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34 Cards in this Set

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  • Back
Diffuse Parenchymal Lung Disease (Interstial Lung disease)
Idiopathic interstitial lung pnumonias are the most common. They inclue UIPs, NSIP, BOOP, and DIP
Non-specific interstitial pneumonia
-More homogenous
-no honeycombing
Bronchiolitis obliterans organized pneumonia
-Plugs of fibroblasts in airways
-CT shows areas of consolidation
Desquamative Interstitial Pneumonia
-Alveolar spaces filled with alveolar macrophages
-Cigarete smoking related
-No desquamation
-Ground glass opacifications on CT
Hypersensitivity Pneumonitis
-Only disease caused by inhalation of organic particles
-Abnormal immunological reaction of the lung to specific organic antigens
-Size and amount of particle are key factors
-Thermophilic actinomycetes, fungi, and bird droppings are the most common causes
Pathogenesis of hypersensitivity pneumonitis
antigen exposure ->accumulation of inflammatory cells ->lymphocytic alveolitis in more chronic phases -> granuloma formation -> normal repair or end up with fibrosis from continued antigen exposure
someone presents with acute fever, chills, dyspnea, cough. chest xray shows infiltrates
Could be hypersensitivity pneumonitis.
-High resolution CT shows a lot of "ground glass opacifications"
Subacute Hypersensitivity presentation
Symptoms evolve over weeks to months
Chronic hypersensitivity pneumonitis presentation
Same as IPF.
-persistent cough, shortness of breath, sputum production
-HRCT may show fibrotic changes
Diagnosis of Hypersensitivity pneumonitis
-Careful H&P
-PFTs show restrictive pattern
-Diffusion impairment and hypoxemia
-Imaging acutely shows ground glass opacifications and chronically shows more fibrotic tissue
-May have serum precipitating antibodies
-Lung biopsy is the gold standard. Will show honeycombing in lower lungs in chronic
Shipbuilder, construction, automotive, railroad
Think Asbestosis!
-Asbestos is a naturally occuring minral with therm-insulating properties
What can asbestos do?
Induces pleural disease, malignant mesothelioma, bronchogenic cancer, and asbestosis

-98% is the more benign chrysotile fiber
Define Asbestosis
-Interstitial pneumonitis and fibrosis caused by exposure to asbestos fibers. The prevalence increase with duration and intensity of exposure.

-Most cases are progressive
Describe the pathogenesis of asbestosis
-Direct toxic effects
-Release of mediators by inflammatory cells causes more inflammatory cells, fibroblast proliferation, and loss of type I and II pneumocytes
Clinical findings of asbestosis
-Progressive Dyspnea
-Bibasilar inspiratory crackels
-clubbing of fingernails
-PFTs show restriction and low DLCO

-can have honeycomb appearance
Radiographic findings of Asbestosis
-Pleural plaques
-Round atelectasis
-Basilar disease
-interlobar septal fibrosis and honeycombing in advanced cases
How do you diagnose asbestosis?
-Reliable history of exposure with latency period
-Evidence of interstitial fibrosis
-Asbestos fibers or bodies in biopsy are helpful but not necessary
Foundry work, tunneling, sandblasting, pottery making, manufacturers of brink, tiles, glass
-Chronic fibrosis disease of the lungs caused by inhalation of free silica dust
Acute silicosis
presents like ARDS. from massive exposure
Chronic or Simple Silica exposure
More like IPF with chronic cough and dyspnea. Caused by exposure to low concentrations for 20 yrs or more
Pathogenesis of Silicosis
alveolar macrophages eat silica, get activated, release inflammatory mediators, and cause inflammation and fibrosis
Someone comes in asymptomatic and you discover an abnormal chest xray and then restricted PFTs
Think silicosis. Usually it is asymptomatic. 10-15% go on to develop "progressive massive fibrosis"

On CXR, there a upper zone micronodular opacities
Diagnosis of Silicosis
-History of exposure
-Absence of other illnesses
-Reticulo-nodular and nodular infiltrates
-Upper lobe predominance
Associated illnesses with silicosis
-Mycobacterial infections (TB)
-Connective Tissue Disease like Scleroderma
-Lung Cancer
Coal workers pneumoconiosis
-exposure to coal dust and silica
-asymptomatic, small nodual opacities on CXR- ALL OVER
-Need history of exposure and CXR to diagnose
-Doesn't usually go to end stage lung disease
ILD is associated with what?
Connective Tissue Diseases
-unknown etiology
-most associated with scleroderma and rheumatoid arthritis and least with lupus
-The CTD probably causes the ILD in many cases
Discuss Rheumatoid arthritis
-Chronic symmetric polyarthritis, usually insidious onset with fatigue, loss of appetite, vague aches and pains, morning stiffness
-joint deformities
-bone erosion with sinovitis
-soft tissue around joints is swollen
-Subcutaneous nodules
ILD caused by RA
-more common in men
-infiltrates at bases
-mean survival 5 yrs
-might respond to steroids
Discuss Systemic Sclerosis
-Accumulation of connective tissue in skin and visceral organs
-Diffuse cutaneous vs limited cutaneous (CREST)
-Diffuse think antitopoisomerase
-Limited think anticentromere Ab
-Pulmonary HTN predicts a poor prognosis
"Friends say I look younger every day" or I have trouble swallowing
Think scleroderma
ILD related to Scleroderma
-Lung impairment in most patients
-Usually in base of lungs
-Lung disease is most common cause of death with scleroderma
-High risk of bronchogenic cancer too
Teat with cyclophoshamide
Reddish discolored knuckles (Gottron's sign)
Sicca Syndrome (dry and red eyes and dry mouth)
Drug-induced ILD

dyspnea, fever, infiltrates are the usual presentation
Bleomycin- dose dependent
Cyclophosphamide/methotrexate- time dependent

Amiodarone (anitarrythmia)
-DLCO of more than 80% virtually excludes it