Interstitial Lung Disease Flashcards
What are common markers and traits of Interstitial Lung Disease (ILD)
- Interstitial inflammation and/or fibrosis
- Granulomas, honeycombing (widespread fibrosis), and cavitation
- Fibrocalcific pleural plaques (particularly in asbestosis)
- Edema and pulmonary infiltrates (acute illness)
- Increased airway secretion
- Bronchial inflammation and airway remodeling
- Bronchospasm
what are common occupational/environmental that could lead to ILD?
There are a lot of factors that could cause or lead to ILD, the following are the most common:
- Asbestosis
- Coal dust
- Silica
- hypersensitivity pneumonitis (organic exposure)
- Pulmonary talcosis (rare)
What is the general term for ILDs caused by inhalation of particular types of dust?
Pneumoconiosis
What are common characteristics/changes that occur with Asbestosis?
Pleural calcification is a common following inhalation of Asbestos fibers (settle in alveoli)
- presence invokes an inflammatory reaction resulting in fibrotic remodeling of lung parenchyma
What is coal worker Pneumoconiosis (Coal minor lung or black lung) caused by?
Long term exposure to coal dust
- inhaled coal dust builds up in the lungs
- invoking inflammatory process which leads to anatomical remodeling (fibrotic changes to the lung
- usually caused by silica, large amounts of coal dust can elicit an inflammatory response
What are anatomical changes that occur with Pneumoconiosis?
Fibrotic changes to the lung parenchyma that eventually lead to necrotic lungs
- Progressive fibrosis is defined as fibrotic granulomas > 1cm in diameter
what is Pneumoconiosis characterized by?
Presence of pinpoint nodules called coal macules
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What is Hypersensitivity pneumonitis
A cell-mediated immune response of the lungs cause by the inhalation of a variety of agents
- progressive fibrotic changes due to chronic inflammation
- (Grains, Silage, Bird/bat droppings, Wood dust,Etc)
- Farmers lung (caused by moldy hay/grains) is a common example
What is Systemic Lupus Erythematosus
Lupus is a multisystem inflammatory (autoimmune) disorder that affects the lungs in 50-70% of cases
What is the primary anatomic concern with lupus?
Pleura becomes inflamed
- Uremic pulmonary edema is the result of alts of pulmonary intravascular starling forces and increases in pulmonary cap membrane permeability
- meaning, efflux of protein rich fluid from the capillaries into the lung
Characteristics of lupus?
Pleurisy with or without effusion (Most common clinical manifestation)
- Atelectasis
- Diffuse infiltrates
- Pneumonitis
- Diffuse fibrotic changes
- Uremic pulmonary edema (high protein count in the effusion fluid) (increased permeability)
- Diaphragmatic dysfunction (weakening and elevation of the diaphragm)
- Increased risk of infection
What is Idiopathic Pulmonary Fibrosis (IPF)
A chronic, progressive, fibrosing pneumonia of unknown cause that leads to:
- severe dyspnea
- impairment of lung function
- death from respiratory failure
What is Idiopathic Pulmonary Fibrosis thought to be most caused by (pathogenesis of disease?)
Trigger is thought to be by various forms of epithelial damage which include infection/microaspirations from gastroesophageal regurgitation
- Injury to epithelial tissues ages it leading to anatomic remodeling of alveoli and collagen disposition in lung parenchyma
- oxidative stress injury leads to production of profibrotic mediators
Major risk factors thought to cause Idiopathic Pulmonary Fibrosis?
Tobacco smoke, heredity factors, environmental factors, GERD, and microbial
Idiopathic Pulmonary Fibrosis clinical manifestations?
Chronic exertional dyspnea, Short of breath on exertion is insidiously progressive over months or years
- Dry cough
- Prevalenceis higher > age of 60 and in men
- Velcro-like inspiratory crackles
- Digital clubbing may occur with chronic hypoxemia
- Hypoxemia (late stage)
- Moderate to severe IPF patients typically require LTOT
What factors help in the differential of IPF when compared to other ILD?
- pattern of interstitial pneumonia pattern on a CT scan
- Specific HRCT patterns and histopathology in surgical lung biopsy
What are features of interstitial pneumonia pattern?
Bilateral basal and subpleural reticulations (reticular pattern = thickening of interstitial space)
- honeycombing on high res CT
- Subpleural reticulation is a type of reticular interstitial pattern where changes are typically in a peripheral subpleural distribution (adjacent to costal pleural spaces)
Idiopathic Pulmonary Fibrosis (IPF) non pharmacological treatments
- Nutrition management + Tx of other comorbidities + palliative care planning
- LTOT if chronic hypoxemia at rest or exertion
- smoking cessation + pulmonary rehab
- lung transplant at end stage
- Influenza and pneumococcal vaccine
IPF pharmacological treatment
Nintendanib and Pirfenidone
Idiopathic Pulmonary Fibrosis (IPF) Acute Exacerbation Criteria
Diagnosis of IPF followed by:
- Unexplained worsening of dyspnea within the past 30 days
- New bilateral ground-glass opacity or consolidation on HRCT
- Exclusion of congestion heart failure (By ECHO)
What does progression of an Acute IPF exacerbation present (timeline) following acceleration of disease process?
Significant decline in lung function w/diffuse alveolar damage superimposed on fibrotic lungs
- may be associated w/acute infection
How can you rule out a acute infection as a cause of IPF?
Bronchoscopy BAL if person is stable enough to tolerate
