Pulmonary: Restrictive + Vascular + HTN + Miscellaneous Flashcards
Restrictive lung disease are due to what?
Reduced lung compliance (Hard to fill lungs)
Restrictive
FVC?
FEV1?
FEV1/FVC
FVC: reduced
FEV1: Reduced
FEV1/FVC: Normal
Acute restrictive is defined by what symptoms (4)
- Acute dyspena
- Hypoxia
- Bilateral pulmonary infiltrates
- Absence of left heart failure
How are ALI and ARDS related?
ALI is like early stage ARDS
What are ALI/ARDS the most comon cause of?
Non-cardiogenic pulmonary edema
ALI/ARDS is due to what (2)
- Direct injury to lungs
2. Indirect injury due to systemic disease
Injury to lungs for ALI/ARDS is due to what? (3)
- Infections
- Toxins (Sepsis)
- Trauma
ALI/ARDS path?
- Endothelial injury
- Alveolar edema
- Type II pneumocytes injured
Cause of ALI/ARDS damage?
Imbalance of pro-inflammatory and anti-inflammatory cytokines
3 major morphologic changes in ARDS/ALI?
- Hyaline membrane formation2
- Hepatization of lungs
- Vascular congestion
End stage of ARDS/ALI?
Fibrosis and honey-combing
What do pathologists call ARDS/ALI?
DAD = Diffuse alveolar destruction
When do ARDS/ALI symptoms appear?
Within 72 hours of insult
What % die in ARDS/ALI?
30-40% (pretty high)
How well do ARDS/ALI survivors recover?
Complete regain of function within a year
Chronic restrictive lung disease end-stage? (2)
- Diffuse interstitial fibrosis
2. with or without Honeycombing
What data is important for chronic restrictive diagnosis? (2)
- Social
2. Occupational
Causes of chronic restrictive lung diseases? 7
- Asbestos
- Silicosis
- Coal
- Drugs
- Radiation
- High O2
- Immunologic
Definition of idiopathic pulmonary fibrosis?
Progressive interstitial fibrosis
IPF/UIP sex?
Age?
How is this diagnosed
Males
Elderly
Diagnosis of exclusion
How is the process of IPF/UIP in terms of morphology and age of fibrosis?
Heterogeneous
Main symptom of IPF/UIP?
Slow, continuous dyspnea
Sarcoidosis present how? 5
Lung problem in 90%
Noncaseating granulomas
Bilateral hilar and paratracheal lymphadenopathy (potato nodes)
Interstitial fibrosis
How do you diagnose sarcoidosis?
Diagnosis of exclusion of any other granuloma formers (Myco, Fungi)
Common complications of sarcoidosis besides lung? 2
Eye
Skin
Popuation of Sarcoidosis
Age
Ethnicies
Smoking
Younger than 40
Danes, Swedes, US Blacks »_space;» Whites and Asians
Nonsmokers more common
The presumed cause of sarcoidosis?
Abnormal immune regulation in genetically predisposed people that are exposed to some enviromental stimuli
Genetic factors of sarcoidosis (2)
- Familial and racial clustering
2. Putative antigens like viruses, myco, pollen
Constitutional signs
Fever
Fatigue
Myalgias
What if sarcoidosis has uveoparotid involvement
Mikulicz syndrome
What test is elevated in granulomatous disorders and hence sarcoidosis? 2
ACE elevated (granulomas) Increased urine calcium (granulomas)
Hypersensitivity pneumonitis is defined how?
Immune mediated inflammatory disease of distal lung and alveoli due to sensitivity to inhaled antigens like mold, dust, chemicals
How does hypersensitivity pneumonitis affect the lung? (3)
- Decreased diffusion capacity
- Decreased lung compliance
- Decreased lung total volume
acute form hypersensitivity pneumonitis presents how?
Fever, cough, dyspena 4 to 8 hours after exposure
Go on vacation, goes away, gets back to work
Chronic form of hypersensitivity pneumonitis presents how?
Insidiously and not easy to diagnosis
Evidence that suggests hypersensitivity pneumonitis is immune mediated?
BAL fluids with increased pro-inflammatory chemokines
- Increased CD4/CD8 ratio
- Serum Ab’s suggesting Type III
- Noncaseating granulomas suggesting Type IV
Diffuse alveolar hemorrhage syndromes present how (3)
- Hemoptysis
- Anemia
- Diffuse pulmonary infiltrates
Diffuse alveolar hemorrhage syndrome members
Goodpasture and Idiopathic Pulmonary Hemosiderosis
Goodpastures syndrome definition (2)
Hemorrhagic interstitial pneumonitis and crescentis glomerulonephritis
Goodpastures syndrome has what Ab’s?
IgG against BM and glomerulis suggesting Type II hypersenstivity
Lungs in Goodpastures syndrome show what?
Focal necrosis of alveolar walls
Hemorrhage in alveoli
Hemosiderin laden macrophages
Goodpastures syndrome treatment (2)
- Plasmapharesis
2. Immunosuppression
Idiopathic pulmonary hemosiderosis is similar to what? Except for (2)
Presents the exact same as Goodpastures
- No Anti-Basement membrane Ab’s
- No renal disease
Wegener Granulomatosis definition
Vasculitis
Triad of wegener granulomatosis?
- URT crusting
- Lower respiratory disease
- Renal disease
What is the mediator of Wegener granulomatosis?
Auto Ab’s against Type IV collagen in renal glomeruli and lung alveoli
Two necrotizing features of Wegener granulomatosis?
- Necrotizing capillaritis
2. Necrotizing granulomas
Radiographic changes in Wegener’s
Granulomas that cavitate and coalesce
What is Wegener granulomatosis
C-ANCA
Collagen vascular disease can cause pulmonary disease, which members?
SLE
RA
Scleroderma
Dermatomyositis
What do collagen vascular diseases most often cause?
Interstitial pneumonitis
What is the worst ending for collagen vascular disease?
Progressive pulmonary fibrosis leading to end-stage honeycomb lung
Accelerated pulmonary fibrosis in RA patients?
Exposure to what? (3)
Caplan syndrome
Coal, silica, asbestos
Transplantation pulmonary
Acute rejection involves (3)
Fevers, Leukocytosis, Mimicking infections
Pulmonary embolism that occludes pulmonary arteries at bifurcation?
Result?
Saddle embolus
Sudden death
Most pulmonary emboli are what form?
DVT
Studies for pulmonary emboli? (3)
- Doppler studies
- Radiography
- D-Dimer test (positive)
Predisposing conditions for PE’s 7
- Prolonged bed rest
- Surgery to legs or knees
- Bone fractures in the leg
- CHF
- Pregnancy and oral birth control
- Inherited disease
- Mucinous adenocarcinomas
What tests can you do in acute PE situations 2
- DNA analysis
2. Factor V test
Why don’t we see infarctions often in PE?
Where will you normally see a PE infarct?
Dual blood supply
Distal, wedge-shaped, and involves the pleural
How is the pain of a PE infarct described?
What will you hear?
Positional
Friction rub
Most PE’s have what effect?
None, asymptomatic
How does HTN occur with PE’s
Enough small PE’s block off enough arteries to raise resistance
Patients with one PE have what risk for a second?
30% risk
Most often Pulmonary HTN is secondary to what two things?
- Decreased pulmonary vascular cross-sectional area
2. Increased pulmonary vascular blood flow
Pulmonary HTN situations (3)
- COPD/Interstitial lung disease
- Recurrent PE’s
- Heart disease with left to right shunt
Types of secondary pulmonary hypertension? (3)
- Plexiform pulmonary arteriopathy (most) convoluted tortuous path
- Thrombotic pulmonary arteriopathy
- Pulmonary venoocclusive disease
If you see atheromas in pulmonary vessels you have to think what?
Pulmonary hypertension
Morphology of PHTN? 2
- Atheromas
2. Intimal and medial thickening
Patients with P-HTN the most?
Young females
Only hope for pulmonary HTN patients?
Lung transplant
Cancer types in lungs
- Small cell
- Squamous cell
- Adenocarcinoma
Which cancer type in central in lungs?
Squamous
Adeno cancers tend to be where?
Two populations
Peripheral
Females and smokers
Which cancer cavitates the most?
Squamous cell
most common cancer with perineoplastic syndrome?
Small cell
Hypercalcemia with lung cancer?
Squamous cell
Asbestosis is what?
Fibrosis you get from asbestos exposure
What is most common thing we see with asbestos?
Pleural plaques THEN fibrosis
Most common malignancy with asbestosis?
Bronchogenic carcinoma
Which cancer is most associated with asbestsos?
Mesothelioma
Silicosis appears how? (3)
Concentric fibrosis with nodules
Refractory material
TB infection