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interstitial lung disease (ILD)

large number os conditions that involve the parenchyma
-implies inflammatory-fibrotic infiltration of the alveolar walls

pathophysiology of ILD

decreased/low lung compliance
-if the lung is compliant that it is unable to adapt to the environment
restrictive pattern on PFT's (reduced TLC)
reduced diffusion capacity
hypoxemia- initially with exercise, later at rest

what "defines" restrictive lung disease

a low TLC (<80% predicted)

if someone has obstructive and restrictive how would that be described?

a reduced FEV-1% and a reduced TLC
ex- coal miners lung

what else is seen in ILD?

decreased diffusion capacity, DLCO will be low

What are the results on PFT for restrictive?

FEV-1 - decreased
FVC - decreased
FEV-1%- normal or increased
TLC - decreased
FRC - decreased

What are the results on PFT for obstructive?

FEV-1 - decreased
FVC - normal or decreased
FEV-1%- <70%
TLC - normal
FRC - normal or increased

Characteristics of obstructive:

normal or elastic lungs
normal or low elastic recoil
high airway resistance
low expiratory flow rates

Characteristics of restrictive:

Stiff lungs
high elastic recoil
normal airway resistance
high expiratory flow rates

what is the clinical presentation os ILD?

dyspnea on exertion, non-productive cough
tachypnea, bibasilar inspiratory dray crackles

may have: clubbing, cyanosis, signs of cor pulmonale

how to dx ILD

Hx (smoking,travel,occupation,family)
PE, CXR, PFT's, 6-min walk test, HRCT
Bronchoscopy, biopsy

What is more useful for diagnosing ILD? CXR or HRCT/

CXR is no sensitive enough, they can have what appears to be normal or, alveolar filling, reticular/nodular pattern

HRCT gives you a good look at parenchyma , allowing you to inspect for fibrosis, honeycombing, ground glass infiltrates, nodules

What is idopathic pulmonary fibrosis/ usual interstitial pneumonitis (UIP)?

rare, causes fibrosis/scarring- prototype ILD
-symptoms have insidious onset but steadily progress, usually in middle aged-elderly
-more common in males
cause is unknown, associated with smoking

what do the labs show/dx/tx in UIP?

HRCT- peripheral/subpleural abnormalities, lower lobes, honeycomb, cysts
dx- sx biopsy
no proven treatment

what is sarcoidosis w/lofgrens syndrome?

erythema nodosum: raised swollen nodules
bilateral hiliar adenopathy (BHA)

what is sarcoidosis?

-characterized by development of non-caseating granulomas in one or more organs
-cause is unknown, but may be form of hypersensitivity reaction
-typically occurs in young females, balacks
-may involve any organ (resp more common)

what are lab studies for sarcoid

skin tests for allergies
elevated LFT
elevated ACE levels

how is sarcoidosis dx?

-biopsies of affected organ that is demonstrating non-caseating granulomas
-must exclude other granulomatous diseases
-increased CD4/CD8 ratio

what does a CXR for sacroidosis look like?

parenchymal infiltrates
-upper/mid lung zone predominance
distortion and fibrosis

stage 0 of sarcoid on CXR

normal CXR

stage 1 of sarcoid on CXR


stage 2 of sarcoid on CXR

BHA plus interstitial infiltrates

stage 3 of sarcoid on CXR

interstitial infiltrates with no BHA

stage 4 of sarcoid on CXR

advanced fibrosis/honey combing

sarcoidosis signs associated with worse prognosis

higher CXR stage at initial diagnosis
african american
onset >40 y/o
longer duration of symptoms (>6 months)
absence of E. nodosum (Lofgren's syndorme)
involvement of more than 3 organ systems

what are some tx's for sarcoid?

1st line- corticosteroids with a slow taper
2nd line- anti-inflammatory/cyctotoxic agents

what are definite indications for tx of sarcoid?

involvement of
cardiac, eye, CNA, symptomatic hypercalcemia, symptomatic lung disease with decreasing PFT, extensive skin lesions


establishing hx of asbestos exposure and pleuropulmonary manifestation is key
-occupation commonly associated w/ exposure

what are some manifestations of asbestos?

Lung cancer
benign asbestos pleural effusion
pleural plaques

what is "asbestosis"?

ILD with onset 20 years or more after exposure
-no specific/effective therapy
no role for corticosteroids

what is mesothelioma

highly fatal malignancy occurring 30-40 years after onset of asbestos exposure
- no effective therapy
-unrelated to pleural plaques. CA of pleura of the lungs

what are pleural plaques

benign pleural thickenings that are a marker of asbestos exposure

what is hypersensitivity pneumonitis?

extrinsic allergic alveolitis
-immune disease of lung due to repeated/prolonged exposure to variety of organic dusts in a susceptible individual
-pathology=mononuclear cell infiltration/ loosely formed granulomas (no biopsy needed)
-CXR=reticulo-nodular, infiltrates (may normalize between episodes)

what are the 3 forms of hypersensitivity pneumonitis?


explain acute hypersensitivity pneumonitis?

repeated episodes of acute "pneumonia"- cough, fever, chills, dyspnea, crackles, interstitial lung pattern

explain subacute hypersensitivity pneumonitis?

slower onset, less systemic symptoms

explain chronic hypersensitivity pneumonitis?

very slow progressive with chronic interstitial abnormalities on chest x-ray

what are the common antigens of hypersensitivity pneumonitis?

farmer's lung (thermophilic actinomycetes)
bird-fancier's lung (avian proteins)
many agricultural sources reported

how do you diagnose/tx hypersensitivity pneumonitis?

dx- history, serum participating antibodies

tx- avoid antigen exposure
-corticosteroids in some cases

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