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50 Cards in this Set
- Front
- Back
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How do you Dx IPF?
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Biopsy
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What the best Rx for IPF/UIP?
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Transplant
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What other organs/systems are affected by IPF?
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NONE
just lungs |
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Typical Rx plan for IPF?
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Educate
Home O2 and Lasix SQ gamma interferon (doesn't really work) Transplant List |
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Original Hypothesis for pathogenesis of ILD?
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Stimulus
Chronic Inflammation Injury Fibrosis |
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Main guy involved in fibrosis?
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collagen
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Of all the different ILD's, how many have known etiology vs unknown?
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1/3 = known etiology
2/3 = unknown etiology |
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Disorders of Known Etiology?
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Inorganic Dusts
Organic Dusts Drugs Poisons Radiation Therapy Reflux Disease/Aspiration |
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Types of inorganic dusts?
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Coal
Silica Asbestos |
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Types of Organic Dusts?
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Fungi
Bacteria Antigens |
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types of drugs causing ILD?
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Bleomycin
Amiodarone Nitrofurantoin |
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Type of poison causing ILD?
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Paraquat
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Types of ILD's w/ unknown etiology?
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Sarcoidosis
Idiopathic Interstitial Pneumonias Collagen Vascular Disorders Eosinophilic Granuloma Pulmonary Vasculitis |
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Examples of Idiopathic Interstitial Pneumonias?
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IPF
BOOP NSIP DIP |
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Types of collagen vascular disorders that may cause ILD?
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RA
SLE |
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Non-specific Sx's of ILD?
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Dyspnea on Exertion
Non-productive Cough |
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Non-specific signs of ILD on physical?
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Clubbing
Basilar rales or crackles Presence of underlying systemic disease Clubbing and Crackles are often absent w/ Sarcoidosis and Hypersensitivity Pneumonitis |
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Possible CXR findings w/ ILD?
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Small Lung Volume
Interstitial Infiltrates |
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How can Interstitial Infiltrates appear on CXR?
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Reticular
Nodular Reticulonodular Ground Glass Honeycombing |
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Which ILD's predominantly affect the upper lobes?
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Sarcoidosis
Silicosis |
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Which ILD's predominantly affect lower lobes?
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IPF
Asbestosis |
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Which ILD causes a "snow storm" appearance on CXR?
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Coal Worker's
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Which ILD can lead to pleural thickening?
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Asbestosis
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What the best imaging study for ILD's?
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High Res CT (HRCT)
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Why is HRCT so good?
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Suggest Dx
Predict Disease Activity Select Biopsy Site Assess Response to Rx |
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PFT's in ILD pts?
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Dec Lung Volumes
Normal FEV1:FVC Ratio Dec Diffusing Capacity Dec pO2 Dec pO2 w/ exercise Dec Compliance i.e. classic restrictive disease |
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Equation for Compliance?
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Compliance = Volume Change/Pressure Change
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Two ways to get a lung biopsy?
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Bronchoscopy
Surgical Lung Bx |
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Types of Rx for ILD's
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Avoidance
Supportive Care Corticosteroids Other Drugs Transplant |
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Types of Supportive Care?
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O2
Diuretics Antibiotics Vaccination |
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Three Drugs for ILD Therapy?
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Cytoxan
Azathioprine Methotrexate |
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What is the most important part of the history of a patient w/ ILD?
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Environmental and Occupational History
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Which ILD's occur more often in 20-40 year olds?
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Sarcoidosis
Histiocytosis X CT Disease-Associate ILD |
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What age range does IPF mostly appear in?
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Over 50
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What ILD's occur exclusively in premenopausal women?
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Lymphangioleiomyomatosis (LAM)
Tuberous Sclerosis |
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What other ILD's are more common in women?
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CT Disease-associated ILD's
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What are men more prone to get?
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Pneumoconiosis...but only b/c we work around the etiological agents
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Smoking is an important risk factor in which ILD's?
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IPF
Respiratory Bronchiolitis-ILD Desquamative Interstitial Pneumonitis Histioctyosis X |
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Which ILD's occur predominantly in nonsmokers?
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Sarcoidosis
Hypersensitivity pneumonitis |
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Which ILD's have a familial association?
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Familial Pulmonary Fibrosis
Neurofibromatosis Tuberous Sclerosis Sarcoidosis |
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What is the Gold Standard for ILD Dx?
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Surgical Bx
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Difference between active and inactive ILD's?
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Active = alveolitis is present and its possibly reversible
Inactive = end-stage w/ irreversible fibrosis |
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What findings on HRCT are indicative of an active, possibly reversible disease process?
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Ground Glass Opacities
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Who are the Pneumoconioses?
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Silicosis
Asbestosis Coal Worker's |
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Etiology of Hypersenstitivity Pneumonitis?
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Inhalation of various organic substances by a sensitized host
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Type of Inflammation associated w/ Hypersensitivity Pneumonitis?
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Granulomatous
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Sx's of Hypersensitivity Pneumonitis?
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Dyspnea
Fever Wt Loss Malaise all occuring 4-10 hours after exposure |
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Prevalence of lung involvement in sarcoidosis cases?
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90%
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Gender and sarcoidosis?
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more predominant in AA's
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Predicted lifespan post-Dx in IPF pts?
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3-5 years
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