes are

seen.

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EtiologyUnknown, likely immunological basis.

Clinical FeaturesFour stages are identified:

Stage 0: No obvious intrathoracic involvementStage 1: Bilateral hilar lymphadenopathy, oftenaccompanied by arthritis, uveitis and erythemanodosum.Stage 2: Pulmonary parenchyma is also involved,changes in mid and upper zones.Stage 3: Pulmonary infiltrates and fibrosis without

adenopathy.

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Non-caseating granulomasin Sarcoidosis

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Stage I(bilateral hilar adenopathy)

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Stage IIReticular nodules and BHL

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HRCT subpleural nodules

Pulmonary Function

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Pulmonary FunctionNo impairment occurs in stages 0 and 1.

In stages 2 and 3 restrictive changes are seen.Treatment and Prognosis85% of these patients improve spontaneously,

but 15% may develop progressive fibrosis andrespiratory failure.Treatment is other observation, but in

symptomatic patients or deteriorating PFTs treatment recommended.Prednisone 0.5- 1 mg/kg initially, then taperedand continued for 6 months to 1 year.

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Hypersensitivity Pneumonitis Also known as extrinsic allergic alveolitis.Hypersensitivity reaction in the lung occurs inresponse to inhaled organic dust.Example is farmers lung. The exposure may be occupational orenvironmental.The disease occurs from type III and type IV

hypersensitivity reactions.Farmers lung is due to thermophilicactinomyces in moldy hay.

Bird fanciers lung is caused by avian antigen.

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Clinical FeaturesThe disease may occur in acute or chronic forms.

Acute HPDyspnea, fever, malaise and cough appear 4-6 hoursafter exposure.These symptoms continue for 24-48 hours.Physical examination shows fine crackles throughoutthe lungs.

These patients present with progressive dyspnea overa period of years.Chest radiograph may be normal, but may showreticular nodular infiltration.

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Chronic HP

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Chronic HP

These patients present with progressivedyspnea.Physical examination shows bilateral inspiratory

crackles.Chest x-ray shows reticular nodular infiltrationand fibrosis predominantly in upper lobes.

Pulmonary function tests restrictive pattern.Gas exchange shows hypoxemia which worsenson exercise.

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Collagen Vascular Diseases

Several collagen vascular diseases particularlysystemic sclerosis and lupus and rheumatoid

arthritis may lead to systemic sclerosis.Dyspnea is often severe. A definite diagnosis requires surgical lungbiopsy.Treatment is corticosteroids plus cytotoxictherapy.

Pl l Di

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Pleural Diseases

Pneumothorax could be either primary orsecondary.Pleural effusion can be acute or chronic.Pleural effusion is divided into exudate andtransudate.Pleural thickening longstanding pleuraleffusion results in fibrotic pleura which splints

the lung and prevents its expansion.If the disease is bilateral may causerestrictive lung diease.Treatment may be decortication.

Diseases of the Chest Wall

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Diseases of the Chest WallDeformity of thoracic cage such as kyphoscoliosis andankylosing spondylitis.Scoliosis lateral curvature of spine, kyphosis posterior curvature.Cause is unknown, polio and previous tuberculosis.

Patients develop exertional dyspnea, rapid shallowbreathing.Hypoxemia, hypercapnia and cor-pulmonale supervene.Pulmonary function tests show RVP with normaldiffusion.Cause of death is respiratory failure or intracurrentpulmonary infection.Treatment is non-invasive or invasive chronicventilation.

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Neuromuscular Disorders

Diseases affecting muscles of respiration or theirnerve 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 andblood gases.Maximal inspiratory and expiratory pressures arereduced.Treatment is either treating the underlying cause orassisted ventilation.

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