Lecture 8 - restrictive disease Flashcards
What is the def of restrictive lung disease?
Loss of compliance of the lungs and/or chest wall which prevents the lungs from expanding fully.
what are th expected PFTs in restrictive lung disease?
VC, IC, TLC, RV, DLCO
↓ VC
↓ IC
↓ TLC
Normal or ↓ RV
↓ DLCO if restriction pulmonary in origin
S/s of restrictive lung disease are what in a non-advanced state?
Tachypnea Decreased breath sounds : dry inspiratory rales Dyspnea Cough: dry, irritating, non-productive
S/s of restrictive lung disease are what in a advanced state?
V/Q mismatch -> hypoxemia -> pulmonary hypertension -> cor pulmonale
Weight loss: increased work of breathing, decreased appetite
What is the name of abnormal healing response to multiple microscopic sites of acute alveolar injury that progress to fibrosis.
Interstitial Pulmonary Fibrosis
Whats are the causes of inter.pulm.fibro?
Occupational and environmental exposure: Inorganic dusts (silica, coal), Toxic gases, Drugs (ex: amiodarone, anticoag)
Poisons
Connective tissue disorders
- i.e. RA, systemic sclerosis, lupus
Genetic link
What is the most common cause of lung restriction?
Idiopathic Pulmonary Fibrosis
What is a Idiopathic Pulmonary Fibrosis
Chronic progressive irreversible disease of unknown cause.
what are possible causes of idio.pulm.fibro?
Risk factors: epithelial injury, genetic factors, maybe infection, microbes
Older age -> mor at risk, micro injury
Smoking, reflux (RGO) chronic, sleep apnea, ongoing reasearch
What are the s/s of idio.pulm.fibrosis?
Resp
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Body structure and functions
Fatigue
Chronic unproductive cough
Dyspnea: on exertion initially increase at rest with disease progression
Digital clubbing
Rapid/shallow breathing
↓ chest expansion
Cyanosis as disease progresses
Weight loss, decrease in appetite
Sleep disturbances with loss of REM sleep
what are the pulm fct of idio.pulm.fibrosis? Lung capacities FEV1, FVC Compliance DLCO Hypoxemia
↓ static lung volumes and capacities Spirometric function preserved ↓ lung compliance ↓ DLCO hypoxemia with exercise initially -> evident at rest or exaggerated by exercise with disease progression
What is an Interstitial Pulmonary Fibrosis?
An it’s process?
Abnormal healing response to multiple microscopic sites of acute alveolar injury that progress to fibrosis.
Triggered by alveolitis -> response of lung tissus cells -> accumul of fibro & myo blasts -> collagen -> scarring
What are the x-rays findings with pulm fibrosis?
Interstitial pattern is rough with evidence of honeycombing in the bases.
Explain the restrictive /both lung disease cascade
Diapo 13-14 lecture 8
what are the tx for idio.pulm.fibro (8)?
Antifibrotics: Nintedonib, pirfenidone
Smoking cessation
Supplemental O2
Antibiotic therapy for secondary infections
Nutritional support
Candidates for single or double lung transplantation
Might increase mortality rates:
Corticosteroids
Immunosuppressive therapy: cyclophosphamide
what ar the 2 occupational lung diseases?
Mineral dusts (Pneumoconiosis) Asbestos, coal and silica
Organic dusts (Hypersensitivity pneumonitis)
Silicosis:
where does it come from?
Silica dust: Byproduct of industry i.e. Quarry work, sandblasting, pottery making, stone masonry, mining (coal, copper, tin)
Silicosis: when is the onset?
Onset 15-20 years following initial exposure.
Silicosis: what does it cause?
Hard nodular deposits in lung parenchyma & peribronchial vascular regions.
- affects ULs > LLs of the lung
Silicosis: whats the s/s
Asymptomatic or dry cough &
mild dyspnea
Silicosis: whats the complication?
Complication: severe pulmonary
restriction -↓ lung volumes,
hypoxia, severe dyspnea,
predisposition to TB
Coal dust:
- whats the name
- whats the onset
- what does is causes
coal workers pneumoconiosis
“Black lung disease” – 10-15 year latency
Small black nodules or areas of discoloration.
T/F pneumoconiosis is a Pathogenesis related to quantity of silica (amoong others) in the dust.
T
Pathogenesis related to quantity of coal dust and level of quartz (silica) in the dust.
T/F pneumoconiosis is a Chronic bronchitis
T
Chronic bronchitis – black secretions.
What kind of fibrosis pneumoconiosis is?
progressive fibrosis
What pneumoconiosis can form?
Form of centrilobular emphysema
also common.
What causes asbestos in the lungs?
Pleural effusions, plaque development, asbestosis, asbestos-related malignancies
What is the pathogenesis of asbestos?
Pathogenesis:
Macrophages engulf asbestos fibers in
lung and pulmonary interstitium - fibrous
tissue deposition.
Honeycomb appearance of lung parenchyma.
Pulmonary carcinoma, malignant mesothelioma.
Asbestos causes:
A) obstruction/restruction lund disease
B) Incr/decr DLCO
Restrictive lung disease, ↓DLCO
What are the finding of asbestosis in an x-ray? On a CT-scan
x-ray: Asbestosis with calcific pleural plaques and an interstitial parenchymal pattern.
Computed tomogram demonstrating calcific asbestosis-related pleural plaques in the right posterior region (arrows).
What causes hypersensitivity pneumonitis (6)?
Whats the other name of disease?
Caused by microorganism, animal or insect protein moldy hay (Farmer lung) mushroom compost (Mushroom-worker lung) bird droppings (Pigeon-breeder lung) moldy cork ( Suberosis) moldy barley ( Malt-worker lung) insect dust ( Miller lung)
extrinsic allergic alveolitis
hyper.pneumo causes what rxn?
Acute inflammatory response – lymphocytes, plasma cells & eosinophils in alveolar septa and around bronchioles
hypr.pneumo: obstructive or restrictive?
Obstructive pattern acutely
Restrictive pattern with chronic exposure.
hyper.pneumo: what does it look like on x-rays?
CXR similar regardless of type of organic dust inhaled - varies according to the intensity of exposure.
Early stages: reversible, multiple, small (1- to 3-mm), nodular radiodensities scattered bilaterally; lung apices may be spared
Repeated exposures: chronic interstitial (honeycomb) pattern with upper lobe predominance.
How do we treat restrictive lung disease (5)?
Avoidance of exposure to causative agent
Supportive interventions: Smoking cessation
Supplemental O2
Good nutrition
Meds:
Antibiotics for secondary pulmonary infection
Corticosteroids, interferon, collagen-inhibiting agent, cytotoxic drugs (cyclophosphomide, axathioprine)
Temporary invasive or noninvasive mechanical ventilation
Lung transplantation
What are the interventions in physio?
Interventions to increase ventilation:
Breathing exercises
Chest mobility, posture exercises
Pulmonary rehabilitation:
Progressive exercise training -> maximize remaining lung function and -> activity tolerance
- monitor for hypoxemia during exercise!!!
Respiratory muscle strength and endurance training
- Paced breathing/ energy conservation techniques
- Supplemental oxygen for hypoxemic patients
where do you see the honeycomb pattern?
Hypersensitivity pneumonitis
Pulmonary fibrosis
Asbestosis
