pola penyakit restriktif paru

8/13/2019 Pola Penyakit Restriktif Paru

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dr. Heny Anggraeny Lenap


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Restriction means:

decrease in lung volumes (vital capacity, total

lung capacity) normal airflow (normal FEV1/FVC ratio)

diffusing capacity may be decreased in interstitial

types of restrictive lung disease


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Definition:

Reduced lung compliance

More pressure needed to expand lungs Lungs are stiff

Pulmonary function test: Low FEV1, Low FVC (the

ratio FEV1/FVC is normal)


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Types: Chest wall abnormality (not primary lung)

Deformities, kyphoscoliosis

Neuromuscular disease

Primary lung disease Acute: Acute Respiratory Distress Syndrome ARDS

Chronic:

Occupational: Asbestosis, silicosis, coal worker pneumoconiosis

Interstitial lung disease (interstitial pneumonia), Idiopathic pulmonaryfibrosis

Immune diseases: Sarcoidosis, SLE, RA, Wegener

Physical injury: : Radiation

Drugs: Chemotherapy, methotrexate


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Interstitial lung disease e.g. fibrosis of lung Pleural disease e.g. effusion, fibrosis of pleura

Chest wall e.g kyphoscoliosis

Neuromuscular disorder e.g. ALS, stroke Extrapulmonary e.g. massive ascites, obesity

Surgical removal of lung tissue


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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 are

defined by reduced total lung capacity, vitalcapacity and functional residual capacity, but withpreserved air flow.


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Restrictive lung diseases may be divided into thefollowing groups:

Intrinsic lung diseases (diseases of the lungparenchyma)

Extrinsic disorders (extra-parenchymal diseases)


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Gas exchange barrier:

1. Basement membrane

2. Interstitial tissue3. Endothelial cell

4. Epithelial cell


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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.


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Initial injury to cells: endothelial, epithelial This is followed by reaction that end by

interstitial fibrosis stiff lung dyspnea Damage to epithelium and vessels

abnormal ventilation-perfusion Hypoxia cyanosis

Pulmonary hypertension cor pulmonale


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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 tothe etiological factor.


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The chest wall, pleura and respiratory muscles arethe 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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Diseases of the pleura, thoracic cage, decreasecompliance of respiratory system.

There is reduction in lung volumes. Secondarily, atelectasis occurs leading to V/Q

mismatchhypoxemia. The thoracic cage and neuromuscular structures

are a part of respiratory system. Any disease of these structures will cause

restrictive disease and ventilatory dysfunction.


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Occupational Hypersensitivity pneumonitis

Drug-induced

Collagen-vascular disease Cancer / lymphangitic metastasis

Granulomatous disorders

Idiopathic interstitial pneumonias Others


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Asbestosis interstitial disease due to asbestos Berrylium

Hard-metal disease e.g. tungsten-carbide


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Organic materials examples

Thermophilic bacteria, fungi Farmers lung

Fungi

ventilation pneumonitis

Avian proteinsbird breeders lung

Chemicals toluene diisocyanate (TDI) & trimellitic

anhydride (paints, resins, plastics)


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Cytotoxic drugs Bleomycin, BCNU, cyclophosphamide, methrotrexate

Irradiation (usually confined to radiation field)

Non-cytotoxic Nitrofurantoin, Carbamazepine

Amiodarone, Tocainide, Hydralazine,

Methadone IV (talc)

Many others!!


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Rheumatoid arthritis Scleroderma (systemic sclerosis)

Polymyositis & Dermatomyositis

Sjogrens syndrome Less commonly - SLE & MCTD.

*These disease have other lung manifestations*

*Also consider infection and medications*


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Sarcoidosis a multisystem disease of unknownetiology.

Various clinical presentations -> lung is involved in

most patients. Non-caseating granuloma in many tissues and

organs


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> a group of 7 interstitial lung disorders with specificcharacteristics that distinguish them from otherforms of interstitial lung disorders.

> they are not infectious - despite use of the termpneumonias

> they produce inflammation/fibrosis of the lunginterstitial space.


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Idiopathic pulmonary fibrosis (IPF) Nonspecific interstitial pneumonia

Cryptogenic organizing pneumonia (BOOP)

Acute interstitial pneumonia Respiratory bronchiolitis-associated interstitial lung

disease

Desquamative interstitial pneumonia

Lymphocytic interstitial pneumonia


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