Flashcards in 11.20: Pulm III Deck (51)
What is decreased TLC consistent with?
Restrictive lung disease
2 categories of restrictive lung disease?
1. Chest wall disorder
2. Chronic interstitial and infiltrative disease
Characteristics of interstitial lung diseases?
- End inspiratory crackles
- Nodules and irregular lines on cxr
2 Main categories and 3 main types of chronic interstitial lung disease?
a. Usual interstitial pneumonia: 15%
b. Pneumoconiosis: 25%
a. Sarcoidosis: 20%
What is interstitial lung disease?
- Inflammatory and fibrotic disease of alveolar walls and septa leading to reduced pulmonary compliance
What will interstitial lung disease lead to?
- Pulmonary htn.
- Cor pulmonale
"Honey comb lung" is end stage pathology
What is honeycomb lung characteristic of?
End stage restrictive interstitial lung disease
Difference between interstitial lung disease and emphysema?
There is fibrosis seen in interstitial disease but not in emphysema
What is idiopathic pulmonary fibrosis?
- Clinical term for "usual interstitial pneumonia"
Pathogenesis of IPF?
- Repeated epithelial injury leading to TGFbeta
- TGFB leads to fibroblasts
- Fibroblasts deposit collagen and ECM
- Pulmonary fibrosis ensues
Another name for idiopathic pulmonary fibrosis?
- Usual interstitial Pneumonia "UIP"
Another name for Usual interstitial Pneumonia "UIP"?
- Idiopathic pulmonary fibrosis
Characteristics of fibrosis in IPF?
- Non uniform pattern
- Varies in intensity and age
- Early "exuberant" fibroblastic proliferation
What is patchy interstitial fibrosis indicative of?
What is the impact of fibrosis in IFP?
- Patchy fibrosis destroys interstitial architecture
- Contraction of scarring pulls to destroy alveolar spaces
- Cystic spaces are created with Type II pneumocytes
What is cobblestone lung indicative of?
Characteristics of IPF?
- Variable fibrosis more common in lower lobes
- "Cobblestone lung"
- Fibrosis more common in subpleural layers than in paraseptal
Clinical appearance of IPF?
- 40 - 70
- M > W
- Dyspnea on exertion
- Dry cough
- Velcro crackles
Prognosis of IPF/UIP? Treatment?
- Rapid deterioration with mean survival
What is pneumoconioses?
Accumulation of particulate, chemical fume, or vapor in lung and the reaction to its presence
3 main types of pneumoconioses?
1. Anthracosis: coal dust
2. Silicosis: silica from sand blasting
3. Asbestosis: ships, roofing, plumbing
What causes largest risk for development of pneumoconioses?
- Small 1 to 5 micrometer particles have greats danger of settle in linings of small airways and sacs and fester
- Too small to be caught by cilia and mucus
- Too big to be engulfed by macs
How is most inhaled particulate removed?
- Trapped in mucus and removed by ciliary elevator if > 1 microns
Pathogenesis of pneumoconiosis?
- Macs endocytose particles releasing inflammatory mediators
- Fibrogenesis and collagen deposition ensues
Presentation of anthracosis?
- Seen in coal, urban, and smokers
- Inhaled carbon pigment engulfed by interstitial macs
- Accumulates in tissues them moves to lymph
- Will see some black on autopsy but not other problems
** NO SYMPTOMS NOTES
Pathogenesis of simple coal workers pneumoconiosis?
- Same carbon irritant forms macules / nodules
- Patchy fibrosis noted but not extensive
Pathogenesis of progressive massive fibrosis?
- Coalescence of coal nodules into large scars
- Fibrotic, bulky, heavily pigmented black tissue masses
Clinical course of coal workers pneumoconiosis?
Bening with progression to pulmonary dysfunction, pulm htn, or cor pulmonale
What is Caplan syndrome?
- Ptn. w/ rheumatoid arthritis exposed to coal
- Get fibrotic lesions with cavitating nodules on radiology
- Hints that RA alters bodies response to coal
Who is likely to get silicosis?
- Most prevalent occupational disease in world
- Workers in foundries, sandblasting, mines
- Caused by inhalation of crystalline silica engulfed by mac releasing fibrotic mediators
Appearance of silicosis?
- Nodules of "Whorled" hyalinized collagen
- Nodules can coalesce leading to massive fibrosis extending to hilar lymph nodes and pleura
"Eggshell calcification" of lymph nodes
When is egg shell calcification of lymph nodes seen?
What does silicosis lead to increased susceptibility to?
- Silicosis suppresses cell mediated immunity and impairs macrophage phagocytosis
What can silicosis lead to?
2. Pulm htn
3. Cor pulmonale
Disease often associated with silicosis?
Disease often associated with coal workers?
Which form of Pneumoconioses is carcinogenic?
Why is asbestos often used?
3. Head resistant for insulation
Which type of asbestos is most dangerous?
- Rigid, rod like molecule that can poke hole through macrophage
What does asbestos lead to?
1. Interstitial Fibrosis
2. Localized fibrotic pleural plaques
3. Malignant mesothelioma
4. Bronchogenic carcinoma
5. Laryngeal Carcinoma
6. Pleural effusion
What cause malignant mesothelioma?
Hallmark of asbestos?
- Fibrinous "Asbestos bodies"
- Looks like beaded structure with nobby head
- Fe will sit on and coat the asbestos body
How much does asbestos increase cancer risk?
- 55x if a smoker
- 5 x if not a smoker
- 1000x increased risk in mesothelioma
***Family members are at risk through indirect exposure
***Can occur 20 - 40 years after exposure
Characteristics of Sarcoidosis?
- Multisystem disease of unknown etiology
- Characterized by non caseating granulomas
- Diagnosis of exclusion in which TB or fungus must be ruled out when granulomas are seen
What is a granuloma?
- Lymphocytes, macrophages that have fused to form giant cells
Clincial presentation of sarcoidosis?
- 90% with bilateral hilar lymphadenopathy or parenchymal abnormality on chest xray
What leads to sarcoidosis?
- Genetic abnormality with environmental trigger
When are asteroid and schaumann bodies seen?
Manifestations of sarcoidosis?
- SOB with dry cough
- Fever fatigue sweats
- Eye in inflammation: Uveitis
- Erythema nodosum
- Restrictive cardiomyopathy
What is erythema nodosum?
Inflammatory lesions of subcutaneous fat see in skin in sarcoidosis