Restrictive d/o Flashcards
What is idiopathic fibrosing interstitial pneumonia (AKA idiopathic pulmonary fibrosis)?
COMMON!
chronic, progressive interstitial fibrosis caused by inflammation
happens to lung parenchyma
What occurs in the lungs as a result of fibrosis in idiopathic pulmonary fibrosis?
Prevents alveoli from inflating - leads to shunting
Capillaries get occluded by fibrosis - leads to dead space
Capillary membrane thickens - leads to decreased diffusion
What are the signs and sx of idiopathic pulmonary fibrosis?
gradual dyspnea, non-productive cough
inspiratory crackles at lung bases, clubbing of fingers
How will you dx idiopathic pulmonary fibrosis?
Bx: honeycombing
CXR/CT: honeycombing, ground glass opacities
PFT: normal or high (restrictive pattern)
DLCO: decreased
How do you treat idiopathic pulmonary fibrosis?
lung transplant
manage by smoking cessation, 02
What is hypersensitivity (HS) pneumonitis?
lung inflammation affecting alveoli and bronchioles;
caused by exposure to dusts, molds, chemicals
Type IV HS reaction (cell-mediated)
What are some examples of HS pneumonitis?
Farmer’s lung (moldy hay)
Bird Breeder’s lung (bird feces, feathers)
Chemical Worker’s lung (plastics, polyurethane manufacturing)
What are the symptoms of acute HS pneumonitis?
Rapid - flulike w/ dyspnea 6-8h post exposure; resolving
inspiratory crackles
What are the sx of subacute HS pneumonitis?
intermittent and gradual
dyspnea, productive cough, anorexia, wt loss, pleuritis without fever and chills
What are the sx of chronic HS pneumonitis?
NO hx of acute episodes
progressively worsening dyspnea, wt loss, clubbing, tachypnea
What will you see with biopsy of HS pneumonitis?
granulomas, less organized in acute phase
How do you dx HS pneumonitis?
CXR: diffuse interstitial pattern, opacities in lower lung field, apices spared
CT: ground glass appearance
PFT: normal or high (restrictive pattern)
DCLO: decreased
How do you treat HS pneumonitis?
avoid antigen
corticosteroids
and wish them good luck w/ chronic
What is pneumoconiosis?
chronic lung disease due to inhaled particulate matter
chronic (parenchymal) fibrotic lung disease
What are examples of pneumoconiosis?
Silicosis, coal worker’s pneumoconiosis (CWP), berylliosis, and asbestosis
What is silicosis?
exposure to silica/quartz, seen in miners
What are the S/S of silicosis?
asymptomatic
dyspnea on exertion, nonprod cough
How do you dx silicosis?
CXR - nodules, lymphadenopathy or “eggshell calcifications”, fibrosis in upper lobes
What does silicosis put a pt at increased risk for?
2ndary infections like TB and CT disorders
What is coal worker’s pneumoconiosis (cwp)?
inhalation of coal dust
What are the s/s of cwp?
pattern of COPD - worse with smokers
How do you dx cwp?
cxr: small upper lobe nodules, hyperinflation of lower lobes (looks like emphysema)
What is berylliosis?
inhalation of nuclear reactors like electronics, manufacturing places, light bulbs
What are s/s of berylliosis?
similar to silicosis (DOE, nonprod cough)
w/ joint pains, wt loss, fever
How do you dx berylliosis?
beryllium lymphocyte proliferation test
sometimes CXR shows nonspecific abnormalities
Looks like sarcoidosis w/ granulomas, but less lymphadenopathy
How do you tx berylliosis?
steroids and 02
if those fail: methotrexate
What can berylliosis lead to?
increased risk of lung, stomach, colon CA
What is asbestosis?
LT inhalation of asbestos; seen 15-20y post exposure
What are the s/s of asbestosis?
similar to silicosis
(asymptomatic +/- DOE, nonprod cough)
How do you dx asbestosis?
CXR: pleural plaques, honeycombing, in lower lobes
“shaggy heart sign”
Bx: asbestos bodies (brown rods)
How do you tx asbestosis?
Supportive mostly; steroids, 02, lung transplant
smoking cessation
What does asbestosis put pt at an increased risk for?
mesothelioma (most common pleural CA), carcinoma of bronchioles, TB
What should you enjoy?
Every sandwich
What is sarcoidosis (pathophys)?
increased T cell response –> granulomas in the lungs
granulomas –> fibrosis
lungs MC affected, but also skin, eyes, lymph nodes
What causes sarcoidosis?
unknown
genetic and environmental factors lead to disordered immune regulation
Who gets sarcoidosis?
20-40y
higher risk in AA, Northern Europeans, females
What are s/s of sarcoidosis?
1/2 are asymptomatic and found incidentally
pulm: nonprod cough, dyspnea, chest pain
lymphadenopathy
skin: erythema nodosa, lupus pernio (raised discoloration of nose, ear, chin, cheek) - pathognomonic, maculopapular rash, nodules, parotid enlargement
visual: uveitis, conjunctivitis, cataracts/glaucoma
heart: arrhythmias
rheum: arthralgias, fever, malaise, wt loss, hepatosplenomegaly
neuro: CN palsies, lesions
(So basically, pt enters room. Differential: sarcoidosis)
How do you dx sarcoidosis?
1) compatible clinical/radiologic findings
2) presence of noncaseating granulomas on bx
3) exclusion of other diseases
What will you find on radiographs of sarcoidosis?
CXR: bilateral hilar lymphadenopathy, interstitial lung disease pattern
CT high res: LA, nodules, ground glass opacities, infiltrates
can also do Gallium scan
What will PFT of sarcoidosis show?
PFT restrictive in advanced, but can be normal or obstructive pattern
What lab findings will you see with sarcoidosis?
increased ACE levels
hypercalcemia/uria
eosinophilia
cutaneous anergy (dec skin activity to common skin antigens)
How do you treat sarcoidosis?
Many have spontaneous remission w/in 2y
if tx is needed: PO corticosteroids
NSAIDs for MSK sx
What is the prognosis for sarcoidosis?
good!
40% spontaneously resolve
40% better w/ tx
20% irreversible lung injury
