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Session 2 CM pulm10

CM- pulm -10- Restrictive Airway Disease

Diminished Lung Volume Decreased TLC, FVC Normal or elevated FEV1/FVC ratio Restrictive Lung Disease
What are interstitial pulmonary fibrosis; and sarcoidosis restrictive lung diseases
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
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
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)
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
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
what restrictive pathophysiology does the following def apply to : reduces the total compliance and lung volumes are reduced Extrinsic Pathophysiology
What is a common extrinisc disorder in elderly patients with restrictive lung disease kyphoscoliosis
what is the medain survival rate for interstitial pulmonary fibrosis less than 3 yrs
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
The following diseases often are classified as which of the following acute, subacute or chronic -Interstitial pneumonitis, eosinophilic pneumonia, and diffuse alveolar hemorrhage Acute
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
The following diseases often are classified as which of the following acute, subacute or chronic -IPF, Sarcoidosis, and pulmonary histiocytosis X Chronic
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
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
What is the worst grade of dyspnea dyspnea at rest
What are the two divisions of extrinsic restrictive lung disease nonmuscular-eg kyphoscoliosis neuromuscular-
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
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
What labs would you order for intrinsic Rest. airway disease Anemia-Vasculitis
What labs would you order for ext. rest. airway disease elevated CK may indicate myositis
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
What will you possibly see on chest x-ray if the disease is extrinisc restricitve chest wall deformities
why would you perfrom a bronchoalveolar lavage to get cells to narrow diagnosis
If you needed to get a tissue sample for anaylysis from the lung what would you likely perform lung biopsy
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
What is IPF diffuse interstitial pulmonary fibrosis also calle idiopathic pulmonary fibrosis, interstitial pneumonia, cryptogenic fibrosing alveolitis
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
what is the etiologic cause of IPF unknown may be immunological rxn
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
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
what is the etiology of sarcoidosis uk, likely immunological basis
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
also known as extrinsic allergic alveolitis, occurs in response to inhaled organic dust. exposure may be occupational or environmental Hypersensitivity pneumonitis
What is an example of hypersensitivity pneumonitis farmer's lung, bird fancier's lung
what type of hypersensitivity rxn is hypersensitivity pneumonitis type III and Type IV hypersensitivity rxn
what type of hypersensitivity rxn is due to thermophilic actinomyces farmer's lung from moldy hay
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
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
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
What can busulfan, nitrofurantoin, amiodarone, bleomycin, High oxygen concentration, radiation exposure all may cause interstitial disease, interstitial fibrosis
What are the collagen vascular diseases that can lead to systemic sclerosis lupus, rheumatoid arthritis, and systemic sclerosis itself
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
HOw is pleural effusion divided acute or chronic and exudate or transudate
How is pneumothorax related to pleural disease it can be primary or secondary to pleural disease
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
What are some diseases of the chest wall leading to restrictive lung disease kyphoscoliosis, ankylosing spondylitis scoliosis- lateral curvature of spine kyphosis- posterior curvature
How does chest wall disease present patient has exertional dyspnea, rapid shallow breathing. Cause of death is respiratory failure or intracurrent pulmonary infection
what is the tx for chest wall disease non-invasive chronic ventilation
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
What do neuromuscular diseases lead to in respiration lead to dyspnea and respiratory failure
what will you see on PFT's in neuromuscular diseaes reduced FVC, TLC, and FEV1
What is the tx for neuromuscualr respiratory disease tx underlying cause of assisted ventilation
Created by: smaxsmith