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106 Cards in this Set
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Physiological hallmark of restrictive lung diseases
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PROPORTIONAL decrease in lung volumes
TLC, FVC and FEV1 are all reduced. FEV1:FVC is NORMAL due to proportional effect on lung volumes |
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Most common type of restrictive disease
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Interstitial Lung Diseases
-Fibrotic -Inflammatory |
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FEV1:FVC in restrictive disease
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NORMAL
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Compliance in restrictive disease
& physiologic consequence |
Compliance is reduced
Causes increased elastic work of breathing |
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Breathing pattern in restrictive disease
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Rapid, shallow breathing mitigates increased work of breathing
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Significance of DLCO-adj in differentiating cause of restrictive lung disease
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If cause is interstitial, DLCO-adj will decrease. If cause is extrinsic to the lung (eg, chest wall disorder) DLCO-adj will not be affected.
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6 categories of interstitial lung diseases
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1. Collagen Vascular Disease
2. Granulomatous Disease 3. Idiopathic Interstitial Pneumonia 4. Inhaled cause 5. Inherited disease 6. Other ("specific entities") |
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Alveolar causes of restrictive physiology
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Pulmonary edema / ARDS -
alveolar filling decreases compliance |
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4 categories of extraparenchymal causes of restrictive disease
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1. Nueromuscular
2. Thoracic cage abnormality - ankylosing spondylitis - scoliosis 3. Soft tissue disease - obesity - myxedema 4. Space occupying lesion - pleural effusion - tumor |
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4 Collagen vascular diseases causing restrictive disease
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1. Rheumatoid Arthritis
2. Systemic Lupus Erythematosus 3. Scleroderma 4. Dermatomyositis Polymyositis |
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3 Granulomatous diseases causing restrictive disease
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1. Sarcoidosis
2. Pulmonary Langerhaans Cell Histiocytosis (Eosinophilic Granuloma) 3. Hypersensitivity Pneumonitis |
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2 types of Idiopathic Interstitial Pneumonia
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1. Idiopathic Pulmonary Fibrosis
2. Non-IPF (5 kinds) |
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2 inherited restrictive diseases
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1. Familial IPF
2. Tuberous Sclerosis |
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7 "other" types of restrictive disease
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1. Iatorgenic
2. Eosinophilic Pneumonia 3. Lymphangioleiomeiomatosis (LAM) 4. Veno-occlusive disease 5. Alveolar Proteinosis 6. Neoplasm 7. Pulmonary Capillaritis |
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% of pulmonary fibrosis with no known cause
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70%
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Patient profile & risk factors for Idiopathic Pulmonary Fibrosis
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Age 50-70
Male > Female Risks: Smoking Environmental exposure Infection Chronic aspiration Family History |
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Name of IPF histological Pattern
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Usual Interstitial Pneumonia
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4 Histopathological features of Usual Interstitial Pneumonia
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1. Fibroblastic foci in various stages of development
2. Excessive collagen deposition 3. ECM overgrowth 4. Cystic "honeycombing in a peripheral and subpleural distribution |
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Specificity / diagnostic usefulness of Usual Interstitial Pneumonia findings
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Not very specific; found in many interstitial lung diseases, including IPF
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Symptoms of Idiopathic Pulmonary Fibrosis
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-Insidious onset
-Progress over time -Most common = exertional dyspnea Other Sx: Dry cough Fatigue Arthralgias |
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3 Signs of Idiopathic Pulmonary Fibrosis
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1. Fine velcro-like end-inspiratory crackles at the lung bases
2. Digital clubbing 3. Cyanosis due to severe hypoxemia (advanced disease) |
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Lung volume measurements in Idiopathic Pulmonary Fibrosis
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All proportionally reduced; FEV1/FVC is normal or marginally high
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DLCO in Idiopathic Pulmonary Fibrosis
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Diminished, due to damage to gas exchange membranes
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Resting ABG in Idiopathic Pulmonary Fibrosis - 3 findings
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1. Normal or Low PaCO2 (pt may hyperventilate)
2. Increased P(Aa)O2 3. Hypoxemia at rest (advnaced disease) |
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2 Changes with exercise in Idiopathic Pulmonary Fibrosis
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1. P(Aa)O2 further increases
2. High physiologic dead space |
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CXR findings in Idiopathic Pulmonary Fibrosis
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1. Low Lung Volume
2. Reticular or net-like opacities ("interstitial" pattern), mainly in bases **Early stage - pt may have normal CXR despite DOE or abn biopsy |
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3 HiRes CT findings in Idiopathic Pulmonary Fibrosis
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1. Cystic honeycombing
2. Reticular opacities 3. Traction Bronchiectasis **All typically seen in subpleural and basilar regions |
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Gold standard imaging modality to asses extent and nature of restrictive lung disease
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Hi-Res Thin-Section CT
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Diagnosis of Idiopathic Pulmonary Fibrosis
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1. Pt has characteristic Sx&S's
2. PFT shows restrictive disease 3. CXR, HR-CT and lung biopsy to confirm Dx ***Must EXCLUDE infectious, environmental or systemic disease, as well as iatrogenic / drug-induced disease. |
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Prognosis for Idiopathic Pulmonary Fibrosis
-Survival -Cause of death |
Very poor:
Median survival = 3 years after Sx onset Cause of death: -respiratory failure -right heart failure (severe hypercapneia & Cor Pulmonale) |
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Goal of Tx in Idiopathic Pulmonary Fibrosis
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--> Suppress fibrotic process of the disease
There is NO effective treatment |
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Medications used in Idiopathic Pulmonary Fibrosis
- 2 classes - 3 drugs |
Immunomodulators:
-Prednisone -Azathioprine Antioxidants: -Acetylcysteine |
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Surgical Tx for Idiopathic Pulmonary Fibrosis
-Procedure -Pt profile -Outcomes |
May attempt uni- or bi-lateral lung transplant
-Younger patient Outcomes are GOOD - unlike RxTx, transplant does improve overall survival. Pursue this option at time of Dx in younger otherwise healthy pts. |
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Sarcoidosis: Histopathological Hallmark
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Granulomatous Inflammation, especially in pulmonary parenchyma and pulmonary lymph nodes
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Patient profile & risk factors in Sarcoidosis
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Young African American woman living in temperate or cold region
Age 25-35 Women > Men 4x as common in African Americans, but RARE in African blacks & other ethnicities Less common in tropical region |
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Extrapulmonary involvement in Sarcoidsosis
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90% have pulmonary and thoracic lymph node involvment; can involve heart, CNS, liver, spleen, skin, eyes, parotids.
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Cellular milieu and pathology of Sarcoidosis
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Etiology is unknown
Macrophages & T-cells contribute to inflammatory granuloma formation: 1. Recruitment of other monocytes 2. Fibroblast proliferation 3. Induce T-cell replication |
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Biopsy role in Dx of Sarcoidosis
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Non-Caseating Granulomas - found in many diseases. Can't definitively Dx Sarcoidosis by their presence alone
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Causes of Non-caseating granulomas
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1. Mycobacterial or fungal disesae
2. Foreign Body response 3. Lymphoma or regional drainage of malignancy 4. Occupational exposure - eg, Berrylium. 5. Sarcoidosis |
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Granuloma progression in sarcoidosis
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Granuloma evolves into fibrotic scar. Inflammatory cells in granuloma infiltrate alveolar septae.
--> Diffuse pulmonary fibrosis may be the predominant finding. |
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Presenting Sx of Sarcoidosis
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50% are aSx
Sx are non-specific: Fatigue, arthralgias, malaise, weight loss May present with Lofgren Syndrome |
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Clinical exam findings in sarcoidosis
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EXAM: usually normal
May have tachypnea, crackles, rarely wheezing (indicates bronchial involvement). Generalized lymphadenopathy, hepatosplenomegaly, arthritis (signs of systemic inflammation) 25% have skin lesions, eye involvement. Rarely may have arrythmia/conduction block or CNS, hepatic or renal abnormality May have Lofgren Syndrome |
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Lofgren Syndrome
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Bilateral hilar adenopathy on CXR
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Pulmonary function in Sarcoidosis
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VARIABLE
-DLCO is usually reduced; may be only abn in early disease. -Restrictive pattern frequent -Advanced disease may have airway involvement or airflow obstruction ABG may show normal or increased P(Aa)O2 |
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Classic CXR finding in Stage I Sarcoid disease
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Symmetrical bilateral hilar adenopathy (Lofgren Syndrome) without evidence of pulmonary parenchymal involvement.
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DDX for CXR suggesting Stage I Sarcoidosis (4)
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1. TB
2. Fungal infection 3. Lymphoma 4. Metastatic carcinoma ***All of these have associated systemic Sx similar to sarcoidosis |
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CXR characteristic of stage II Sarcoidosis
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Bilateral hilar adenopathy
Diffuse symmetric pulmonary involvement with parenchymal lesions -Ranges from reticulations to nodular pattern |
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CXR characteristic of stage III sarcoidosis
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Diffuse pulmonary involvement
NO hilar adenopathy |
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CXR characteristic of stage IV sarcoidosis
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Extensive scarring and honeycombing
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Diagnostic significance of CXR findings of fibrosis suggestive of sarcoidosis
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NOT diagnositc alone; fibrosis associated with sarcoidosis is indistinguishable from other etiologies of fibrosis
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Diagnosis of sarcoidosis
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Clinical picture - S&Sx
Radiographic findings- hilar adenopathy or diffuse nodular reticulation Biopsy - non-caseating granulomas (necessary for Dx) May use bronchoscopy / bronchoalveolar lavage |
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Prognosis for Sarcoidosis
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Resolves spontaneously in 90% of Stage I cases (only hilar lymphadenopathy)
May have some residual Sx Satge II or III aSx does not need Tx |
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Tx of Sarcoidosis
-When to initiate tx -Specific tx |
Worsening fxnl status or extrapulmonary involvement --> Start Tx
Mainstay = corticosteroids May also use Methotrexate or Imatinib |
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Cause of hypersensitivity Pnuemonitis
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1. Exposure to organic dust particles of specific size
2. Adverse effect of Rx, eg Methotrexate |
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Reversibility of Hypersensitivity Pneumonitis
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-Usually reversible
-Lasting damage under what circumstances? (side 3) |
Long duration
large amount of exposure high frequency exposure type of inhaled dust vigor and nature of immune response |
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What determines whether pt will develop hypersensitivity pneumonitis on exposure to dust?
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1. Genetics
2. Environmental / host factors --Most ppl exposed to the specific type of dust DO develop Ab response but DO NOT develop hypersensitivity pneumonitis reaction |
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Most important hypersensitivity pneumonitis triggers
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1. Thermophilic actinomycetes
2. Bird droppings 3. Aerosols / chemicals in plastics industry |
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Pathogenic mechanism of hypersensitivity pneumonitis
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Immunologic; specific mechanism has not been identified.
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Farmer's Lung:
-Type of dust inhaled -Specific antigens |
Caused by moldy hay.
Ag's are: -Micropolyspora Faeni -Thermoactinomyces vulgaris |
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Pneumonitis due to contaminated air conditioning / heating system
-Source of dust inhaled -Specific antigens |
Caused by dust inhaled from furnace or air conditioner.
Ag's are: -Micropolyspora Faeni -Thermoactinomyces vulgaris |
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Sequoiosis
-Type of dust -Specific antigens |
Caused by inhaling Redwood dust.
Specific antigen is Graphium-species bacteria |
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Bird fancier's lung: Cause?
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Exposure to endogenous proteins in bird droppings
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Cheese washer's lung: Type of dust and specific antigen
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Caused by inhaling cheese particles. Speicifc antigen is Penicillium casei
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Pathologic hallmark of hypersensitivity pneumonitis & typical histopathological findings
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Granulomatous interstitial pneumonia consisting if lymphocytes, foamy macrophages, and plasma cells
Prominent, patchy interstitial infiltrate beginning near terminal bronchioles, may extend widely into parenchyma. Most patients have intra-alveolar infiltrate. Non-caseating granulomas may form in different distribution than sarcoidosis. |
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Clinical timing of acute hypersensitivity pneumonitis
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-Most common presentation
-Occurs 4-8 hours after exposure, prsists for several hours and usually spontaneously remits after removal of offending antigen. |
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Chronic hypersensitivity pneumonitis: Cause, progression and relation to presence of antigen
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-Caused by long-duration / repeated exposure to the antigen. Shows irreversible pulmonary fibrosis.
May continue to progress even after removal of offending antigen. |
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Symptoms of acute hypersensitivity pneumonitis
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Cough, dyspnea, malaise
**Fever/chills, myalgias --> Easy to mistake for bacterial infection |
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Physical exam findings of acute hypersensitivity pneumonitis
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Acutely ill, febrile, dyspneic patient
Bibasilar crackles, which persist until episode subsides May develop pleural effusion |
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Lab findings in acute hypersensitivity pneumonitis
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Leukocytes (rarely, includes eosiniphils)
Elevated Ig levels **60% are positive for Rheumatoid Factor Serum values normailze after episode |
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Symptoms & signs of chronic hypersensitivity pneumonitis
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Symptoms:
Progressive dyspnea, chronic cough, malaise, weight loss. Signs: Crackles on lungs exam May have clubbing |
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Pulmonary Function Tests typical of acute/chronic hypersensitivity pneumonitis
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**Both forms show similar results.
-Consistent with restrictive lung disease -May also show obstructive component DLCO is decreased due to interstitial inflammation ***Hypoxemia is usually mild if present at all in acute presentation, but is very common in chronic form, even at rest. |
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Chest imaging in acute hypersensivity pneumonitis
1. CXR 2. Hi-Res CT |
CXR:
Patchy, ill-defined parenchymal densities May have pleural effusion 2. Hi-Res CT: Ground-glass opacities in centrilobular distribution, mainly in upper lobe. |
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CXR findings in chronic hypersensitivity pneumonitis
HRCT findings |
CXR:
Similar to idiopathic pulmonary fibrosis: reticular interstitial opacities HRCT: Volume loss. cystic honeycombing, reticulated opacities |
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Diagnosis of hypersensitivity pneumonitis
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Most definitive = challenge with suspected offending agent.
Can also diagnose empirically based on symptom relief with removal of antigen. Based largely on clinical picture. Can test for specific antibodies, but 50% of aSx ptshave same result.\ Bronchoalveolar lavage give supporting evidence of lymphocytosis, low CD4:CD8 ratio, but not diagnostic. |
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Treatment for hypersensitivity pneumonitis
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remove offending agent & prevent further exposure.
Corticosteroids for acute severe case, however not beneficial if continued exposure to trigger. |
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Collagen vascular diseases associated with idiopathic pulmonary fibrosis (6)
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1. Rheumatoid Arthritis
2. Systemic Lupus Erythematosis 3. Systemic sclerosis 4. Dermatomyositis / Polymyositis 5. Mixed CT disease 6. Sjogren syndrome |
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Percent of idiopathic pulmonary fibrosis with underlying vascular disease
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15%
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4 manifestations of asbestosis
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1. Interstitial Asbestosis
2. Pleural disease - asbestos plaques or effusion 3. Brochogenic carcinoma 4. Mesthelioma |
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Pathophysiology of asbestosis
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Inhaled fibers deposited in lower respiratory tract
Fibers are directly toxic Cause macrophages and pneumocytes to release inflammatory mediators: Reactive oxygen species Nitrogen free radicals Cell injury from inflammation leads to progressive fibrosis |
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Histopathology of asbestosis
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Iron-coated asbestos fibers may or may not be present. Lack of fibers does not exclude dx of asbestosis.
Pathologic pattern is Usual Interstitial Pneumonia |
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Chronology of exposure and Sx onset in asbestosis
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Sx onset is generally years after exposure
More severe exposure produces Sx sooner |
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S&Sx of asbetosis
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Closely resemble Idiopathic Pulmonary Fibrosis
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CXR in asbestosis
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Bilateral patchy reticular infiltrates with associated pleural plaques
**End stage disease may be indistinguishable from other fibrotic disease |
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Pulmonary Function Tests in Asbestosis
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Generally show restrictive defect.
Low DLCO is often the earliest finding. |
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ABG in asbestosis
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May show hypoxemia at rest in advanced disease ( severe scarring)
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Diagnosis of asbestosis
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Hx of exposure
Clinical presentation CXR Usually don't need biopsy |
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Biopsy of asbestosis
-Findings -Role in diagnosis |
Shows Usual Interstitial Pneumonia and sometime asbestos crystals.
Helpful in diagnosis but usually not necessary |
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Treatment of asbestosis
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NO TREATMENT; pts receive care as those with Idiopathic Pulmonary Fibrosis.
1. Stop smoking 2. Supportive: -O2 -Pulmonary rehab 3. prevention of respiratory infection 4. Rdiographic monitoring for malignancy development. |
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Silicosis pathophysiology
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Silica particle deposit in alveoli
Macrophages become activated & release inflammatory mediators Free radicals Inflammation Cell death Fibrosis |
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Histopathologic hallmark of Silicosis
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Silicotic Nodules
Central zone of Hyaline surrounded by concentric collagen Outer zone has active inflammation and nodule progression |
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S&Sx of silicosis
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Variable
May develop acute silicosis w massive exposure: Dyspnea, weight loss, fatigue Otherwise takes years to develop; may presnet with only abnormal X-ray or with progressive dyspnea and cough. |
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"Simple silicosis"
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Has characteristic CXR but no Sx or evidence of pulmonary dysfunction
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Chronic silicosis / Progressive Massive Fibrosis: PFT
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PFT's show mixed restrictive / obstructive disease
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3 characteristic CXR patterns of Silicosis
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1. Simple silicosis: small, upper lobe nodules. No other S&Sx.
2. Chronic silicosis / progressive massive fibrosis: caolescing nodules with upper lobe fibrosis, lower lobe hyperinflation / air trapping, and hilar adenopathy / calcification 3. Acute silicosis: Basilar alveolar filling pattern. |
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Diagnosis of silicosis
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Clincial: Hx of exposure, abnormal CXR, exclusion of other possible causes.
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Treatment of silicosis
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No standard Tx.
Avoid further exposure Can give bronchodilators for obstructive Sx or O2 for hypoxemia |
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Coal worker's lung / Coal Worker's Pneumoconiosis / Black Lung disease
Cause |
Inhalation of coal dust, which is a mix of carbon and silicon; pathogensis may be related to silicosis.
Anthracite coal is higher risk than soft coal |
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Pathophysiology of Coal Worker's Lung
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Coal particles in alveoli and respiratory bronchioles cause localized inflammation.
Macrophages & fibroblasts form macules Localized emphysematous changes develop |
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Clinical features of coal workers disease
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Often aSx with small upper lobe nodules on CXR
Progressive dyspnea and cough in worsening disease May have black sputum in severe disease |
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Pulmonary Function Tests in coal workers lung
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Mixed obstructive/restrictive profile
Low DLCO Hypoxemia |
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Diagnosis & treament of coal workers lung
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Dx is via Hx of exposure and clinical findings.
Treatment is supportive |
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Chemical Pneumonitis:
etiologic agents |
Direct toxins:
Phosgene, nitrogen dioxide, sulfur dioxide, metal fumes |
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Nitrogen dioxide pulmonary injury: pathophysiology
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Acute non-cardiogenic pulmonary edema, may progress to Adult Respiratory Distress Syndrome;
Direct airway injury extends all the way into parenchyma Inflammatory reaction acuses increased permeability; can lead to pulmonary hemorrhage |
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Clinical features
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Acute: Sx manifest 3-30 hours after exposure:
Airway irritation / mucosal burning; Dyspnea & chest tightness; however low level exposure may not cause Sx Pt may be hypotensive due to fluid loss through edema Diffuse bilateral crackles Pulmonary edema CXR shows diffuse bilateral infiltrates |
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Long-term outcomes of nitrogen dioxide pneumonitis
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Often major pulmonary impairments continue
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Treatment for nitrogen dioxide pneumonitis
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Supportive only. Often requires mechanical ventilation. Corticosteroids do have proven effect.
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