COPD vs RLDCOPD vs RLD

Review of Lung Anatomy and Review of Lung Anatomy and PhysiologyPhysiology

Which is the correct order of largest Which is the correct order of largest diameter to smallest diameter?diameter to smallest diameter?a) primary bronchi -> secondary bronchi a) primary bronchi -> secondary bronchi -> tertiary bronchi -> alveoli-> tertiary bronchi -> alveolib) alveoli -> primary bronchi -> b) alveoli -> primary bronchi -> secondary bronchi -> tertiary bronchisecondary bronchi -> tertiary bronchic) tertiary bronchi -> secondary bronchi -c) tertiary bronchi -> secondary bronchi -> primary bronchi -> alveoli> primary bronchi -> alveolid) all are the same size d) all are the same size

Review of Lung Anatomy and Review of Lung Anatomy and PhysiologyPhysiology

Which is the correct order of largest Which is the correct order of largest diameter to smallest diameter?diameter to smallest diameter?a) primary bronchi -> secondary a) primary bronchi -> secondary bronchi -> tertiary bronchi -> alveolibronchi -> tertiary bronchi -> alveolib) alveoli -> primary bronchi -> b) alveoli -> primary bronchi -> secondary bronchi -> tertiary bronchisecondary bronchi -> tertiary bronchic) tertiary bronchi -> secondary bronchi -c) tertiary bronchi -> secondary bronchi -> primary bronchi -> alveoli> primary bronchi -> alveolid) all are the same size d) all are the same size

How many lobes do the right and left lung How many lobes do the right and left lung have?have?a) right -> 2, left -> 3 a) right -> 2, left -> 3 b) right -> 3, left -> 2b) right -> 3, left -> 2c) right -> 2, left -> 1c) right -> 2, left -> 1d) right -> 1, left -> 2 d) right -> 1, left -> 2

How many lobes do the right and left lung How many lobes do the right and left lung have?have?a) right -> 2, left -> 3 a) right -> 2, left -> 3 b) right -> 3, left -> 2b) right -> 3, left -> 2c) right -> 2, left -> 1c) right -> 2, left -> 1d) right -> 1, left -> 2 d) right -> 1, left -> 2

How many alveoli are found in the lung?How many alveoli are found in the lung?a) 100 million a) 100 million b) 200 millionb) 200 millionc) 250 millionc) 250 milliond) d) 300 million 300 million

How many alveoli are found in the lung?How many alveoli are found in the lung?a) 100 million a) 100 million b) 200 millionb) 200 millionc) 250 millionc) 250 milliond) 300 milliond) 300 million

What anatomic structure lies beneath the What anatomic structure lies beneath the lungs?lungs?a) tracheaa) tracheab) bronchib) bronchic) c) diaphragmdiaphragmd) larynx d) larynx

What anatomic structure lies beneath the What anatomic structure lies beneath the lungs?lungs?a) tracheaa) tracheab) bronchib) bronchic) diaphragmc) diaphragmd) larynx d) larynx

When the trachea comes to a fork in to When the trachea comes to a fork in to road, this is called the ______?road, this is called the ______?a) larynxa) larynxb) epiglottisb) epiglottisc) lungsc) lungsd) bronchial tubes d) bronchial tubes

When the trachea comes to a fork in to When the trachea comes to a fork in to road, this is called the ______?road, this is called the ______?a) larynxa) larynxb) epiglottisb) epiglottisc) lungsc) lungsd) bronchial tubesd) bronchial tubes

TT or F Individual diseased lobules of the lungs or F Individual diseased lobules of the lungs can be surgically removed because major blood can be surgically removed because major blood vessels and bronchi do not cross the connective vessels and bronchi do not cross the connective tissues of the lung. tissues of the lung.

T or T or FF The alveoli split into numerous number of The alveoli split into numerous number of generations. generations.

TT or F Surfactant reduces the surface tension at or F Surfactant reduces the surface tension at the respiratory membrane. Without surfactant the respiratory membrane. Without surfactant the alveoli would collapse on expiration. the alveoli would collapse on expiration.

The pulmonary vein carries ______ to the heart.The pulmonary vein carries ______ to the heart.a) oxygenated blooda) oxygenated bloodb) unoxygenated blood b) unoxygenated blood

Which cells make surfactant?Which cells make surfactant?a) type I cellsa) type I cellsb) type II cellsb) type II cellsc) WBCc) WBCd) RBC d) RBC

The tracheal bifurcation is located:The tracheal bifurcation is located:                    a) manubriosternal notch, 2nd riba) manubriosternal notch, 2nd rib                    b) 4th ribb) 4th rib                    c) at the costochondral junctionc) at the costochondral junction                    d) 1.5 inches above the clavicled) 1.5 inches above the clavicle

COPDCOPD

Definition of COPDDefinition of COPD Classification and Epidemiology of COPDClassification and Epidemiology of COPD Causes of COPDCauses of COPD Signs and Symptoms of COPDSigns and Symptoms of COPD Diagnosis of COPDDiagnosis of COPD Management of COPDManagement of COPD

Restrictive Lung DiseaseRestrictive Lung Disease

Definition of RLDDefinition of RLD Classification and Epidemiology of RLDClassification and Epidemiology of RLD Causes of RLDCauses of RLD Signs and Symptoms of RLD vs COPDSigns and Symptoms of RLD vs COPD Management of COPDManagement of COPD

Chronic Obstructive Pulmonary Chronic Obstructive Pulmonary DiseaseDisease

characterized by the presence of airflow characterized by the presence of airflow obstruction which is generally progressiveobstruction which is generally progressive

chronic bronchitis or emphysema chronic bronchitis or emphysema may be accompanied by airway may be accompanied by airway

hyperreactivityhyperreactivity may be partially reversible may be partially reversible

Chronic bronchitisChronic bronchitis is defined clinically as the is defined clinically as the presence of a chronic productive cough for 3 presence of a chronic productive cough for 3 months during each of 2 consecutive years months during each of 2 consecutive years (other causes of cough being excluded). (other causes of cough being excluded).

EmphysemaEmphysema is defined as an abnormal, is defined as an abnormal, permanent enlargement of the air spaces distal permanent enlargement of the air spaces distal to the terminal bronchioles, accompanied by to the terminal bronchioles, accompanied by destruction of their walls and without obvious destruction of their walls and without obvious fibrosis. fibrosis.

Based on pooled data from a number of Based on pooled data from a number of studies, global prevalence of COPD was studies, global prevalence of COPD was 7.5% (2006)7.5% (2006) chronic bronchitis alone was 6.4%chronic bronchitis alone was 6.4% emphysema alone was 1.8% emphysema alone was 1.8%

M > F; 40-69 years oldM > F; 40-69 years old M > F in terms of mortalityM > F in terms of mortality

Chronic Bronchitis - PathologyChronic Bronchitis - Pathology

Mucous gland enlargementMucous gland enlargement is the histologic hallmark is the histologic hallmark Structural changes include: atrophy of airways, focal Structural changes include: atrophy of airways, focal

squamous metaplasia, ciliary abnormalities, variable squamous metaplasia, ciliary abnormalities, variable amounts of airway smooth muscle hyperplasia, amounts of airway smooth muscle hyperplasia, inflammation, and bronchial wall thickeninginflammation, and bronchial wall thickening

Neutrophilic infiltrates accumulate in the submucosaNeutrophilic infiltrates accumulate in the submucosa Respiratory bronchioles Respiratory bronchioles mononuclear inflammatory mononuclear inflammatory

process, lumen occlusion by mucous plugging, goblet process, lumen occlusion by mucous plugging, goblet cell metaplasia, smooth muscle hyperplasia, and cell metaplasia, smooth muscle hyperplasia, and distortion due to fibrosisdistortion due to fibrosis

=> airflow limitation by allowing airway walls to deform and => airflow limitation by allowing airway walls to deform and narrow the airway lumen.narrow the airway lumen.

Emphysema - PathologyEmphysema - Pathology 3 morphologic patterns3 morphologic patterns

centriacinar emphysemacentriacinar emphysema• focal destruction limited to the respiratory bronchioles and focal destruction limited to the respiratory bronchioles and

the central portions of acinus. the central portions of acinus. • associated with cigarette smoking; most severe in the upper associated with cigarette smoking; most severe in the upper

lobes. lobes. panacinar emphysemapanacinar emphysema

• involves the entire alveolus distal to the terminal bronchioleinvolves the entire alveolus distal to the terminal bronchiole• Most severe in the lower lung zones and generally develops Most severe in the lower lung zones and generally develops

in patients with homozygous alpha1-antitrypsin (AAT) in patients with homozygous alpha1-antitrypsin (AAT) deficiency deficiency

distal acinar emphysema or paraseptal emphysemadistal acinar emphysema or paraseptal emphysema• least common form and involves distal airway structures, least common form and involves distal airway structures,

alveolar ducts, and sacsalveolar ducts, and sacs• localized to fibrous septa or to the pleura and leads to localized to fibrous septa or to the pleura and leads to

formation of bullae formation of bullae may cause pneumothorax. may cause pneumothorax.

Causes of COPDCauses of COPD

SmokingSmoking Air pollutionAir pollution Airway hyperresponsivenessAirway hyperresponsiveness Alpha-1-antitrypsin deficiencyAlpha-1-antitrypsin deficiency

Only known genetic factor for COPDOnly known genetic factor for COPD <1% incidence in the US<1% incidence in the US

Most patients with COPD have smoked Most patients with COPD have smoked at leastat least 20 cigarettes per day for 20 or more years 20 cigarettes per day for 20 or more years

commonly occurs in the fifth decade of lifecommonly occurs in the fifth decade of life Common symptoms in the history of a patient:Common symptoms in the history of a patient:

A productive cough or an acute chest illness; cough A productive cough or an acute chest illness; cough usually is worse in the mornings and produces a small usually is worse in the mornings and produces a small amount of colorless sputum. amount of colorless sputum.

BreathlessnessBreathlessness is the most significant symptom, but it is the most significant symptom, but it usually does not occur until the sixth decade of life. usually does not occur until the sixth decade of life.

Wheezing may occur in some patientsWheezing may occur in some patients Disease progression Disease progression intervals between acute intervals between acute

exacerbations become shorter; cyanosis and right exacerbations become shorter; cyanosis and right heart failure may occur. heart failure may occur.

Anorexia and weight loss Anorexia and weight loss worse prognosis worse prognosis

The RR increases proportionally to disease severity. The RR increases proportionally to disease severity. use of accessory respiratory muscles and paradoxical indrawing use of accessory respiratory muscles and paradoxical indrawing

of lower intercostal spaces of lower intercostal spaces cyanosis, elevated jugular venous pulse (JVP), and peripheral cyanosis, elevated jugular venous pulse (JVP), and peripheral

edema edema Measurement of Measurement of forced expiratory time (FET)forced expiratory time (FET) maneuver maneuver

FET of more than 6 seconds indicates considerable expiratory FET of more than 6 seconds indicates considerable expiratory flow obstruction (ie, FEV1/forced vital capacity (FVC) <50%). flow obstruction (ie, FEV1/forced vital capacity (FVC) <50%).

Thoracic examination reveals:Thoracic examination reveals: hyperinflation (barrel chest)hyperinflation (barrel chest) wheezing, diffusely decreased breath sounds, wheezing, diffusely decreased breath sounds, hyperresonance on percussion hyperresonance on percussion prolonged expiration. prolonged expiration. coarse crackles beginning with inspiration and wheezes coarse crackles beginning with inspiration and wheezes

frequently are heard on forced and unforced expirationfrequently are heard on forced and unforced expiration

DiagnosesDiagnoses Imaging StudiesImaging Studies

Chest radiograph Chest radiograph • Frontal and lateral chest radiographs reveal signs of Frontal and lateral chest radiographs reveal signs of

hyperinflation, including a flattening of the diaphragm, hyperinflation, including a flattening of the diaphragm, increased retrosternal air space, and a long narrow heart increased retrosternal air space, and a long narrow heart shadow. shadow.

• Rapid tapering vascular shadows accompanied by Rapid tapering vascular shadows accompanied by hyperlucency of the lungs are signs of emphysema. hyperlucency of the lungs are signs of emphysema.

• prominent hilar vascular shadows with possible right prominent hilar vascular shadows with possible right ventricular enlargement and opacity in the lower retrosternal ventricular enlargement and opacity in the lower retrosternal air space air space pulmonary hypertension pulmonary hypertension

Computed tomography scan Computed tomography scan • High-resolution CT (HRCT) scan is more sensitive than the High-resolution CT (HRCT) scan is more sensitive than the

standard chest radiograph. standard chest radiograph. • HRCT scan is highly specific for diagnosing emphysema, and HRCT scan is highly specific for diagnosing emphysema, and

the outlined bullae are not always visible on a radiograph. This the outlined bullae are not always visible on a radiograph. This information does not alter therapy; information does not alter therapy; therefore, a CT scan is not therefore, a CT scan is not useful in the routine care of patients with COPD.useful in the routine care of patients with COPD.

Pulmonary function tests Pulmonary function tests essential for the diagnosis and assessment of the severity of essential for the diagnosis and assessment of the severity of

disease; helpful in following its progress. disease; helpful in following its progress. FEV1FEV1 is a reproducible test and is the most common index of is a reproducible test and is the most common index of

airflow obstruction. airflow obstruction. Lung volume measurements may document an increase in total Lung volume measurements may document an increase in total

lung capacity, functional residual capacity, and residual volume. lung capacity, functional residual capacity, and residual volume. The vital capacity decreases. The vital capacity decreases.

Carbon monoxide diffusing capacity is decreased in proportion Carbon monoxide diffusing capacity is decreased in proportion to the severity of emphysema. to the severity of emphysema.

Arterial blood gases reveal mild-to-moderate hypoxemia Arterial blood gases reveal mild-to-moderate hypoxemia without hypercapnia in the early stages. As the disease without hypercapnia in the early stages. As the disease progresses, hypoxemia becomes more severe and progresses, hypoxemia becomes more severe and hypercapnia supervenes. hypercapnia supervenes.

ManagementManagement

goal of management: improve daily living goal of management: improve daily living and the quality of life by preventing and the quality of life by preventing symptoms and the recurrence of symptoms and the recurrence of exacerbations by preserving optimal lung exacerbations by preserving optimal lung function. function.

educate the patient about the disease and educate the patient about the disease and encourage the patient to participate encourage the patient to participate actively in therapy. actively in therapy.

Smoking cessation continues to be the Smoking cessation continues to be the most important therapeutic intervention. most important therapeutic intervention. smoking cessation plan smoking cessation plan essential part of a essential part of a

comprehensive management plancomprehensive management plan low success rates low success rates addictive power of addictive power of

nicotine, the conditioned response to nicotine, the conditioned response to smoking-associated stimuli, and psychological smoking-associated stimuli, and psychological problems, including depression, poor problems, including depression, poor education, and forceful promotional education, and forceful promotional campaigns by the tobacco industry. campaigns by the tobacco industry.

Oral and inhaled medications are used for Oral and inhaled medications are used for patients with stable disease to reduce dyspnea patients with stable disease to reduce dyspnea and improve exercise tolerance. and improve exercise tolerance.

Most of the medications employed are directed Most of the medications employed are directed at 4 potentially reversible causes of airflow at 4 potentially reversible causes of airflow limitation in a disease state that has largely limitation in a disease state that has largely fixed obstruction:fixed obstruction:

(1)(1) bronchial smooth muscle contractionbronchial smooth muscle contraction(2)(2) bronchial mucosal congestion and edemabronchial mucosal congestion and edema(3)(3) airway inflammationairway inflammation(4)(4) increased airway secretion.increased airway secretion.

MedicationsMedications

Inhaled steroidsInhaled steroids Decrease frequency and severity of exacerbations; Decrease frequency and severity of exacerbations;

slows rate of decline of COPDslows rate of decline of COPD BronchodilatorsBronchodilators

Methylxanthines, B2 agonistsMethylxanthines, B2 agonists Anti-cholinergic agentsAnti-cholinergic agents

Ipratropium bromideIpratropium bromide Phosphodiesterase IV inhibitorsPhosphodiesterase IV inhibitors

Reduction of inflammatory response; nausea is the Reduction of inflammatory response; nausea is the principal adverse reactionprincipal adverse reaction

CilomilastCilomilast

AntibioticsAntibiotics Prevent infection from Prevent infection from Streptococcus pneumoniae, Streptococcus pneumoniae,

Haemophilus influenzae,Haemophilus influenzae, and and Moraxella catarrhalisMoraxella catarrhalis Patients who benefited most were those whose Patients who benefited most were those whose

exacerbations were characterized by at least 2 of the exacerbations were characterized by at least 2 of the following: increases in dyspnea, sputum production, following: increases in dyspnea, sputum production, and sputum purulence and sputum purulence

Mucolytics – N-acetylcysteineMucolytics – N-acetylcysteine Oxygen therapyOxygen therapy

Specialists recommend long-term oxygen therapy for Specialists recommend long-term oxygen therapy for patients with a PaO2 of less than 55 mm Hg, a PaO2 patients with a PaO2 of less than 55 mm Hg, a PaO2 of less than 59 mm Hg with evidence of polycythemia, of less than 59 mm Hg with evidence of polycythemia, or cor pulmonale or cor pulmonale

Surgical CareSurgical Care

BullectomyBullectomy Lung volume reduction surgeryLung volume reduction surgery

candidates for LVRS have symptoms secondary to candidates for LVRS have symptoms secondary to severe emphysema, marked hyperinflation), and CT severe emphysema, marked hyperinflation), and CT scan evidence of heterogeneous emphysema scan evidence of heterogeneous emphysema

resect 20-30% of each lung from the upper zones.resect 20-30% of each lung from the upper zones. mortality rate of 0-18% with complications, including mortality rate of 0-18% with complications, including

pneumonia and prolonged air leakspneumonia and prolonged air leaks Lung transplantationLung transplantation

patients selected to receive a transplant should have patients selected to receive a transplant should have a life expectancy of 2 years or less due to COPD. a life expectancy of 2 years or less due to COPD.

Other forms of ManagementOther forms of Management

DietDiet Inadequate nutritional status associated with Inadequate nutritional status associated with

low body weight in patients with COPD is low body weight in patients with COPD is associated with impaired pulmonary status, associated with impaired pulmonary status, reduced diaphragmatic mass, lower exercise reduced diaphragmatic mass, lower exercise capacity, and higher mortality rates capacity, and higher mortality rates

The American Thoracic Society (ATS) has The American Thoracic Society (ATS) has recommended the clinical staging of recommended the clinical staging of COPD severity according to lung function. COPD severity according to lung function. Stage IStage I is FEV1 of equal or more than 50% of is FEV1 of equal or more than 50% of

the predicted value the predicted value Stage IIStage II is FEV1 35-49% of the predicted is FEV1 35-49% of the predicted

valuevalue Stage IIIStage III is FEV1 less than 35% of the is FEV1 less than 35% of the

predicted value. predicted value.

The predictors of mortalityThe predictors of mortality AgingAging Continued smokingContinued smoking Accelerated decline in FEV1Accelerated decline in FEV1 Moderate-to-severe airflow obstruction,Moderate-to-severe airflow obstruction, Poor bronchodilator responsePoor bronchodilator response Severe hypoxemiaSevere hypoxemia Hypercapnia, Hypercapnia, Development of cor pulmonaleDevelopment of cor pulmonale Overall poor functional capacity.Overall poor functional capacity.

Pulmonary RehabilitationPulmonary Rehabilitation

A multidisciplinary team approach A multidisciplinary team approach physician, dietitian, nurse, respiratory therapist, physician, dietitian, nurse, respiratory therapist,

exercise physiologist, physical therapist, occupational exercise physiologist, physical therapist, occupational therapist, recreational therapist, cardiorespiratory therapist, recreational therapist, cardiorespiratory technician, pharmacist, psychosocial professionalstechnician, pharmacist, psychosocial professionals

Emphasizes Emphasizes patient and family education, patient and family education, smoking cessation, medical management (eg, smoking cessation, medical management (eg, oxygen, immunization), respiratory and chest oxygen, immunization), respiratory and chest physiotherapy, physical therapy with physiotherapy, physical therapy with bronchopulmonary hygiene, exercise, vocational bronchopulmonary hygiene, exercise, vocational rehabilitation, and psychosocial supportrehabilitation, and psychosocial support

Components of pulmonary rehabilitation Components of pulmonary rehabilitation outpatient settingoutpatient setting tailored to the needs of the individual patient. tailored to the needs of the individual patient. provide all patients who complete the program with guidelines for provide all patients who complete the program with guidelines for

continuing at home continuing at home Education is key to comprehensive pulmonary rehabilitationEducation is key to comprehensive pulmonary rehabilitation. The . The

educational component prepares the patient and families to be educational component prepares the patient and families to be actively involved in providing care. This reliance on patients to actively involved in providing care. This reliance on patients to assume charge of their care is known as collaborative self-assume charge of their care is known as collaborative self-management. management.

Exercise training is a mandatory component of pulmonary Exercise training is a mandatory component of pulmonary rehabilitation. rehabilitation.

aerobic lower extremity endurance exercises regularly to enhance aerobic lower extremity endurance exercises regularly to enhance performance of daily activities and reduce dyspnea. performance of daily activities and reduce dyspnea.

Upper extremity exercise training improves dyspnea and allows Upper extremity exercise training improves dyspnea and allows increased activities of daily living requiring the use of upper extremities. increased activities of daily living requiring the use of upper extremities.

Breathing retraining techniques (eg, diaphragmatic, pursed lip Breathing retraining techniques (eg, diaphragmatic, pursed lip breathing) may improve the ventilatory pattern and prevent dynamic breathing) may improve the ventilatory pattern and prevent dynamic airway compression.airway compression.

Restrictive Lung DiseaseRestrictive Lung Disease

characterized by reduced lung volume due to:characterized by reduced lung volume due to: alteration in lung parenchyma alteration in lung parenchyma disease of the pleura, chest wall, or neuromuscular disease of the pleura, chest wall, or neuromuscular

apparatusapparatus

In physiological terms:In physiological terms: reduced total lung capacity (TLC), vital capacity, or reduced total lung capacity (TLC), vital capacity, or

resting lung volume. resting lung volume. Accompanying characteristics are preserved airflow Accompanying characteristics are preserved airflow

and normal airway resistance, which are measured as and normal airway resistance, which are measured as the functional residual capacity (FRC). the functional residual capacity (FRC).

ClassificationClassification Intrinsic lung diseases or diseases of the lung Intrinsic lung diseases or diseases of the lung

parenchymaparenchyma inflammation or scarring of the lung tissue (interstitial inflammation or scarring of the lung tissue (interstitial

lung disease) or result in filling of the air spaces with lung disease) or result in filling of the air spaces with exudate and debris (pneumonitis). exudate and debris (pneumonitis).

characterized according to etiological factorscharacterized according to etiological factors Includes idiopathic fibrotic diseases, connective tissue Includes idiopathic fibrotic diseases, connective tissue

diseases, drug-induced lung disease, and primary diseases, drug-induced lung disease, and primary diseases of the lungs diseases of the lungs

Extrinsic disorders or extraparenchymal Extrinsic disorders or extraparenchymal diseasesdiseases Chest wall, pleura, and respiratory muscles Chest wall, pleura, and respiratory muscles Result in lung restriction, impaired ventilatory function, Result in lung restriction, impaired ventilatory function,

and respiratory failure (eg, nonmuscular diseases of and respiratory failure (eg, nonmuscular diseases of the chest wall, neuromuscular disorders)the chest wall, neuromuscular disorders)

EpidemiologyEpidemiology

KyphoscoliosisKyphoscoliosis is a common extrinsic disorder. It is a common extrinsic disorder. It is associated with an incidence of mild is associated with an incidence of mild deformities amounting to 1 case per 1000 deformities amounting to 1 case per 1000 persons, with severe deformity occurring in 1 persons, with severe deformity occurring in 1 case per 10,000 persons case per 10,000 persons

For intrinsic lung diseases, studies cite an For intrinsic lung diseases, studies cite an overall prevalence of 3-6 cases per 100,000 overall prevalence of 3-6 cases per 100,000 persons, with a prevalence of persons, with a prevalence of idiopathic idiopathic pulmonary fibrosis (IPF)pulmonary fibrosis (IPF) of 27-29 cases per of 27-29 cases per 100,000 persons. 100,000 persons.

Causes of RLDCauses of RLD Intrinsic lung diseases Intrinsic lung diseases

Collagen vascular diseases, including scleroderma, polymyositis, Collagen vascular diseases, including scleroderma, polymyositis, dermatomyositis, systemic lupus erythematosus, rheumatoid dermatomyositis, systemic lupus erythematosus, rheumatoid arthritis, and ankylosing spondylitis, are a cause of restrictive lung arthritis, and ankylosing spondylitis, are a cause of restrictive lung disease. disease.

drugs and other treatments (eg, nitrofurantoin, amiodarone, gold, drugs and other treatments (eg, nitrofurantoin, amiodarone, gold, dilantin, bleomycin, bischloroethylnitrosourea [BCNU or dilantin, bleomycin, bischloroethylnitrosourea [BCNU or carmustine], cyclophosphamide, methotrexate, radiation). carmustine], cyclophosphamide, methotrexate, radiation).

primary or unclassified diseases may include sarcoidosis, primary or unclassified diseases may include sarcoidosis, pulmonary histiocytosis X, LAM, pulmonary vasculitis, alveolar pulmonary histiocytosis X, LAM, pulmonary vasculitis, alveolar proteinosis, eosinophilic pneumonia, and BOOP. proteinosis, eosinophilic pneumonia, and BOOP.

Inorganic dust exposure (eg, silicosis, asbestosis, talc, Inorganic dust exposure (eg, silicosis, asbestosis, talc, pneumoconiosis, berylliosis, hard metal fibrosis, coal worker's pneumoconiosis, berylliosis, hard metal fibrosis, coal worker's pneumoconiosis) may cause restrictive lung disease. pneumoconiosis) may cause restrictive lung disease.

Organic dust exposure (eg, farmer's lung, bird fancier's lung, Organic dust exposure (eg, farmer's lung, bird fancier's lung, bagassosis, and mushroom worker lung, which all cause bagassosis, and mushroom worker lung, which all cause hypersensitivity pneumonitis) is another cause.hypersensitivity pneumonitis) is another cause.

Idiopathic fibrotic disorders: These may include acute interstitial Idiopathic fibrotic disorders: These may include acute interstitial pneumonia, IPF (usually interstitial pneumonitis), lymphocytic pneumonia, IPF (usually interstitial pneumonitis), lymphocytic interstitial pneumonitis, desquamative interstitial pneumonitis, and interstitial pneumonitis, desquamative interstitial pneumonitis, and nonspecific interstitial pneumonitis. nonspecific interstitial pneumonitis.

Causes of RLDCauses of RLD Extrinsic disorders Extrinsic disorders

Nonmuscular diseases of the chest wall, in which Nonmuscular diseases of the chest wall, in which kyphosis can be idiopathic or secondary, may kyphosis can be idiopathic or secondary, may cause restrictive lung disease. The most common cause restrictive lung disease. The most common cause of secondary kyphoscoliosis is cause of secondary kyphoscoliosis is neuromuscular disease (eg, polio, muscular neuromuscular disease (eg, polio, muscular dystrophy). Fibrothorax, massive pleural effusion, dystrophy). Fibrothorax, massive pleural effusion, morbid obesity, ankylosing spondylitis, and morbid obesity, ankylosing spondylitis, and thoracoplasty are other causes. thoracoplasty are other causes.

Neuromuscular diseases manifest as respiratory Neuromuscular diseases manifest as respiratory muscle weakness and are due to myopathy or muscle weakness and are due to myopathy or myositis, quadriplegia, or phrenic neuropathy myositis, quadriplegia, or phrenic neuropathy from infectious or metabolic causes.from infectious or metabolic causes.

PathologyPathology

In cases of intrinsic lung disease In cases of intrinsic lung disease reduction in all lung volumes; expiratory reduction in all lung volumes; expiratory airflow is reduced in proportion to lung airflow is reduced in proportion to lung volume.volume. Arterial hypoxemia due to V/Q mismatch; Arterial hypoxemia due to V/Q mismatch;

impaired diffusion of oxygen which contributes impaired diffusion of oxygen which contributes a little towards hypoxemia at rest but is a little towards hypoxemia at rest but is primarily the mechanism of exercise-induced primarily the mechanism of exercise-induced desaturation.desaturation.

Hyperventilation at rest and exerciseHyperventilation at rest and exercise

In cases of extrinsic disorders of the pleura and In cases of extrinsic disorders of the pleura and thoracic cage thoracic cage reduced lung compliance, and, reduced lung compliance, and, hence, lung volumes are reduced; gas hence, lung volumes are reduced; gas distribution is nonuniform, resulting in ventilation-distribution is nonuniform, resulting in ventilation-perfusion mismatch and hypoxemia.perfusion mismatch and hypoxemia. In kyphoscoliosis, the Cobb angle is an indication of In kyphoscoliosis, the Cobb angle is an indication of

the severity of disease. An the severity of disease. An angle greater than 100°angle greater than 100° is is usually associated with respiratory failure.usually associated with respiratory failure.

neuromuscular disorders neuromuscular disorders respiratory pump can be respiratory pump can be impaired at the level of the central nervous system, impaired at the level of the central nervous system, spinal cord, peripheral nervous system, spinal cord, peripheral nervous system, neuromuscular junction, or respiratory muscle. neuromuscular junction, or respiratory muscle.

A careful history of occupation, travel, A careful history of occupation, travel, habits, hobbies, exposures, and HIV risk habits, hobbies, exposures, and HIV risk factors is critical to help identify any factors is critical to help identify any etiologic agent. etiologic agent.

Duration of illness, smoking history, Duration of illness, smoking history, medication history, occupational and medication history, occupational and environmental exposure environmental exposure

Symptoms of intrinsic diseases Symptoms of intrinsic diseases Progressive exertional dyspnea is the predominant symptom. Progressive exertional dyspnea is the predominant symptom.

Grading the level of dyspnea is useful as a method to gauge the Grading the level of dyspnea is useful as a method to gauge the severity of the disease and to follow its course. severity of the disease and to follow its course.

A dry cough is common and may be a disturbing sign. A A dry cough is common and may be a disturbing sign. A productive cough is an unusual sign in most patients with diffuse productive cough is an unusual sign in most patients with diffuse parenchymal lung disorders. parenchymal lung disorders.

Hemoptysis or grossly bloody sputum occurs in patients with Hemoptysis or grossly bloody sputum occurs in patients with diffuse alveolar hemorrhage syndromes and vasculitis. diffuse alveolar hemorrhage syndromes and vasculitis.

Wheezing is an uncommon manifestation but can occur in Wheezing is an uncommon manifestation but can occur in patients with lymphangitic carcinomatosis, chronic eosinophilic patients with lymphangitic carcinomatosis, chronic eosinophilic pneumonia, and respiratory bronchiolitis. pneumonia, and respiratory bronchiolitis.

Chest pain is uncommon in most instances of the disease, but Chest pain is uncommon in most instances of the disease, but pleuritic chest pain can occur in patients with rheumatoid pleuritic chest pain can occur in patients with rheumatoid arthritis, systemic lupus erythematosus, and some drug-induced arthritis, systemic lupus erythematosus, and some drug-induced disordersdisorders

Symptoms of extrinsic disorders Symptoms of extrinsic disorders Nonmuscular diseases of the chest wall affect patients with Nonmuscular diseases of the chest wall affect patients with

kyphoscoliosis. Patients younger than 35 years tend to be kyphoscoliosis. Patients younger than 35 years tend to be asymptomatic, whereas middle-aged patients develop dyspnea, asymptomatic, whereas middle-aged patients develop dyspnea, decreased exercise tolerance, and respiratory infections. decreased exercise tolerance, and respiratory infections.

The cause of respiratory failure is often multifactorial and is The cause of respiratory failure is often multifactorial and is secondary to spinal deformity, muscle weakness, disordered secondary to spinal deformity, muscle weakness, disordered ventilatory control, sleep disordered breathing, and airway ventilatory control, sleep disordered breathing, and airway disease. disease.

Neuromuscular disorders occur as the respiratory muscle Neuromuscular disorders occur as the respiratory muscle weakness progresses. Patients develop dyspnea upon exertion, weakness progresses. Patients develop dyspnea upon exertion, followed by dyspnea at rest, and their condition ultimately followed by dyspnea at rest, and their condition ultimately advances to respiratory failure. Acute and chronic respiratory advances to respiratory failure. Acute and chronic respiratory failure, pulmonary hypertension, and cor pulmonale eventually failure, pulmonary hypertension, and cor pulmonale eventually ensue.ensue.

chest wall disorders show obvious massive obesity and an chest wall disorders show obvious massive obesity and an abnormal configuration of the thoracic cage (eg, kyphoscoliosis, abnormal configuration of the thoracic cage (eg, kyphoscoliosis, ankylosing spondylitis). ankylosing spondylitis).

Abnormal breath sounds:Abnormal breath sounds: Velcro crackles (interstitial lung disorders) Velcro crackles (interstitial lung disorders) Inspiratory squeaks or scattered, late, inspiratory high-pitched rhonchi Inspiratory squeaks or scattered, late, inspiratory high-pitched rhonchi

(bronchiolitis)(bronchiolitis) Cyanosis at rest is a late manifestation of advanced disease. Cyanosis at rest is a late manifestation of advanced disease. Digital clubbing is common in those with IPF and is rare in others Digital clubbing is common in those with IPF and is rare in others

(eg, those with sarcoidosis or hypersensitivity pneumonitis). (eg, those with sarcoidosis or hypersensitivity pneumonitis). Extrapulmonary findings, including erythema nodosum, suggest Extrapulmonary findings, including erythema nodosum, suggest

sarcoidosis. A maculopapular rash can occur in those with sarcoidosis. A maculopapular rash can occur in those with connective tissue diseases, or it may be drug-induced. Raynaud connective tissue diseases, or it may be drug-induced. Raynaud phenomenon may be present in patients with connective tissue phenomenon may be present in patients with connective tissue diseases, and telangiectasia is present in those with scleroderma. diseases, and telangiectasia is present in those with scleroderma. Peripheral lymphadenopathy, salivary gland enlargement, and Peripheral lymphadenopathy, salivary gland enlargement, and hepatosplenomegaly are signs of systemic sarcoidosis.hepatosplenomegaly are signs of systemic sarcoidosis.

Cor pulmonale occurs in the late stages of pulmonary fibrosis or Cor pulmonale occurs in the late stages of pulmonary fibrosis or advanced kyphoscoliosis. Pulmonary hypertension and cor advanced kyphoscoliosis. Pulmonary hypertension and cor pulmonale pulmonale a loud P2, right-sided precordial lift, and right-sided a loud P2, right-sided precordial lift, and right-sided gallopgallop..

DiagnosisDiagnosis

Depends on the underlying causeDepends on the underlying cause Blood chemistry and serum markersBlood chemistry and serum markers Imaging Modalities (CXR, CT scan)Imaging Modalities (CXR, CT scan) Pulmonary function testsPulmonary function tests

All disorders are associated with a restrictive All disorders are associated with a restrictive defect with a reduction in TLC, FRC, and defect with a reduction in TLC, FRC, and residual volume (RV). residual volume (RV).

Bronchoalveolar lavageBronchoalveolar lavage BiopsyBiopsy

ManagementManagement

Treatment depends on the specific Treatment depends on the specific diagnosis, which is based on findings from diagnosis, which is based on findings from the clinical evaluation, imaging studies, the clinical evaluation, imaging studies, and lung biopsy.and lung biopsy.

Corticosteroids, immunosuppressive Corticosteroids, immunosuppressive agents, and cytotoxic agents are the agents, and cytotoxic agents are the mainstay of therapy for many of the mainstay of therapy for many of the interstitial lung diseasesinterstitial lung diseases

Patients with nonmuscular chest wall disorders and Patients with nonmuscular chest wall disorders and neuromuscular disease may develop problems with neuromuscular disease may develop problems with ventilation and gas exchange during sleep. The effect of ventilation and gas exchange during sleep. The effect of decreased chest wall and lung compliance or decreased decreased chest wall and lung compliance or decreased muscle strength is hypercapnia and hypoxemia, which muscle strength is hypercapnia and hypoxemia, which occurs initially during sleep. occurs initially during sleep.

Treatment of neuromuscular diseases includes Treatment of neuromuscular diseases includes preventive therapies to minimize the impact of impaired preventive therapies to minimize the impact of impaired secretion clearance and the prevention and prompt secretion clearance and the prevention and prompt treatment of respiratory infections.treatment of respiratory infections.

Treatment for massive obesity consists of weight loss, Treatment for massive obesity consists of weight loss, which causes dramatic improvement in pulmonary which causes dramatic improvement in pulmonary function test findingsfunction test findings

In advanced disease, when respiratory failure develops, In advanced disease, when respiratory failure develops, these patients are treated with mechanical ventilation these patients are treated with mechanical ventilation

CHRONIC BRONCHITIS (DIRTY CHEST)

LOBAR PNEUMONIA

PNEUMONIA WITH CONSOLIDATION

BILATERAL BRONCHOPNEUMONIA

PULMONARY ABSCESS

EMPHYSEMA