ILD Flashcards
______ primarily due to deficiency of surfactant in immature lungs
Respiratory distress syndrome (RDS)
*common in preterm infants
the more _____ the infant is, the more likely to suffer RDS
preterm
32 weeks more likely to have RDS than 36 weeks
what is the physical exam of RDS?
presents within minutes-hours after birth
worsens over 1st 48 hrs of life
tachypnea
nasal flaring
expiratory grunting
intercostal, subxiphoid, subcostal retractions
cyanosis
urine output in 1st 24-48 hr is low due to increased vasopressin
peripheral edema
what is the first line of treatment for baby with RDS?
CPAP
what are common CXR findings with RDS?
low lung volume
classic reticulograndular ground glass appearance with air bronchograms
what labs may you see with RDS?
ABG - hypoxemia
hyponatremia due to water retention
what other diseases must you distinguish RDS from?
transient tachypnea of the newborn (more mature infant) bacterial PNA pneumothorax cyanotic congenital heart disease ILD
how to prevent RDS?
prevent preterm birth antenatal steroids (given to all women 23-34 weeks gestation at risk of preterm delivery in next 7 days)
how to manage RDS?
nasal CPAP
OR
intubation + surfactant therapy + mechanical ventilation
if CPAP not working in baby, what do you do next?
intubate baby and administer surfactant therapy
non-TB mycobacteria infections (NTM) can cause what 4 clinical syndromes?
pulmonary disease (MAC and mycobacterium kansaii) superficial lymphadenitis (MAC) disseminated disease (MAC) skin and soft tissue infection (mycobacterium marinum and mycobacterium ulcerans)
unlike TB, NTM are not due to ______?
human-to-human or animal-to-human transmission
what are S&S of MAC?
looks like TB but less severe
fatigue, malaise, weakness
cough
dyspnea, chest discomfort
what is the clinical presentation in those with underlying lung disease and MAC?
primarily white, middle-aged, or elderly men, often alcoholics and/or smokers with underlying COPD
what is the clinical presentation in those without underlying lung disease and MAC?
nonsmoking women over age 50 who have interstitial pattern on CXR
what diagnostic test distinguishes MAC vs TB?
positive skin test
what antibiotics recommended for MAC?
azithromycin + rifampin + ethambutol
how long is antibiotic treatment for MAC usually?
15-18 months
what are etiologies to ILD?
sarcoidosis
exposures (asbestos, coal dust, aluminum)
drugs (macrobid, amiodarone)
idiopathic
what is the common S&S for ILD?
progressive DOE and nonproductive (dry) cough
what are physical exam findings for ILD?
crackles
inspiratory squeaks
what are CXR findings for ILD?
abnormal CXR ground-glass apperance hazy opacity reticular "netlike" MOST COMMON honeycombing
what PFTs will you note with ILDs?
decreased TLC
decreased FEV1 and FVC
normal FEV1/FVC ratio or increased
what are PFTs of an obstructive disease (i.e. asthma)?
TLC increased
FVC normal
FEV1/FVC ratio decreased
what is the bronchodilator challenge?
for obstructive diseases
give pt bronchodilator (beta agonist inhaled)
if FEV1 improves over 12%, obstruction may be reversible or partially reverisble
what may be the only PFT finding in early ILD?
reduced diffusing capacity of lung for carbon monoxide (DLCO)
what is the gold standard for diagnosing ILD?
lung biopsy
what are the indications for lung biopsy?
atypical or progressive symptoms
age < 50 y/o
fever, weight loss, hemoptysis
ILD symptoms with normal or atypical CXR
what are the types of lung biopsies?
fiberoptic bronchoscopy with transbronchial lung biopsy
thoracoscopy
open lung biopsy
what are complications of ILD?
PH leading to cor pulmonale
pneumothorax
elevated cancer risk
progressive respiratory insufficiency
you determine ILD, but no clinical recovery or specific systemic disease yet? what are your next steps and what are steps after those?
bronchoalveolar lavage or transbronchial lung biopsy (TLB)
THEN if no specific dx
HRCT, if still nothing THEN
lung biopsy
define pneumoconiosis
any disease of the respiratory tract due to inhalation of dust particles (asbestotis, silicosis)
list facts about pneumoconiosis
asbestotis presents after 10-15 years of exposure
age of dx at 40-75 y/o
construction is high risk occupation
asbestos linked to lung cancer and malignant mesothelioma
what is almost always associate with asbestos exposure?
mesothelioma
what is the physical exam findings for asbestosis?
no specific S&S
insidious onset - dyspnea, reduced exercise tolerance
digital clubbing (30-40% pts)
what are CXR findings for asbestosis?
opacities in lower lungs and pleural plaques
what is needed to diagnose asbestosis usually?
consistent H&P, symptoms, and CXR
what are the PFT findings of asbestosis?
decreased TLC
FEV1/FVC ratio normal
most important preventative measures against asbestosis?
smoking cessation
avoid exposure
protective mask
list facts on sarcoidosis
multisystem inflammatory disease with unknown etiology noncaseating granulomas in lungs prevalence in African Americans 20-40 y/o female predominance asymptomatic to multi-system disease
what are physical exam findings for sarcoidosis?
fever, anorexia, arthralgias (MOST COMMON 3)
DOE and dry cough
arthritis, cranial nerve palsies, visual disturbances, and erythema nodusum
classify the different stages on CXR for sarcoidosis (stage 0-4)
stage 0: normal
stage 1: hilar adenopathy
stage 2: hilar adenopathy + diffuse infiltrates
stage 3: only diffuse parenchymal infiltrates
stage 4: pulmonary fibrosis
what are the PFTs for sarcoidosis?
decreased DLCO
decreased TLC
decreased FEV1
FEV1/FVC ratio normal
what are lab findings with sarcoidosis?
hypercalcemia
increased ESR
increased serum protein
increased serum ACE
how to diagnose sarcoidosis?
fiberoptic bronchoscopy with transbronchial biopsy
how to manage sarcoidosis?
consult pulmonologist
NSAIDS
corticosteroids - MAINSTAY for severe disease
monitor CXR and ACE level
list facts on granulomatosis with polyangiitis (aka Wegener’s granulomatosis)
immune-mediated, systemic vasulitis necrotizing granulomas of upper/lower respiratory tracts renal involvement/glomerulonephritis multiple organ system involvement 40-50 y/o (men or women) can result in ESRD
what are common upper airway symptoms associated with Wegener’s granulomatosis?
rhinorrhea, purulent/bloody nasal discharge
oral/nasal ulcers, sinus pain
what are PFT findings in Wegener’s granulomatosis?
restrictive/obstructive pattern
decreased DLCO
decreased lung volume if diffuse interstitial involvement present
what does CXR look like with Wegener’s granulomatosis?
opacities
nodules which may cavitate
what does CT look like with Wegener’s granulomatosis?
irregular and stellate-shaped peripheral pulmonary arteries (vascultitis sign)
what are lab findings with Wegener’s granulomatosis?
increased ESR (greater than 100)
leukocytosis, thrombocytosis, normocytic anemia
increased BUN/Cr
+ ANCA
how to diagnose Wegener’s granulomatosis?
difficult to distinguish
compatible clinical presentation and histopathology
how to manage Wegener’s granulomatosis?
consult rheumatologist
treat with immunosuppressant (methotreaxate or cyclophosphamide) and glucocorticoid
lists facts about idiopathic pulmonary fibrosis
most common of the 7 idiopathic interstitial PNAs
inflammation and fibrosis of lung parenchyma
diagnosis made efter excluding other causes of ILD
70% are current/former smokers
slight male predominance
insidious onset
what is most common symptom of IPF?
DOE
what are physical exam findings with IPF?
inspiratory crackles
digital clubbing
what are CXR findings with IPF?
bilateral diffuse reticular or reticulonodular infiltrates, periphery and bases
what diagnostic tool to meet criteria for dx of IPF?
lung biopsy (thoracoscopy or open)
what are PFT findings for IPF?
decreased TLC
normal FEV1/FVC ratio
how to manage IPF?
consult pulmonologist
evaluated for lung transplant (most common indication = IPF)
