Restrictive Pulmonary Disorders Flashcards
The disease process is often chronic and can lead to irreversible
interstitial fibrosis causing derangement of gas exchange
Usually present with exertional dyspnea and dry cough of insidious
onset
Idiopathic Pulmonary Fibrosis
What is the most common diagnosis in Interstitial lung diseases patients?
idiopathic pulmonary fibrosis
CXR findings in this disease:
Ground-glass, nodular, or “hazy” appearance that may progress to
“honeycomb” lung
idiopathic pulmonary fibrosis
“Velcro” crackles is a characteristic finding of what disease?
idiopathic pulmonary fibrosis
What is the most common diagnosis presenting with interstitial lung disease?
Idiopathic Fibrosing Interstitial Pneumonia
Multisystem disease of unknown etiology characterized by granulomatous inflammation
Inflammatory disease, chronic multisystem disorder: can affect lung, skin, eye, liver, lymph nodes
Sarcoidosis
What location is the number one place for involvement in sarcoidosis?
Lung
Sarcoidosis incidence highest in what demographic(s)?
North American blacks and northern European whites (Nordic) – Norway, Sweden, Denmark, Iceland, Finland
What is the hallmark in sarcoidosis?
“Granuloma”
In sarcoidosis, what percentage of patients present with pulmonary findings?
> 90%
What are some extrapulmonary manifestations of sarcoidosis?
S – skin: erythema nodosum, lupus pernio
H – heart: conduction abnormalities
O – ocular: uveitis
P – parotid gland enlargement
What enzyme is increased in 66% of sarcoidosis patients?
Angiotensin Converting Enzyme
What test is a definitive diagnosis in sarcoidosis?
Biopsy via bronchoscopy or mediastinoscopy
Noncaseating granulomas should have you suspect what disease?
sarcoidosis
Sarcoidosis involvement where is associated with the worst prognosis?
Involvement of parenchyma
Sarcoidosis involvement located solely here is associated with the best prognosis?
Outlook is best if only hilar adenopathy
Chronic fibrotic lung diseases caused by inhalation of various dusts
Pneumoconiosis
Clinically important pneumoconiosis include:
Asbestosis
Silicosis
Coal worker’s pneumoconiosis
Which pneumoconiosis is described below?
usually rare by itself
Pleural plaques
Cigarette smoking accelerates disease and risk of lung CA
Associated with mesothelioma
Asbestosis
Which pneumoconiosis is described below?
typically seen in construction workers
Prolonged inhalation of agent causes formation of small rounded opacities throughout lungs
Increased incidence of pulmonary TB (30x) and atypical mycobacterial disease – unsure why
Progressive pulmonary fibrosis
May be rapidly fatal in <2 years
Often presents 20 years after exposure
Silicosis
Which pneumoconiosis is described below?
chronic fibrotic lung disease caused by inhalation of this worker’s exposure at work
coal dust is ingested by alveolar macrophages
Coal worker’s pneumoconiosis
What is the oldest known occupational pulmonary disease?
Silicosis
CXR findings in what disease?
“egg shell” calcification 🡪 very thin calcification
around the nodule
More commonly in upper lobes
Silicosis
