Interstitial Lung Disease DSA II Flashcards
churg strauss syndrome
systemic necrotizing vasculitis
-upper and lower resp tracts
preceded by allergic disorders
peripheral and lung eosinophilia
IgE increase
rashes
CXR for churg strauss
bilateral patchy, fleeting infiltrates
diffuse nodular infiltrates
diffuse reticulonodular infiltrates
biopsy for churg strauss
granulomatous angiitis or vasculitis
Tx for churg strauss
corticosteroids
chronic eosinophilic pneumonia
pulmonary interstitium and alveolar spaces are infiltrated by eosinos and to lesser extent macrophages
clinical for chronic eosinophilic pneomonia
over weeks to months
-fever, weight loss, dyspnea, productive cough
pulmonary infiltrates with peripheral distribution
increased eosinophils in peripheral smear
BAL with increased eosinophils
Tx of chronic eosinophilic pneumonia
dramatic response to corticosteroids within days to weeks
therapy - prolonged for months - prevent recurrence
pulmonary alveolar proteinosis
primary path process affects alveolar spaces
filled with proteinacious phospholipid material
-components of surfactant
clinical for pulmonary alveolar proteinosis
dyspnea, cough, bilateral alveolar infiltrates
high res CT for pulmonary alveolar proteinosis
crazy paving pattern
thickened interlobular septa accompanied by groundglass alveolar filling
nocardia
superimposed resp infection
with pulmonary alveolar proteinosis
Tx for pulmonary alveolar proteinosis
whole lung lavage
good prognosis
whole lung lavage
gold standard treatment for pulmonary alveolar proteinosis
-saline into lungs with percussion to dislodge material in lungs
fluid then removed
repeated until no more material is recovered (when fluid is clear)
hypersensitivity pneumonitis
hyperimmune respiratory syndrome caused by inhalation of wide variety of allergic antigens that are usually rganic
bacteria, fungi, protozoa, animal proteins, reactive chemicals
Tx for hypersensitivity pneumonitis
avoid exposure
corticosteroids speed resolution
intermittent pulmonary and systemic symptoms, progressive interstitial CXR changes, non-resolving pneumonia
suspect hypersensitivity pneumonitis
agriculture, cattle farming, bird keeping, contaminated home ventilation, hot tubs
abrupt onset of cough, dyspnea, fever, chest pain, following heavy exposure to offending antigen, Sx 4-6 hours after exposure
subsides 24 hours after removal from exposure
acute hypersensitivity pneumonitis
gradula development of symptoms and less severe intensity
subacute hypersensitivity pneumonitis
insidious progressive dyspnea, cough, weight loss, fatigue, pulmonary fibrosis and resp failure
chronic hypersensitivity pneumonitis
thermophilic actinomycetes
moldy hay, grain, silage - farming - dairy cattle
hypersensitivity pneumonitis
also from ventilation - water-related contamination
-humidifier, hot tub, air conditioner
mycobacterium avium intracellular complex
hypersensitivity pneumonitis
from ventilation and water-related contamination
CXR for hypersensitivity pneumonitis
acute disease
-diffuse reticulonodular infiltrates
chronic disease
-diffuse interstitial fibrosis
high res CT for hypersensitivity pneumonitis
ground glass opacities early
chronic - with traction bronchiectasis, honeycombing, and fibrosis
PFT for hypersensitivity pneumonitis
restrictive, small lung volume, decreased diffusion capacity
sarcoidosis
systemic granulomatous disease
-non-caseating granuloma
can effect any organ
unknown etiology
age 40-60yo
-peak 20-40yo
3.5x higher than african americans
more in women
sarcoidosis
pulmonary not necessary for Dx
abnormal CXR with mediastinal and hilar adenopathy
may lead to chronic disease
-pulmonary fibrosis
lofgrens syndrome
acute manifestation of sarcoidosis
- erthyema nodosum
- arthritis
- bilateral hilar lymphadenopathy
favorable prognosis
fatigue, night sweats, fever, weight loss, cough, dyspnea, wheeze, rash, macules, hyper/hypopigmentation, erythema nodosum, dry eyes, pain, redness, blurry vision, arrhythmia, palpitations, syncope, HAs, numbness, epigastric pain, RUQ pain, jaundice, swelling and joint stiff, muscle pain, nasal congestion, sinus pressure
sarcoidosis
rales, exp whezing, infiltration of old scars/tattoos, macolopapular lesions, erythema nodosum uveitis, iritis, scleral plaque, irregular cardiac rhythm, CN II, VII, VIII neuropathies, hepatomegaly, arthritis, palpable adenopathy
sarcoidosis
lupus pernio
affect nose, cheeks, ears in sarcoidosis
yellow nodules in eyes
can be bx to make dx of sarcoidosis
calcium in sarcoidosis
disorder of vit D
nephrolithiasis, nephrocalcinosis, hypercalcuria
Dx of sarcoidosis
clinical, radiographic, histologic evidence of noncaseating granuloma in one or more organs
bronchoscopy with transbronchial lung bx
also elevated ACE - but this is nonspecific
pathognomonic for sarcoidosis
lofgrens syndrome
staging of sarcoidosis
CXR
0 normal
1 bilateral hilar adenoapthy
2 bilateral hilar adenopathy and parenchymal infiltrates
3 parenchymal infiltrates without lymphadenopathy
4 advanced parenchymal disease with fibrosis
PFT for sarcoidosis
restrictive ventilatory defect
- concurrent obstruction
- decrased DLCO
other testing for sarcoidosis
opthalmology exam EKG CVC CMP serum ACE level Bx of involved organ
Tx of sarcoidosis
only for symptomatic patient**
prednisone - daily for 3 months
-monitor with FVC
taper off if improvement
sarcoidosis prognosis
spontaneous remission in 50% of patients at 3 years
lofgrens - best prognosis
2/3 remission after 10 years
1/3 with unrelenting disease
less than 5% die
silicosis
fibrotic lung disease cased by inhalation of crystalline silica in form of quartz
risk for silicosis
mining tunneling excavating quarrying stonework foundries sandblasting ceramics
chronic silicosis
most common
20 or more years after exposure
silicotic nodule
-whorled hyalinized collagen fibers with peripheral zone of dust laden macrophages
asymptomatic - or complain of dyspnea and productive cough
accelerated silicosis
nodules after 3-10 years after exposure
-progressive clinical course
acute silicosis
6 months to 2 years after exposure
dyspnea, cough, weight loss, resp failure and death
alveolar filling process
progressive massive fibrosis
with silicosis
lesions are at least 1 cm in diameter and often larger
upper lobes
resp failure, cor pulmonale, weight loss, death
diseases associated with silicosis
TB COPD chronic bronchitis collagen vascular disease rheumatoid arthritis scleroderma lung cancer
CXR for silicosis
symmetric nodular pattern involving upper lobes
hilar lymphadenopathy with eggshell calcification is strongly suggestive
progressive massive fibrosis - coalescence of nodules with larger mass lesions
acute silicosis displays air space and interstitial pattern on x-ray
PFT for silicosis
normal early in chronic silicosis
later mixed pattern obstructive and restrictive
Dx of silicosis
x-ray changes and history of exposure
Tx of silicosis
irreversible
avoid further exposure
TB test
smoking cessation
consider lung transplant
