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172 Cards in this Set

  • Front
  • Back
Acute respiratory worsening (over less than 4 weeks) in patients with diffuse pulmonary infiltrates is most often the result of _____ or ______.
Acute respiratory worsening (over less than 4 weeks) in patients with diffuse pulmonary infiltrates is most often the result of INFECTION or EDEMA.
Diffuse parenchymal lung diseases that can present with acute respiratory worsening include what?
Diffuse parenchymal lung diseases that can present with acute respiratory worsening include the VASCULITIDES, EOSINOPHILIC PNEUMONIA, and ACUTE INTERSTITIAL PNEUMONIA.
Imaging of choice for diffuse parenchymal lung disease?
High-resolution CT of the chest is more sensitive and specific than chest radiography for diagnosis of diffuse parenchymal lung disease.
A false-positive high-resolution CT finding for diffuse parenchymal lung disease due to atelectasis in gravity-dependent lung zones. Improves with prone imaging at full inspiration.
Pseudoreticulation
Most causes of drug-induced pulmonary disease are the result of a _________ reaction with presenting symptoms of fatigue, low-grade fever, and cough.
Hypersensitivity-type Reaction
How long will it take to develop radiation pneumonitis s/p chemoradiation?
Radiation pneumonitis occurs 1 to 6 months after radiation therapy has been completed.
Response rate of systemic corticosteroids in treating radiation pneumonitis?
Systemic corticosteroids have an estimated response rate of 80% in treating radiation pneumonitis and can result in rapid clinical improvement.
Diffuse parenchymal lung diseases associated with smoking include?
Pulmonary Langerhans cell histiocytosis

Respiratory bronchiolitis–associated interstitial lung disease

Desquamative interstitial pneumonia.
Accelerated worsening of previously stable idiopathic pulmonary fibrosis

The clinical context is acute worsening (less than 1 month) of respiratory symptoms and gas exchange, along with new lung infiltrates occurring in the absence of identifiable infection, pulmonary embolism, or cardiac dysfunction
Acute Exacerbation of IPF

Accelerated worsening of previously stable idiopathic pulmonary fibrosis is a specific diagnosis called acute exacerbation of idiopathic pulmonary fibrosis, which is frequently the cause of death in patients with the disorder.
A form of sarcoidosis that presents with fever, erythema nodosum, polyarthralgias, and hilar lymphadenopathy.
Lofgren Syndrome
A rare progressive cystic lung disease occurring almost exclusively in women in their third or fourth decade as a sporadic disorder or in conjunction with tuberous sclerosis.
Lymphangioleiomyomatosis
Drugs known to cause diffuse pneumonitis include what 2 common chemotherapy medications?
Bleomycin and Methotrexate
Physical finding common in fibrosing interstitial lung disease; >80% in idiopathic pulmonary fibrosis; less common in sarcoidosis.
Crackles
Physical exam finding suggestive of small airways disease (bronchiolitis)
Mild-inspiratory "squeeks"
Physical exam finding related to Pulmonary hypertension secondary to interstitial lung disease or as a specific feature (scleroderma, pulmonary Langerhans cell histiocytosis); occurs with severe restrictive disease
Increased intensity P2, right ventricular lift, tricuspid regurgitation murmur
Peripheral physical exam finding common in idiopathic pulmonary fibrosis (30%); uncommon/rare in respiratory bronchiolitis interstitial lung disease, cryptogenic organizing pneumonia, and collagen vascular disease
Clubbing
DPLDs that may present with acute worsening leading to fulminant respiratory failure due to diffuse alveolar hemorrhage include?
Vasculitides (e.g. Wegener's granulomatosis, Churg-straus syndrome, and microscopic polyangitis)
Acute interstitial pneumonia can present as ARDS without known cause
Hamman-Rich Syndrome
Characterized by airspace consolidation with indistinct margins, the presence of air bronchograms, and the silhouette sign when bordering adjacent soft tissue.
Consolidation
Characterized by increased alveolar attenuation through which lung architecture can still be identified.
Ground-glass opacification
DPLDs most commonly associated with cystic disease include?
Pulmonary Langerhans Cell Histiocytosis

Lymphagiomyomatosis

although idiopathic pulmonary fibrosis can also cause cystic changes in areas of honeycombing
DPLD most commonly associated with a small nodular pattern distributed centrally along the pulmonary vascular bundles
Sarcoidosis
2 most common DPLDs associated with upper lobe involvement?
Hypersensitivity Pneumonitis

Sarcoidosis
2 most common DPLDs associated with lower lobe involvement?
Idiopathic pulmonary fibrosis

Asbestosis
DPLDs associated with central portions of the lung?
Sarcoidosis

Pulmonary Alveolar Proteinosis
DPLDs associated with peripheral portions of the lung?
Idiopathic pulmonary fibrosis

Chronic eosinophilic pneumonia

Cryptogenic organizing pneumonia
Pattern of mosaic distribution in interstitlal lung disease (refers to a nonspecific pattern of ground-glass infiltration that variably involves neighboring pulmonary lobules) is most likely associated with what class of diseases?
This pattern can be seen in pulmonary vascular disease and airways disease.
"Crazy Paving" on a HRCT
Pulmonary Alveolar Proteinosis
Interstitial lung disease + pleural plaques
Asbestosis
Pleural Effusions in a patient with interstitial lung disease should prompt what further work up?
Pleural effusions are uncommon in most DPLDs, and therefore their presence may help distinguish DPLD from other diseases. Pleural effusions are commonly associated with pulmonary edema or with lymphangiitic spread of malignancy but are rarely associated with interstitial lung disease and should prompt consideration of heart failure or lymphangiitic involvement of tumor.
Mortality and Complication rate associated with VATS (Video Assisted Thoracoscopic Surgery)?
VATS lung biopsy has a reported mortality rate of 2% and a complication rate of between 5% and 10%.
When should surgical lung biopsy be considered?
High-risk patients with little potential benefit from surgical lung biopsy include those with suspected idiopathic pulmonary fibrosis who have a DLCO less than 20% of predicted or an accelerated clinical decline. Surgical lung biopsy is most informative when HRCT findings are used to direct surgical sampling of two or more lobes. Surgical lung biopsy specimens can provide sufficient tissue to assess the histopathologic architecture, which may be required to establish a diagnosis of usual interstitial pneumonia and nonspecific interstitial pneumonitis.
Occurs 6 to 24 months after radiation therapy and represents a long-term fibrotic sequela of lung damage, most often within the radiation field. Symptoms are uncommon, but patients with previous marginal lung function may have worsening dyspnea. The fibrotic process is irreversible.
Radiation Fibrosis

Note: this is clinically distinct from radiation pneumonitis, although the diseases may have overlapping clinical presentations. Radiation fibrosis can occur in patients with or without a history of radiation pneumonitis.
Treatment of choice for patients with Interstitial Lung Disease associated systemic sclerosis
Cyclophosphamide

Oral corticosteroid therapy has not consistently proved to be beneficial. A recent randomized, placebo-controlled trial demonstrated less decline of lung volume in patients with systemic sclerosis treated with cyclophosphamide compared with placebo.
Epidemiology associated with Rheumatoid arthritis–associated interstitial lung
Rheumatoid arthritis–associated interstitial lung disease occurs in as many as 50% of patients with rheumatoid arthritis, may precede musculoskeletal joint disease, and is more common in men than in women.
What drug should be avoided (if possible) in Rheumatoid Arthritis-associated interstitial lung disease?
Methotrexate

Treatment of rheumatoid arthritis–associated interstitial lung disease is similar to treatment of systemic rheumatoid arthritis. Treatment of rheumatoid arthritis with methotrexate can lead to drug-induced lung disease, which is clinically difficult to distinguish from new-onset or worsening rheumatoid arthritis–associated interstitial lung disease. In addition, atypical pulmonary infections in patients treated with immunosuppressive therapy may mimic interstitial lung disease.
An allergic, inflammatory lung disease also called extrinsic allergic alveolitis. It results from exposure to airborne allergens, which cause a cell-mediated immunologic sensitization.
Hypersensitivity Pneumonitis
Pathophysiology of hypersensitivity pneumonitis
Recurrent exposure then leads to inflammatory cytokine recruitment of CD8 lymphocytes, which results in granuloma formation and, over time, may produce parenchymal lung fibrosis.
Hypersensitivity pneumonitis associated with working with moldy corks
Suberosis
Hypersensitivity pneumonitis associated with working with animal pelts
Furrier's Lung
Inorgnaic antigen associated with "Chemical Lung"
Diisocyanates
Classic HRCT findings in hypersensitivity pneumonitis
HRCT should be considered in the diagnostic evaluation for all patients with possible hypersensitivity pneumonitis except those whose chest radiograph, symptoms, and exposure to a known antigen source make the diagnosis highly likely. Classic HRCT findings include a pattern of ground-glass opacification and centrilobular nodules in an upper- and mid-lung distribution. The centrilobular nodules are thought to represent cellular bronchiolitis, and therefore HRCT expiratory imaging often demonstrates air-trapping. Subacute or chronic disease often has a similar pattern of centrilobular nodules and ground-glass opacification, but these findings are associated with reticular lines, honeycomb changes, and traction bronchiectasis. The distribution of findings is most often upper- and mid-lung, but when the disease is severe, it can involve the entire lung. Associated findings include volume loss as the fibrotic disease becomes advanced.
Treatment that should be considered for patients with Hypersensitivity Pneumonitis and hypoxemia or failure to improve after elimination of exposure
Oral Corticosteroids
Typically presents in a young (less than 40 years of age) current smoker. Pneumothorax occurs in 25% of patients with the disorder. The temporal pattern of the disease tends to be subacute, with gradual worsening of cough and dyspnea. Pulmonary function studies typically reveal an obstructive pattern with low DLCO in those with more severe parenchymal involvement, and those with mild disease may have obstructive, restrictive, or normal lung function. HRCT showing a pattern of nodules accompanied by thin-walled cysts in an upper and middle lung-field distribution is virtually pathognomonic. What's the treatment of this disease?
Pulmonary Langerhans cell histiocytosis (formerly called histiocytosis X or eosinophilic granuloma)

The initial treatment is smoking cessation. The disease may stabilize in patients who stop smoking, but most patients show evidence of gradual progression. In patients with the related systemic disease of Langerhans cell histiocytosis, chemotherapy with novel agents such as cladribine (2-chlorodeoxyadenosine [2-CdA]) appears to be effective, but such therapy has not been evaluated for treatment of smoking-related pulmonary Langerhans cell histiocytosis without systemic disease.
A form of bronchiolitis that occurs in most smokers and is occasionally severe enough to cause clinical symptoms and characteristic radiographic abnormalities. Affected patients present with the gradual onset of persistent cough and dyspnea with impaired gas exchange; pulmonary function tests show an obstructive or mixed obstructive/restrictive pattern. Chest radiograph may be normal but usually shows a pattern of bronchial wall thickening and alveolar infiltrates in a central and basilar distribution. HRCT shows a pattern of centrilobular nodules with air-trapping and scattered ground-glass attenuation. These HRCT findings are also seen, although less extensively, in asymptomatic smokers.
Respiratory bronchiolitis–associated interstitial lung disease (RB-ILD)
Current diagnostic criteria classify the diagnosis as “definite” when a surgical lung biopsy specimen shows usual interstitial pneumonia in the appropriate clinical–radiographic context.

The clinical context is typically a middle-aged or older person with interstitial lung disease in whom no cause can be identified. Presenting manifestations generally include dry cough and dyspnea without systemic symptoms. Physical examination characteristically shows bibasilar dry inspiratory crackles. Digital clubbing is present in 30% of patients. Laboratory tests are not helpful unless there is clinical suspicion for underlying connective tissue disease. Pulmonary function tests show physiologic impairment of oxygen uptake and restriction of lung volumes. The disease is characteristically slowly progressive. HRCT findings are important in the diagnostic evaluation and may obviate the need for surgical lung biopsy. HRCT features that suggest usual interstitial pneumonia include reticular opacities and honeycombing with a peripheral and basilar predominance and minimal or no ground-glass opacifications. If these features are not present, surgical biopsy is required.
diopathic pulmonary fibrosis

Constitutes 25% to 35% of clinically encountered interstitial lung diseases.

However, the pattern of usual interstitial pneumonia is not specific for idiopathic pulmonary fibrosis.
Management of Idiopathic Pulmonary Fibrosis?
Because of the poor prognosis of idiopathic pulmonary fibrosis (2- to 3-year mortality rate 50%) and the lack of effective treatment, management of newly diagnosed disease is challenging. Traditional treatment with corticosteroids and cytotoxic therapies is not known to affect survival, and the risk of these therapies may outweigh their benefit. When appropriate, patients should be referred for lung transplantation or considered for enrollment in clinical trials.
A rare idiopathic disorder that shares many clinical and radiologic features with idiopathic pulmonary fibrosis.

HRCT features of nonspecific interstitial pneumonitis include basilar or mid-lung reticular changes, rarely with honeycombing, and a dominant pattern of ground-glass opacification in the mid- and lower-lung zones. A diagnosis requires open lung biopsy.

The histopathologic pattern of the disorder includes lymphoplasmacytic interstitial infiltration in a uniform pattern that disrupts the normal lung architecture and may be accompanied by chronic fibrosis.

The prognosis and response to treatment are better when the pattern involves cellular inflammation rather than fibrosis.

The diagnosis depends on excluding histopathologic features of organizing pneumonia, usual interstitial pneumonia, and diffuse alveolar damage.
Nonspecific interstitial pneumonitis

The histopathologic pattern of nonspecific interstitial pneumonitis is most frequently the manifestation of connective tissue disease–associated interstitial lung disease, hypersensitivity pneumonitis, drug-induced lung disease, or HIV infection. A patient with a new diagnosis of nonspecific interstitial pneumonitis requires evaluation for diseases known to cause that pattern, especially connective tissue disease.

Treatment consists of corticosteroids with or without cytotoxic agents.

Prognosis is better than for idiopathic pulmonary fibrosis but remains poor for patients with a dominant fibrotic pattern on lung biopsy specimen.
When organizing pneumonia occurs as a primary histopathologic process without any associated cause, it is called...
Cryptogenic Organizing Pneumonia
typically affects middle-aged nonsmokers, with both sexes being equally affected. The temporal pattern of disease onset and progression of symptoms are subacute. Symptoms usually consist of cough with fatigue, low-grade fever, anorexia, and weight loss. Most patients are initially diagnosed with viral pneumonitis or bacterial pneumonia after 1 to 2 weeks of symptoms. It is often considered 6 to 8 weeks later, usually after one or more courses of ineffective antimicrobial therapy. The chest radiograph most commonly shows a pattern of multiple patchy alveolar opacities with or without air bronchograms. Their distribution is peripheral and bilateral; the infiltrates may be migratory with resolution of established opacities as new areas appear on serial imaging. Imaging may also be nonspecific, showing interstitial infiltrates and alveolar opacification or showing one or more rounded nodules that may be interpreted as malignancy. Pulmonary function tests may show an obstructive or restrictive pattern.
Cryptogenic Organizing Pneumonia

Diagnosis requires organizing pneumonia as the primary histopathologic finding in conjunction with clinical exclusion of known associated conditions. Surgical lung biopsy is the preferred technique, although bronchoscopic lung biopsy occasionally provides sufficient tissue for diagnosis.
Treatment of Cryptogenic Organizing Pneumonia
Although some patients with mild disease improve without treatment, systemic corticosteroids result in rapid symptomatic improvement and gradual resolution of radiologic findings. Corticosteroid therapy should be continued at a dose of 1.0 to 1.5 mg/kg of prednisone for 1 to 3 months, with a prolonged taper over 6 to 12 months. The disorder commonly recurs during corticosteroid tapering. The prognosis is generally good and only occasionally requires long-term treatment with corticosteroid-sparing cytotoxic therapy.
Acute onset, constitutional symptoms, Raynaud phenomenon, “mechanic’s hands,” arthritis, and interstitial lung disease + Anti-Jo-1 Antibodies
Antisynthetase Syndrome
Pleural Plaques on a CXR are most commonly a result of what exposure
Pleural plaques are the most common sequelae of asbestos exposure and typically develop bilaterally, with a latency of 10 to 20 years.
Bilateral interstitial fibrosis of the lung parenchyma caused by inhalation of asbestos fibers, with a latency of 20 to 30 years.
Asbestosis
Aside from asbestos exposure, what are other causes of pleural plaques (especially a unilateral pleural plaque)?
Previous pneumothorax

Previous empyema

Mesothelioma

Lymphoma

Multiple Myeloma

Pleural Metastases

The callus formed after a rib fracture may also mimic pleural thickening.

Talc, mica, tin, barite, and silica may also cause pleural plaques, although some of these materials may simply be contaminated with asbestos.
Consists of more extensive fibrosis of the pleura than occurs with circumscribed pleural plaques. Thickening extends into the visceral pleura and obliterates the costophrenic angles.

It is more likely to be associated with pulmonary impairment, including breathlessness and restrictive pulmonary physiology.
Diffuse Pleural Thickening

Patients with diffuse pleural thickening may develop hypercapnic respiratory failure as their lungs become encased by a thick pleural rind that prevents the lungs from expanding.

Surgical decortication has shown mixed results and is not routinely recommended.
The result of infolding of thickened visceral pleura with collapse of the adjacent peripheral lung.

It can present clinically as single or multiple masses, which must be distinguished from a malignancy and can be progressive and associated with pulmonary impairment consisting of dyspnea and restrictive pulmonary physiology.

The classic radiographic finding is a “comet tail” on chest CT scan extending from the hilum toward the base of the lung and then sweeping into the inferior pole of the lesion
Rounded Atelectasis
Aside from asbestosis, what are other conditions that have been associated with rounded atelectasis?
Parapneumonic effusions

Heart failure

Dressler Syndrome

Pulmonary infarcts

Chest trauma with hemothorax
The most extensively studied occupational exposures with epidemiologic and pathologic data to support a causative link to COPD are...
Coal

Silica

Cadmium
Approximately what percentage of COPD is work-related?
In 2003, the American Thoracic Society published a statement on the occupational contribution to the overall burden of airways disease, which concluded that 15% of cases of both asthma and COPD are likely to be work related.

Another study based on U.S. National Health and Nutrition Examination Survey (NHANES) III data estimates the percentage of COPD attributable to work at 19% overall and 31% in never-smokers.

Unlike occupational asthma, occupational COPD has no distinct definition. The clinical and pathologic presentation of most occupational COPD is indistinguishable from nonoccupational COPD, and there are no laboratory tests or pulmonary physiology tests that aid in establishing the diagnosis as work-related. The diagnosis of occupational COPD is currently mostly based on inference from results of population studies to causation in the individual patient. The treatment is the same, except removal from the exposure is added.
What are the 5 different pathophysiologic processes associated with pleural effusions?
(1) transpleural pressure imbalance (e.g. transudative effusion of heart failure)

(2) increased capillary permeability (exudative effusion of pneumonia)

(3) impaired lymphatic drainage (exudative effusion of malignancy)

(4) transdiaphragmatic movement of fluid from the peritoneal cavilty (transudative effusion of hepatic hydrothorax)

(5) pleural effusions of extravascular origin (exudative effusion of chylothorax)
Most common cause of all pleural effusions
Transudate from Heart failure

[Parapneumonic and malignant effusions are the most common exudates]
Pleural fluid lymphocytosis (at least 80% lymphocytes) narrows the differential diagnosis of the exudate to what disorders commonly?
Tuberculous effusion

Chylothorax

Lymphoma

Yellow Nail Syndrome

Rheumatoid pleurisy

CABG

Sarcoidosis

Acute Lung Rejection

Uremic Pleurisy
Pleural fluid pH less than 7.30 narrows the differential diagnosis of the exudate to what disorders?
Complicated parapneumonic effusion

Esophageal rupture

Chronic rheumatoid pleurisy

Malignancy

Lupus pleuritis

Tuberculosis effusion
An increased pleural fluid amylase concentration (pleural fluid/serum amylase ratio greater than 1.0) limits the differential diagnosis to what disorders?
Pancreatic disease (acute pancreatitis or pancreaticopleural fistula)

Esophageal rupture

Malignancy, most commonly adenocarcinoma of the lung.
Transudative pleural effusion, lymphedema, and yellow dystrophic nails
Yellow Nail Syndrome
Ascites, pleural effusion, and benign ovarian tumor
Meigs Syndrome
If pleural pH measurement is not available at the institution what other test can be ordered that correlates with pH of the pleural fluid?
The pathophysiologic mechanisms resulting in low pleural fluid pH and pleural fluid glucose are interrelated. Therefore, if pleural fluid pH measurement is not available in an institution, the same information can be gained by identifying a pleural fluid glucose concentration less than 60 mg/dL (3.33 mmol/L) with normal plasma glucose or a pleural fluid/plasma glucose ratio less than 0.50.
In pleural effusions associated with pneumonia, the presence of loculated pleural fluid, pleural fluid pH less than 7.20, pleural fluid glucose less than 60 mg/dL (3.33 mmol/L), positive pleural fluid Gram stain or culture, or the presence of gross pus in the pleural space will warrant what further management steps?
Catheter or Chest tube placement as the patient will have a likely poor response to antibiotics alone
Treatment for a thoracic empyema
Thoracic empyema (pus in the pleural space) develops when antibiotics are not given and the pleural space is not drained in a timely manner. In this case, video-assisted thorascopic surgery (VATS) is indicated to break down loculations and drain pus from the pleural cavity.

Early antibiotics directed at the pneumonia and timely small-bore chest tube drainage (with or without fibrinolytic agents) for complicated parapneumonic effusions (defined as a pleural fluid pH less than 7.30, glucose less than 60 mg/dL [3.33 mmol/L], LDH greater than 1000 U/L, fluid loculation, or positive Gram stain or culture) typically can preclude the need for VATS or thoracotomy.
Pleural fluid pH less than 7.30, glucose less than 60 mg/dL [3.33 mmol/L], LDH greater than 1000 U/L, fluid loculation, or positive Gram stain or culture)
Definition for a complicated parapneumonic effusion
What are options for patients with multiple recurrent symptomatic pleural effusions in a patient with malignancy that is not responsive to chemotherapy?
Patients with recurrent, symptomatic malignant pleural effusions not responsive to chemotherapy require drainage for relief of dyspnea. Drainage can be accomplished either by placing an indwelling catheter as an outpatient or performing pleurodesis (typically an inpatient procedure) using a chemical agent, such as large-particle talc, as a slurry through a chest tube or by poudrage (the surgical application of powder) at VATS. A large, multicenter trial using large-particle talc showed the procedure to be safe with no episodes of respiratory failure.
What is the treatment for a patient with a positive tuberculin skin test and a lymphocyte-predominant, exudative pleural effusion, without an alternative diagnosis
Should be treated with the same 6-month multidrug regimen used for treatment of pulmonary tuberculosis. There are insufficient data to support the use of adjunctive corticosteroids in the treatment of tuberculous effusions.

Tuberculous pleural effusions, whether or not treated with antituberculosis drugs, resolve in 4 to 16 weeks. However, a patient with a tuberculous effusion who does not receive drug therapy has a 65% chance of developing pulmonary or extrapulmonary tuberculosis within the next 5 years.
Visceral pleural restriction from a remote inflammatory process that results in transpleural pressure imbalance and a transudative pleural effusion
Trapped lung

However, the effusion often presents as a protein-discordant exudate that is diagnostically problematic. The protein concentration in pleural fluid of vascular origin depends on the protein reflection coefficient, solvent filtration into the pleural space, and the bulk flow of fluid by way of the pleural lymphatic vessels. In trapped lung, solvent filtration may not be increased and lymphatic bulk flow may be impaired, resulting in an increased total protein concentration. Therefore, finding a pleural effusion with protein in the exudative range makes the diagnosis of trapped lung likely. If the patient has no significant symptoms from a small trapped lung, observation is appropriate. However, with significant visceral pleural restriction and a large pleural effusion, decortication should be recommended if the underlying lung on CT scan is relatively normal.
Typically present 3 weeks (range 3 days to 1 year) after coronary artery bypass graft surgery; they usually have pleuritic chest pain and typically dyspnea associated with a pleural effusion, pleural or pericardial friction rub, fever, left lower lobe infiltrates, leukocytosis, and an increased erythrocyte sedimentation rate.
Post-cardiac injury syndrome
Next step for a patient with pulmonary nodules that are smaller than 4 mm detected on CT scans in patients at low risk for lung cancer (no history of a first-degree relative with lung cancer or significant radon or asbestos exposure) and the nodules are unchanged compared to previous imaging.
No follow up is indicated
First step in the evaluation of a pulmonary nodule.
Review previous imaging
What are the risks of lung cancer if a patient is committed to smoking cessation?
After smoking cessation, the risk of lung cancer decreases for about 15 years and then it remains about 2x that of a never-smoker.
What are the 3 most important factors in the treatment and prognosis of lung cancer?
The three most important factors in the treatment and prognosis of lung cancer are cell type, cancer stage, and performance status of the patient.
Most cost effective way to assess for preoperative staging of patients with known or suspected non–small cell lung cancer.
PET scanning or PET-CT
Treatment for Stages I or II NSCLC?
Surgical Resection
Lesions larger than what size are considered masses in the lung?
3 cm
What is the risk of lung cancer in a patient who has smoked 40 py compared to a nonsmoker?
The risk of a current smoker with a 40-pack-year smoking history is approximately 20 times that of someone who has never smoked.
In a smoker with airway obstruction on PFTs, what is the increased risk of cancer?
The presence of airway obstruction on pulmonary function testing is associated with about a four- to sixfold increase in the risk for lung cancer.
Asbestos exposure + Smoking increases the risk of cancer by how much?
50-90x
What % of patients with bronchogenic carcinoma have a paraneoplastic syndrome?
10%
What are potential explanations of False-Negative PET scans in the evaluation of pulmonary nodules?
False-negative PET scans may occur with low-grade tumors, such as bronchoalveolar cell carcinoma, carcinoid tumor, and tumors less than 1 cm in diameter.
Treatment of Limited-stage SCLC
Chemotherapy and Radiation Tx
Treatment of extensive-stage disease SCLC
Chemotherapy only
When should photodynamic therapy be considered?
Photodynamic therapy appears to be most appropriate for radiographically occult superficial squamous cell tumors but is also used in other forms of NSCLC; laser therapy and brachytherapy may be used in either NSCLC or SCLC. Photodynamic therapy may also be used for palliation of extensive endobronchial disease.
When should laser endobronchial resection be considered?
Laser endobronchial resection should be considered in patients who do not respond to other treatment modalities, have an identifiable bronchial lumen, and show evidence of functioning lung tissue beyond the level of the endobronchial obstruction. Palliation of symptoms is often immediate, although temporary, and median survival after laser resection is approximately 6 months.
When should brachytherapy be considered?
Brachytherapy is the intraluminal application of radiation and is generally used in patients who have previously received maximal doses of external-beam radiation. Brachytherapy is appropriate for both intrinsic and extrinsic malignant airway obstruction when functioning lung may be maintained or regained by achieving airway patency. Response rates range from 30% to 80%, with success more likely in patients who had a favorable response to previous external-beam radiation. Hemorrhage or fistula formations are the most frequent complications.
Describe palliation strategies of prosthetic airway stents
Bronchoscopic placement of prosthetic airway stents can palliate both intrinsic and extrinsic malignant obstruction. Silastic stents (which are removable), metal stents, and combinations of metal and silastic materials have been used. Symptomatic improvement after regaining airway patency may be immediate and impressive. Complications with the silastic stents include migration, mucus obstruction, and granulation tissue formation. Cough may be troublesome, even when dyspnea is improved. The metal stents become incorporated into the bronchial wall and may be irretrievable or removed with great difficulty, and the formation of granulation tissue is more prevalent. Malignant growth may occur through the wall of the uncovered metal stents or proximal or distal to either type of stent.
Low-grade malignant neoplasms that consist of neuroendocrine cells and account for 1% to 2% of all tumors of the lung. Patients may present with hemoptysis, have evidence of bronchial obstruction, or be asymptomatic.
Carcinoid Tumor

he association of carcinoid syndrome (flushing and diarrhea) with bronchial carcinoid tumor is rare.
Treatment of Pulmonary Carcinoid Tumor
Surgical resection is often curative, and in the absence of nodal metastasis, the 10-year survival rate for patients with typical carcinoid tumors is greater than 90%.

Prognosis is worse in patients with tumors larger than 3 cm or in the presence of nodal metastasis.
Term to describe and significance of a carcinoid tumor w/ histologic evidence of increased mitotic activity, nuclear pleomorphism, and/or necrosis.
"Atyipical Carcinoid"

Atypical carcinoids tend to have a higher rate of metastasis and to be larger at diagnosis than typical carcinoid tumors. The 5-year survival rate for patients with atypical carcinoid tumors is approximately 60% to 70%, and, when feasible, surgery is the therapy of choice.
Approxiamtely what % of all malignant nodules are metastases?
Approximately 10% to 30% of all malignant nodules resected from the lung are metastases.
Common sources of pulmonary metastases
Carcinomas that are frequent sources for pulmonary metastases include those of the head and neck, colon, kidney, breast, and thyroid gland, as well as melanoma.
DDx of an anterior mediastinal mass?
Masses in the anterior mediastinum are usually one of 5 T’s: Thyroid (or parathyroid) tumors, Thymoma (or thymic carcinoma), Teratoma (or germ cell tumors), Thomas Hodgkin disease, and T-cell lymphoma.
Weakness + thymoma suggests ?
Myasthenia Gravis

Approximately 35% to 50% of patients with a thymoma have myasthenia gravis, and approximately 15% of patients with myasthenia gravis have a thymoma.
Paraneoplastic syndromes associated with thymomas?
Myasthenia gravis

Pure red cell aplasia

Hypogammaglobulinemia
DDx of a mass in the middle mediastinum
Enlargement of lymph nodes (?mets)

Lymphoma

Granulomatous diseases (Sarcoidosis, Fungal infections, Pulmonary TB)

Giant lymph node hyperplasia (Castleman Disease)

Pericardial or bronchogenic cysts

Vascular masses or enlargements

Diaphragmatic hernias
DDx of masses seen in the posterior mediastinum
Masses most often originate from the esophagus or from neural tissue.

Esophageal lesions include leiomyomas, fibromas, and lipomas.

Neural tumors include neurofibroma and neurilemmoma (schwannoma).
Endobronchial obstruction in a nonsmoking young adult...what's your most likely dx?
A carcinoid tumor is the most likely tumor in a young person who has never smoked and who has evidence of endobronchial obstruction. Bronchial carcinoid is a slow growing tumor that originally was classified as an adenoma but has been reclassified as a malignant neoplasm because of its ability to metastasize. Most bronchial carcinoid tumors are located in proximal airways and cause symptoms by either obstructing an airway or bleeding. Common presenting symptoms include cough or wheeze, hemoptysis, and recurrent pneumonia in the same pulmonary lobe. The carcinoid syndrome is caused by systemic release of vasoactive substances such as serotonin, and the most typical features include cutaneous flushing and diarrhea. Bronchial carcinoids are not commonly associated with the carcinoid syndrome because of their relatively small amount of serotonin production.
Most common antibody present in a sensory neuropathy associated with small cell carcinoma.
ANNA-1
______ should be considered in patients with asthma who require more than minimal medication to control their disease.
Allergy evaluation
Test that has a high negative predictive value for asthma, and a negative test can therefore be used to exclude asthma.
Methacholine challenge test
Adding ____________ in patients whose asthma is not controlled on low- to medium-dose inhaled corticosteroids is more effective than doubling the dose of inhaled corticosteroids.
long-acting β2-agonist inhalers
Preferred class of medications for the treatment of patients with aspirin-sensitive asthma, exercise-induced asthma, and virus-induced wheezing.
Leukotriene modifiers
Class of medications that should be used for long-term control of asthma during pregnancy
Inhaled corticosteroids
As of 2010, what is the annual mortality rate from having asthma in the United States?
4000 deaths from asthma annually in the United States
What is the utility of checking sputum eosinophils in asthma?
Eosinophils in the sputum are associated with increased airway hyperresponsiveness and propensity toward exacerbations, although, at this time, examination of sputum for eosinophils is of limited clinical utility in patients with stable asthma.
What significance does the a neutrophilic phenotype of asthma have?
Neutrophils contribute significantly to the pathogenesis of persistent asthma, especially severe persistent asthma, or viral disease–induced asthma exacerbations. If neutrophils are present, the disease tends to be less reversible with inhaled bronchodilators, less responsive to corticosteroid therapy, and associated with reduced lung function. There are no medications that specifically target this neutrophilic phenotype of asthma. However, there is limited evidence that macrolide antibiotics and 5-lipoxygenase inhibitors may be effective in treating neutrophilic asthma.
Abrupt onset of severe symptoms, often with rapid improvement. Monophonic wheezing heard loudest during either inspiration or expiration.The preferred diagnostic test is direct visualization during symptoms or flow volume loops. May closely mimic asthma, particularly in young adults.
Vocal cord dysfunction
Voluminous sputum production, often purulent, sometimes blood tinged. Suspect when physical examination shows clubbing or crackles with wheezing or with peribronchial thickening on chest radiograph.
Bronchiectasis
What % defines significant airway obstruction reversibility?
Significant reversibility of obstruction is indicated by a 12% or greater improvement in FEV1 after administration of a short-acting inhaled β2-agonist
The use of peak flow meters may be a consideration for which type of patients who have asthma?
Patients who are poor perceivers of airway obstruction or who have more severe asthma symptoms may benefit from regular home monitoring using peak flow meters

Peak flow meters can be used at home for serial measurement of lung function and to assess the relationship of lung function to symptoms. In patients with poor perception of airway obstruction, monitoring peak expiratory flow rate (PEFR) may help detect the loss of asthma control. When peak flow meters are used for managing asthma in outpatient settings, the highest value of PEFR obtained during a 2-week period of stability is regarded as a patient’s personal best. Obtaining subsequent values within 20% of the personal best suggests that the disease is stable. PEFR reduction of 20% to 50% from personal best indicates a moderate increase in airway obstruction, whereas a greater than 50% reduction suggests severe airway obstruction.
When should you use a methacholine challenge test?
In patients who have symptoms suggestive of asthma but normal spirometry, bronchoprovocation with methacholine can help establish the presence of airway hyperresponsiveness.

Methacholine is administered by inhalation in increasing concentrations through a dosimeter-activated nebulizer. Spirometry is obtained after inhalation of each dose of methacholine to determine the effect on FEV1. Increasing doses of methacholine are given until there is a 20% or greater reduction in FEV1. The dose-response curve is used to calculate the methacholine concentration leading to a 20% reduction in FEV1, which is referred to as the provocative concentration 20% (PC20). In normal persons, the PC20 is typically greater than 16 mg/mL; most patients with asthma have a PC20 of less than 8 mg/mL.

Cigarette smoking, chronic obstructive pulmonary disease, allergic rhinitis, and recent viral respiratory tract infection can lead to increased airway hyperresponsiveness and a positive methacholine challenge test. Therefore, the best clinical use of the methacholine challenge test is to exclude asthma in patients who have atypical symptoms because it has a high negative predictive value. However, a positive test should be correlated with other features of asthma before reaching a clinical diagnosis.
What is the utility of FeNO?
Measurement of exhaled nitric oxide is sometimes used as an indirect method of assessing airway inflammation in asthma. It is noninvasive, easy to do, and reproducible. In several studies, increased exhaled nitric oxide has been shown to correlate with asthma exacerbation and with eosinophilic inflammation.

Inhaled corticosteroids decrease the levels of exhaled nitric oxide so predictably that measurement of nitric oxide levels can be used to test adherence with inhaled corticosteroids.

An approach to asthma management based on measurement of exhaled nitric oxide has been proposed, but there are no randomized clinical trials that support this approach.
Describe the term Reactive Airways Dysfunction Syndrome
Exposure to high levels of irritants (for example, chlorine gas, bleach, or ammonia) can result in significant airway injury, which can lead to persistent airway inflammation and dysfunction with airway hyperresponsiveness and obstruction. After a single exposure, typically accidental, the patient may develop chronic and persistent cough, shortness of breath, and chest tightness. This is known as the reactive airways dysfunction syndrome (RADS).
Most common infectious cause of asthma
Rhinovirus
Association of Asthma and GERD?
Gastroesophageal reflux disease (GERD) is common in patients with asthma and can lead to chronic cough resembling that of cough-variant asthma or may result in worsening asthma control and severity.

Patients with refractory asthma should be evaluated for GERD. Although heartburn is a common symptom in GERD, many patients do not have typical symptoms and can be asymptomatic. Therefore, in patients with difficult-to-control asthma, lifestyle modifications and a trial of acid suppression with a proton pump inhibitor should be considered.

Acid suppression neutralizes the acidity of the gastric juices but does not prevent reflux. Therefore, patients with GERD and asthma should be encouraged to adopt lifestyle changes to reduce reflux, including avoiding meals close to bedtime, avoiding certain foods, and elevating the head of the bed.

If GERD is still suspected, a 24-hour esophageal pH monitoring study should be considered.
Diagnosis and Treatment of Allergic Bronchopulmonary Aspergillosis?
Affected patients typically have eosinophilia, elevated serum levels of circulating IgE (total and specific IgE against A. fumigatus), and a positive skin test for Aspergillus.

The treatment of ABPA is similar to that of asthma in general, but the initial therapy involves systemic corticosteroids until the disease is controlled; the corticosteroid dosage is then tapered, and IgE levels are monitored. Untreated, ABPA can result in permanent lung damage and fibrosis.
Exercise-Induced Asthma
Exercise-induced bronchospasm (EIB) can develop in most patients with asthma if they do high-intensity exercise. The occurrence of EIB is related to the degree of ventilation with significant contribution of cooling and drying of the airways. The symptoms typically occur during or shortly after exercise, peak within 5 to 10 minutes after stopping the activity, and resolve in less than 30 minutes. The disorder can be confused with vocal cord dysfunction, which can also be induced by exercise. Exercise challenge testing helps confirm the diagnosis. Typically, a 15% reduction in FEV1 after intense exercise is compatible with the diagnosis.

Affected patients do not have to limit their participation in sports or other physical activities. Approximately 10% of the athletes on the U.S. Olympic teams in recent years had EIB. The bronchospasm can be prevented by adequate warm-up before exercise, by wearing a mask or scarf over the mouth in cold weather, or by therapy with short- or long-acting β2-agonists or leukotriene antagonists. Short-acting β2-agonists given 10 to 15 minutes before exercise can prevent EIB for up to 3 hours. In some patients, EIB is the only manifestation of asthma, and such patients require treatment with intermittent short-acting inhaled β2-agonists before exercise without regular use of long-term controller therapy.
Treatment of vocal cord dysfunction?
Treatment involves speech therapy, behavior modification, and patient education.

During an acute attack, inhalation of a helium-oxygen mixture and continuous positive airway pressure can relieve the symptoms of VCD.
What % of patients with asthma may develop bronchoconstriction after taking aspirin?
Up to 20% of adults with asthma develop bronchoconstriction after taking aspirin or other NSAIDs.

Patients with asthma, especially those with poorly controlled disease, should be asked about the use of these analgesics and advised to avoid taking them.

Patients who must use aspirin should be referred for desensitization.

The mechanism for aspirin-induced asthma involves increased leukotriene levels, and, as such, patients with mild disease may benefit from the use of leukotriene modifying agents (montelukast, zafirlukast) as part of their asthma treatment.
What is the significance of homozygosity of position 16 of the beta-2 adrenergic receptor?
Questions were raised about the safety of β2-agonists in a subgroup of patients with asthma who are homozygous for arginine (Arg/Arg) rather than glycine at position 16 of the β2-adrenergic receptor.

Studies done by the Asthma Clinical Research Network suggested that this polymorphism in the receptor could lead to an unfavorable response to a short-acting β2-agonist when used regularly but not when used as needed.
What are some significant side effects to discuss with patients regarding the use of leukotriene modifiers?
Leukotriene modifiers have been associated with the development of the Churg-Strauss syndrome in patients with asthma who are tapering the dosage of oral corticosteroids. It is unclear whether this is due to unmasking preexisting Churg-Strauss syndrome or a direct causal relationship of the drug.

Recently, neuropsychiatric side effects, including agitation, depression, and suicide, have been reported in patients taking leukotriene modifiers. Therefore, the FDA advised stopping these drugs if patients start experiencing neuropsychiatric symptoms.
A recombinant monoclonal antibody that binds to the Fc portion of IgE antibody, is approved for use in patients with moderate to severe, persistent allergic asthma
Omalizumab

By binding to the free circulating IgE, omalizumab prevents IgE from attaching to its receptors on the surface of basophils and mast cells, thus limiting IgE cross-linking and release of mediators. The amount of anti-IgE antibody given to patients needs to be adjusted based on the patients’ weight and circulating serum IgE level so that the majority of free IgE in the serum is bound and therefore unavailable to attach to the surface of basophils and mast cells. Treatment with omalizumab improves asthma control, reduces exacerbations, and lowers the required dose of corticosteroids. Omalizumab is given as a subcutaneous injection every 2 to 4 weeks based on patients’ weight and serum IgE levels. There is a small risk for serious anaphylactoid reactions, which have occurred in roughly 1 of 1000 patients. Therefore, treated patients need to be observed for at least 2 hours after the initial three doses and for 1 hour after subsequent treatments. Like other biologic medications, omalizumab is expensive, with therapy costing up to $36,000 per year. Therefore, it should be reserved mainly for patients with severe, poorly controlled asthma who do not respond to other treatments.
Most common cause of excessive daytime sleepiness?
lack of sleep
What are ways of enhancing compliance to therapy for OSA?
Using heated humidification and educating the patient about the benefits of positive airway pressure therapy can enhance compliance to therapy for obstructive sleep apnea.
Hypoventilation (high PCO2) and a diminished response to hypercapnia is associated with what?
Hypercapnic central sleep apnea
Characterized by a normal or low waking PCO2 and an increased ventilatory response to hypercapnia.
Nonhypercapenic central sleep apnea
Muscular dystrophy, myotonic dystrophy, amyotrophic lateral sclerosis, and poliomyelitis are associated with ________________, such as alveolar hypoventilation, that may precede waking abnormalities in respiration by several months to years.
nocturnal ventilatory impairment
The patients may have episodic hypoventilation during sleep when function of those muscles is less reliable and especially during rapid eye movement sleep when accessory muscles are hyperpolarized and unavailable.
Patients with prominent awake respiratory accessory muscle
A rare disorder characterized by recurring periods of excessive amounts of sleep and altered behavior)
Kleine-Levin Syndrome
Defined as a significant reduction in airflow (a decrease in the peak thermal sensor amplitude by at least 90% from baseline) for at least 10 seconds that occurs despite efforts to breathe and that is secondary to upper airway obstruction.
Obstructive Sleep Apnea
Characterized by a reduction in nasal pressure of at least 30% of baseline for at least 10 seconds that is accompanied by at least 4% oxygen desaturation.
Obstructive hypopnea
How do you assess severity in OSA?
he severity of obstructive sleep apnea (OSA) is commonly based on the apnea-hypopnea index (AHI; sum of apneas and hypopneas per hour of sleep): mild sleep apnea consists of an AHI 5 to 15/hour; moderate sleep apnea, AHI 16 to 30/hour; and severe sleep apnea, AHI greater than 30/hour. It is estimated that 24% of men aged 30 to 60 years and 9% of similarly aged women have OSA (AHI at least 5/hour).
Pathophysiology of OSA
The patency of the upper airway is maintained during the waking state by activation of muscles that dilate this region; the dilating activity of these muscles is significantly reduced during sleep and is exceeded by forces that promote closure of the airway. The airway is more vulnerable to closure in patients with OSA than in unaffected persons. In a patient whose airway has closed, airway patency is reestablished during brief arousals that accompany the termination of apnea-hypopneas. Closure recurs with the resumption of sleep. Respiratory events generally occur less frequently during non–rapid eye movement sleep than in rapid eye movement sleep. In some persons, respiratory events occur predominantly or exclusively in the supine sleep position.

Apnea-hypopneas are commonly associated with oxygen desaturation, mild to moderate hypercapnia, and changes in blood pressure and heart rate; there is a relative bradycardia and reduction in blood pressure during respiratory events, and tachycardia and an increase in blood pressure during the termination of apnea-hypopneas. Premature ventricular contractions may also occur.
Risk Factors for OSA
Risk factors for OSA include excessive body weight, abnormalities of craniofacial anatomy, male sex, underlying medical or neurologic disorders (myxedema, acromegaly, and stroke), alcohol use, certain medications (muscle relaxants, sedatives, opioids, and anesthetics), and aging.
Complications of untreated OSA
Patients with untreated OSA have a greater likelihood of developing coronary artery disease, acute myocardial infarction during sleep, systemic and pulmonary arterial hypertension, heart failure, recurrent atrial fibrillation, stroke, insulin resistance, mood disorders, and parasomnias. OSA may also negatively affect quality of life and academic and occupational performance, may increase the risk of vehicular and work-related accidents, and may increase the overall mortality rate.
Treatment considerations for OSA?
Therapy is recommended for all patients with an AHI at least 15/hour and for patients with excessive daytime sleepiness, insomnia, impaired cognition, mood disorder, hypertension, ischemic heart disease, or stroke and an AHI between 5 and 15/hour.

Positional sleep therapy consists of measures to prevent sleep in a supine position for patients whose OSA occurs exclusively or predominantly during a supine sleep position and whose AHI normalizes during sleep in a lateral or prone sleep position.

Supplemental oxygen is not recommended as a primary therapy for OSA and should not be used to replace positive airway pressure therapy. Oxygen therapy may be beneficial if marked oxygen desaturation related to respiratory events does not respond to positive airway pressure therapy alone. No pharmacologic agent is consistently effective in treating OSA. Patients should be counseled not to drive or engage in other potentially dangerous activities unless their OSA is optimally treated and they are fully alert.
Characterized by the recurrent cessation or reduction of airflow lasting for at least 10 seconds secondary to an absence of inspiratory effort.
Central Sleep Apnea
Recurring periods of gradually waxing and waning tidal volume (crescendo-decrescendo ventilation) with repetitive central apnea-hypopneas alternating with prolonged hyperpneas (increased depth of breathing. Can develop in patients with heart failure or neurologic disorders, with a prevalence of about 10% in patients with strokes or renal failure
Cheyne-Stokes Respirations
Risk Factors for Central Sleep Apnea
Risk factors for primary CSA include a high carbon dioxide ventilatory drive (that is, increased response to hypercapnia) and male sex.

CA can develop acutely after ascent to high altitudes. In patients with heart failure, the likelihood of CSA is increased in persons older than 60 years and those with atrial fibrillation and low awake PCO2.
Below what max inspiratory pressure or forced expiratory volume puts a patient at risk for sleep-related hypoventilation and hypoxemia?
Patients with maximal inspiratory pressures less than 60 cm H2O or forced expiratory volume less than 50% of predicted are at higher risk for sleep-related hypoventilation and hypoxemia, which occur predominantly during REM sleep.
Drug of choice to treat high-altitude pulmonary edema?
Nifedipine, which inhibits hypoxic pulmonary arterial vasoconstriction, has been used both to prevent and to treat HAPE.
How can one test for whether a patient with a chronic respiratory disorder will require additional in-flight
oxygen therapy?
The hypoxia inhalation test (inhalation of a 15% oxygen–85% nitrogen mixture to simulate 8000 feet) or regression formulas may be used to determine whether a patient needs additional in-flight supplemental oxygen.
What % of DVT's re-embolize even when treated.
Many patients die of PE in the first 2 weeks after presentation. During this time, almost 5% of DVTs re-embolize, even when treated. Stable patients usually tolerate these incidental emboli, but hemodynamically compromised patients may not.
What is the role of IVC Filters in patients with unstable PE?
In patients with unstable PE, insertion of an inferior vena cava filter decreases the short-term re-embolization rate by nearly 80%. Although some filters may increase the long-term risk of symptomatic DVT recurrence, they may be lifesaving during unstable PE. Newer filters are removable and may offer the same short-term protection with fewer long-term complications.
Indication for thrombolytic therapy in PE?
In general, patients with persistent shock and those requiring ongoing norepinephrine therapy should be considered for treatment with thrombolytic agents unless the patient is at high risk for bleeding.
What are the risks associated with use of thrombolytic therapy?
Thrombolytic therapy is costly and is associated with significant risks. A review of clinical trials disclosed a 2.1% rate of intracranial hemorrhage and 1.6% rate of fatal intracranial hemorrhage when thrombolytic agents were used for thromboembolic disease. Therefore, thrombolytic therapy should likely be reserved for management of patients with persistent hypotension due to PE who have no contraindications. However, patient selection is difficult, and these agents should be administered only by physicians experienced in their use.
What is the role of echocardiography in the management of PEs?
The use of echocardiography in pulmonary embolism is controversial. Although echocardiographic findings consistent with right heart strain are associated with worse outcomes, there are no clinical data to determine how echocardiographic findings would alter the risk/benefit ratios of any of the interventions mentioned above. Some experts recommend echocardiography in all patients with PE to help identify those in whom additional interventions may become necessary. Others recommend that additional interventions be limited to those with more clinical evidence of cardiopulmonary deterioration. Therefore, the decision to perform echocardiography and the use of the results in management decisions must be based on individualized clinical judgment.
How do we anticoagulate patients with PE?
During the first 5 or so days of therapy for PE, heparin, LMWH, or fondaparinux is necessary because these drugs prevent propagation of thromboemboli; warfarin is used to prevent recurrence. Warfarin is initiated simultaneously with heparin, and both therapies are overlapped for a minimum of 4 to 5 days and until the INR has reached the therapeutic range for two measurements taken 24 hours apart.
In what percentage of patients with significant PE go on to develop chronic thromboembolic pulmonary HTN (CTEPH)?
1-2% of patients

Thromboemboli within the pulmonary arteries become remodeled into large occlusive scars, causing chronic thromboembolic pulmonary hypertension (CTEPH). The vessel wall becomes massively thickened and the lumen progressively narrowed or obliterated.

The reason why some patients with PE develop CTEPH is unclear. Most patients who develop CTEPH were previously diagnosed with acute PE or DVT, but their initial presentations appear, in retrospect, indistinguishable from those of patients whose acute clots resolve.

No modality of treatment of the initial acute PE has been shown to protect against CTEPH. However, posttreatment V/Q scans may detect patients prone to develop chronic disease, especially if performed in patients whose cardiopulmonary function does not return to normal. In patients with abnormal scans, echocardiography can clarify the condition of the right ventricle and, by inference, of the pulmonary vasculature.
How do most patients with CTEPH present?
Patients with CTEPH often present with unexplained progressively worsening dyspnea in the absence of (or out of proportion to) airway or pulmonary parenchymal disease.

The predisposing acute PE may have been asymptomatic or undiagnosed, and therefore only about 50% of patients have a history of clinically detected PE.
What is the workup associated with diagnosing CTEPH?
A V/Q scan invariably shows perfusion defects, although the size of the defects frequently underestimates the extent of disease. This finding can help distinguish CTEPH from small-vessel PAH, in which perfusion defects on V/Q scan are minimal. Echocardiography often shows right ventricular hypertrophy, a sign of advanced disease. Although measurement of the pulmonary artery pressure may be imprecise, some indication of right ventricular dysfunction usually can be observed.

Pulmonary angiography can confirm the diagnosis and determine whether the large-vessel obstruction is amenable to surgical endarterectomy. In CTEPH, the embolic material is organized into the arterial wall, and angiography shows gradual tapering of the lumen, with webs and luminal irregularities being evidence of ineffectual attempts at recanalization.
Angiography shows gradual tapering of the lumen, with webs and luminal irregularities being evidence of ineffectual attempts at recanalization
CTEPH
Definitive treatment for CTEPH
Pulmonary thromboendarterectomy
Mutation associated with Familial PAH?
BMPR3 (bone morphogenic protein receptor 2 gene) - Autosomal Dominant
Appetite suppressant associated with PAH?
Fenfluramine
Scattered, "moth eaten” perfusion pattern in the peripheral lung zones seen on V/Q scan
Suggests PAH, but V/Q may be normal as well
Workup associated with PAH?
PAH should be distinguished from secondary forms of pulmonary hypertension, in which the elevated pulmonary vascular resistance is a result of parenchymal damage, chronic or nocturnal hypoxia, or pulmonary venous disease. The clinical presentation of idiopathic PAH is similar to that of other forms of pulmonary hypertension. The symptoms tend to be nonspecific, including progressively worsening dyspnea and dizziness, although these symptoms usually occur in the absence of (or out of proportion to) pulmonary disease or left-sided heart disease. Physical examination may disclose signs of elevated pulmonary artery pressure and right ventricular strain (loud P2, fixed split S2, tricuspid regurgitation, elevated jugular venous distention). Chest radiographs may show enlarged pulmonary arteries, right atrium, and right ventricle. Echocardiography in more advanced stages may show ventricular hypertrophy. Echocardiography may give reasonable estimates of pulmonary artery pressure and cardiac output, although it is not sensitive enough to rule out PAH or quantify its severity. Echocardiography is also useful to rule out left-sided heart disease and congenital heart disease. The V/Q scan in PAH is either normal or shows a scattered, “moth eaten” perfusion pattern in the peripheral lung zones. Additional testing to rule out other causes of pulmonary hypertension includes pulmonary function tests, polysomnography (if sleep-disordered breathing is suspected), liver chemistry tests, and serologic tests for HIV infection or connective tissue disease.
First-line therapy for patients with severe (New York Heart Association [NYHA] functional class IV) idiopathic PAH and severe systemic sclerosis–associated PAH.
Epoprostenol IV

Treprostinil, which is given subcutaneously three or four times daily, and iloprost, which is inhaled six to ten times daily, are more convenient for the patient and may be used in less severe forms of the disease.
Endothelin receptor antagonist that may reverse some of the hemodynamic effects of PAH?

They are used most commonly in moderate PAH and have been shown to improve cardiac index, pulmonary vascular resistance, respiratory comfort, functional class, and survival.
Bosentan and Ambrisentan
Most significant side effect associated with use of Bosentan for PAH?
Annually, about 10% of patients treated with bosentan develop elevated aminotransferase levels, which are sufficient to require cessation of therapy in 3% of patients.

Ambrisentan, a newer agent, has a much lower rate of aminotransferase elevation, but there is not as much clinical experience with this drug in PAH.
Role of sildenafil in PAH?
Pulmonary vascular smooth muscle dilatation is mediated by cyclic guanosine 3′-5′ monophosphate (cGMP), which is induced by nitric oxide and degraded by phosphodiesterases.

Sildenafil, a phosphodiesterase inhibitor, has been found to be useful in PAH. In patients with milder PAH, sildenafil can improve exercise performance, symptoms, and hemodynamics.
Role of anticoagulation in PAH?
Anticoagulation is used as an adjunct to therapy in many forms of pulmonary hypertension. Pathologic specimens of patients with pulmonary hypertension often disclose in situ thrombosis of the pulmonary vascular bed. Although anticoagulation for PAH has not been tested in large randomized clinical trials, retrospective data and a prospective trial referencing historical controls suggest that anticoagulation is associated with improved survival, especially in patients with nonuniform blood flow on perfusion scans.
Role of Calcium Channel Blockers in PAH?
Although early studies of calcium channel blockers suggested that they may be of benefit in many patients with PAH, only about 5% of patients have any long-term response. Because of the availability of safer and more effective medications, calcium channel blockade is no longer recommended as first-line therapy for patients with PAH.
Role of adjunctive surgical treatment in PAH?
Although medical therapy for PAH is improving, patients with severe advanced disease may require lung transplantation. In some patients, right ventricular failure may be so severe that atrial septostomy is required to improve hemodynamics until transplantation is possible. Although randomized controlled trials have not been performed, one small case series showed modest improvement in hemodynamics and clinical performance after percutaneous atrial septostomy in selected patients with severe pulmonary hypertension.
4 generally accepted indications for placement of an IVC filter for pulmonary embolism
There are four generally accepted indications for placement of an inferior vena cava filter:

(1) absolute contraindication to anticoagulation (for example, active bleeding);

(2) recurrent pulmonary embolism despite adequate anticoagulation therapy;

(3) bleeding complication of anticoagulation therapy; and

(4) hemodynamic or respiratory compromise severe enough that a subsequent pulmonary embolism might be lethal.