ILD Flashcards
Function of the interstitium
Support the lung
Maintain fluid
Help repair and remodel lungs
How is ILD characterized
Broken into:
Acute
Subacute
Chronic
Alveolar walls infiltrated by:
Cells
Fluid
Connective tissue
What happens to diseases that originate in the interstitium
Usually impact other tissues
Where do diseases of the interstitial move to?
Bronchi
Alveolar walls
Adjacent alveolar spaces
ILD pathophysiologic pathway
Injury
Inflammation
Disordered Repair of Involved Tissue
Pulmonary Fibrosis
End-Stage Lung Disease
What does ILD arise from
Inflammation (not infection)
Cells and elements in the interstitium
Collagen fibers
Elastic fibers
Cells:
Fibroblast
WBC (7%) (Lymphocytes)
Alveolar Mac (93%)
Granulocytes (<1%)
What does ILD do to the adjacent pulmonary structures
Destruction of alveoli and adjacent pulmonary capillaries
Fibrotic thickening of bronchioles, alveolar ducts, and alveoli
Granulomas
Honeycombing and cavity formation
Fibrocalcific pleural plaques (asbestosis)
What are the less common side effects of ILD
Bronchospasm
Excessive bronchial secretions (inflammation)
How are ILD disorders grouped
Occupational
Environmental
Therapeutic
Autoimmune
Idiopathic interstitial pneumonia
Specific pathologies
Miscellaneous ILD (everything else)
What two groups of exposures encompasses occupational and environmental ILD
Inorganic and organic
Inorganic ILD associated exposures
Asbestos
Coal dust
Silica
Beryllium
Aluminum
Barium
Clay
Iron
Certain talcs
Where is asbestos found in
Fire-fighting suits
Fireproof paint
Insulation
Roofing materials
Ropes
Steam pipe material
Where is abestos useful
Acoustic products
Automobile undercoating
Brake lining
Cements
Clutch casings
Floor tiles
What size are asbestos fibers
0.1-1um (lodged and not released)
Size of products deposited in the parenchyma
0.8-3 um
How does asbestos appear under a microscope
Brown or orange baton-like structure
Name the nodules caused by coal workers pneumoconiosis
Pinpoint Coal macules
Where do coal macules develop
First and second respiratory bronchioles
How is “complicated” CWP characterized
Fibrotic nodules greater than 1 cm
What are CWP nodules composed of
Dense collagenous tissue with black pigment
What causes fibrotic changes
Silica
Where might you find silica
beaches and playgrounds
How is silica created
Breaking down stone, rock, concrete, brick, block, and mortar
What are you at risk of exposure to with fracking
Crystalline Silica
How many people are exposed to silica at work in the U.S.
2.3 million
What causes silicosis
Chronic inhalation of crystalline, free silica or silicon dioxide particles
What is silicosis characterized by
Small round nodules scattered throughout the lungs (may be asymptomatic)
What is complicated silicosis
When nodules merge and form large masses of fibrous tissues (usually upper lobes and perihilar regions)
ILD irritant gases
Chlorine
Ammonia
Ozone
Nitrogen dioxide
Phosgene
How many people out of 100,000 have ILD
81 people (not rare, but some might be)
Whose’s at risk for developing ILD
Exposed people and people with select systemic disease
What type of material causes hypersensitivity pneumonitis
Organic
How does hypersensitivity pneumonitis develop
Immune response detects antigens and produces antibodies that initiate inflammatory response (pneumonitis)
Sprinters into other names
Disease syndromes associated with hypersensitivity pneumonitis
Farmers lung
Bagassosis
Sequoiosis
Humidifier lung
Bird-breeder’s lung
Antigens associated with organic exposure
Thermophilic
Nonthermophilic
Fungi
Amoebae
Animal protein
Chemicals
What therapeutic medications exposures cause ILD
Antibiotics
Anti-inflammatory meds
Cardiovascular meds
Drugs that induce lupus
Miscellaneous agents
Antibiotic medication that induces hypersensitivity pneumonitis
Nitrofurantoin
Disease-modifying anti-rheumatic drug that induces hypersensitivity pneumonitis
Sulfasalazine
Anti-inflammatory agent that induces hypersensitivity pneumonitis
Methotrexate
Cardiovascular medication that causes hypersensitivity Pneumonitis
Amiodarone
Miscellaneous agent that causes hypersensitivity pneumonitis
Oxygen
How does medication cause pulmonary fibrosis
Unknown
How much radiation usually causes ILD/ fibrosis
Greater than 6,000 rad over 6 weeks
How long after radiation does it take for the development of acute pneumonitis
2-3 months
When after radiation does late fibrotic stage happen
6-12 months accompanied with pleura effusion
(Can develop without acute pneumonitis)
What causes radiation induced lung disease
Unknown
Autoimmune diseases that can cause ILD
Rheumatoid Arthritis
Sarcoidosis
Lupus/ erythematosis
How does Rheumatoid Arthritis cause ILD
The relieving medications could cause lung complications
What percent of people with Lupus have pulmonary complications
50-70%
What does sarcoidosis do to the lungs
Tubercles form (they are non-case acting granulomas)
What group is at a higher risk of sarcoidosis
Black women
What age is sarcoidosis likely to show up
10-40 years old
(Peak: 20-30)
What immunoglobulin increase with sarcoidosis
IgM, IgG and IgA
What is idiopathic interstitial pneumonias
A group without specific pathogen/ we don’t know the cause
What is the incidence of idiopathic pulmonary Fibrosis in North America and Europe
3-9 per 100,000 a year
What does a CT of idiopathic pulmonary fibrosis look like
Usual Interstitial pneumonia
(Honeycombing and traction bronchiectasis)
What ILD “specific pathology” should we remember
PULMONARY VASCULITIDES
Wegener’s granulomatosis
What does wegeners look like in the lungs
Numerous nodules of 1-9 cm diameter (usually in the upper lobes
Describe Goodpasture’s syndrome
Attacks lungs and kidneys
Causes recurrent episodes of pulmonary hemorrhage (pulmonary fibrosis in some)
Other names for goodpasture
Anti-GMB disease
Anti glomerular basement membrane disease
Physicians call it GMB
What happens with goodpastures
Circulating antibodies are directed to antigens and produces intrinsic to glomerular BM and alveolar membrane
What is the result of Goodpasture
Glomerulonephritis
Alveolar hemorrhage
Incidence and survival after diagnosis of anti-GMB
Less that 2 per million population
Survival after 15 weeks
50% massive pulmonary hemorrhage
50% die from chronic renal failure
What autoimmune disease other than RA causes damage to multiple organs
Sarcoidosis
What does sarcoidosis look like
Granulomas on multiple organs (including lymph nodes)
How is sarcoidosis diagnosed
Bronchoscopy or biopsy
What is usual pneumonia on a high resolution CT Scan a sign of
Idiopathic interstitial lung disease
Name a feature of usual interstitial pneumonia seen on a high resolution CT Scan
Honeycombing
Name two antifibrotics
Nintedanib
Pirfenidone
Ways to get a tissue sample for ILD
Trans-bronchial biopsy
Video assisted thoracoscopic surgery
Thoracotomy
