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Diffuse Infiltrative Lung Disease


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Define diffuse infiltrative lund disease
Non-infectious, non-malignant processes of lower respiratory tract

--> restrictive ventilatory impairment, diffuse interstitial infiltrates

Progressive and fatal
What % of DPLD pts are asymptomatic?
Most common presentation of DPLD
Cough Abnormal CXR
Physiological impairment
What functional abnormalities are seen in DPLD?
Reduced TLC, VC, FEV1, RV
FEV1/FVC = normal
Abnormal gas exchange
What is the best way to dx DPLD using radiograph?
HR CAT scan is most sensitive way
What physical findings are most common in DPLD?
Crackles (common in IPF, rare in granulomatous dz)
Joint deformities
Skin changes
Signs of cor pulmonale
What are general features of sarcoidosis?
More common in?
Unknown etiology
non-caseating granulomas
10x more common in blacks
Most common in 20-30 yo
Organ dysfxn from presence of granulomas in tissue
What is the common presentation of sarcoidosis?
12-35% asymptomatic
Cough, dyspnea
Any pulmonary sx
Constitutional sx
Extrapulmonary involvement
What are the most common findings of extrapulmonary sarcoidosis
CNS involvement
What is seen on CXR in sarcoidosis?
Bilateral hilar adenopathy (most common)
Diffuse reticular, reticulonodular, nodular
Multiple large nodules
Diffuse alveolar infiltrates
Can be **normal**
What is the dx for sarcoidosis based on?
Dx of exclusion
What is the prognosis for sarcoidosis?
1/3 resolve spontaneously
1/3 stay the same
some have permanent loss of lung fxn (including severe fibrosis)
Death (5%)
Other than sarcoidosis, what are the other granulomatous diseases?
Hypersensitivity pneumonitis
Chronic beryllium disease
What are some sources that can cause hypersensitivity pneumonitis?
mammalian and avian protiesn
Thermophilic bacteria and fungi
What are the different syndromes of acute hypersensitivity pneumonitis?
Acute form
Subacute form
Chronic form
What are the sx of acute hypersensitivity pneumonitis?
After intermittenet and intense exposure wth sx 4-8 hrs exposure
What are teh sx of subacute and chronic hypersensitivity pneumonitis
Continual low level exposures
Insidious onset of dyspnea and fatigue

Chronic can have irreversible lung damage
What does the acute hypersensitivity pneumonitis look like on CXR?
Diffuse Ground glass appearance or air-space consolidations
What does the subacute hypersensitivity pneumonitis look like on CXR?
Fine nodular or reticulonodular pattern
What does the chronic hypersensitivity pneumonitis look like on CXR?
Mostly reticular pattern
Where is the lung damage in hypersensitivity pneumonitis?
Upper 2/3 of lung
What can be used to treat pneumonitis?
Early dx and Ag avoidance are key
Corticosteroids for tx of acute disease
What are the different forms of silicosis?
Simple and Complicated
What is simple silicosis?
Asymptomatic or chronic cough from exposure of silicon dioxide
Where is the abnormality in silicosis? What is seen on CXR in simple silicosis?
Upper zone
Small rounded opaacities <10mm diameter
What disease increases TB risk significantly?
What is complicated silicosis?
Progressive massive fibrosis
Occurs when smaller opacities coalesce
What sx are seen in complicated silocosis?
Minimal to severe dyspnea
What is seen on CXR in complicated silicosis?
Confluent nodules >10mm
What is the morphology of chronic beryllium disease? LOCATION!
Non-caseating granulomas in lung parenchyma and hilar nodes
What are examples of interstitial inflammation/fibrosis?
Bleomycin toxicity
Chronic radiation fibrosis/bronchiectasis
Idiopathic interstitial pneumonia
What things are risk factors for bleomycin toxicity?
Renal insufficiency
Dose received
Age >70
Radiation to chest
Supplemental O2
When are changes from chronic radiation seen?
4-6 monhts after therapy
When does fibrosis from radiation become stable?
9-12 months after
What is seen on physical exam in asbestosis?
Basal crackles
Clubbin (late stages)
Lower lobe linear opacities
Early fibrotic changes with HR CAT scan
What are complications of asbestos exposure?
Pleural effusion (clear spont)
Malignant mesothelioma
Lung CA increased risk
What is idiopathic interstitial pneumonia?
Group of disease w/o identifiable cause --> diffuse parenchymal lund dz
How is IIP classified?
Histological patterns
What is mean age of onset of IIP?

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