Obstructive vs. Restrictive lung disease Flashcards
Obstructive features
- Blockage of the airways or bronchi and/or bronchioles
- Caused by secretions, inflammation or bronchoconstriction
- normal lung volume but decreased expiratory function
- FEV/FVC % decreased
Restrictive features
- Lung disease that does not allow full expansion of the lungs.
- Multiple causes.
- Decreased total lung volumes.
- shrunk FEV/FVC
Obstructive lung diseases
Asthma Inhalant occupational asthma Bronchitis Emphysema COPD=Chronic Obstructive Pulmonary Disease Bronchiectiasis Cystic Fibrosis
Restrictive
- Goodpasture’s Syndrome
- Wegner’s Granulomatosis
- Kyphoscoliosis
INTERSTITIAL LUNG DISEASE
-Inhaled Lung Diseases
Inorganic dust
Pneumoconiosis
Asbestosis
Silicosis
Coal Worker’s Pneumoconiosis
-Organic dust
Hypersensitivity pneumonitis
Eosinophilic pneumonia
INTERSTITIAL LUNG DISEASE
- Sarcoidosis
- Connective Tissue
- Rheumatoid Arthritis
- Scleroderma
- Idiopathic interstitial pneumonias
- Idiopathic pulmonary fibrosis (usual -interstitial pneumonia or UIP)
- Cryptogenic organizing pneumonia formerly called (BOOP)
- Acute interstitial pneumonia (Hamman-Rich Syndrome)
Goodpasture’s Syndrome
-Restrictive lung disease
-Progressive autoimmune disease of the lungs and kidneys
-Produces intra-alveolar hemorrhage and glomerulonephritis
E: caused by anti-glomerular basement membrane (anti-GBM) antibodies
S/Sx: hemoptysis and dyspnea
Lab: +anti-GBM antibody in serum or biopsy
Dx:
-CXR: bilateral alveolar infiltrates
-PFT: restrictive pattern on spirometry
-Hypoxia
Tx:
-Plasmapheresis and Corticosteroids
-Nephrectomy and dialysis in severe cases
Wegener’s Granulomatosis
- Restrictive lung disease
- Small vessel vasculitis with predilection for the respiratory tract and kidney
- Immunologic injury of vessels with secondary inflammatory changes
- Classic triad: upper respiratory vasculitis, sinus or nasal polyps, lower respiratory vasculitis and glomerlonephritis
- Lab: +ANCA, Increased ESR
- Tx: Cyclophosphamide (decreases immune response) +/- prednisone
Kyphoscoliosis
-Skeletal abnormality that leads to chronic deterioration in lung function
-Causes increased stiffness of the chest wall
-PFT-reduced lung functions; restrictive lung pattern, cant fully expand chest wall to get a whole breath
S/Sx: dyspnea, use of accessory muscles
-Chronic hypercapnic respiratory failure may occur
-Tx: possible mechanical ventilatory support, BiPAP (help with CO2), corrective intervention when angulation >40degrees.
Interstitial Lung Disease (ILD)
- AKA Diffuse Parenchymal Lung Disease
- Alveolitis leading to diminished lung compliance and restrictive lung disease
- E: infectious, drug-related, occupational
- S/Sx: dyspnea, cough
- CXR: “honeycomb lung”, ground-glass infiltrates
- Dx: biopsy
- Tx: +/- corticosteroids
- Diffuse parenchymal lung damage effecting interstitium, alveoli, and airways.
- Diseases are classified together because of similar clinical presentation, chest xray findings and physiologic or pathologic manifestations.
- The most common identifiable causes of ILD are exposure to occupational and environmental agents; especially inorganic or organic dusts (bacteria or fungi), drug-induced pulmonary toxicity and radiation-induced lung injury.
Pneumoconiosis
3 kinds: Asbestosis, Silicosis, Coal worker’s Pneumoconiosis
Asbestosis
- Diffuse interstitial cellular and fibrotic reaction of the lung to inhaled asbestos fibers
- Asbestos found in old homes, plumbing, floors, insulation, brakes
- S/Sx: breathlessness, digital clubbing, crackles in the lower lobes, may cause pleurisy with effusion
- CXR: hazy infiltrates in lower lung zones, interstitial fibrosis, thickened pleura, pleural plaques appear on the diaphragms or lateral chest wall
Cancer causing
- Mesothelioma-diffuse interstitial cell disease
- Bronchogenic carcinoma
- Increased risk of lung cancer with Asbestos exposure and smoking history 16 fold, 1ppd. Increase risk of lung cancer 9 fold, <1ppd. Increased risk of lung cancer with asbestos exposure alone,
Silicosis
-Diffuse fibrotic reaction of the lungs to inhalation of free crystalline silica
S/Sx:
-Silica found in sand, quartz, and talc
-Progression can lead to myobacterial or mycotic infections
-CXR: numerous small, rounded opacities scattered throughout the lungs and hilar lymph nodes may be calcified.
Coal Worker’s Pneumoconiossis
- Chronic fibrotic lung disease caused by inhalation of coal dust
- S/Sx: dyspnea, cough, inspiratory crackles, clubbing, cyanosis
- CXR: small opacities are prominent in the upper lung fields
- Dx: PFT with restrictive dysfunction and reduced diffusion capacity
- Tx: supportive, possibly corticosteroids, smoking cessation
Hypersensitivity Pneumonitis
- Also called extrinsic allergic alveolitis.
- Abnormal pulmonary lung function testing with restrictive pattern and reduced diffusing capacity.
- Immunologic reaction to an inhaled agent.
- Agricultural dusts, bioaerosols and certain reactive chemicals.
- Most common is Farmer’s Lung-farming, vegetable or dairy cattle workers.
- Exposure to ventilation systems and water reservoirs.
- Bird and poultry handlers.
- Animal handlers
- Grain and flour processing
- Lumber mill and construction
- Plastics, paint/epoxy, electronic industries
- Textile workers
Treatment-eliminate or decrease allergen exposure
-Protective devices dust filters, mask
-Oral or IV steroids for subacute and chronic
Don’t always help!
Acute Hypersensitivity Pneumonitis Clinical presentation
- Usually abrupt onset (4-6 hrs after exposure) with fever, chills, malaise, nausea, cough, chest tightness and dyspnea.
- PE: tachypnea and diffuse fine crackles
- Xray- transient patchy, micronodular opacities
- Removal for exposure improves symptoms 12 hours to several days with resolution within several weeks.
Subacute Hypersensitivity Pneumonitis
- Gradual development of productive cough, dyspnea, fatigue, anorexia and weight loss
- Xray more extensive changes with infiltrates and nodular opacities
- Symptoms usually resolve but slower than acute.
- Systemic steroids usually necessary.
Chronic Hypersensitivity Pneumonitis
- May lack history of acute episodes
- Usually report with insidious onset of cough, dyspnea, fatigue, and weight loss.
- Digital clubbing may be seen.
- Disabling and irreversible respiratory findings due to pulmonary fibrosis and may be difficult to differentiate between idiopathic pulmonary fibrosis (IPF).
- Diagnosis by video-assisted thoracoscopic lung biopsy (VATS)
Eosinophillic Pneumonia
- Rare disease, can occur at any age.
- Patients usually between 20 and 40 yrs old.
- Men are affected approximately twice as often as women.
- S/Sx: acute febrile illness of less than three weeks. Nonproductive cough and dyspnea. Malaise, myalgias, night sweats, pleuritic chest pain.
- PE: fever (often high), tachypnea, bibasilar inspiratory crackles and occasionally rhonchi. Low SpO2 levels or hypoxia.
- Labs not too helpful, may see elevated neutrophils with increased ESR
- Xray-subtle reticular or ground glass opacities
- Bronchoalveolar lavage with high eosinolphils
- PFTs with restrictive lung pattern and reduced diffusing capacity
- Lung biopsy but diagnosis based usually on clinical presentation and chest xray.
- Treatment with steroids and respiratory support as needed.
ILD - connective tissue disease
- Polymyositis/dermatomyositis (ILD in 10% of cases)
- Rheumatoid arthritis
- Systemic lupus erythematosus
- Scleroderma (70% of patients have ILD)
- Mixed connective tissue disease
Idiopathic causes of ILD
- Sarcoidosis
- Idiopathic interstitial pneumonias
- Idiopathic pulmonary fibrosis (usual interstitial pneumonia or UIP)
- Cryptogenic organizing pneumonia formerly called (BOOP)
- Acute interstitial pneumonia (Hamman-Rich Syndrome)
Sarcoidosis
-Multi-organ disease of unknown cause
-Incidence 10-20 in 100,000 persons with diagnosis between age 10-40 70% of the time.
-3-4x increase in black individuals and 2.4% chance of having sarcoidosis vs 0.85% in whites
-Characterized by noncaseating granulomatous inflammation in affected organs
-S/Sx: nonproductive cough, dyspnea on exertion, insidious onset, chest discomfort
+/- fever, malaise
-Lab: leukopenia, eosiophilla, increased ESR,
-Hypercalcemia, hypercalcuria, increased ACE levels
-CXR: bilateral hilar and right paratracheal adenopathy and diffuse reticular infiltrates
-Dx: transbronchial biopsy confirms diagnosis
-Assoc: erythema nodosum, enlarged parotid gland, arthritis, cardiomyopathy, uveitis, renal stones
-Tx: 90% respond to steroids; SLOW taper
Idiopathic Pulmonary Fibrosis (UIP)
-Inflammation and fibrosis of the alveolar walls and air spaces without known cause
S/Sx:
-Exertional dyspnea and dry non-productive cough
-Digital clubbing
-Fever, fatigue, anorexia, weight loss
Dx:
-CXR-diffuse ground-glass, nodular, reticular or reticulonodular infiltrates
-PFT: restrictive pattern with decreased FVC and FEV1
Tx: +/- steroids, immunosuppressive agents, Mucomyst
Cryptogenic organizing pneumonia orBronchiolitis Obliterans Organizing Pneumonia (BOOP)
- a rare lung condition
- small airways (bronchioles) and air exchange sac (alveoli) become inflamed with connective tissue.
- usually starts with a flu-like illness associated with fever, malaise, fatigue and cough.
- cough may be persistent and troubling.
- shortness of breath with exertion and weight loss occurs in about half of patients.
- form of interstitial [within the fine supporting tissue of the lung] pneumonia of unknown origin.
- Very similar interstitial pneumonias can be seen in association with connective tissue diseases such as lupus erythematosis, several drug exposures and malignancies.
- PE: other interstitial pneumonias but the chest x-ray and chest CT scan are distinctive
- findings are not definitive and a lung biopsy is recommended for confirmation. -Pulmonary function tests are nonspecific.
- tends to be persistent and not self limited.
- Current therapy involves relatively high doses of corticosteroids [e.g. prednisone] for several months depending upon the response.
- Other immunosuppressive drugs [e.g. cyclophosphamide] may also be used. -Treatment usually results in significant improvement.
- recurrences are common and patients should be periodically monitored with chest radiography, especially in the first year after treatment.
Acute Interstitial Pneumonia(Hamman-Rich Syndrome)
- Rare and fulminant form of diffuse lung injury.
- Acute interstitial pneumonia with the most acute onset of interstitial pneumonias and rapidly progressive course.
- Presents similarly to acute respiratory distress syndrome (ARDS)
- Effects previously healthy people without history of lung disease. Frequency equal in men and women.
- No association with smoking.
- Most patients are over age 40 with mean age 50-55 yrs old.
Acute Interstitial Pneumonia
- Chest xray- diffuse alveolar damage
- Cause is unknown
- S/Sx: Prodromal illness with muscle aches, chills, arthralgias and malaise 7-14 days.
- Most present with fever, cough and progressive, SEVERE shortness of breath. Usually will have hypoxemia at presentation. Most require intubation and ventilator support for a few days.
- PE: Tachypnea, low SpO2, diffuse crackles
- Severe hypoxemia on ABGs.
- Bronchoscopy (to ruleout other causes) VATS lung biopsy (identifies diffuse alveolar damage-pathology)
- Treatment-supportive care. High dose IV steroids. Empiric antibiotics often given.
- Lung transplant
