Test 5 Ch. 27 Interstitial Lung Diseases Flashcards
This refers to a broad group of inflammatory lung disorders
interstitial lung disease (ILD)
ILD is also called (3)
- diffuse interstitial lung disease
- fibrotic interstitial lung disease
- pulmonary fibrosis
More than _____ disease entities are characterized by _____, ________, or _______ inflammatory of the alveolar walls by cells, fluid and connective tissue
180
acute
subacute
chronic
If ILD is left untreated the inflammatory process can progress to
irreversible pulmonary fibrosis
The anatomic alterations of ILD may involve the (3)
- bronchi
- alveolar walls
- adjacent alveolar spaces
In severe cases the extensive inflammation leads to…(4)
- pulmonary fibrosis
- granulomas
- honeycombing
- cavitation
During this stage of any ILD, the general inflammatory condition is characterized by edema and the infiltration of a variety of WBC in the alveolar walls and interstitial spaces
Acute stage
This stage may be followed by future interstitial thickening, fibrosis, granulomas, and in some cases, honeycombing and cavity formation. Pleural effusion may be present
Chronic stage
Interstitial lung disorders produce a
restrictive lung conditions
Pathologic and structural associated w/ chronic ILD: (only 1 that does not have atelectasis)
- Destruction of the alveoli and adjacent pulmonary capillaries
- Fibrotic thickening of the respiratory bronchioles, alveolar ducts and alveoli
- Granulomas
- Honeycombing and cavity formation
- Fibrocalcific pleural plaques
- Bronchospasm
- Excessive bronchial secretions
B/c there are more than 180 different pulmonary disorders classified as ILD, it is helpful to group them according to (3)
- their occupation or environmental exposure
- disease associations
- specific pathology
Occupation/ Environment : Inorganic Substance Exposure
- Asbestosis
- Coal dust
- Silica
- Beryllium
- Aluminum
- Barrium
- Clay
- Iron
- Certain talcs
Occupation/ Environment: Organic Exposure
- Hypersensitivity pneumonitis
- Moldy hay
- Silage
- Modly sugar cane
- Mushroom compost
- Barley
- Cheese
- Wood pulp, bark, dust
- Cork dust
- Bird droppings
- Paintis
Occupation/ Environment: Medication: illicit drugs
- Antibiotics
- Anti-inflammatory agents
- Cardiovascular agents
- Chemotherapeutic agents
- Drug-induced systemic lupus erythemaatosus
- illicit drugs
Occupation/ Environment: other
- Radiation
- Irritant gases
Exposure to asbestos may cause ____________, a form of ILD
asbestosis
There are two primary types of asbestos:
- amphiboles (crocidolite, amosite, anthophyllite)
- chrysotile (most commonly used in industry)
Asbestos fibers typically range from __ to ___ in length and are about ___ in diameter
50 to 100;
0.5 um
The ____________ have the longest and strongest ___________.
chrysotiles;
fibers
What is most common in diagnosis in pts with an asbestos exposure history
Pleural calcification
The pulmonary deposition and accumulation of large amounts of coal dust cause
coal worker pneumoconiosis (CWP)
CWP is also known as
coal miner lung and black lung
Simple CWP is characterized by the presence of
pinpoint nodules called coal macules (black spots)
The coal macules often develop around _____ and _________- generation respiratory bronchioles and cause adjacent alveoli to retract, which is called ________ emphysema
first and second;
focal
This is characterized by areas of fibrotic nodules greater than 1 cm in diameter
progressive massive fibrosis (PMF)
The fibrotic changes in CWP are usually caused by _______
silica
This is caused by the chronic inhalation of crystalline, free silica, or silicon dioxide particles
Silicosis
Silica is the main component of more than % of the rocks of the earth
95
Simple silicosis is characterized by…….. scattered through the lungs
small rounded nodules
No single nodule is greater than
9 mm in diameter
Pts w/ silicosis are usually
symptom- free
This is characterized by nodules that coalesce and form large masses of fibrous tissue, usually in the upper lobes and perihilar regions
Complicated silicosis
In severe cases of complicated silicosis the fibrotic regions may undergo
tissue necrosis and cavitate
_____________ is a steel-gray, lightweight metal found in certain plastics and ceramics, rocket fuels and xray tubes
Beryllium
This is characterized by the development of granulomas and a diffuse interstitial inflammatory reaction
The more complex form of beryllium
Is a cell-mediated immune response of the lungs caused by inhalation of a variety of offending agents or antigens
Hypersensitivity pneumonitis
Other names for Hypersensitivity Pneumonitis (2)
- allergic alveolitis
- extrinsic allergic
Hypersensitivity Pneumonitis antigens include
- grains
- silage
- bird droppings
- feathers
- wood dust (redwood and maple)
- cork
- dust
- animal pelts
- coffee beans
- fish meal
- mushroom compost
- mold that grows on sugar cane
- barley
- straw
The inhalation of moldy hay causes
farmer’s lung
What are by far the largest group of agents associated with ILD
chemotherapeutics (anticancer agents)
What are the names of the major offenders for anticancer agents
- Bleomycin
- mitomycin
- busulfan
- cyclophosphamide
- methotrexate
- carmustine
BCNU
An antibacterial drug used in the treatment of urinary tract infections is associated with ILD
Nitrofurantoin
The excessive long-term administration of oxygen (oxygen toxicity) is known to cause diffuse
pulmonary injury and fibrosis
What is the cause of drug-induced ILD?
it is unknown
What is the diagnosis confirmed by in drug induced ILD?
open lung biopsy
____________ therapy is the management of cancer may cause ILD
Radiation
Radiation-induced lung disease is commonly divided into two major phases:
- acute pneumonitic phase
- late fibrotic phase
Is rarely seen in pts who receive a total of less than 3500 rad, but if doses in excessive 6000 rad, over 6 weeks almost always causes ILD, near the radiated areas
Acute pneumonitic
The acute pneumonitic phase develops about ___ to __ months after exposure
2 to 3
The late phase of fibrosis may develop (3)
- immediately
- w/o an acute pneumonitic period
- after symptom free latent period
When fibrosis does develop it generally happens after ___ to ____ months after radiation exposure
6 to 12
What is often associated with the late fibrotic phase?
Pleural effusion
The precise cause of radiation therapy is
unknown
radiation- therapy is diagnosed by (2)
- history of recent radiation
- open lung biopsy
The inhalation of irritant gases may cause
acute chemical pneumonitis and in severe cases ILD
This is characterized by chronic hardening and thickening of the skin caused by new collagen formation
Scleroderma
A rare autoimmune disorder that affects the blood vessels and connective tissue
Progressive systemic sclerosis (PSS)
PSS causes fibrosis degeneration of the connective tissue of the
skin, lungs, and internal organs, especially the esophagus, digestive tract, and kidney
Scleroderma is most commonly seen in _________ ages……
women 30 to 50 y/o
This is an inflammatory joint disease
Rheumatoid Arthritis
Rheumatoid Arthritis may involve the lungs in the form of (5)
- Pleurisy w/ or w/o effusion
- interstitial pneumonitis
- necrobiotic nodules w/ or w/o cavities
- Caplan syndrome
- pulmonary hypertension secondary to pulmonary vasculitis
What is the most common Pulmonary complication associated w/ rheumatoid arthritis
pleurisy
Who develops rheumatoid pleural complication
men
Rheumatoid interstitial pneumonitis is characterized by (3)
- alveolar wall fibrosis
- interstitial and intraalveolar mononuclear cell infiltration
- lymphoid nodules
This is progressive pulmonary fibrosis of the lung commonly seen in coal miners
Caplan syndrome
Caplan syndrome is characterized by ___________ ________ in the lung periphery that often undergoes ________ _____________ and in some cases calcification
rounded densities
cavity formation
This is a lymphocytic infiltration that primarily involves the salivary and lacrimal (eyes) glands and manifests by dry mucous membranes, usually the mouth an eyes
Sjogren Syndrome
Pulmonary involvement frequently occurs in Sjögren syndrome includes (3)
- pleurisy w/ or w/o effusion
- interstitial fibrosis that is indistinguishable from that of other collagen vascular disorders
- infiltration of the lymphocytes, low lymphocytes the TB tree mucus glands, mucous plugging, atelectasis and secondary infections
Sjögren syndrome occurs most often in……..
And is commonly associated w/ rheumatoid arthritis (___% of pts w/ Sjögren syndrome)
women (90%);
50%
___________________ is a diffuse inflammatory disorder of the striated muscles that primarily weakens the limbs, neck and pharynx
Polymyositis
When erythematous skin rash accompanies the muscle weakness. What is this called
Dermatomyositis
Pulmonary involvement develops in response to
- recurrent episodes of aspiration PNA caused by esophageal weakness and atrophy
- hypostatic PNA secondary to a weakened diaphragm
- drug-induced interstitial pneumonitis
Polymyositis-dermatomyositis is seen more often in…….. at about ___ ratio
women then men ; 2:1 ratio
This a multi-system disorder that mainly involves the joints and skin, it can also cause damage to kidneys, lungs, nervous system and heart
Systemic lupus erythematosus (SLE)
Involvement of the lungs appears in SLE about
50% to 70% of cases
Pulmonary manifestations are characterized by
- Pleurisy w/ or w/o effusion
- atelectasis
- diffuse infiltrates and pneumonitis
- diffuse ILD
- uremic pulmonary edema
- diaphragmatic dysfunction
- infections
What is the most common pulmonary complication of SLE?
Pleurisy w/ or w/o effusion
A common chronic disorder of unknown origin characterized by the formation of tubercles of non necrotizing epithelial tissue (non casting granulomas)
Sarcoidosis
Common sites for sarcoidosis are:
- eyes
- lungs
- spleen
- liver
- skin
- mucous membranes
- lacrimal and salivary glands
One of the clinical laboratory hallmarks of sarcoidosis is an increase in
all three immunoglobulin, IgM, IgG, IgA
Sarcoidosis is more common in
African America
Who is affected more by sarcoidosis
Women
Is progressive and inflammatory disease w/ varying degrees of fibrosis and in severe cases honeycombing
Idiopathic pulmonary fibrosis (IPF)
IPF is more chronic and death usually occurs in ___ to ___ years
4 to 10
This is characterized by connective tissue plugs in the small airways and mononuclear cell infiltration of the surrounding parenchyma
Cryptogenic organizing PNA (COP) or Bronchiolitis obliterans organizing PNA (BOOP)
COP is idiopathic and is one of the ILD in which both
restrictive and obstructive findings
This is a diffuse pulmonary disorder characterized by fibrosis and accumulation of lymphocytes in the lungs
Lymphocytic interstitial PNA (LIP)
LIP is commonly associated with
lymphoma
The diagnosis of LIP requires a
surgical lung biopsy
This is a rare lung disease involving the smooth muscles of the airways and affects women of childbearing age
Lymphangioleiomyomatosis (LAM)
The diagnosis of LAM is confirmed w/ an
open lung biopsy
LAM’s disease slowly progresses over ___ to ___ years, ending in ______ resulting from ______________ _____________.
2 to 10 years;
death;
ventilatory failure
This is a smoking-related ILD characterized by middling zone star-shaped nodules w/ adjacent thin-walled cysts
Pulmonary Langerhans Cell Histiocytosis (PLCH)
Diagnosis is confirmed histologiically by
tissue biopsy
This is a condition of unknown cause in which the alveoli become filled w/ protein and lipids
Pulmonary Alveolar Proteinosis (PAP)
What does the chest xray typically reveals in PAP
Bilateral infiltrates, prominent in the perihilar regions (butterfly pattern)
What are the 2 diagnosis for PAP
- transbronchial or open lung biopsy
- Analysis of fluid removed during WLL
This is characterized by inflammation and destruction of the pulmonary vessels
Pulmonary Vasculitides
The major categories in pulmonary vasculitides are: (3)
- Wegner granulomatosis
- Churg- Strauss syndrome
- lymphomatous granulomatosis
This is a disease of unknown cause that involves two organ systems: kidneys and lungs
Goodpasture syndrome
In the lungs recurrent episodes of
pulmonary hemorrhage and hemoptysis and in some cases pulmonary fibrosis
In the kidneys there is a glomerulonephritis characterized by infiltration of antibodies within the
glomerular basement membrane (GBM)
Goodpasture disease is usually seen in
young adults
With appropriate tx, the __ year survival rate is almost ___%
5;
80%
YELLOW PAGES
What are the clinical manifestations for ILD
- increased AC membrane thickness
- excessive bronchial secretions
Vital signsof ILD (3)
- Increased RR
- Increased HR
- Increased BP
Chest Assessment Findings for ILD: (6)
- increased tactile and vocal fremitus
- Dull percussion note
- Bronchial breath sounds
- Crackles
- Pleural friction rub
- Whispered pectoriliquy
Lab Findings in ILD
Increased hematocrit and hemoglobin (polycythemia)
Chest Xray in ILD
- Bilateral reticulondular
- Irregularly shaped opacities
- Granulomas
- Cavity formation
- Honeycombing
- Pleural effusion
- Pleural thickening
Which disease process demonstrates an increased pulmonary diffusion capacity (DLCO)
- Goodpasture syndrome
- Idiopathic Pulmonary Hemosiderosis
This is an entity of unknown cause that is characterized by recurrent episodes of pulmonary hemorrhage similar seen in Goodpasture syndrome
Idiopathic Pulmonary Hemosiderosis
This is characterized by infiltration of eosinophils and lesser extent macrophages into the alveolar and interstitial spaces
Chronic Eosinophilic PNA
Diagnosis of chronic esinophillic PNA
open lung biopsy
General management for ILD: MEDs 2
- Corticosteroids
- Immunosuppressive Agents
What the Respiratory Protocols for ILD (2)
- Oxygen Therapy
- Mechanical Ventilation
What is the tx for Goodpasture syndrome?
Plasmapheresis, which removes the anti-GBM antibodies
What is the tx for PAP
- WLL
- Bronchial lavage
Questions from the back
Which of the following is another name for hypersensitivity pneumonitis
Extrinsic allergic alveolitis
Which of the following are considered pulmonary vasculitides
- Wegener granulomatosis
- Lymphomatoid granulomatosis
- Churg- Strauss syndrome
Which asbestos is most commonly used in the industry?
chrysotile
What is another name amphiboles (a type of asbestoses) (3)
- crocidolite
- amosite
- anthophyllite
Which of the following disorders is associated with desquamative interstitial PNA and unusual interstitial PNA
Idiopathic pulmonary fibrosis
Which of the following are systemic connective tissue disease
- Rheumatoid Arthritis
- Sjögren syndrome
Which of the following pulmonary function study findings are associated with chronic ILD
- Decreased FEVT
- Decreased FVC
Which of the following hemodynamic indices are associated w/ advance or severe ILD
- Increased CVP
- Increased PA
Which of the following chest assessment findings is associated w ILD
- Bronchial breath sounds
Which of the following oxygenation indices are associated with pneumoconiosis
- Increased O2ER
- Decreased SvO2
The fibrotic changes that develop in coal work pneumoconiosis usually result from
- silica
Which of the following g is associated with ILD
- pleural friction rub
- dull percussion note
- cor pulmonale
- Elveated PA
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Inspection of ILD (4)
- Cyanosis
- Digital Clubbing
- Peripheral Edema and Venous Distention
- Nonproductive cough
Because polycythemia and cor pulmonale are associated with chronic ILD, the following may occur: (3)
- Distended neck veins
- pitting edema
- Enlarged and tender liver
