Flashcards in Interstitial Lung Diseases Deck (75):
respiratory disease stress is common in...
(aka hyaline membrane disease)
Cause of RDS?
deficiency of surfactant in immature lung --> makes gas exchange difficult
Infants with RDS usually present within...
minutes to hrs after birth, worsens over 1st 48 hrs of life
Signs of RDS
tachypnea, nasal flaring, expiratory grunting, retractions, cyanosis
decreased BS, pale, decreased peripheral pulses, low UOP, peripheral edema
Clinical course of RDS
uncomp RDS usually progressd 48-72 hrs & improves as endogenous surfactant production is increased
exogenous surfactant dramatically improves pulm func. -> sxs resolve -> shorter course
CPAP also improves clinical course
CXAY of pt with RDS?
low lung volume
classic reticulogranular ground glass appearance with air bronchograms
ABG in pt with RDS typically shows...
hypoxemia that responds to O2
as disease progresses, may see hyponatremia due to water retention
In RDS diagnosis is...
clinical, must distinguish from other causes
How can we prevent RDS?
prevent pre-term birth when possible
Antenatal steroids given to all women 23-34 wks gestation at risk of preterm delivery in the next 7 days
Management of RDS?
or Intubation + surfactant therapy + mechanical ventilation
How is ARDS different from RDS?
ARDS is acute respiratory distress syndrome in adults, is an acute, diffuse, inflammatory lung injury that leads to:
-decreased pulmonary vascular permeability
-increased lung weight and loss of aerated tissue-assoc. w/ high mortality rate
caused by direct or indirect injury to the lung
What are the 4 clinical syndromes that can be caused by Non-TB mycobacteria infections (NTM)?
1. pulmonary disease (esp. in older pts with cystic fibrosis
2. superficial lymphadenitis
3. Disseminated disease (in severely immunocompromised)
4. skin and soft tissue infection
Main cause of NTM lung infections?
MAC (mycobacterium avium complex)
these organisms are acquired from the environment
Signs/sxs of MAC pulmonary disease?
looks like TB but less severe and often indolent
-fatigue, malaise, weakness, cough, dyspnea, chest discomfort
lung exam often norm
MAC clinical presentation in those with underlying lung disease?
-white, middle aged or elderly men often alcoholics and/or smokers with underlying COPD
resembles TB clinically &radiographically but usu. less severe
MAC clinical presentation in those w/p lung disease
non smoking women >50 y/p who have interstitial pattern on CXR
How do you dx MAC pulmonary disease?
positive sputum culture x 2
other dx have been excluded
What is bronchiectasis?
chronic condition where walls of the bronchi are thickened from inflammation and infection
Do you always need to tx MAC pulmonary disease?
NO, anti-myobacterial drugs can be dif. to tolerate
If fibrocavitary disease > TREAT
if nodular bronchiectasis > depends on presentation/status of pt
What abx should you give to a pt with MAC pulmonary disease?
depends on susceptibility
usually: Azithromycin + Rifampin + Ethambutol
for usually 15-18 months (tx until cultures -)
Interstitial lung disease aka...
What is interstitial lung disease?
includes variety of chronic lung disorders
nonmalignant/noninfectious but serious
How is the lung affected in ILD?
-lung parenchyma is damaged
-walls of the alveoli become inflamed
-Scarring (fibrosis) begins in the interstitium and the lung becomes stiff
Causes of ILD?
diseases (sarcoidosis, neurofibromatosis), exposures (asbestos), drugs (Macrobid), Idiopathic
Clinical presentation of ILD?
progressive dyspnea on exertion and non productive cough
(usually NO wheezing of CP)
PE in ot with ILD might reveal?
crackles, inspiratory squeals, cor pulmonale
advanced disease: cyanosis, digital clubbing
extrapulmonary: ass. w/ other disease ie. erythema nodusum in sarcoidosis
ground-glass appearance often early finding-hazy opacity assoc. with inflammation
reticular "netlike" most common
honeycombing --> poor prognosis
What other diagnostic tests can you use to dx ILD?
- will see restrictive defect: decreased TLC, decreased FEV1 and PVC but normal FEV1/FVC ratio
-may be norm or may show hypoxemia or respiratory alkalosis
-minor extension of bronchoscopy, allows for cellular analysis
Lung Biopsy **
What is a normal FEV1/FVC ratio?
If a patient has a + bronchodilator challenge this indicates...
obstructive disease (like asthma)
not restrictive disease (like ILD)
What is meant by V/Q mismatch?
there will be some areas of the lungs which are better perfused than ventilated and some areas that are better ventilated than perfused
What is diffusing capacity?
the ability of has to cross from air > interstitium > lung
may have reduced diffusing capacity of lung for carbon monoxide
Indications for lung biopsy?
atypical or progressive sxs
Age <50 y/o
fever, weight loss, hemoptysis
ILD sxs with norm or atypical CXR
predict prognosis before initiating therapies with serious side effects, etc.
Types of lung biopsies?
fiberoptic bronchoscopy with transbronchial lung biopsy- less invasive but less info
open lung biopsy
Complications of ILD?
pulmonary HTN, cor pulmonale, pneumothorax, elevated CA risk, progressive respiratory insufficiency
What are some known causes of ILD?
inorganic dust (asbestos), organic dust (bacteria), gases, fumes
chemo meds, abx (Macrobid), radiation tx
What is pneumoconiosis?
any disease of the respiratory tract due to inhalation of dust particles
-asbests, silicosis, coal worker's disease, berylliosis
males> females due to work hx
What are some occupations associated with risk of asbestosis?
construction, auto mechanics, pipe fitters, plumbers, welders, janitors, shipyard workers
Is mesothelioma asbestosis?
NO, it is associated with asbestos but isn't asbestosis
It is a form of CA almost always associated with asbestos exposure
-exposure can be short term
-CA develops in mesothelium
-not caused by smoking
-poor prognosis (med. survical 6-12 mo after presentation)
no specific signs/sxs
insidious onset-dyspnea, reduced exercise tolerance. usually dry cough
Asbestosis dx studies?
CXR- opacities in lower lungs, thickened pleura, pleural plaques
Definitive: open lung biopsy
PFTs - restrictive pattern
histopathology shows fibrosis and asbestos bodies
management of asbestosis?
consult pulm-may need long term O2
smoking cessation, eliminate exposure
No effective therapy/drugs not effective
Vaccinate: flue, pneumovax
Classic pt with Asbestosis?
Course of asbestosis?
lung damage is irreversible
smoking + asbestosis= 59 x more likely to get lung CA
elevated risk for mesothelioma
What is sarcoidosis?
multi-system inflammatory disease of unknown etiology
non-caseating granulomas-predominantly in the lungs
What is a granuloma?
a small inflammatory nodule of macrophages-produced in response to injury/infection, boy's attempt to "wall off" something
besides the lungs, sarcoidosis can also affect...
heart, liver, spleen, joints, skin and bones
Sarcoidosis is most common in...
10x greater in African Americans
Sxs of sarcoidosis?
asymptomatic- multi-system disease
-fever, anorexia, arthralgias, DOE, dry cough, CP
-extrapulmonary: arthritis, CN palsies, erythema nodusum
pts can have spontaneous resolution, 1/3 develop chronic disease
CXR stages in sarcoidosis?
1: hilar adenopathy
2: hilar adenopathy + diffuse infiltrates
3: only diffuse parenchymal infiltrates
4: pulmonary fibrosis
PFTs in sarcoidosis?
isolated decreased in DLCO
restrictive pattern with advanced disease (decreased TLC, norm FEV/FEV1)
What labs would you check if you are concerned for sarcoidosis?
serum protein - excess immunoglobulins
serum ACE- elevated in 60%
In most causes dx of sarcoidosis requires...
biopsy- fiberoptic bronchoscopy with transbronchial biopsy
Classic pt with sarcoidosis?
female, African American, 30ish, non smoker
she's an ACE
How do you manage sarcoidosis?
-most pts resolve spontaneously- give NSAIDS for sxs
-if severe disease --> corticosteroids (Prednisone)
-monitor: CXRS, ACE level
What is granulomatosis with polyangiitis?
aka Wegener's granulomatosis
immune mediated systemic vasculitis
characterized by necrotizing granulomas of the upper and lower respiratory tracts
75% of pts with granulomatosis with polyangiitis have...
sig. morbidity-> can results in ESRD
Sxs of granulomatosis with polyangiitis
upper airway: most common- oral/nasal ulcers, rhinorrhea, purulent/bloody nasal DC
lower airway: dyspnea, cough, pleuritis pain, hemoptysis
Pulmonary func. in pt with granulomatosis with polyangiitis?
restrictive and obstructive patterns found
CXR in pt with granulomatosis with polyangiitis? CT?
highly variable -opacities, nodules which may cavitate
blood vessels leading to nodules & cavities, irregular and stellate-shaped peripheral pulmonary aa. "vasculitis sign"
Hilar adenopathy should prompt concern for...
sarcoidosis or infection (TB or fungal pna)
Additional dx tests for granulomatosis with polyangiitis
-leukocytosis, thrombocytosis, normocytic anemia
-BUN/Cr, proteinuria if renal involvement
- + ANCA (antineutrophil cytoplasmic abs)
classic pt with Granulomatosis with Polyangiitis?
"Gary granulomatosis with polyangiitis"
male or female
upper airway sxs- in the nose
Goes to AA (+ANCA Abs)
Management for granulomatosis with polyangiitis?
may tx with immunosuppressant (methotrexate or cyclophosphamide) &glucocorticoid
Prognosis for granulomatosis with polyangiitis
with cyclophosphamide- 80% 8 yr survival rates
What is idiopathic pulmonary fibrosis?
aka usual interstitial pna
most common of the 7 idiopathic interstitial pnas
inflammation & fibrosis of the lung and parenchyma
Dx of idiopathic pulmonary fibrosis is made..
after excluding other causes of ILD
-DOE, nonproductive cough
bilateral diffuse reticular or reticulonodular infiltrates in periphery and bases
Additional dx tests for IPF?
HRCT- reticular opacities, honeycombing indicates poor prognosis
PFTs- restrictive pattern
Histopathology of IPF?
predominantly fibrosis and scant numbers of inflammatory cells: usual interstitial pneumonitis
Classic pt with IPF
a common man (common)
Management of IPF?
eval for lung transplant- most common indication for lung transplant