CM- pulm -10- Restrictive Airway Disease
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| Diminished Lung Volume Decreased TLC, FVC Normal or elevated FEV1/FVC ratio | Restrictive Lung Disease
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| What are interstitial pulmonary fibrosis; and sarcoidosis | restrictive lung diseases
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| What are restrictive lungs disease defined as | diseases that are caused by reduced lung volume due to intrinsic lung diseases that alter lung parenchyma or extrinisc diseases that effect pleura, chest wall or thoracic neuromusculature
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| What are intrinsic lung restrictive diseases defined as | diseases of the lung parenchyma, Cause inflamattion or scarring of the actual lung tissue- Interstitial lung disease
-Filling air space with exudate - pneumonitis
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| what are some etiologic factors that characterize intrinsic lung diseas | idiopathic fibriotic disease
connective tissue diseases
drug-induced lung diseases
primary diseases of the lung (sarcoidosis)
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| How would you define extrinsic restrictive lung diseases | extraparenchymal diseases ie diseases that affect the chest wall, pleura and respiratory muscles
cause lung restrcition and impaired ventilatory function and respiratory failure
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| what restrictive pathophysiology does the following def apply to : reduce all lung volumes by excessive elastic recoil of the lungs. Expiratory airflow is reduced in proportion to lung volume. | Intrinsic Pathophysiology
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| what restrictive pathophysiology does the following def apply to : reduces the total compliance and lung volumes are reduced | Extrinsic Pathophysiology
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| What is a common extrinisc disorder in elderly patients with restrictive lung disease | kyphoscoliosis
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| what is the medain survival rate for interstitial pulmonary fibrosis | less than 3 yrs
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| What is an acute vs subacute vs a chronic restrictive lung disease | acute- lasts days to weeks
subacute- lasting weeks to months
chronic-lasting months to years
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| The following diseases often are classified as which of the following acute, subacute or chronic -Interstitial pneumonitis, eosinophilic pneumonia, and diffuse alveolar hemorrhage | Acute
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| The following diseases often are classified as which of the following acute, subacute or chronic -sarcoidosis, drug-induced interstitial lung disease, alveolar hemorrhage syndrome and connective tissue diseases | subacute
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| The following diseases often are classified as which of the following acute, subacute or chronic -IPF, Sarcoidosis, and pulmonary histiocytosis X | Chronic
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| What are some risk factors for developing restrictive lung disease | -smoking
-prior medication use of
Common drugs include nitrofurantoin, amiodarone, gold, chemotherapeutic agents, procainamide and hydralazine
-Radiation tx
-FX
-Occupational History
-Environmental exposure
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| what are the s/sx of intrinsic restrictive airway diseases | -predominant is Progressive exertional dyspnea
-Dry Cough (common and disturbing sign)
-Hemoptysis (w/ specific diseases)
Wheezing is not common
Chest pain is not common
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| What is the worst grade of dyspnea | dyspnea at rest
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| What are the two divisions of extrinsic restrictive lung disease | nonmuscular-eg kyphoscoliosis
neuromuscular-
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| What will you find on physical exam of intrinsic restrictive airway disease | velcro crackles- common in most with interstitial lung disorders
Digital Clubbing- common with IPF
Extrapulmonary findings- Erythema nodosum-sarcoidosis; Raynauds- scelroderma
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| What will you typically find on Physical Exam in extrinisic restrictive airway disease | severe kyphoscoliosis
massive obesity
pleural disorders- Decreased tactile fremitus, dullness on percussion, decreased breath sounds
Neuromuscular diseases- accessory muscle use, rapid shallow breathing
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| What labs would you order for intrinsic Rest. airway disease | Anemia-Vasculitis
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| What labs would you order for ext. rest. airway disease | elevated CK may indicate myositis
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| what will you see on chest x-ray if the restrictive disease is intrinsic | reticular pattern "ground glass" increased interstitial markings, bilateral hilar lymphadenopathy w/ sarcoidosis
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| What will you possibly see on chest x-ray if the disease is extrinisc restricitve | chest wall deformities
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| why would you perfrom a bronchoalveolar lavage | to get cells to narrow diagnosis
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| If you needed to get a tissue sample for anaylysis from the lung what would you likely perform | lung biopsy
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| what is the tx for restrictive airway disease | corticosteroids, immunosuppresive agents and cytotoxic agents
Extrinsic- may need help breathing especially at night and if obese lose weight
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| What is IPF | diffuse interstitial pulmonary fibrosis
also calle idiopathic pulmonary fibrosis, interstitial pneumonia, cryptogenic fibrosing alveolitis
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| What is the pathology of IPF | thickening of interstitium
initially, infiltration with lymphocytes and plasma cells.
later fibroblasts lay down thick collagen bundles
Changes occur irregularly in lung eventually leads to honeycomb lung with destruction of alveolar architecture
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| what is the etiologic cause of IPF | unknown may be immunological rxn
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| How does it present clinically | affects adults in late middle age
progressive extertional dyspnea, later at rest
non productive cough
on PE, clubbing, fine inspiratory crackles both lungs
develop respiratory failure terminally
disease progression is insidious survival <3yrs
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| lung disease characterized by granulomatous tissue is a systemic disease which involes eyes, brain, lungs, bones, kidneys, skin, liver and spleen. non-caseating granuloma composed of histiocytes, giant cells, and lymphocytes | sarcoidosis
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| what is the etiology of sarcoidosis | uk, likely immunological basis
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| What are the clinical features of sarcoidosis | stage 0: no intrathoracic involvement
stage 1: bilateral hilar lymphadenopathy often w/ arthritis, uveitis, erythema nodosum
stage 2: pulm parenchyma is involved changes in mid and upper zones
stage 3: pulm infiltrates and fibrosis w/o adenopathy
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| also known as extrinsic allergic alveolitis, occurs in response to inhaled organic dust. exposure may be occupational or environmental | Hypersensitivity pneumonitis
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| What is an example of hypersensitivity pneumonitis | farmer's lung, bird fancier's lung
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| what type of hypersensitivity rxn is hypersensitivity pneumonitis | type III and Type IV hypersensitivity rxn
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| what type of hypersensitivity rxn is due to thermophilic actinomyces | farmer's lung from moldy hay
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| what is the pathology of hypersensitivity pneumonitis | infiltration of alveolar walls with lymphocytes, plasma cells and histiocytes
loosely formed granulomas
fibrotic changes in advanced disease
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| How does acute Hypersensitivity pneumonitis present | dyspnea, fever, malaise and cough 4-6 hours after exposure
symptoms for 24-48 hours
fine crackles through lungs
progressive dyspnea over period of years
may have reticular nodular infiltration
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| how does chronic hypersensitivity pneumonitis present clinically | progressive dyspnea
PE 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 worsens with exercise
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| What can busulfan, nitrofurantoin, amiodarone, bleomycin, High oxygen concentration, radiation exposure | all may cause interstitial disease, interstitial fibrosis
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| What are the collagen vascular diseases that can lead to systemic sclerosis | lupus, rheumatoid arthritis, and systemic sclerosis itself
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| How do you dx systemic sclerosis and what is the tx | first dyspnea will be severe then definite dx from surgical lung biopsy confirms
tx is corticosteroids plus cytotoxic therapy
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| HOw is pleural effusion divided | acute or chronic and exudate or transudate
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| How is pneumothorax related to pleural disease | it can be primary or secondary to pleural disease
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| What can develop from longstanding pleural effusion | can develop plueral thickening results in fibrotic pleura which splints the lungs and prevents expansion
if disease if bilateral may cause restrictive lung disease
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| What are some diseases of the chest wall leading to restrictive lung disease | kyphoscoliosis, ankylosing spondylitis
scoliosis- lateral curvature of spine
kyphosis- posterior curvature
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| How does chest wall disease present | patient has exertional dyspnea, rapid shallow breathing.
Cause of death is respiratory failure or intracurrent pulmonary infection
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| what is the tx for chest wall disease | non-invasive chronic ventilation
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| What are neuromuscular diseases that lead to restrictive lung disease | diseases affecting muslces of respiration or their nerve supply
eg. polio, guillaine barre syndrome, ALS, myasthenia gravis, muscular dystrophy
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| What do neuromuscular diseases lead to in respiration | lead to dyspnea and respiratory failure
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| what will you see on PFT's in neuromuscular diseaes | reduced FVC, TLC, and FEV1
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| What is the tx for neuromuscualr respiratory disease | tx underlying cause of assisted ventilation
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