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70 Cards in this Set
- Front
- Back
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What are the Interstitial lung diseases?
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A group of lung parenchymal disorders with common
-Clinical features -Radiologic features -Pathophysiologic features |
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What is the hallmark of interstitial lung disease?
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Involvement of the interstitium
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What is involved in ILD along with interstitial involvement?
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Cellular and non-cellular infiltration within alveolar septa and the alveoli
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What are 2 synonyms for ILD?
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-Infiltrative lung disease
-Diffuse parenchymal lung disease |
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What is the main difference between ILD and COPD/emphesyma?
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ILD: parenchymal disorder - of the interstitial tissue
Obstructive: disorder of the trachea to the resp bronchiole |
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What is TLC in
-ILD -Obstructive disorders |
ILD = decreased
Obstructive = increased |
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What is the incidence of ILD?
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Much less common than COPD
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What are the most common causes of ILD?
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Occupation
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What are the 4 main phases in ILD?
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1. Injury to parenchymal cells and acute alveolitis
2. Chronic alveolitis 3. Derangement of collagen interstitium - Fibrosis 4. End stage lung |
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When does ILD become irreversible?
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When collagen gets deranged in phase 3
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What does Chronic ILD refer to?
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Fibrous widening of the interstitium (alveolar walls)
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What are the 2 mechanisms involved in Chronic interstital lung disease?
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-Primary interstitial widening
-Accretion |
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What is accretion?
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The incorporation of fibrous tissues FROM THE AIR SPACES into the interstitium
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What is Primary interstitial widening?
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Fibroblast Activation and proliferation for collagen production WITHIN THE INTERSTITIUM.
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If ever, when could a definitive diagnosis of the actual TYPE of ILD be made?
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During early stages of disease
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What happens to the lungs in advanced stages of ILD?
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Honeycombing, End stage lung - undifferentiable between etiologies.
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What do you have to do to make a definitive diagnosis at advanced stages of ILD?
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Rely on the clinical presentation - occupation, travel, and social history
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What 3 professions have to collaborate in order to come up with a diagnosis in ILD?
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-Pathology
-Radiology -Clinical |
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What is the time frame of acute ILD? Chronic?
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Acute = days to weeks
Chronic = months to years |
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What is the most common presenting symptom of ILD?
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Dyspnea
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What will be heard on auscultation in ILD?
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Bronchovesicular breath sounds with bibasilar rales - VELCRO
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What are 5 main complications of ILD?
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RIPPS
-RVH/Cor pulmonale -Infections -Pneumothorax -Pulmonary embolism -Steroid complications |
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How is diffuse ILD discovered in most cases?
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When CXR is done for respiratory symptoms
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What is the most productive part of the exam in diagnosing diffuse ILD?
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The history
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What is the definition of Pneumoconiosis?
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Non-neoplastic lung disease in response to the inhalation of mineral dusts in the workplace.
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Does pneumoconiosis only refer to inhaling mineral dusts?
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No, also organic and inorganic particulate matter, chemical fumes, and vapors.
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What factors determine the development of pneumoconioses?
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-Amt of dust retained in the lung parenchyma/airways
-Size, shape, and buoyancy of the particles -Particle solubility/reactivity -Other effeccts of irritants ie smoking |
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What are 2 alternative types of Pneumoconiosis? Which form is worse? Why?
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-Fibrogenic - worse because restrictive fibrosis occurs
-Nonfibrogenic |
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What are the 4 most common types of Fibrogenic Pneumoconiosis?
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-Coal worker's (carbon dust)
-Silicosis -Silicatosis -Asbestosis |
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Why is Asbestos bad?
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It is related to a whole host of 2ndary diseases - both neoplastic and non-neoplastic
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What are the 2 main types of Asbestos fibers?
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1. Serpentine
2. Amphibole |
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Which type of asbestos fiber is worse? Why?
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Amphibole - because it is short and brittle and goes to the distal lung causing more damage
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Why is Serpentine asbestos not as bad?
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Because it is flexible and easily expelled
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What is a common lung disease that results from asbestosis?
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Pleural effusions and plaques
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What can be seen on microscopic exam of lung tissue in Asbestosis?
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Asbestos bodies
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What are asbestos bodies?
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Iron containing objects that stain positive with Prussian blue
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What cell abnormalities can be seen on high power in asbestosis?
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Multinucleated Giant cells with hemosiderin pigment
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What is the most COMMON form of ILD?
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Idiopathic interstitial pneumonia
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What is the most common form of idiopathic interstitial pneumonia called?
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UIP - Usual Interstitial pneumonitis
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What is UIP synonymous with?
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IPF: idiopathic pulmonary fibrosis
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How was the current terminology for UIP devised?
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By the collaboration of all areas of expertise - Pathology, Clinical medicine, Radiology.
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If I remember nothing else about UIP, what should I remember?
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UIP has a poor prognosis; 50% of patients die within 5 years
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IPF:
UIP: |
IPF = idiopathic pulm fibrosis
UIP = usual interstitial pneumonia |
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What are the clinical manifestations of UIP?
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-Dyspnea
-Nonspecific fever/weightloss, fatigue, aches/pains, etc |
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What will be seen on pathologic exam of lungs with UIP?
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Interstitial pneumonitis and fibrosis
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What is the clinical course of UIP like?
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Unpredictable
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What does UIP end in?
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End stage lung and Cor pulmonale
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What is the nature in which UIP spreads through the lungs?
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Early: in peripheral parts
Later: throughout the lungs including the center regions |
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What does end stage lung look like grossly?
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A honeycomb
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What is a more pathological term for the honeycomb look?
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Reticular Nodular Pattern
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Why are the bronchi throughout the lungs dilated in UIP?
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Because they are surrounded by fibrosis which pulls them open.
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What is the bronchial dilation in UIP called?
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TRACTION BRONCHIECTASIS
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What are the honeycomb spaces in UIP lined by?
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Very broad, thick bands of fibrous tissue - the interstitial walls are just thick.
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Is the honeycomb pattern variable or uniform throughout the lungs in UIP?
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Patchy - there may be spots of completely normal tissue, just adjacent to severe honeycombing
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What do patches of blue fibrosis adjacent to patches of very cellular pink collagen indicate?
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That there were repeated insults to the lungs resulting in new fibrosis (blue) in addition to old (pink).
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If an 35 yo African American woman presents in respiratory distress and develops hilar adenopathy the next day, think:
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SARCOIDOSIS
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What is the ratio of sarcoidosis in AAs vs Whites?
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10:1
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What is the predominent sex that gets sarcoidosis? What age?
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Females 2:1 over males
Age <40 years |
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What is the etiology of sarcoidosis?
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Unknown
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What organs are involved in 90% of sarcoid cases?
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-Lungs
-Lymph nodes |
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What are 5 common lab findings in sarcoidosis?
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-Hypercalcemic
-Hypercalciuric -Hypergammaglobulinemia -Elevated LFTs -Raised ACE levels |
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Why is increased ACE significant? What is it NOT useful for?
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As an indicator of disease activity; not diagnostic..
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What is the course of Sarcoidosis like?
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Unpredictable - could resolve in a few months, or continue chronically
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What is the worst form of sarcoid?
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Sarcoid heart
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What type of inflammation is seen in Sarcoidosis?
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Noncaseating granulomatous
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What should you do if you suspect sarcoidosis due to seeing granulomas on chest XR?
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Culture and workup for TB
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What will you see on CXR in sarcoidosis?
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-Reticular nodular shadowing
-Hilar adenopathy |
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What is seen in late stage sarcoid of the lung?
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Honeycombing - at that point undifferentiable from UIP
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What is the hallmark characteristic of the granulomas seen in Sarcoidosis?
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They extend right up to the pleural surface and are present in the INTERSTITIUM.
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What does NOT occur in the granulomas in sarcoidosis?
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Necrosis
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