Pathoma Respiratory Part II Flashcards
Group of diseases characterized by airway obstruction + air trapping; decreased FVC, FEV1, FEV1/FVC ratio; increased TLC, RV, FRC
Obstructive lung dz:
- chronic bronchitis
- emphysema
- asthma
- bronchiectasis
Chronic productive cough (due to excessive mucus production) lasting at last 3 months over at least two years; characterized by hypertrophy of bronchial mucinous glands (goblet cells); leads to increased thickness of mucus glands relative to bronchial wall thickness (Reid index > 50%; normal
Chronic bronchitis
- Productive cough
- Cyanosis (“blue bloaters”) - mucus plugs trap CO2 –> increased PaCO2 + decreased PaO2
- Increased risk of infection and cor pulmonale
Chronic bronchitis
Highly associated with SMOKING –> pollutants –> irritation and inflammation –> mucinous gland hypertrophy + hyperplasia
Chronic bronchitis
Destruction of alveolar airs sacs –> loss o f elastic recoil and collapse of airways during exhalation; due to imbalance of proteases and antiproteases
Emphysema
Smoking –> inflammation + protease-mediated damage
CENTRIACINAR emphysema in UPPER lobe
Inflammation in the lungs normally leads to
release of proteases by neutrophils and macrophages
Alpha-1-antitrypsin normally …
neutralizes proteases
Lack of anti-protease leaves the air sacs vulnerable to protease-mediated damage; mutated protein accumulates in the ER of hepatocytes (pink, PAS+ globules on biopsy_–> cirrhosis; due to PiZ mutation
Alpha-1-antitrypsin deficiency –> PANACINAR emphysema in LOWER lobes
- Dyspnea + cough with minimal sputum
- Prolonged expiration w/ pursed lips (“pink puffers”)
- Weight loss
- Increased AP diameter of chest (“barrel chest”)
- Hypoxemia due to destruction of capillaries in the alveolar sac and cor pulmonale are late complications
Emphysema
Type I HS –> reversible AW bronchoconstriction
Atopic asthma
Allergens –> TH2 –> IL4 + IL5 + IL10
Re-exposure –> IgE mediated activation of mast cells –> release of preformed histamine granules + LTC4, LTD4, LTE4 –> bronchoconstriction, inflammation and edema
Asthma (early phase reaction)
Mediates class switch to IgE
IL-4
Attracts eosinophils
IL-5
Stimulates TH2 cells and inhibits TH1
IL-10
Asthma: Allergens –> TH2 –> ?
IL4, IL5, IL10
Inflammation, especially MAJOR BASIC PROTEIN derived from eosinophils, damages cells and perpetuates bronchoconstriction
Late-phase reaction
Curschmann spirals (spiral-shaped mucus plugs) Charcot-Leyden crystals (eosinophil-derived)
Asthma
Besides allergens, what else can provoke asthma
- Exercise
- Viral infxn
- Aspirin (NASAL POLYPS)
- Occupational exposures
Permanent dilatation of bronchioles and bronchioles and bronchi; loss of airway tone results in air trapping; due to necrotizing inflammation with damage to airway walls.
Bronchiectasis
Causes of bronchiectasis include:
- CF
- Kartagener syndrome
- Tumor or foregoing body
- Necrotizing inflection
- Allergic bronchopulmonary aspergillosis (ABPA)
What is Kartagener syndrome?
Inherited defect of the dynein arm, which is necessary for ciliary movement –> sinus, infertility (poor motility of sperm), + situs inversus
Cough, dyspnea, foul-smelling sputum –> hypoxemia w/ cor pulmonale + secondary amyloidosis
Bronchiectasis
Characterized by restrictive filling of the lungs; decreased TLC, FEV1, FVC; increased FEV1: FVC ratio; most commonly due to interstitial diseases of the lung; may also arise with chest wall abnormalities (obesity)
Restrictive pulmonary dz
Cyclical lung injury –> TGF-Beta from injured pneumocytes –> Fibrosis of lung interstitium
Idiopathic pulmonary fibroiss
What are the secondary clauses of interstitial fibrosis?
- Bleomycin
- Amiodarone
- Radiation therapy
Diffuse fibrosis on CT with end-stage honeycomb lung
Idiopathic pulmonary fibrosis
Treatment for pulmonary fibrosis?
Transplant
Interstitial fibrosis due to chronic exposure to small particles that are fibrogenic –> alveolar macrophages engulf foreign particles that induce fibrosis
Pneumoconioses
Massive exposures leads to diffuse fibrosis (“black lung”) associated with rheumatoid arthritis (Caplan syndrome)
Coal Workers’ Pneumoconiosis (exposure to carbon dust)
Mild exposure to carbon results in WHAT? (collections of carbon laden macrophages that are not clinically significant)
Anthracosis
Fibrotic nodules in upper lobes of the lung; leads to impaired phagolysosome formation by macrophages + increased risk for TB
Silicosis (seen in sandblasters and silica miners)
Noncaseating granulomas in the lung, lilac lymph nodes, and systemic organs –> increased risk for lung cancer
Berylliosis, seen in beryllium miners and workers in the aerospace industry
Fibrosis of lung and pleura (plaques) with increased risk for lung carcinoma (MORE COMMON) and mesothelioma; lesions may contain long, golden brown fibers with associated iron (ferruginus body) which confirm exposure
Asbestosis
Noncaseating granulomas + hilar lymphadenopathy in an African American female due to CD4+ helper T cell response to an unknown antigen –> restrictive lung disease;
Sarcoidosis
Uveitis
Cutaneous nodules or erythema nodosum
Dry eyes / mouth (salivary + lacrimal glands)
Restrictive lung disease
Sarcoidosis
- Dyspnea or cough (most common presenting sx)
- Elevated serum ACE
- Hypercalcemia (1-alpha hydroxyls activity of EPITHELIOD HISTIOCYTES (diagnostic) converts vitamin D to its active form)
- Stellate inclusions (“asteroid bodies”) are often seen w/i giant cells of the granulomas
Sarcoidosis
Treatment for sarcoidosis
Steroids, but often resolves spontaneously
Granulomatous rxn to inhaled organic antigens (e.g. pigeon-breeder’s lung); presents with fever, cough, and dyspnea hours after exposure; resolves w. removal of the exposure (chronic exposure –> interstitial fibrosis)
Hypersensitivity pneumonitis
Definition of pulmonary HTN
MAP > 25 (normal is 10)
Characterized by atherosclerosis of the pulmonary trunk, smooth muscle hypertrophy of pulmonary arteries, and intimal fibrosis; leads to right ventricular hypertrophy with eventual cor pulmonale
Pulmonary HTN
PLEXIFORM LESIONS
Pulmonary HTN
Saddle emboli
sudden death
Classically seen in young adult females; some familial forms related to INACTIVATING mutation in BMPR2 –> proliferation of vascular smooth muscle
Primary pulmonary HTN
Due to hypoxemia or increased volume in the pulmonary circuit; may also arise with recurrent PE
Secondary pulmonary HTN
Diffuse damage to the alveolar-capillary interface – leakage of protein-rich fluid –> edema that combines with necrotic epithelial cells to form HYALINE MEMBRANES
ARDS
“White out” on x ray (diffuse alveolar collapse on expiration)
ARDS
Hypoxemia and cyanosis with respiratory distress due to thickened diffusion barrier and collapse of air sacs (increased surface tension)
ARDS
Activation of neutrophils in ARDS induces protease- and free radical mediated damage of
type I and type II pneumocytes
Respiratory distress due to inadequate surfactant levels
Neonatal RDS
What produces surfactant?
Type II pneumocytes; phosphatidylcholine (lecithin) is the major component
Components of surfactant
Lecithin (phosphatidylcholine) and sphingomyelin –> L:2 ratio > 2 = mature lungs
Risk factors for NRDS
Prematurity C-section delivery (steroids increase synthesis and release of surfactant) Maternal diabetes (insulin decreases surfactant)
Increased respiratory distress after birth, tachypnea w/ use of accessory muscles; grunting; hypoxemia + cyanosis; diffuse granular (“ground glass”) appearance on CXR
NRDS
Hypoxemia in infant w/ NRDS increases risk of:
persistence of PDA
necrotizing enterocolitis
Supplemental O2 given to infant w/ NRDS
Blindness
Lung damage w. NRDS
Bronchopulmonary dysplasia
Most common cancers by incidence
- Breast/prostate
- Lung
- Colorectal
Key risk factors for lung caner
Cigarettes, radon, asbestos
Coin-lesion on xray
- Granuloma
- Bronchial hamartoma
- Small cell
- Non-small cell
The firs thing you should do is review previous CXRs.
Surgery does not help which lung cancer?
Small cell
Benigh tumor composed of lung tissue and cartilage; often calcified on imaging
Hamartoma
Pleural involvement is classically seen with
adenocarcinoma
Distended head and neck veins with edema and blue discoloration of arms and face
Superior vena cava syndrome
Hoarseness
Involvement of recurrent laryngeal nerve
Diaphragmatic paralysis
Involvement of the phrenic nerve
What are the mutagenic components of cigarettes?
Polycyclic aromatic hydrocarbons; arsenic;
Horner syndrome
Pancoast tumor (apical tumor)
Poorly differentiated; arises from neuroendocrine (Kulchisky) cells
Small cell carcinoma
Keratin pearls or intercellular bridges
Squamous cell carcinoma
Lung cancer: glands or mucin on histology
adenocarcinoma
Poorly differentiated large cells (with no keratin bridges intercellular bridges, glands, or mucin)
Large cell carcinoma
Columnar cells that grown along preexisting bronchioles and alveoli; arises from Clara cells
Bronchioalveolar carcinoma
Well differentiated neuroendocrine cells; chromogranin positive
Carcinoid tumor
Multiple “cannon ball” nodules on imaging
Metastasis to lung
Lung cancers associated with smoking
Small cell
Squamous cell
Adenocarcinoma
Large cell carcinoma
Lung cancers not associated with smoking
Bronchioalveolar carcinoma
Carcinoid tumor
Most common tumor in male smokers
Squamous cell carcinoma
Another (not the most common) tumor associated with male smokers
Small cell carcinoma
Most common tumor in nonsmokers and female smokers
Adenocarcinoma
Central tumors
Small cell
Squamous cell
Peripheral tumors
Adenocarcinoma
Bronchioalveolar
Can be central or peripheral
Large cell
Carcinoid
Polyp-like mass in the bronchus
Central carcinoid tumor
Rapid growth and early metastasis; may produce ADH or ACTH (Cushing) or cause Eaton-Lambert syndrome
Small cell
May produce PTHrP leading to hypercalcemia
squamous cell
May present with pneumonia-like consolidation on imaging
Bronchioalveolar
Pneumothorax in a tall, thin, young man
Spontaneous pneumothorax due to rupture of emphysematous bleb, trachea shifts towards collapse
Pneumothorax following penetrating chest wall injury
Tension pneumothorax; trachea is pushed OPPOSITE to side of injury
Trachea shifts away from the pneumothorax
Tension pneumothorax
Trachea shifts toward the pneumothoraz
Spontaneous pneumothoraz
Highly associated with occupational exposure to asbestos
mesothelioma
Recurrent pleural effusions, dyspnea, and chest pain
Mesothelioma
