Restrictive Lung dz packet - Ms. Anna Flashcards
What is restrictive lung dz characterized by?
Decreased lung volume and decreased lung capacity due to either alteration in the lung parenchyma or disease of the pleura, chest wall, or neuromuscular apparatus
Describe how alteration in lung parenchyma relates to restrictive lung dz?
“intrinsic” or “interstitial” dz
Disease causes inflammation, scarring, or results in filling of air spaces with exudate or debris
Describe how diseases of pleura, chest wall, or neuromuscular apparatus relate to restrictive lung disease?
“extrinsic”
Chest wall, pleura, or respiratory muscles are not functioning normally
What are some examples of extrinsic disease that involve the chest wall?
Trauma
Kyphoscoliosis
Ankylosing spondylitis
Neuromuscular disease like myasthenia gravis/guillain barre
morbid obesity
scleroderma
What are some drug induced causes of interstitial lung disease?
Chemotherapy
Methotrexate
Amiodarone
Macrobid
What are some autoimmune related causes of interstitial lung disease?
RA
SLE
Scleroderma
Polymyositis
Dermatomyositis
Sjogrens syndrome
What is the most common hypothesized etiology of interstitial lung disease?
Both environmental and genetic factors contribute
Usually idiopathic
Most identifiable causes include infectious, drug-related, or environmental/occupational
What does interstitial lung disease cause?
thickening of the interstitium, a part of the lung’s structure
Inflammation, scarring, or extra fluid can result in the thickening
Can be acute or chronic
What are the pathophysiological changes in ILD?
After injury/exposure:
-A repair process is initiated resulting in progressive, patchy, and diffuse thickening of the alveolar walls with connective tissue and inflammatory cells
What is the functional abnormality of ILD?
Reduced lung volume/capacity
What are the symptoms of ILD?
Dyspnea**
-usually insidious and progressive
Dry chronic cough
Extrapulmonary symptoms may be noted depending on dz
What are the signs of ILD?
Early - normal
Advanced:
-tachypnea
-bilateral crackles
-clubbing
-right sided heart failure
What all should be in the workup for ILD?
CXR
PFTs
CT chest
Blood tests
Bronchoscopy with biopsy
Surgical biopsy
What are the common CXR findings in ILD?
Low lung volumes
Patchy “ground glass” appearance
Hallmark: reticular or nodular opacities
What are the common Chest CT findings in ILD?
HRCT:
Reticular opacities
interlobular septal thickening
Honeycombing (late)
Nodules
Traction bronchiectasis
What will be seen on spirometry in ILD?
Decrease lung volumes
Decrease vital capacity
What is the use of Bronchoscopy with biopsy and surgical biopsy for ILD?
Bronchoscopy with biopsy is diagnostic for sarcoidosis
Surgical biopsy is the definitive diagnostic study
What are the pharmacological treatment options for ILD?
Corticosteroids
Cytotoxic agents
Immunosuppressive agents
What are the non-pharmacological treatment options for ILD?
Smoking cessation
Supplemental O2 therapy
Avoid exposure/DC meds
Tx pulmonary infections
Pulmonary rehabilitation
Lung transplant (definitive)
Define idiopathic pulmonary fibrosis?
A subtype of interstitial lung disease that results in fibrosis of the lungs without a known cause
What is the etiology of idiopathic pulmonary fibrosis?
Cause is unknown
More commonly effects older adults (65+)
Hypothesized genetic link
What is the pathogenesis of idiopathic pulmonary fibrosis?
An inciting incident disrupts homeostasis of alveolar epithelial cells causing diffuse epithelial cell activation and abnormal cell repair
Exaggerated accumulation of extracellular matrix - destruction of the lung parenchyma
Idiopathic pulmonary fibrosis clinical manifestations?
limited to lungs - systemic symptoms rare
Onset is gradual and not often diagnosed for 1-2 years
Most common complaint is dyspnea, 2nd is non-productive cough
What is found on PE for idiopathic pulmonary fibrosis?
End-respiratory crackles
What is found on CXR for idiopathic pulmonary fibrosis?
Diffuse reticular opacities; not diagnostically specific
What is seen on HRCT for idiopathic pulmonary fibrosis?
Diffuse reticular pattern, honeycombing
Describe use of a 6-minute walk test in idiopathic pulmonary fibrosis?
If O2 drops below 88%, mortality risk is increased
If HR fails to decline 1-2 min after exercise, indicates poor prognosis
What is a helpful diagnostic test for idiopathic pulmonary fibrosis?
Bronchoalveolar lavage with fluid analysis
What is the diagnostic criteria for idiopathic pulmonary fibrosis?
> 65 years old
Idiopathic dz
Restrictive pattern on PFT
Progressive scarring on CXR
honeycombing on HRCT
How is idiopathic pulmonary fibrosis treated?
Treat underlying diseases
Supplement O2 if sat <88% on room air
Smoking cessation
Vaccinations
Symptom management
Drug therapy - anti-fibrotic drugs, immunosuppressants
Lung transplant is the ONLY TX to prolong survival
What is the prognosis for idiopathic pulmonary fibrosis?
Poor
2-5 years after diagnosis
What is the etiology of asbestosis?
A group of naturally occurring, heat-resistant fibrous silicates that are found:
-shipyards
-construction sites
-pipe fitters
-insulation
-cement
-shingles
-vinyl floor
-textiles
What is the typical presentation of asbestosis?
about 10-20 years after exposure:
-Chronic SOB
-Clubbing
-Inspiratory crackles
What are the CXR findings of asbestosis?
Linear streaking at bases
Diffuse infiltrate
Pleural calcifications
What is to be noted about CT of chest in asbestosis?
Best imaging method
What are the complications for asbestosis?
Pulmonary HTN
Right sided HF
Progressive pulmonary insufficiency
Malignancy - lung and mesotheliomas (6x)
What is the etiology of silicosis?
Inhalation of free silica:
rock mining
stone cutting
sand blasting
pottery
What are the CXR findings of silicosis?
Small round opacities
Calcifications of hilar nodes (strong indication)
Eggshell or popcorn calcification
What is the prognosis of silicosis?
Simple silicosis is usually asymptomatic with no effects of PFTs
What are the complications of silicosis?
Higher incidence of pulmonary TB
Chronic exposure can lead to scarring or fibrosis
Acute silicosis can be rapidly progressive and lead to cardiorespiratory failure
What is the etiology of coal worker’s pneumoconiosis?
Chronic inhalation of dust from high-carbon coal and rarely graphite
Typically 20+
Alveolar macrophages ingest dust leading to coal macules 2-5mm in diameter
What is found of CXR for coal worker’s pneumoconiosis?
Diffuse small opacities prominent un upper lobes
nonspecific
Caplan syndrome: Necrotic nodules in lung tissue - RA or CWP
What is the prognosis for coal worker’s pneumoconiosis?
Can be asymptomatic with little to no change in PFT
Prognosis depends on severity and length of exposure
Severity of disease may depend on the type of coal
What is the etiology of hypersensitivity pneumonitis?
Immune system disorder that occurs in some people after they breathe in certain organic antigens triggering inflammation
What are specific triggers for hypersensitivity pneumonitis?
Non-asthmatic, nonatopic inflammatory pulmonary disease:
Bacteria or mycobacteria
Fungi or molds
Bird excrements/feathers
Animal furs
Mold in air conditioners or ventilation
Bacteria in hot tub
Moldy hay
Contaminated food
What are the clinical manifestations for hypersensitivity pneumonitis?
Sudden onset of fever/chills, malaise, cough, SOB, nausea
4-8 hours after exposure
Crackles, tachypnea, tachycardia, and possibly cyanosis
*very sick patients
What imaging findings are associated with hypersensitivity pneumonitis?
CXR:
Diffuse reticular consolidation pattern
Fibrosis in late/chronic stages
HRCT:
ground glass appearance
What is the treatment/prognosis for hypersensitivity pneumonitis?
Severe - steroids
Based on many factors including identification and avoidance of agents
If not diagnosed properly or poorly controlled, disease can lead to irreversible lung damage
What is sarcoidosis?
A multisystem disorder characterized by accumulation of noncaseating granulomas in involved tissues
Bilateral hilar lymphadenopathy = sarcoidosis
What is the etiology of sarcoidosis?
Unknown
Both genetic and environmental factors likely involved
It is not malignant
What is the epidemiology for sarcoidosis?
AA
F>M
northern european caucasians
Onset usually in third or fourth decade
General manifestations of sarcoidosis?
Malaise, fever, and dyspnea
Arthralgias
anorexia
What are the pulmonary manifestations of sarcoidosis?
Dyspnea on exertion
cough
chest pain
***Crackles are UNCOMMONWh
What are some other manifestations of sarcoidosis?
Ocular and skin involvement
Cardiac involvement
Hepatosplenomegaly
Encephalopathy
Renal involvement
What are the key points of sarcoidosis clinical manifestions?
Multiorgan disease that primarily effects the lung tissue
Granulomas can infiltrate any organ leading to pathology
Some patients may be asymptomatic
What is the skin finding in sarcoidosis?
lupus pernio
What is the ocular finding in sarcoidosis?
Granulomatous uveitis
How is sarcoidosis worked up?
exclude other disorders and determine what body systems are affected
Labs:
-CBC leukopenia
-elevated CSR/ACE levels
-R/O TB
-Hypercalciuria may be present
What is the imaging/diagnostic procedure for sarcoidosis?
Bilateral hilar adenopathy
Transbronchial biopsy via fiberoptic bronchoscopy is diagnostic
How are asymptomatic sarcoidosis patients treated?
Need close monitoring:
-Serial eye examination
-CXR
-EKG
-Labs
How should mod-severe sarcoidosis be treated?
oral corticosteroid (prednisone)
-long term therapy over months to years
-serum ACE levels usually fall with clinical improvement
Describe the stages of sarcoidosis?
Stage 0: no sign of granulomas
Stage 1: Granulomas in lymph nodes only
Stage 2: Granulomas in both lymph nodes and lungs
Stage 3: Granulomas present in the lungs only
Stage 4: Scarring of the lung tissue and permanent damage
