Obstructive and Restrictive Pulmonary Disease (Handorf) Flashcards
What is pulmonary emphysema?
irreversible enlargement of air spaces distal to the terminal bronchioles
What are the two pathological causes of emphysema?
- destruction of walls
2. dilation of alveoli
Most significant etiology of emphysema
smoking
Why does emphysema cause “more suffering” than lung cancer, according to Handorf?
more common
emphysema patients live longer
emphysema is progressive
some lung cancers can be cured
Centrilobar pattern of emphysema is characteristic of:
smoking damage
Panlobar pattern of emphysema is characteristic of:
alpha-1-antitrypsin deficiency
Fundamental unit of lung; includes terminal br, resp br, alveolar duct and alveoli
acinus
Centriacinar emphysema initially affects:
What usually causes this, and in what portion of the lungs?
respiratory bronchioles
smokers
upper lobes and apices
Panacinar emphysema initially affects
What usually causes this, and in what portion of the lungs?
peripheral structures, such as alveoli and ducts; it later extends to affect resp bronchioles
alpha-1-antitrypsin deficiency
lower basal zones
What is the pathophys of emphysema? How do the two causes overlap?
- Smoking produces ROS, which signal neutrophils via IL8, LTB4, TNF
- elastase (from neutrophils and resultant from inactivation of antiproteinases via ROS) damages tissue
smoking creates a function alpha-1-antitrypsin deficiency
What is bullous emphysema?
How would you realize you had this?
damaged alveoli extend to form exceptionally large air spaces (especially in apices)
abnormal air space ruptures (often healthy young adults); fluid leaks into pleural space potentially causing lung collapse
*this happened to Zach, minus the pneumothorax.
How is chronic bronchitis defined?
clinical condition characterized by persistent cough with sputum production for at least 3 months in at least 2 consecutive years
*Robbins defines it pathologically as mucus gland hypertrophy with hypersecretion and bronchiolitis
What causes chronic bronchitis?
chronic irritation
microbiologic infections
What is bronchiectasis?
permanent abn dilation of airways
Where do chronic bronchitis and emphysema intersect?
both involve destruction of alveolar walls with inflammatory cell involvement
*is this right?
Gross pathology of asthma
- pulm hyperexpansion, with and without atelectasis
- bronchial mucus plugs due to hypersecretion
Micro pathology of asthma
–bronchial lumen mucus with: eosinophils, neutrophils, creola bodies, Curschmann spirals, Charcot-Leyden crystals
–airways remodeling: sm muscle hyperplasia and hypertrophy, submucosal gland expansion, thickened basement membrane, fibrosis
What is pneumoconioses?
non-neoplastic lung reaction to inhalation of mineral dusts, org and inorg particulates, chemical fumes or vapors
Types of pneumoconioses
- coal miners lung (anthracosis to prog massive fibrosis; large blackened scars)
- silicosis (most common)
- asbestosis
- berylliosis
- farmers lung (bagassosis, org dust)
- byssinosis (org dust exposure + asthma)
Causes fibrocalcific plaques on the pleural side of the diaphragm
pneumoconioses from asbestos
What causes ferruginous bodies?
inhaled asbestos particles which are ingested by macrophages and coated with iron salts
What is sarcoid characterized by?
NONcaseating granulomas in many tissues and organs
Who is the STEP patient with sarcoidosis?
middle aged AAF
Where are sarcoid lesions commonly found?
lungs
eyes
skin
lymph nodes
What is likely the cause of sarcoidosis?
infectious, organic or inorganic particles begin an immune reaction
What are strong contributors to sarcoidosis susceptibility?
macrophage HLA and BTNL2 alleles
What initiates the formation and maintenance of sarcoid granulomas?
CD4 T cells
What causes progressive fibrosis in certain sarcoidosis patients?
Th2 activated alveolar macrophages which stimulate fibroblast prolif and collagen production
What are characteristics of advanced sarcoidosis?
bronchiectasis, emphysema, fibrosis, pulm HTN
