pola penyakit restriktif paru
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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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