Interstitial Lung Disease Flashcards
1
Q
Interstitial Lung Disease
A
- Large group of disorders that affect the alveolar wall that ultimately lead to diffuse scarring or fibrosis
- Misnomer: Interstitial lung disease affects all components of the alveolar wall which include epithelial cells, endothelial cells, and the interstitium
2
Q
Interstitial lung disease
Clinical Manifestations
A
- Clinical Manifestations
- Dyspnea, noticed initially on exertion
- Nonproductive cough
- Crackles prominent at lung bases
- Clubbing may be present
3
Q
Interstitial lung disease
Pathophysiology
A
- Pathophysiology
- Decreased compliance (increased stiffness) of lungs
- Decreased lung volumes
- Loss of alveolar-capillary surface area resulting in impaired measured diffusion
- Abnormalities in small airway function without generalized airflow obstruction
- Hypoxemia without CO2 retention
- Pulmonary hypertension
4
Q
Interstitial lung disease
Imaging
A
- Chest x-ray
- Reticular – increased linear markings
- Reticulonodular – increased linear and small nodular markings
- 10% have normal radiographic findings
- High-resolution computed tomography (HRCT)
- Can distinguish inflammation from fibrosis
- Picks up early changes
- Specific pattern may be suggestive of a particular diagnosis
5
Q
Interstitial lung disease
Testing
A
- Tissue specimens for diagnosis
- Thorascopic lung biopsy
- Transbronchial biopsy via flexible bronchoscopy
- Bronchoalveolar lavage
- PFT
- Restrictive pattern
- Decreased lung volumes
- hypoxia
6
Q
Interstitial Lung Disease
Classification
A
- Classification
- Known Etiology ~ 35%
- Inhaled inorganic dusts (pneumoconiosis such as asbestosis or silicosis)
- Organic antigens ( hypersensitivity pneumonitis)
- Known Etiology ~ 35%
- Unknown Etiology ~65%
- Idiopathic interstitial pneumonias
- Connective tissue diseases (rheumatic disease)
- Sarcoidosis
7
Q
Interstitial Lung Disease
Unknown Etiology – continued
A
- Unknown Etiology – continued
- Less Common
- Pulmonary Langerhans cell histiocytosis
- Lymphangioleiomyomatosis
- Goodpasture’s Syndrome
- Wegener’s granulmomatosis
- Chronic eosinophilic pneumonia
- Pulmonary alveolar proteinosis
- Less Common
8
Q
Interstitial Lung Disease
Pathologic Process
A
- Pathologic Processes
- 1) inflammatory process in the alveolar spaces – alveolitis
- 2) scarring – fibrosis
- Both processes often occur simultaneously
- Granuloma : localized collection of cells called epithelioid histocytes (phagocytic or macrophage), generally accompanied by T-lymphocytes, multinucleated giant cells. Noncaseating in interstitial lung diseases. Examples include hypersensitivity pneumonitis and sarcoidosis
9
Q
Idiopathic Interstitial Pneumonias
A
- Usual interstitial pneumonia
- Desquamative interstitial pneumonia
- Respiratory bronchiolitis interstitial lung disease
- Nonspecific interstitial pneumonia
- Acute interstitial pneumonia
- Cryptogenic organizing pneumonia
- Lymphocytic interstitial pneumonia
10
Q
Usual interstitial pneumonia
A
- Patchy areas of parenchymal fibrosis and interstitial inflammation interspersed between areas of relatively preserved lung tissue
- Fibrosis is the most prominent component of the pathology
- Honeycombing – cystic airspace from retraction of surrounding fibrotic tissue
- Most important disorder in this category is idiopathic pulmonary fibrosis
11
Q
Desquamative interstitial pneumonia
A
- More homogenous than UIP
- Large number of intraalveolar mononuclear cells
- Less prominent inflammation and little associated fibrosis
- Uniform process
- Minimal architectural distortion
- Pigmented macrophages in respiratory bronchioles secondary to smoking
- Smoking is believed to be an important underlying cause
12
Q
Respiratory bronchiolitis interstitial lung disease
A
- Related to DIP
- Associated with pigmented macrophages which are present within the lumen of respiratory bronchioles
- Interstitial inflammation is not present
- Almost always associated with smoking
- Most important intervention is smoking cessation
13
Q
Nonspecific interstitial pneumonia
A
- Mononuculear cell infiltration within the alveolar walls
- Uniform process
- Fibrosis is variable, but less than UIP
- Idiopathic or connective tissue disorder
- Better prognosis
14
Q
Acute interstitial pneumonia
A
- Organizing or fibrotic stage of alveolar damage, which is the histologic pattern seen in ARDS.
- No initial trigger identified.
- Histology shows fibroblast proliferation and type II pneumocyte hyperplasia in the setting of what appears to be organizing alveolar damage
15
Q
Cryptogenic organizing pneumonia
A
- Organizing fibrosis (granulation tissue) in small airways
- Mild degree of chronic interstitial inflammation
- Intraluminal airway involvement is a key feature
- Idiopathic
- Differential: infections, toxic inhalants, or connective tissue disease
16
Q
Idiopathic pulmonary fibrosis
A
- No recognizable inciting agent
- Dysregulated pattern of fibrosis in response to alveolar epithelial injury
- Presents between ages 50-70
- Insidious onset
- Dyspnea is the most common complaint
- Rales on lung examination
- Patients frequently have clubbing
17
Q
Idiopathic pulmonary fibrosis
Imaging
A
- Chest x-ray
- Interstitial pattern that is generally bilateral and relatively diffuse
- More prominent at lung bases, particularly in the peripheral subpleural regions
- High Resolution CT
- Interstitial densities that are patchy, peripheral, subpleural, and associated with small cystic spaces.
- Honeycombing – indicated irreversible fibrosis
18
Q
Idiopathic pulmonary fibrosis
Dx & Px
A
- Diagnosis made with surgical lung biopsy
- HRCT with classic pattern of honeycombing – diagnostic in patient too frail for lung biopsy
- Prognosis
- Poor with mean survival at time of diagnosis 2-5 years
- No proven effective treatment
19
Q
Desquamative interstitial pneumonia
A
- Smokers
- Subacute
- Ground glass appearance on imaging
- Lung biopsy with a uniform accumulation of intraalveolar macrophages with little or no fibrosis
- Prognosis better than IPF
- Improves with smoking cessation and may respond to corticosteroids
20
Q
Nonspecific interstitial pneumonia
A
- Nonspecific interstitial pneumonia
- Ground glass appearance on imaging – inflammation
- Lung biopsy with predominantly inflammatory response in the alveolar walls , with little fibrosis
- Prognosis depends on degree of fibrosis
- Patients often respond to corticosteroids
21
Q
Cryptogenic organizing pneumonia
A
- Connective tissue plug in small airway accompanied by mononuclear cell infiltration of the surrounding lung parenchyma
- Large majority have no specific cause
- Chest x-ray often mimics pneumonia with one or more alveolar infiltrates
- Subacute presentation over weeks to months with constitutional as well as respiratory symptoms
- Response to steroid is dramatic and occurs over days to weeks.
- Therapy is usually prolonged for months to prevent relapse
22
Q
Acute Interstitial Pneumonia
A
- Acute disease that begins with the clinical picture of ARDS but without any usual inciting causes
- Imaging with ground glass appearance, alveolar filling
- Histology of diffuse alveolar damage, often showing some organization and fibrosis
- Mortality is high
- Small percentage of patients do well with clinical resolution and no long term sequelae
23
Q
Interstitial lung disease
A
- Complicating Connective Tissue Disease
- Rheumatoid Arthritis
- Systemic Lupus Erythematosis
- Progressive systemic sclerosis (Scleroderma)
- Polymyositis-dermatomyositis
- Sjogren’s Syndrome
- Overlap Syndromes
24
Q
Interstitial lung disease
Connective Tissue Disorders
A
- Connective Tissue Disorders
- Usually patients have evidence of underlying disease first, but sometimes lung manifestations can proceed diagnosis by several years
- Pulmonary involvement is more common than clinically suspected
- Often UIP, but sometimes NSIP, or COP
- Lower lobe more common than upper lobe disease
25
Rheumatoid Arthritis
* Primary manifestation is inflammatory joint disease
* Most common site of thorax involvement is the pleura
* Pleurisy, pleural effusions or both
* Lung parenchymal involvement includes one or multiple nodules or the development of interstitial lung disease
* Interstitial lung disease is usually mild, but can be severe in some cases
26
Systemic lupus erythematosus
* Multisystem disease that affects joints and skin
* Serious organ involvement includes kidneys, lungs, nervous system, and heart
* Involvement of the thorax includes pleural disease presenting with pleuritic chest pain, pleural effusion, or both
* Acute pneumonitis in which infiltrates often involve the alveolar spaces as well as the alveolar walls
* Less frequently chronic interstitial lung disease – extensive fibrosis usually not prominent
27
Progressive systemic sclerosis ( scleroderma)
* Most obvious manifestation involve the skin and small blood vessels
* Other organ system involvement include GI tract, lungs, kidneys, and heart
* Pulmonary involvement tends to be severe with significant scarring of the pulmonary parenchyma
* Pulmonary fibrosis strongly associated with an autoantibody to topoisomerase I (antitopoisomerase I, also called Scl70)
* Pulmonary Artery Hypertension – small pulmonary vessel disease independent of the fibrosis
28
Polymyositis-dermatomyositis
* Muscles and skin are the primary sites of the inflammatory process
* Interstitial lung disease is relatively infrequent
* Respiratory difficulty secondary to weakness of the diaphragm
* Involvement of the striated muscles in the proximal esophagus may lead to dysphagia and recurrent aspiration pneumonia
29
Pulmonary Langerhans Histiocytosis
* Also called eosinophilic granuloma of the lung or pulmonary histiocytosis X
* Differential diagnosis of unexplained interstitial disease in the young or middle aged adult
* Histiocytic cell appears to be an antigen-presenting dendritic or phagocytic cell called a Langerhans cell
* Cytoplasmic rod like structures called X bodies (Burbeck Granules) which can be seen by electron microscopy
* Light microscopy reveals histiocytes, eosinophils, lymphocytes, macrophages, and plasma cells
30
Pulmonary Langerhans Cell Histiocytosis
* Starts as a peribronchiolar distribution and later becomes more diffuse
* Disease occurs almost exclusively in smokers
* Chest x-ray
* Nodular or reticulonodular disease
* Upper lobe prominent
* HRCT
* Small cysts in addition to the nodular and reticulonodular changes
* Cysts may rupture and cause pneumothorax
* Some cases extensive honeycombing
31
Pulmonary Langerhans Cell Histiocytosis
Natural Hx
* Natural history is variable
* Self limited in some with radiographic and functional changes stabilizing over time, especially with smoking cessation
* In others, more extensive disease and significant functional impairment
* No clearly effective treatment
* Corticosteroids used if smoking cessation alone is ineffective
•
•
32
Lymphangioleiomyomatosis
* Rare pulmonary disease
* Characterized by proliferation of atypical smooth muscle cells around lymphatics, blood vessels, and airways, accompanied by numerous small cysts throughout the pulmonary parenchyma
* Occurs almost exclusively in women of childbearing age
* Develops in 30-40% of female patients with the genetic condition tuberous sclerosis complex
33
Lymphangioleiomyomatosis
Clinical Manifestation
* Clinical Manifestation
* Dyspnea and cough
* Vascular involvement may lead to hemoptysis
* Lymphatic obstruction may produce a chylous pleural effusion
* Airway involvement may lead to airflow obstruction
* Rupture of subpleural cysts can lead to spontaneous pneumothorax
34
Lymphangioleiomyomatosis
Imaging
* Chest x-ray
* Reticular pattern, cystic changes
* Lung volumes normal or increased
* HRCT
* Cystic disease throughout the pulmonary parenchyma
* Pulmonary Function Testing
* Obstructive disease, restrictive disease, or both
35
Goodpasture’s Syndrome
* Affects lungs and kidneys
* Patients have episodes of pulmonary hemorrhage, and pulmonary fibrosis may develop
* Patient have glomerulonephritis characterized by linear deposits of anti-glomerular basement membrane antibody
* Anti-GBM is an antibody against type IV collagen in their own GBM
* Anti-GBM cross reacts with the basement membrane of the alveolar wall leading to pulmonary manifestations
36
Goodpasture’s Syndrome
Tx
* Treatment
* Plasmapheresis is capable of directly removing anti-GBM antibodies from the circulation
* Immunosuppressive therapy such as prednisone or cyclophosphamide are given to decrease the formation of anti-GBM antibiodies in conjunction with the plasmapharesis
37
Wegener Granulomatosis
* Upper respiratory tract, lungs, and kidneys
* Upper respiratory tract and lungs – necrotizing small-vessel granulomatous vasculitis
* Kidneys with focal glomerulonephritis
* Chest x-ray
* Nodules, infiltrates, cavitation
* Diffuse interstitial lung disease is not characteristic
* cANCA +
38
Wegener Granulomatosis
Tx
* Treatment
* Cyclophosphamide
* Prednisone
39
Churg-Strauss Syndrome
* Systemic necrotizing vasculitis
* Affects upper and lower respiratory tracts
* Preceded by allergic disorders such as asthma , allergic rhinitis, sinusitis or drug reaction
* Peripheral and lung eosinophilia
* Increased immunoglobulin E levels
* Rashes
40
Churg-Strauss Syndrome
Imaging
Testing
Tx
* Chest x-ray
* Bilateral patchy, fleeting infiltrates
* Diffuse nodular infiltrates
* Diffuse reticulonodular infiltrates
* Biopsy
* Granulomatous angiitis or vasculitis
* Treatment
* Corticosteroids
41
Chronic eosinophilic pneumonia
* Pulmonary interstitium and alveolar spaces are infiltrated by eosinophils and to a lesser extent macrophages
* Clinical Manifestations
* Occurs over weeks to months
* Fever, weight loss, dyspnea, and productive cough
* Pulmonary infiltrates with a peripheral distribution and more suggestive of alveolar than interstitial disease
* Increased eosinophils in the peripheral smear
* BAL with increased eosinophils
42
Chronic eosinophilic pneumonia
* Treatment
* Dramatic response to corticosteroids within days to weeks
* Therapy must often be prolonged for months to prevent recurrence
43
Pulmonary alveolar proteinosis
* Primary pathologic process affects the alveolar spaces, not the alveolar cells
* Alveolar spaces are filled with a proteinaceous phospholipid material that represents components of pulmonary surfactant
•
44
Pulmonary alveolar proteinosis
* Clinical manifestations
* Dyspnea and cough
* Bilateral alveolar infiltrates
* HRCT crazy paving pattern produced by thickening of interlobular septa accompanied by ground –glass alveolar filling
* Superimposed to respiratory infections - Nocardia
* Treatment
* Whole-lung lavage
* Prognosis is good
45
Hypersensitivity Pneumonitis
* Hyperimmune respiratory syndrome caused by inhalation of a wide variety of allergic antigens that are usually organic
* Causative antigens include bacteria, fungi, protozoa, animal proteins, and reactive chemicals
* Treatment
* Identify causative antigen and avoid exposure
* Prevent progressive permanent lung damage
* Corticosteroids hasten resolution
46
Hypersensitivity Pneumonitis
•Suspect Hypersensitivity Pneumonitis
* Suspect Hypersensitivity Pneumonitis
* Intermittent pulmonary and systemic symptoms
* Progressive pulmonary symptoms with interstitial chest x-ray changes
* Non-resolving pneumonia
* Workplace exposures
* Agricultural and cattle farming
* Recreational exposures
* Bird keeping
* Home exposures
* Contaminated home ventilation
* Hot tubs
47
Hypersensitivity Pneumonitis
•Clinical Presentation
* Clinical Presentation
* Acute
* Abrupt onset of cough, dyspnea, fever, and chest pain following heavy exposure to the offending antigen
* Symptoms begin 4-6 hours after exposure
* Subside within 24 hours of removal from exposure
* Subacute
* More gradual development of symptoms
* Less severe intensity
* Chronic
* Insidious progressive dyspnea, cough, weight loss, and fatigue
* Most progress toward pulmonary fibrosis, with respiratory
48
Hypersensitivity Pneumonitis
•Professions and Hobbies Affected
* Professions and Hobbies Affected
* Farmers
* Bird keepers or breeders
* Woodworkers
* Office workers with forced-air ventilation
* Cheese makers
* Plastic industry workers
* Metal workers
* Painters/refinishers
* Lifeguards
* Machine workers
49
Hypersensitivity Pnemonitis
•Common etiologies
* Common etiologies
* Farming and agriculture – especially dairy cattle
* Thermophillic actinomycetes in moldy hay, grain, or silage
* Bird and Poultry Handling
* Animal proteins from droppings, feather, or serum proteins
* Pigeons, parakeets, budgerigars, canaries, chickens, ducks, and turkeys
* Ventilation and water-related contamination
* Humidifiers, hot-tubs, air conditioners, unventilated showers, or contaminated basements
* Thermophillic actinomycetes
* Mycobacterium avium-intracellulare complex organisms
50
Hypersensitivity Pneumonitis
Imaging
* Chest x-ray
* Acute disease diffuse reticulonodular infiltrates
* Chronic disease with diffuse interstitial fibrosis
* HRCT
* Ground glass opacities early
* Chronic with traction bronchiectasis, honeycombing, and fibrosis
* PFT’s
* Restriction, small lung volumes, decreased diffusion capacity
51
Sarcoidosis
* Sarcoidosis is a systemic granulomatous disease of unknown etiology characterized pathologically by the non-caseasting granuloma.
* Can effect almost any organ
* 90% of patients present with mediastinal or hilar lymphadenopathy and parenchymal lung disease
* Occurs at any age, but most commonly between the ages of 40-60
* Incidence 3.5 times higher in black Americans compared to white Americans
* Women have a higher incidence than men
•
52
Sarcoidosis
Involvement
* Pulmonary involvement frequent, but not necessary for the diagnosis
* Initial presentation may be an abnormal chest x-ray with mediastinal and hilar adenopathy
* 50% of patients have no symptoms at the time of diagnosis
53
Sarcoidosis
•Lofgren’s Syndrome
* Lofgren’s Syndrome
* Acute manifestation of sarcoidosis
* Erythema nodosum
* More common in women
* Arthritis
* More common in men
* Bilateral Hilar Lymphadenopathy
* Prognosis is favorable
54
Sarcoidosis
Etiology
•Sarcoidosis may also cause a more chronic disease leading to pulmonary fibrosis and increased morbidity and mortality
55
Sarcoidosis
Sx
* Symptoms
* General: fatigue, fever, night sweats, and weight loss.
* Pulmonary: Cough, dyspnea with exertion, wheeze, chest discomfort – 50% of patients have only respiratory symptoms
* Skin: rash, macules, papules, nodules, hyperpigmentation or hypopigmentation, erythema nodosum
* Ocular: gritty or dry eyes, pain, redness, and or blurred vision
56
Sarcoidosis
•Symptoms – continued
* Symptoms – continued
* Cardiac: arrhythmia, palpitations, near-syncope, syncope, lower extremity edema
* Nervous System: headaches, blurred vision, numbness, weakness, instability, seizures
* Gastrointestinal: epigastric pain, early satiety, RUQ pain, jaundice
* Musculoskeletal: swelling and joint stiffness, joint pain, muscle pain
* Upper Respiratory tract: nasal congestion, sinus pressure, nasal discharge
57
Sarcoidosis
Signs
* Signs:
* Pulmonary: rales, expiratory wheezing, can also be normal
* Skin: Infiltration of old scars and tattoos, Maculopapular lesions of face, neck , upper back, and extremities, Lupus pernio of nose, cheeks, ears, and lips, Erythema nodosum
* Ocular: uveitis, iritis, scleral plaques, enlarged lacrimal glands
* Cardiac: usually normal, irregular rhythm
58
Sarcoidosis
•Signs –continued
* Signs –continued
* Nervous System: cranial nerves II, VII, and VIII neuropathies, gait instability, weakness
* GI: hepatomegaly, splenomegaly, jaundice
* Musculoskeletal: Arthralgias, arthritis.
* Lymphatic: common sites of palpable adenopathy include cervical, supraclavicular, axillae, and inguinal regions
59
Sarcoidosis
•Organ Involvement:
* Organ Involvement:
* Pulmonary: 90% present with mediastinal and hilar lymphadenopathy or parenchymal lung disease
* Skin Disease: 25-30% of patients
* Lesions have predilection for scars and tattoos
* Erythema Nodosum: 10 % of patients
* Red, hot , tender subcutaneous nodule most common on the shins
* Biopsy of erythema nodosum is not useful since these are not sarcoid granulomas
* Lupus pernio: affects the nose, cheeks, and ears
60
Sarcoidosis
•Ocular Involvement
* Ocular Involvement
* 25% have eye involvement
* Lacrimal gland enlargement and conjunctival involvement are common
* Yellow Nodules – conjunctiva can be biopsied to make diagnosis
* Uveitis can present with pain, photophobia, lacrimation, and redness
* Ocular symptoms can precede pulmonary disease by 20 years.
61
Sarcoidosis
•Cardiac Involvement:
* Cardiac Involvement:
* Cardiac granulomas found in 25% of sarcoid patients at autopsy
* Cardiac involvement only suspected in 5% of patients
* Atrioventricular block
* Ventricular arrhythmias
* Left ventricular dysfunction
* Sudden death
62
Sarcoidosis
•Neurologic Involvement:
* Neurologic Involvement:
* Nervous system granulomas in 25% of patients at autopsy
* 10% of sarcoid patients have neurologic symptoms
* Neurologic symptoms precede diagnosis in 75% of patients with neurosarcoidosis
* Granulomas can occur in the base of the brain, hypothalamus, and pituitary gland
* Most common cranial nerves affected are VII, II, and VIII
* Myelopathy and peripheral neuropathy can occur
63
Sarcoidosis
•Liver and Spleen Involvement:
* Liver and Spleen Involvement:
* Fever, weight loss, and jaundice are present in less than 5% with liver involvement
* 20% of patients will have hepatomegaly
* 5-15 % of patients will have splenomegaly
64
Sarcoidosis
•Bone and Joint Involvement:
* Bone and Joint Involvement:
* Most patients have arthralgias
* 35% of patients develop arthritis
* Ankles and knees most commonly affected
65
Sarcoidosis
•Calcium Metabolism:
* Calcium Metabolism:
* Disorder of vitamin D occurs in 50% of patients
* May result in nephrolithiasis, nephrocalcinosis, and hypercalcuria
* 10-14% of chronic sarcoidosis patients have at least one symptomatic renal stone
66
Sarcoidosis
Dx
* Diagnosis is made on clinical, radiographic, and histologic evidence of noncaseating granulomas in one or more organs
* Lofgren’s syndrome alone is pathognomonic for sarcoidosis and may not require biopsy for diagnosis
* Biopsy should be taken from the safest site such as skin, peripheral lymph nodes, lacrimal glands, or conjunctiva
* Bronchoscopy with transbronchial lung biopsy has a diagnostic yield of at least 85%
•
67
Sarcoidosis
Tx and Results
* Bronchoalveolar lavage may be remarkable for lymphocytosis and an elevated CD4/CD8 ratio, however this is not diagnostic
* Sarcoid granulomas produce ACE. ACE levels are elevated in 60% of patients, but this is nonspecific
68
Sarcoidosis
DDx
* Differential Diagnosis:
* Infection: Mycobacteria, Fungi, Spirochetes, Bacteria, Parasites
* Neoplasia: Lymphoma, Carcinoma
* Extrinsic allergic alveolitis: Animal protein(bird fancier’s lung), Fungi(farmer’s lung)
* Chemical: silica, beryllium
* Immunologic: Crohn’s disease, primary biliary cirrhosis, vasculitis(Wegener’s polyangiitis)
69
Sarcoidosis - Testing
Imaging/Staging
* Chest x-ray staging
* Stage 0: Normal
* Stage 1: Bilateral hilar adenopathy
* Stage 2: Bilateral hilar adenopathy and parenchymal infiltrates
* Stage 3: Parenchymal infiltrates without lymphadenopathy
* Stage 4: Advanced parenchymal disease with fibrosis
* PFT
* Restrictive ventilatory defect, concurrent obstruction, decreased DLCO
70
Sarcoidosis - Testing
* Ophthalmology Examination
* EKG
* Consider Cardiac Event Monitoring and Echocardiography
* CBC
* CMP to assess liver, renal function, and hypercalcemia
* Serum ACE level not sensitive or specific enough
* Biopsy of involved organ
71
Sarcoidosis Treatment
* Should only be initiated for symptomatic patients.
* Prednisone 20-40 mg daily for 3 months. Monitor for improvement with FVC.
* If improved wean to 10-15 mg daily for 6-9 months, then taper off.
* Cytotoxic and immunosuppressives are used for those not responding to prednisone.
72
Sarcoidosis Prognosis
* Spontaneous remission in 50% of patients at 3 years.
* Lofgren’s with the best prognosis
* 2/3 remission after 10 years.
* 1/3 with unrelenting disease
* Less than 5% die from sarcoidosis
73
Silicosis
•A fibrotic lung disease cause by the inhalation of crystalline silica in the form of quartz
74
Silicosis
Industries at Risk
* Industries at Risk
* Mining
* Tunneling
* Excavating
* Quarrying,
* Stonework
* Foundries
* Sandblasting
* Ceramics
* Recently stressed denim jean manufacturing - sandblasting
75
Silicosis
•Clinical Presentations:
* Clinical Presentations:
* Chronic silicosis
* Accelerated silicosis
* Acute silicosis
* Progressive massive fibrosis
76
Silicosis
•Chronic Silicosis
* Chronic Silicosis
* Most common for of the disease
* Occurs 20 or more years after the exposure
* Hallmark pathology is the silicotic nodule characterized by whorled hyalinized collagen fibers with a more peripheral zone of dust laden macrophages
* Patients may be asymptomatic or complain of dyspnea and a productive cough
77
Silicosis
•Accelerated silicosis
* Accelerated silicosis
* Nodules develop after 3-10 years of exposure
* Clinical course is progressive
78
Silicosis
•Acute silicosis
* Acute silicosis
* Develops in 6 months to 2 years after exposure
* Symptoms: dyspnea, cough, and weight loss that rapidly progress to respiratory failure and death
* Pathology consistent with an alveolar filling process
79
Silicosis
•Progressive massive fibrosis
* Progressive massive fibrosis
* Lesions are at least 1cm in diameter and often larger
* Lesions usually involve the upper lobes
* Leads to respiratory failure, cor pulmonale, weight loss, and death
80
Silicosis
•Associated Diseases
* Associated Diseases
* Tuberculosis
* COPD and Chronic Bronchitis
* Collagen Vascular Disease
* Rheumatoid Arthritis
* Scleroderma
* Lung Cancer
81
Silicosis
•Chest x-ray
* Chest x-ray
* Symmetric nodular pattern involving the upper lobes
* Hilar adenopathy with eggshell calcification is strongly suggestive
* Progressive massive fibrosis is characterized by coalescence of the nodules with larger mass lesions
* Acute silicosis displays air space and interstitial pattern on x-ray
82
Silicosis
PFT
* Pulmonary Function Testing
* Normal early in chronic silicosis
* Later mixed pattern of obstruction and restriction
83
Silicosis
Dx
* Diagnosis
* Based on history of exposure and characteristic x-ray changes
84
Silicosis
•Management
* Management
* Disease is irreversible
* Avoid further exposure
* Tb Testing
* Smoking cessation
* Consider lung transplants in acute and accelerated silicosis
85
Coal Worker’s Pneumoconiosis
* Results from the deposit of coal dust in the lung
* Risk increases with the intensity of exposure and carbon content
* Anthracite is most toxic
86
Coal Worker’s Pneumonconiosis
•Risk Factors
* Risk Factors
* Underground miners at more risk than surface miners
87
Coal Worker’s Pneumoconiosis
•Pathology
* Pathology
* Coal macule consisting of macrophages laden with coal dust within the walls of respiratory bronchioles and adjacent alveoli
* Coal nodules
* Progressive massive fibrosis may be seen
88
Coal Worker’s Pneumoconiosis
•Clinical presentation
* Clinical presentation
* Usually no symptoms or signs
* Can have symptoms of bronchitis
* May lead to progressive massive fibrosis
89
Coal Worker’s Pneumoconiosis
•Associated Diseases
* Associated Diseases
* Silicosis is also common in coal workers
* Scleroderma and rheumatoid arthritis
* Caplan Syndrome: rheumatoid arthritis with large cavitary pulmonary nodules associated with silicosis and coal worker’s pneumoconiosis
* Tb not increased unless associated silicosis
* Lung cancer not increased
90
Coal Worker’s Pneumoconiosis
•Chest x-ray
* Chest x-ray
* Resembles silicosis
* Small rounded opacities in the lung parenchyma
* Can progress to Progressive Massive Fibrosis with nodules from 0.5 to 5 cm
91
Coal Worker’s Pneumoconiosis
PFT
* Pulmonary Function Testing
* Normal in early phase
* Often obstructive later on
* May show restriction if pulmonary fibrosis present
* Coal dust has been shown to cause emphysema
92
Coal Worker’s Pneumoconiosis
Dx
* Diagnosis
* Coal Dust Exposure
* Chest x-ray
93
Coal Worker’s Pneumoconiosis
•Management
* Management
* Avoid Exposure
* Stop Smoking
94
Asbestosis
* Chronic fibrotic interstitial lung disease secondary to prolonged inhalation of asbestos fibers.
* Twenty year latency between disease and exposure is common
95
Asbestosis
•Industries at risk
* Industries at risk
* Mining
* Milling
* Transportation of asbestos
* Building demolition
* Brake lining
* Shipbuilding
* Insulations
* Fireproofing
* Asbestos use in construction banned in the 1980’s
96
Asbestosis
•Pathology
* Pathology
* Ferruginous bodies, asbestos bodies
* Found in sputum or BAL fluid
97
Asbestosis
* Clinical Presentation
* Signs
* Clinical Presentation
* Symptoms
* Dyspnea
* Dry cough
* Chest tightness/pain
* Signs
* Inspiratory crackles
* clubbing
98
Asbestosis
•Associated Diseases
* Associated Diseases
* Mesothelioma
* 30-40 year latency
* Incurable
* 9-18 month survival
* Lung Cancer
* Risk 4-6 times higher
* Pleural Effusion
* Usually small, exudative, blood stained and contains leukocytes
* Most clear spontaneously
99
Asbestosis
•Associated Diseases
* Associated Diseases
* Mesothelioma
* 30-40 year latency
* Incurable
* 9-18 month survival
* Lung Cancer
* Risk 4-6 times higher
* Pleural Effusion
* Usually small, exudative, blood stained and contains leukocytes
* Most clear spontaneously
100
Asbestosis
•Chest x-ray
* Chest x-ray
* Pleural plaques
* Pleural effusion 10-15 year latency
* Pleural thickening
* Rounded atelectasis with “comet tail”
* Lower lobe and subpleural disease prominent
101
Asbestosis
PFT
* PFT
* Restrictive pattern, but may be obstructive
102
Asbestosis
Dx
* Diagnosis
* Reliable history of asbestos exposure
* Appropriate lag time between exposure and disease
* Lung fibrosis on chest x-ray or CT
* Restrictive PFT
* Bilateral inspiratory crackles
* Clubbing of fingers or toes
* Exposure and x-ray essential, others confirmatory
103
Asbestosis
•Management
* Management
* No effective treatment
* Avoid exposure
* Stop smoking
* Lung transplantation
104
Beryllium Disease
•Industries at risk
* Industries at risk
* Aerospace
* Electronics
* Ceramics
* Metal
* Nuclear
* Telecommunications
* Tool and die
* Welding
105
Beryllium Disease
•Clinical Presentations
* Clinical Presentations
* Acute Toxic Pneumonitis: high exposure can lead to a hypersensitivity response that is now rare due to better recognition of beryllium associated disease
106
Beryllium Disease
Signs & Symptoms:
* Symptoms:
* Dyspnea
* Cough
* Chest pain
* Signs:
* Blood tinged sputum
* Crackles
107
Beryllium Disease
•Chronic beryllium disease:
* Chronic beryllium disease: clinical features are similar to sarcoidosis ranging from asymptomatic to severe granulomatous restrictive lung disease
* Symptoms:
* Dyspnea
* Cough
* Chest pain
* Weight loss
* Fatigue
* Arthralgias
108
Beryllium Disease
* Chronic beryllium disease - continued
* Signs
* Chronic beryllium disease - continued
* Signs
* Crackles
* Onset: 20 or more years after exposure
* An inquiry into possible beryllium exposure must be made in all potential sarcoidosis patients
109
Beryllium disease
•Chest x-ray
* Chest x-ray
* Enlarged hilar or mediastinal nodes
OR
•Multiple lung nodules
OR
* Both
* Later stages patchy fibrosis, hyperinflation, and honeycombing
110
Beryllium disease
Dx
* Diagnosis:
* Documented exposure to beryllium
* Evidence of lung disease
* Positive BeLPT( beryllium lymphocyte proliferation test performed on blood or BAL fluid
111
Beryllium disease
•Management
* Management
* Avoid further exposure
* Stop Smoking
* Steroids
112
Case 2
## Footnote
* 75 year old male with an abnormal chest x-ray. He had a normal chest x-ray six months ago. He has a 60 pack year smoking history. Three weeks ago he noticed myalgias in his shoulder and neck along with fatigue and a sinus infection. He was treated with antibiotics 2 weeks ago by another doctor with no results. In the past four days he has had hemoptysis with blood streaked sputum. He has had a 20 lb weight loss for the past month. Exam reveals a temperature of 99F. Scattered crackles are heard bilaterally. There are no skin findings and no joint findings. Your office chest x-ray shows bilateral lung nodules, some of which have cavitated.
* Hgb 9.2g/dL
* WBC 10,700
* Na 131
* K 5.2
* CA 8.0mg/dl
* Creatinine 6 mg/dl
* UA 51-100 rbc/hpf, 4-10 WBC/hpf
* The most likely diagnosis is:
* A. Acute interstitial fibrosis
* B. Disseminated histoplasmosis
* C. Wegner’s granulomatosis
* D. Metastatic bronchoalveolar cell carcinoma
* E. Goodpasture’s syndrome
Case 2
## Footnote
* The most likely diagnosis is:
* A. Acute interstitial fibrosis - no b/c there are cavitary nodules and blood in the urine
* B. Disseminated histoplasmosis - usually pt lives in an indemic area and no blood in urine
**_•C. Wegner’s granulomatosis - Pt Sx's match_**
* D. Metastatic bronchoalveolar cell carcinoma
* E. Goodpasture’s syndrome
* Differential Diagnosis of Cavitary Lung Lesions
* C Carcinoma: squamous cell, melanoma, cervical, sarcoma metastasis
* A Autoimmune: Wegner’s, rheumatoid lung
* V Vascular: bland/septic emboli
* I Infection: TB, fungal (coccidio, aspergillosis, cryptosporidia, nocardia) bacterial ( esp. GNR, staph, strep
* T Trauma
* Y Young congenital lesions (bronchogenic cyst or communicating sequestration
113
Case 2
## Footnote
Differential Diagnosis of Cavitary Lung Lesions
Case 2
## Footnote
Differential Diagnosis of Cavitary Lung Lesions
* C Carcinoma: squamous cell, melanoma, cervical, sarcoma metastasis
* A Autoimmune: Wegner’s, rheumatoid lung
* V Vascular: bland/septic emboli
* I Infection: TB, fungal (coccidio, aspergillosis, cryptosporidia, nocardia) bacterial ( esp. GNR, staph, strep
* T Trauma
* Y Young congenital lesions (bronchogenic cyst or communicating sequestration
114
Case 3
## Footnote
* 61 year old male has worsening shortness of breath over the last year. He now becomes short of breath walking only a short distance, and has trouble with simple activities of daily living. This resolves when he lies down. He has a hacking cough that is nonproductive. He admits to a 50 pack year history of smoking, though he quit 5 years ago. Past medical history is significant for atrial fibrillation, hypertension, rheumatoid arthritis, and depression. Current medications include amiodarone, hydrochlorothiazide, and methotrexate.
* BP 135/85 mm Hg, P 83/min, RR 25/min, and Temp 98.6 F Chest examination reveals diffuse, dry, “Velcro-like” crackles two thirds of the way up the chest. Cardiac examination shows an elevated jugular venous pressure and a widely split S2. The extremities have 1+ pitting lower extremity edema and marked clubbing. A chest radiograph shows mild bibasilar interstitial reticular markings and some possible atelectasis.
* PFT’s show:
* FVC 2.31 L 52% of predicted
* FEV1 1.89 L 51% of predicted
* FEV1/FVC 0.81 98% of predicted
* Which of the following is the most appropriate next step in management?
* A. Perform a methacholine challenge test.
* B. Empiric trial of interferon and steroids
* C. Start him on high dose oral corticosteroids
* D. Stop amiodarone and methotrexate, follow pulmonary function tests
* E. Initiate bronchodilator therapy with albuterol and add an inhaled steroid
Case 3
## Footnote
* Which of the following is the most appropriate next step in management?
* A. Perform a methacholine challenge test.
* B. Empiric trial of interferon and steroids
* C. Start him on high dose oral corticosteroids
**_•D. Stop amiodarone and methotrexate, follow pulmonary function tests_**
•E. Initiate bronchodilator therapy with albuterol and add an inhaled steroid
115
Case 3
Amiodarone Lung
Case 3
* Amiodarone Lung
* Usually 2-4 months at doses greater than 400 mg/day
* Lipid laden foamy alveolar macrophages
* Organizing pneumonia
* 25% of cases. Mimics infectious pneumonia
* ARDS
* Post surgical. Diffuse alveolar damage with interstitial pneumonitis 1-4 days post intubation.
* Diffuse alveolar hemorrhage
* Rare. First few days to 6 months
* Solitary Pulmonary Mass
* rare
116
Case 4
## Footnote
* 50 year old male complains of increasing shortness of breath with exercise over the last year. Previously he has been healthy. He denies any fever, palpitations, hemoptysis, or weight loss. He denies any occupational exposure. He reports a dry cough. He does not take any medications and has no known drug allergies. He denies a smoking history. His oxygen saturation is 93% on room air. Lungs have a fine crackle pattern. Heart is regular. Examination of the extremities shows clubbing. Chest x-ray reveals diffuse linear opacities. Pulmonary function tests show a restrictive pattern. He has a decreased diffusion capacity.
* Which of the following is the most likely diagnosis?
* A. Acute interstitial pneumonia
* B. Asbestosis
* C. Idiopathic cardiomyopathy
* D. Idiopathic pulmonary fibrosis
* E. Sarcoidosis
Case 4
## Footnote
* Which of the following is the most likely diagnosis?
* A. Acute interstitial pneumonia
* B. Asbestosis
* C. Idiopathic cardiomyopathy
**_•D. Idiopathic pulmonary fibrosis_**
•E. Sarcoidosis
117
Case 5
## Footnote
* 27 year old female complains of feeling short of breath recently. Her exercise tolerance has dwindled and she must rest at the top of a flight of stairs. She had been previously healthy and had attributed the change to deconditioning. She denies fever, but has an occasional dry cough. She has no allergies and does not smoke or use illicit drugs. On review of systems, she sleeps well, is able to lie flat in bed, has not had any rashes and there has been no change in bowel habits or vison. Temp 98.6 F, BP 132/68 mm Hg, pulse 88/min, and RR 18/min. O2 Sat on room air is 98% resting and 92% after 5 minutes of exercise. Spirometry is normal.
* Physical examination reveals scattered crackles, but no wheezes in the lungs, normal cardiac rhythm, with no murmurs, no cyanosis, clubbing, or edema of the extremities. Chest x-ray reveals bilateral hilar lymph nodes. Purified protein derivative (PPD) is negative. Pulmonary function testing demonstrates a restrictive ventilatory defect.
Which of the following is the next most appropriate step?
1) Begin empiric antimicrobial therapy with a macrolide antibiotic
2) Obtain a transbronchial lymph node biopsy
3) Obtain a Quantiferron Gold test
4) Start her on inhaled corticosteroids
Case 5
## Footnote
Which of the following is the next most appropriate step?
1) Begin empiric antimicrobial therapy with a macrolide antibiotic
**_2) Obtain a transbronchial lymph node biopsy_**
3) Obtain a Quantiferron Gold test
4) Start her on inhaled corticosteroids
118
Case 6
## Footnote
* 61 year old female with 3 day history of progressive shortness of breath, nonproductive cough, and fever. At present, the patient is able to ambulate 15 feet before becoming dyspneic. Prior she was functional and walked 2 miles in the evening after dinner with her husband. Additionally, she report one episode of hemoptysis the evening before this presentation. The hemoptysis was approximately 3 ml and maroon. She has had no prior episodes of hemoptysis and denies any lightheadedness. She has a history of SLE, and 5 years ago was diagnosed with cerebritis and lupus nephritis by renal biopsy
* The patient is on prednisone and trimethoprim/sulfamethoxazole.
* Her vital signs are: Temp 100.4F, BP 151/87 mm Hg, pulse 98/min, RR 16/min and oxygen sat of 91% on 6L O2 by nasal cannula. Chest x-ray reveals diffuse infiltrates bilaterally.
If pulmonary function tests reveal an increased DLCO in this patient, which of the following is the most likely diagnosis?
A. ARDS
B. Diffuse alveolar hemorrhage
C. Pneumonia
D. Pulmonary edema
E. Pulmonary embolism
Case 6
## Footnote
If pulmonary function tests reveal an increased DLCO in this patient, which of the following is the most likely diagnosis?
A. ARDS
**_B. Diffuse alveolar hemorrhage - also polycythemia_**
C. Pneumonia
D. Pulmonary edema
E. Pulmonary embolism
119
Case 7
## Footnote
•30 year old male is brought to the ER after a syncopal episode. He has not had episodes in the past. He has no fever, chills, shakes, nausea, or vomiting. He denies chest pain or palpitations. He takes no medications and has no allergies. He denies drug use. Vital signs are stable. Cardiac examination is unremarkable. Rhythm strip reveals nonsustained ventricular tachycardia. Laboratory studies are normal. Chest x-ray reveals bilateral hilar adenopathy. He has a history of a positive skin test for anergy. His serum calcium is elevated. Echocardiogram reveals segmental wall motion abnormality. He undergoes a biopsy of the myocardium and you are awaiting the results.
You correctly ascertain that upon receiving the biopsy results you will?
A. Observe at home with a holter monitor
B. Begin treatment for a disorder associated with noncaseating granulomas
C. Administer a PPD test
D. Perform a DLCO test
E. Begin therapy with antifungals for treating histoplasmosis
Case 7
## Footnote
You correctly ascertain that upon receiving the biopsy results you will?
A. Observe at home with a holter monitor
**_B. Begin treatment for a disorder associated with noncaseating granulomas_**
C. Administer a PPD test
D. Perform a DLCO test
E. Begin therapy with antifungals for treating histoplasmosis
120
Case 9
## Footnote
* 30 year old male with a 15 pack year history of smoking. Three weeks ago he developed a URI, treated with Azithromycin.
* Three days ago he developed marked SOB and a cough associated with marked hemoptysis. Examination reveals a BP of 160/95 and a RR of 20. Heart exam reveals a sinus tachycardia. His lips are cyanotic. He has 2+ pretibial edema. His BUN is 60 and Creatinine is 4.0. Urinalysis demonstrates microscopic hematuria.
Which of the following would help confirm the most likely diagnosis?
A. DLCO
B. Anti Glomerular Basement Membrane Antibody ( Anti-GBM)
C. C-ANCA
D. Alpha-1-Antitrypsin
E. Kveim test, Serum Calcium, Serum ACE levels
Case 9
## Footnote
Which of the following would help confirm the most likely diagnosis?
A. DLCO
**_B. Anti Glomerular Basement Membrane Antibody ( Anti-GBM)_**
C. C-ANCA
D. Alpha-1-Antitrypsin
E. Kveim test, Serum Calcium, Serum ACE levels
121
Case 10
## Footnote
* 37 year old African American man is seen with raised red lesions on the anterior aspect of both legs. He has difficulty breathing, especially while walking at a fast pace, associated with a dry cough for the last 2-3 months. He denies fever, night sweats, weight loss, or any other symptoms. He has never smoked. BP 135/80 mm Hg, Pulse 52/min, RR 14/min. He is afebrile. Auscultation of his chest reveals discreet dry rales bilaterally with no wheezing. Heart auscultation reveals an irregular rhythm. Examination of his lower extremities shows raised red-purplish lesions on both anterior aspects of both legs. His PaO2 is 97 mm Hg at rest on room and becomes 94 mm Hg with exercise
* An EKG shows episodes of second-degree AV block.
* A chest x-ray reveals a diffuse bilateral reticulonodular pattern and bilateral hilar adenopathy. A bronchoscopy with transbronchial biopsy reveals noncaseating granulomas A PFT shows a decreased TLC and RV with an FEV1/FVC of 95%of predicted. Laboratory studies show minimally elevated calcium and ACE levels. Other lab studies are normal.
* After you review this patient’s clinical presentation and findings, which of the following is the most appropriate treatment plan at this time?
* A. Begin therapy with high-dose systemic corticosteroids
* B. Follow ACE levels, Chest x-rays, and O2 sats, and employ “watchful waiting”
* C. Place a transvenous pacemaker
* D. Start treatment for TB
* E. Start an appropriate antibiotic regimen accepted for this condition
Case 10
## Footnote
•After you review this patient’s clinical presentation and findings, which of the following is the most appropriate treatment plan at this time?
**_•A. Begin therapy with high-dose systemic corticosteroids_**
* B. Follow ACE levels, Chest x-rays, and O2 sats, and employ “watchful waiting”
* C. Place a transvenous pacemaker
* D. Start treatment for TB
* E. Start an appropriate antibiotic regimen accepted for this condition
122
Case 11
## Footnote
* 75 year old male comes to the office complaining of severe dyspnea that has been ongoing for the past 1 year. He denies a cough, chest pain, hemoptysis, or weight loss. He has a significant smoking history of two pack per day for the past 50 years. He has no other medial issues. He has no allergies and takes no medications
* He worked as a stone engraver until 10 years ago when he retired. His vital signs are stable. Pulmonary examination reveals end expiratory crackles bilaterally. He has clubbing. His chest x-ray reveals multiple sub centimeter nodules and eggshell calcifications of hilar lymph nodes.
Which of the following is the most likely diagnosis
A. Adenocarcinoma
B. Asbestosis
C. Idiopathic pulmonary fibrosis
D. Silicosis
E. Tuberculosis
Case 11
## Footnote
Which of the following is the most likely diagnosis
A. Adenocarcinoma
B. Asbestosis
C. Idiopathic pulmonary fibrosis
**_D. Silicosis_**
E. Tuberculosis
123
Case 12
## Footnote
* 50 year old female presents with acute dyspnea. Her symptoms began with dry cough, shortness of breath, malaise, and fever seen days earlier. She is brought to the ER in acute hypoxemic ventilatory failure. pH 7.30, PaCO2 65, PaO2 55 on 100% FiO2 intubated and on ventilator.
* Her chest x-ray demonstrates diffuse alveolar infiltrates and air space consolidation suggestive of ARDS.
* A CT scan reveals bilateral air apace consolidation with areas of ground glass opacities with little honeycombing. Septal thickening and subpleural distribution of the opacities is noted.
* A lung biopsy reveals diffuse alveolar damage.
* She is treated with mechanical ventilation, steroids, and antibiotics. She dies two days later.
Which of the following is the most likely diagnosis?
A. Acute interstitial pneumonia
B. Asbestosis
C. Idiopathic cardiomyopathy
D. Idiopathic pulmonary fibrosis
E. Sarcoidosis
Case 12
## Footnote
Which of the following is the most likely diagnosis?
**_A. Acute interstitial pneumonia_**
B. Asbestosis
C. Idiopathic cardiomyopathy
D. Idiopathic pulmonary fibrosis
E. Sarcoidosis
124
Case 14
## Footnote
* 49 year old man has a chest x-ray as part of a pre-op workup before gastric bypass surgery. The surgeon who reviews the film is concerned because there appear to be small nodules in the lateral aspects of the left lung field. A high resolution CT scan is ordered, which further defines these nodules.
* After review by the radiologist, the opacities seen on chest radiograph are felt to be pleural-based. At least eight focal areas of pleural plaques are visible, as well as areas of diffuse pleural thickening, subpleural linear densities, and areas of basilar fibrosis. Although the patient states that he has no respiratory complaints and he never smokes, a set of pulmonary function tests reveal a borderline restrictive pattern and a diminished diffusion capacity of the lung for carbon monoxide (DLCO)
Which of the following aspects of this patient’s history is most likely to explain these abnormal findings?
A. Dietary/nutritional review
B. Family medical history
C. Occupational history
D. Review of symptoms
E. Travel history
Case 14
## Footnote
Which of the following aspects of this patient’s history is most likely to explain these abnormal findings?
A. Dietary/nutritional review
B. Family medical history
**_C. Occupational history_**
D. Review of symptoms
E. Travel history
125
Case 15
## Footnote
•37 year old male presents with 2-3 weeks of increasing dyspnea, occasional hemoptysis, sinusitis, and on episode of epistaxis. Renal function is normal. His UA reveals 10-15 RBCs and 2 WBCs. His chest x-ray demonstrates bilateral nodular infiltrates. One is cavitary.
Which of the following is true?
A. His diagnosis is most likely Tuberculosis
B. His diagnosis is most likely Goodpastures syndrome
C. He will have a positive test for c-ANCA
D. He will have an elevated ACE level
E. He will soon develop congestive heart failure
Case 15
## Footnote
Which of the following is true?
A. His diagnosis is most likely Tuberculosis
B. His diagnosis is most likely Goodpastures syndrome
**_C. He will have a positive test for c-ANCA_**
D. He will have an elevated ACE level
E. He will soon develop congestive heart failure
126
Case 17
## Footnote
* 27 year old female presents to the ER with cough, fever, dyspnea, weight loss, malaise, and night sweats for the past week. She complains of recent onset of wheezing during this time as well. Her CBC demonstrates a marked eosinophilia with over 35% eosinophils. Her chest x-ray demonstrates peripheral, nonsegmental alveolar infiltrates and possible non-cavitating lung lesions. You suspect asthma and a superimposed infection. You treat her with albuterol by inhalation and large doses of oral corticosteroids. The infiltrates on the chest x-ray resolve in 2 days and she becomes asymptomatic.
* She is sent home and returns to the ER in three weeks with the same symptoms. She has also now developed symptoms of diarrhea and abdominal pain with diarrhea.
Your correct diagnosis is?
A. Asthma with associated mucus plugs and atelectasis
B. Bronchiectasis
C. Chronic eosinophilic pneumonia
D. Churg – Strauss Syndrome
E. Goodpasture’s variant
Case 17
## Footnote
Your correct diagnosis is?
A. Asthma with associated mucus plugs and atelectasis
B. Bronchiectasis
C. Chronic eosinophilic pneumonia
**_D. Churg – Strauss Syndrome - assoc w/naussea and diarrhea_**
E. Goodpasture’s variant
127
Case 18
## Footnote
•Patient is a 50 year old female that presents with increased difficulty breathing, cough, and exertional dyspnea. She is a non-smoking housewife. She is married. Her husband is a wood worker and fixes / remodels old buildings and warehouses for a living. Physical exam demonstrates bibasilar fine crackles on auscultation along with the presence of clubbing. Chest x-ray shows basilar opacities and bilateral calcified pleural plaques.
Based upon the history and these findings, you expect to diagnose?
A. Idiopathic pulmonary fibrosis
B. Asbestosis
C. BOOP
D. Kaplan’s syndrome
E. Collagen vascular disease of the lung
Case 18
## Footnote
Based upon the history and these findings, you expect to diagnose?
A. Idiopathic pulmonary fibrosis
**_B. Asbestosis_**
C. BOOP
D. Kaplan’s syndrome
E. Collagen vascular disease of the lung
128
Case 1
## Footnote
•65 year old male that has been retired for the past four years. He is sent to you for evaluation because of an abnormal chest x-ray. As you review the x-ray you note that there are rounded opacities, 1-5 mm in size in the upper lung zones. Egg-shell calcifications are noted in the region of the hilar and mediastinal lymph nodes. He tells you that he has been having increasing SOB for the last 2 years. He is thin and using accessory muscles of respiration. BP is 140/80, Pulse 90 and regular, Lung sounds exhibit decreased flow and some fine crackles at the end of expiration.
Based upon your presumptive diagnosis, you correctly
A. Apply a PPD intermediate skin test
B. Place this patient on a Beta 2 agonist
C. Place this patient on inhaled corticosteroids
D. Start him on chemotherapy
E. Order an echocardiogram
Case 1
## Footnote
Based upon your presumptive diagnosis, you correctly
**_A. Apply a PPD intermediate skin test_**
B. Place this patient on a Beta 2 agonist
C. Place this patient on inhaled corticosteroids
D. Start him on chemotherapy
E. Order an echocardiogram
129
Case 2
## Footnote
•65 year old male who retired to Pueblo, CO after working the mines of West Virginia for twenty-five years. Over the years he has had increasing difficulty with shortness of breath. He can no longer walk up the stairs to his bedroom, and now sleeps on the living room sofa. Physical exam reveals right-sided heart failure with 2+ pretibial edema, a prolonged expiratory phase, and diffuse wheezing.
The underlying pathology in this patient is:
A. Localized fibrous plaques or, rarely, diffuse pleural fibrosis
B. Coalescence of particle containing macules that form discrete areas of interstitial fibrosis causing distention of the respiratory bronchioles, forming focal areas of emphysema.
C. The development of noncaseating granuloma formation.
D. Fibrosis secondary to the effects of anti-GBM antibody
E. The development of chronic mucopurulent infections causing dilation of the respiratory bronchioles.
Case 2
The underlying pathology in this patient is:
A. Localized fibrous plaques or, rarely, diffuse pleural fibrosis
**_B. Coalescence of particle containing macules that form discrete areas of interstitial fibrosis causing distention of the respiratory bronchioles, forming focal areas of emphysema._**
C. The development of noncaseating granuloma formation.
D. Fibrosis secondary to the effects of anti-GBM antibody
E. The development of chronic mucopurulent infections causing dilation of the respiratory bronchioles.
130
Case 3
## Footnote
•35 year old coal miner that has a history of rheumatoid arthritis. He is being treated with Methotrexate, gold injections, and Aspirin. Your are asked to consult based upon an abnormal chest x-ray that demonstrates multiple calcified pulmonary nodules throughout the lung fields bilaterally. The presence of a pleural effusion is also noted in the left lower lung field.
You correctly diagnose:
A. Silicosis
B. Caplan’s syndrome
C. Sarcoidosis
D. Miliary Tuberculosis
E. Coal Workers Pneumoconiosis
Case 3
You correctly diagnose:
A. Silicosis
**_B. Caplan’s syndrome_**
C. Sarcoidosis
D. Miliary Tuberculosis
E. Coal Workers Pneumoconiosis
131
Case 4
## Footnote
* 47 year old roofer from Malawi who is seeing you because of increased shortness of breath of increasing duration. He smokes 1 pack of “Lucky Strike” cigarettes daily and has done so for the last thirty years. He has been in the US for the last two years and now works in the roofing industry here in Colorado. He has a chronic non productive cough. His chest x-ray demonstrates small, irregular, shadows in lower lung zones along with thickened pleural calcified plaques present under diaphragms and lateral chest wall.
* Physical exam reveals fine end respiratory crackles heard more predominantly in the lung bases bilaterally and digital clubbing is noted.
* Pulmonary function testing shows a decreased vital capacity, decreased total lung capacity, and a decreased DLCO.
You expect to find which of the following on lung biopsy?
A. Caseating granulomas
B. Discrete pale nodules in the upper zones of the lungs that have coalesced into hard, collagenous scars.
C. Golden brown, fusiform rods with a translucent center consisting of particulate fibers coated with an iron-containing proteinaceous material
D. A lymphocytic alveolitis leading to pulmonary fibrosis
E. Lymphocytes, plasma cells, and macrophage aggregates resulting in interstitial fibrosis and obliterative bronchiolitis
Case 4
## Footnote
You expect to find which of the following on lung biopsy?
A. Caseating granulomas
B. Discrete pale nodules in the upper zones of the lungs that have coalesced into hard, collagenous scars.
**_C. Golden brown, fusiform rods with a translucent center consisting of particulate fibers coated with an iron-containing proteinaceous material_**
D. A lymphocytic alveolitis leading to pulmonary fibrosis
E. Lymphocytes, plasma cells, and macrophage aggregates resulting in interstitial fibrosis and obliterative bronchiolitis
132
Case 5
## Footnote
* 64 year old male with a chief complaint of increasing SOB, a 20 lb weight loss over the last three months, and non pleuritic chest pain.
* On examination he has dullness to percussion on the right lung base and bilaterally diminished breath sounds that is more pronounced on the right. He has positive JVD, distant heart sounds, and a pulsus paradoxus of 18 mm. There is low voltage on the EKG.
* Chest x-ray reveals a nodular, irregular pleural thickening noted on the right side with a right sided pleural effusion and a pericardial effusion with a greatly enlarged heart. He has never smoked. He immigrated to the United States from Ireland where he was employed as a ship builder and “shipyard worker for 50 years. He has helped build battle ships, cruise ships, and heavy freighters.
* You perform a thoracentesis to retrieve pleural fluid for diagnostic purposes, expecting to find:
* A. Pleural fluid that stains positive for Acid Fast Bacilli.
* B. Pleural fluid analysis with an uncharacteristically low random sugar.
* C. Pleural fluid analysis compatible with a transudate as seen in CHF
* D. Pleural fluid analysis with beryllium residue
* E. Pleural fluid analysis with cells compatible with malignant mesothelioma
Case 5
## Footnote
* You perform a thoracentesis to retrieve pleural fluid for diagnostic purposes, expecting to find:
* A. Pleural fluid that stains positive for Acid Fast Bacilli.
* B. Pleural fluid analysis with an uncharacteristically low random sugar.
* C. Pleural fluid analysis compatible with a transudate as seen in CHF
* D. Pleural fluid analysis with beryllium residue
**_•E. Pleural fluid analysis with cells compatible with malignant mesothelioma_**
133
Case 5
Light Criteria for Exudate
Case 5
## Footnote
Light Criteria for Exudate:
1. Pleural fluid protein/serum protein \> 0.5
2. Pleural fluid LDH/Serum LDH \> 0.6
3. Pleural fluid LDH more than two-thirds normal upper limit for serum
134
Case 6
## Footnote
•35 year old male who has a complaint of progressive dyspnea on exertion, anorexia, night sweats, and a 10 lb weight loss over the last five months. He is employed as a nuclear technician at a power plant in Louisiana. Because of hurricane Katrina, he evacuated La, and is now in Fort Worth. On examination, he has mildly decreased breath sounds bilaterally. The remainder of the examination is normal. Chest x-ray examination reveals bilateral fibrosis with marked bilateral hilar adenopathy. He was previously diagnosed as having sarcoidosis. You review his chart and note that he has positive skin tests to mumps and trichophyton, a normal serum ACE level, and a normal serum calcium.
Because you are a “sharp clinician” you correctly:
A. Perform a pleural biopsy looking for asbestosis
B. Perform a BAL for a beryllium lymphocyte proliferation test
C. Perform a bronchoscopy looking for a non-caseating granuloma.
D. Perform a BAL looking for ACE levels in the aspirate
E. Perform a parotid gland biopsy for Heerfordt’s syndrome
Case 6
Because you are a “sharp clinician” you correctly:
A. Perform a pleural biopsy looking for asbestosis
**_B. Perform a BAL for a beryllium lymphocyte proliferation test_**
C. Perform a bronchoscopy looking for a non-caseating granuloma.
D. Perform a BAL looking for ACE levels in the aspirate
E. Perform a parotid gland biopsy for Heerfordt’s syndrome
135
Case 6
Heerfordt’s disease
Case 6
## Footnote
Heerfordt’s disease
* A variant of Sarcoidosis
* Characterized by nonsuppurative parotitis, uveitis, mild fever, and facial nerve paralysis
136
Case 9
## Footnote
* 25 year old male who has recently immigrated from Sri Lanka. He complains of dry cough, malaise, episodic fevers, and intermittent wheezing. These symptoms are worse at night.
* He has lost 25 pounds over the last 6 months.
* On his CBC he has a marked eosinophilia with an absolute eosinophil count that is 2000 eosinophils/ml. His IGE level is markedly elevated. His Chest x-ray shows a diffuse infiltrate with multiple nodules. His sedimentation rate is 40. Charcot-Leyden crystals are seen in his sputum specimen.
Based upon your suspected diagnosis, you correctly treat him with?
A. Corticosteroids
B. Albuterol
C. Diethylcarbamazine
D. Azithromycin
E. Isoniazid
Case 9
## Footnote
Based upon your suspected diagnosis, you correctly treat him with?
A. Corticosteroids
B. Albuterol
**_C. Diethylcarbamazine_**
D. Azithromycin
E. Isoniazid
Pt has:
* Wuchereria bancrofti which causes
* Elephantiasis
137
Case 10
## Footnote
•14 year old female with cough and wheezing, transient pulmonary infiltrates, and blood eosinophilia. When she has the episodes of wheezing her eosinophil count is markedly elevated and she runs temperatures as high as 100F. Her fever and episodes of wheezing resolve in 3-4 days and she then becomes asymptomatic. Her WBC count is 15,000 during and attack with 40% eosinophils.
With this most recent episode, she also presents with abdominal pain, nausea, vomiting, and x-ray evidence of an acute GI obstruction. You correctly diagnose?
A. Churg-Strauss Syndrome
B. Loeffler’s Syndrome
C. Allergic aspergillosis
D. BOOP
E. Sarcoidosis Pulmonary Variant
Case 10
## Footnote
With this most recent episode, she also presents with abdominal pain, nausea, vomiting, and x-ray evidence of an acute GI obstruction. You correctly diagnose?
A. Churg-Strauss Syndrome
**_B. Loeffler’s Syndrome - hook worm_**
C. Allergic aspergillosis
D. BOOP
E. Sarcoidosis Pulmonary Variant
138
Case 12
## Footnote
•38 year old female who was diagnosed and treated for non-metastatic breast cancer 16 months ago. Her treatment consisted of a right mastectomy that went well and radiation therapy that ended 12 months ago. She now complains of a low-grade fever, night sweats, dyspnea on exertion, increased sputum production, and occasional cough. Her O2 sat level is 92%.
You correctly expect that?
A. She will have had chronic remodeling of her lung tissue resulting in pulmonary fibrosis
B. She now demonstrates lymphangitic spread of her primary tumor.
C. She now has an opportunistic infection secondary to her altered state of immunity
D. She will develop bronchiectasis
E. She will require bronchodilator therapy.
Case 12
## Footnote
You correctly expect that?
**_A. She will have had chronic remodeling of her lung tissue resulting in pulmonary fibrosis_**
B. She now demonstrates lymphangitic spread of her primary tumor.
C. She now has an opportunistic infection secondary to her altered state of immunity
D. She will develop bronchiectasis
E. She will require bronchodilator therapy.
139
Case 16
## Footnote
* 50 year old male with a history of sinusitis, allergies, and severe persistent asthma for the past ten years. He is on SABA’s, LABA, inhaled steroids, cromolyn, and the leukotriene inhibitor, singulair.
* He now presents with marked respiratory difficulty, a neuritic pain in his legs, arms, and hands. His skin demonstrates erythematous maculopapules resembling erythema multiforme. He has marked abdominal pain with rebound on exam. He also reports decreased urination.
* His chest x-ray demonstrates marked right sided infiltrates, bilateral patchy infiltrates, and areas with diffuse nodular infiltrates. His eosinophil count is also markedly elevated with 25% of his cells being eosinophils. p-ANCA levels are elevated. His IgE level is elevated. His BUN and creatinine are elevated.
Which of the following is the most likely diagnosis?
A. Wegener’s granulomatosis
B. Churg-Strauss syndrome
C. Desquamative interstitial fibrosis
D. Sarcoidosis – Stage III
E. Loeffler’s Syndrome
Case 16
## Footnote
Which of the following is the most likely diagnosis?
A. Wegener’s granulomatosis
**_B. Churg-Strauss syndrome_**
C. Desquamative interstitial fibrosis
D. Sarcoidosis – Stage III
E. Loeffler’s Syndrome
140
Case 19
## Footnote
•27 year old ranch hand brought in with the onset of severe respiratory distress. He is one of the three patients brought into the ER with similar symptoms. He was working at the farm doing generalized cleaning, and had this sudden onset.
Case 19
## Footnote
You correctly diagnose?
A. Farmer’s lung secondary to exposure to Thermophilic Actinomycetes
B. RADS (Reaactive Airways Dysfunction Syndrome)
**_C. Silo Filler’s Disease - Nitrogen Dioxide_**
D. Strep Pneumonia
