Interstitial Lung disease Flashcards
How is ILD characterized?
ILD is characterized by acute, subacute or chronic inflammatory infiltration of alveolar walls by cells, fluid and connective tissue
What is the result if the inflammation from ILD is left untreated?
If untreated, the inflammatory processes can progressively develop into irreversible pulmonary fibrosis
What is the occurrence of ILD in the united states?
81 out of every 100000 people have some form of ILD
What are the basic functions of the interstitium?
Support the lung structurally
Maintain fluid balance
Help repair and remodel the lungs
Describe the basic pathophysiologic pathway of ILD
Injury (inhaling shit)
Inflammation
Disordered repair of involved tissue
Pulmonary fibrosis
End stage lung disease
What happens to the alveoli in ILD?
The alveoli can be destroyed along with the adjacent pulmonary capillaries
What is the order of movement of ILD in regards to the tissues?
Moves from bronchi to alveolar walls to adjacent alveolar spaces
Describe what happens to the tissues as a result of ILD
Fibrotic thickening of the respiratory bronchioles alveolar ducts and alveoli
What can form in the lungs as a result of ILD?
Granulomas
Just what the fuck is a granuloma?
A granuloma is an aggregation of macrophages (along with other cells) that forms in response to chronic inflammation. This occurs when the immune system attempts to isolate foreign substances that it is otherwise unable to eliminate
What can form in the lungs specifically in patients with asbestosis?
Fibrocalcific pleural plaques
Describe how ILD changes the compliance of the lungs
ILD severely decreases compliance
What inorganic exposures can result in ILD?
Asbestos
Coal dust
Silica
Why is the size of asbestos fibers significant?
Asbestos fibers can be <1 micrometer in size which can allow them to be deposited very deeply in the respiratory tract, notably in the parenchyma
If you were looking at a tissue sample from someone who has asbestosis, what would it look like?
Asbestos fibers would appear within the thickened walls of the alveoli as brown or orange baton shaped structures
What can form in the lungs as a result of exposure to coal dust?
Pinpoint nodules called coal macules
Where do coal macules tend to be found?
Coal macules tend to be found in the first and second generation respiratory bronchioles and cause the adjacent alveoli to retract
What is the characteristic of complicated coal workers pneumoconiosis?
Coal workers pneumoconiosis is characterized by areas of fibrotic nodules greater than 1 cm in diameter
The nodules are composed of dense collagenous tissue with black pigmentation
Complicated CWP is characterized by nodules coalescing and forming large masses of fibrotic tissues
What is silicosis characterized by?
The formation of small rounded nodules scattered throughout the lungs
Describe hypersensitivity pneumonitis
Immune system detects antigens and produces antibodies that initiate an inflammatory response on future exposures
Pneumonitis develops after repeated/prolonged exposure to the antigen
What is complicated silicosis characterized by?
Same as complicated CWP
Nodules coalesce and form large masses of fibrous tissues, usually in the upper lobes and peripheral regions
What are examples of antibiotics that can result in ILD?
Bitrofurantoin - used for UTIs
Sulfazalazine - treating rheumatoid arthritis
What are examples of antiinflammatory agents that are linked to ILD?
Aspirin
Methotrexate
What are cardiovascular agents that are linked to ILD?
Amiodarone
T/F: Oxygen is linked to ILD
True, thats why we aggressively wean people
At what level of radiation do most patients have to worry about ILD?
> 6000 rad
Describe scleroderma
A relatively rare autoimmune disorder of the blood vessels and connective tissue
What does scleroderma do to the body?
Causes fibrous degeneration of the connective tissue of the skin, lungs and internal organs particularly in the esophagus, digestive tract, and kidney
Where are the effects of scleroderma most common found
The esophagus, digestive tract and kidneys
Can also affect the lungs
What does scleroderma of the lung appear as?
ILD and fibrosis
What does scleroderma do to the lungs
Causes significant scarring of the lung parenchyma
What are some pulmonary complications associated with scleroderma?
Diffuse interstitial fibrosis
Severe pulmonary hypertension
Pleural disease
Aspiration pneumonitis (secondary to esophageal involvement)
In what population is scleroderma most commonly seen?
Women, 30-50 yo
What is the link between rheumatoid arthritis and lung complications?
Medications used to relieve RA potentially raise risk for lung complications
RA can also be associated with fibrosis of the lungs and nodule formation
What mucous glands can Sjogrens syndrome effect that harm the lungs?
Tracheobronchial mucous glands
Glands atrophy, plugs form, atelectasis results, secondary infection to follow
How does Sjogrens syndrom affect the lungs?
Pleurisy with/without effusion
Interstitial fibrosis
Lymphocyte infiltration of tracheal bronchial mucous glands resulting in mucous gland atrophy, mucous plug formation, atelectasis and secondary infection
What occurs primarily in Sjogrens syndrome?
Lymphocytic infiltration of the salivary and lacrimal glands that results in dry mucous membranes in eyes and mouth
What population is more affected by Sjogrens syndrome?
Women
How does polymyositis/dermatomyositis affect the lungs?
Recurrent episodes of aspiration PNA due to weakening of pharynx muscles
Hypostatic PNA secondary to weakened diaphragm
Drug induced interstitial pneumonitis
Describe polymyositis/dermatomyositis
Diffuse inflammatory disorder of striated muscles that weakens limbs, neck, and PHARYNX
Describe lupus (systemic lupus erythematosus)
Autoimmune disease where the body attacks its own tissues
People with it are said to have a distinctive rash
What are the pulmonary manifestations of lupus?
Pleurisy with or without effusion
Atelectasis
Diffuse infiltrates and pneumonitis
Diffuse ILD
Uremic pulmonary edema
Diaphragmatic dysfunction
Infections
What forms during sarcoidosis?
Tubercles-noncaseating granulomas
What increases in patients with sarcoidosis?
IgM, IgG, IgA
What are the most commonly affected organ in patients with sarcoidosis?
Lungs
Who is sarcoidosis most common in?
African americans
Women
Ages 10-40
What does it mean if a disease is classified as idiopathic?
its an authoritative way of saying we dont know what causes this
What does the imaging pattern “usual interstitial pneumonia” generally mean?
Idiopathic pulmonary fibrosis
How might a CT appear in a patient with IPF (idiopathic pulmonary fibrosis)?
Honeycombing
Traction bronchiectasis (dilation of bronchi or bronchioles in areas of ILD)
Tissue plugs are found in what disease process?
Cryptogenic organizing pneumonia
Aka bronchiolotos obliterans organizing PNA
What happens in cryptogenic organizing PNA?
Connective tissue plugs in the small airways
Mononuclear cell infiltration of surrounding parenchyma
Accumulation of lymphocytes in the lungs along with fibrosis is characteristic of what disease?
Lymphocytic interstitial PNA
What happens in lymphocytic interstitial PNA
Diffuse pulmonary disorder characterized by fibrosis and accumulation of lymphocytes in the lungs
What is commonly associated with lymphocytic interstitial PNA?
Lymphoma (cancer of the lymphatic system)
What is required to diagnose Lymphocytic interstitial PNA?
Surgical lung biopsy
The common pathology of inflammation and destruction of pulmonary vessels is characteristic of what group of disorders?
Pulmonary vasculitides
What does Goodpastures syndrome cause in the kidneys?
Glomerulonephritis
What is the pulmonary manifestation of granulomatosis with polyangiitis (wegeners granulomatosis)?
Numerous nodules in the lungs 1-9 cm in diameter and more commonly found in the upper lobes
Larger lesions that may cavitate
What does Goodpasture’s syndrome cause in the lungs?
Recurrent episodes of pulmonary hemmorrhage
Pulmonary fibrosis
What is the incidence of anti-GBM disease?
Less than 2 per million
What else is Goodpasture’s syndrome called?
Anti GBM disease
Anti glomerular basement membrane disease
What is the survival diagnosis for anti-GBM disease?
50% die from massive pulmonary hemorrhage
50% die from chronic renal failure
What do patients suffering from idiopathic pulmonary hemosiderosis experience?
Recurrent episodes of pulmonary hemorrhage
What happens in chronic eosinophilic PNA?
Alveoli and interstitium are infiltrated by eosinophils and macrophages
How does chronic eosinophilic PNA present on a CXR?
Photographic negative ie lots of black spaces
What are the main clinical manifestations of ILD?
Increased alveolar capillary membrane thickness
Excessive bronchial secretions
What vital signs might be increased on a patient with ILD?
RR
HR
BP
If you perform a chest assessment on a patient suspected of having ILD, what findings would indicate the presence of ILD?
Increased tactile and vocal fremitus
Dull percussion note
Bronchial breath sounds
Fine dry crackles (velcro)
Pleural rub
Whisper pectoriloquy
During a physical examination, what might you observe in a patient with ILD
Cyanosis
Digital clubbing
Peripheral edema and venous distention
Enlarged and tender liver
What would a PFT look like from a person with ILD?
Decreased capacities, particularly FVC
Normal or increased FEV1/FVC ratio
Decreased volumes
What seemingly unrelated conditions may be indicative of ILD?
Dry mouth
Tooth or gum erosion
Abnormalities of the eyes or skin
Why would the FEV1/FVC remain normal or even increase?
ILD increases the elastic recoil force of the lungs making it easier to expel air
Which disease processes are exceptions when considering ILD and DLco?
Goodpasture’s syndrome
Idiopathic pulmonary hemosiderosis
ILD has what effect on hemodynamic indices?
Increased pulmonary arterial pressure
Increased pulmonary vascular resistance
What happens to the diffusion capacity in patients with ILD?
Diffusion capacity decreases as a result of increase AC membrane thickness
What might you see on a CBC from a patient you suspect to have ILD?
Increased hematocrit
Increased hemoglobin
A patient with ILD or suspected of ILD might have what findings on a CXR?
Bilateral reticulonodular pattern
Irregularly shaped opacities
Granulomas
Cavity formation
Honeycombing
Pleural effusion
What the fuck does reticulonodular mean?
Reticular = net like
Node = small defined mass of tissue
What might the CXR of an individual with asbestosis look like?
Cloudy or ground glass opacity
Might have diaphragmatic or pleural plaques
How do sarcoid cysts appear on a CXR?
They look like a honeycomb pattern
How can you test for ILD?
CXR (not adequate to diagnose)
High resolution CT scan
Bronchoscopy for BAL
Biopsy
What is the difference in resolution between a normal CT scan and a high resolution CT scan?
Normal resolution of 1-2 mm
High resolution of 100-200 micrometers
What is the gold standard for assessing ILD?
High Resolution CT scan
What is the best way to care for individuals with ILD?
By supporting their physical condition, understanding medications, supporting effective nutrition and education surrounding movement patterns that will allow them to conserve energy and improve their quality of life
T/F: Lung transplants are not a viable option for patients with ILD
False. They can be hard to come by but they are a good option for individuals with severe ILD
What is the focus of palliative care?
On maintaining quality of life
