Interstitial lung diseases Flashcards
usual interstitial pneumonia
patchy ares of parenchymal fibrosis and interstitial inflammation interspersed btwn normal areas
honeycombing
most important in this category-. idiopathic pulmonary fibrosis
honeycombing
cystic airspace from retraction of surrounding fibrotic tissue
desquamative interstitial pneumonia
more homogenous than UIP
large number of intra-alveolar mononuclear cells
less prominent inflammation and little associated fibrosis
uniform process
minimal architectural distortion
pigmented macros secondary to smoking
smoking important underlying cause
Respiratory bronchiolitis interstitial lung disease
related to DIP associated w/pigmented macros interstitial inflammation not present almost always a smoke smoking cessations
non-specific interstitial penumonia
mononuclear cell infiltration w/in alveolar walls uniforma process fribrosis variable, but less the UIP idiopathic or CT disorder better prognosis
acute interstitial pneumonia
organizing or fibrotic stage of alveolar damage- pattern seen in ARDS
no initial trigger identified
histo-fibroblast proliferation and type II pneumocyte hyperplasia
cytogenic organizing penumonia
organizing fibrosis (granulation tissue) in small airways mild degree of chronic interstitial inflammation intraluminal airway involvement key idiopathic
cytogenic organizing penumonia Ddx
infections, toxic inhalants, or CT disease
idiopathic pulmonary fibrosis
dysregulated pattern of fibrosis due to epi injury 50-70yrs insidious onset dyspnea is most common complaint rales on lung exam clubbing
idiopathic pulmonary fibrosis CXR
interstitial pattern that is generally bilateral and relatively diffuse
more prominent at bases and periphery
idiopathic pulmonary fibrosis high resolution CT
interstital densities that are patchy, peripheral, subpleural and associated w/small cystic spaces
honeycombing- irreversible damage
Dx idopathic pulmonary fibrosis
surgical lung biopsy
HRCT w/classic pattern of honeycombing diagnostic in patient too frail for lung biopsy
idiopathic pulmonary fibrosis prognosis
poor w/mean survival 2-5yrs
desquamative interstitial pneumonia
smokers subacute ground glass appearance on imaging lung biopsy w/uniform accumulation of intraalveolar macros w/little or no fibrosis prognosis better than IPF may respond to corticosteroids
nonspecific interstitial pneumonia
ground glass due to inflammation
lung biopsy- inflammatory response in alveolar walls w/little fibrosis
nonspecific interstitial pneumonia prognosis
depends on degree of fibrosis
nonspecific interstitial pneumonia Tx
corticosteroids
cryptogenic organizing pneumonia
CT plug in small airway accompanied by mononuclear cell infiltration of surrounding lung parenchymal
subacute presentation over weeks to months w/constititional as well as respiratory symptoms
cryptogenic organizing pneumonia CXR
mimics pneumonia w/one or more alveolar infiltrates
cryptogenic organizing pneumonia Tx
response to steroids is dramatic and occurs over days to weeks
therapy is usually prolonged for months to prevent relapse
acute interstitial pneumonia
acute disease that begins w/clinical picture of ARDS, but w/o inciting cause
Histo- diffuse alveolar damage, often showing some organization and fibrosis
mortality is high
acute interstitial pneumonia CXR
ground glass
alveolar filling
complicating CT diseases
RA SLE progressive systemic sclerosis (scleroderma) polymyositis-dermatomyositis Sjogrens syndrome overlap syndromes
CT disorders
usually patients have evidence of underlying disease first, but lung disease can proceed diagnosis
often UIP, but sometimes NSIP or COP
lower lobe more common then upper lobe disease
RA
joints
pleura
lungs- nodules or interstitial lung disease (usually mild)
SLE
joints, skin, organs
pleural disease
acute pneumonitis- alveolar walls and spaces
chronic interstitial lung disease
progressive systemic sclerosis
aka scleroderma skin, small blood vessels organs scarring of pulmonary parenchyma pulmonary fibrosis associated a/Ab to topoisomerae I pulmonary artery HTN
polymyositis dermatomyositis
mm and skin are priamry sites of inflammatory process
interstitial lung disease uncommon
respiratory difficulty secondary to diaphragm weakness
pulmonary langerhans histocytosis
aka eosinophilic granuloma of lung
histioctic cell appears to be an Ag presenting dendritic or phagocytic cell called langerhans cell
x-bodies (rod like)
histiocytes, eos, lymphocytes, macros, plasma cells
pulmonary langerhans histocytosis clinical
starts as peribronchiolar distribution -> diffuse
smokers
unexplained interstitial disease in young or middle aged
pulmonary langerhans histocytosis CXR
nodular or reticulonodular disease
upper lobe prominent
pulmonary langerhans histocytosis HRCT
small cysts in addition to nodular and reticulonodular changes
cysts may rupture and cause penumothorax
some cases extensive honeycombing
pulmonary langerhans histocytosis course
natural history is variable
self limited-> extensive disease
no proven Tx
smoking cessation, corticosteroids
lymphangioleiomyomatosis
rare pulmonary disease
characterized by proliferation of atypical smooth mm around vessels
women of childbearing age
tuberous sclerosis complex genetically predisposes
lymphangioleiomyomatosis clinical
dyspnea and cough
vascular-> hemoptysis
lymph ostruction -> chylous pleural effusion
airway obstruction
rupture of cysts -> spontaneous pneumothorax
lymphangioleiomyomatosis CXR
reticular pattern, cystic changes
lung volumes normal or increased
lymphangioleiomyomatosis HRCT
cystic disease throughout parnechyma
lymphangioleiomyomatosis PFT
obstructive, restrictive, both
Goodpastures Tx
palsmapheresis immunosupression (prednisone)
wagners
cANCA
URTI
wagners Tx
cyclophosphamide
prednisone
churg-strauss
upper and lower respiratory tracts preceded by allergic disorders peripheral and lung eos incrased IgE rashes
churg-strauss CXR
bilateral patchy, fleeting infiltrated
diffuse nodular infiltrates
difuse reticulonodular infiltrates
churg-strauss biopsy
granulomatous angiitis or vasculitis
churg-strauss Tx
corticosteroids
chronic eosinophillic pneumonia
pulmonary interstitium and alveolar spaces infiltrated by eos and macros
chronic eosinophillic pneumonia clinical
weeks-months
fever, weight loss, dyspnea, productive cough
pulmonary inflitrates w/peripheral distruction
increased esos in peripheral smear
BAL w/increased eos
chronic eosinophillic pneumonia Tx
dramatic response to corticosteroids w/in days to weeks
prolonged for months to prevent recurrence
pulmonary alveolar proteinosis
primary pathologic process affects the alveolar spaces, not alveolar cells
spaces filled w/proteinaceous phospholipid
pulmonary alveolar proteinosis clinical
dyspnea and cough b/l alveolar infiltrates HRCT crazy paving pattern due to thicking of interlobular septa ground glass superimposed nocardia infection
pulmonary alveolar proteinosis Tx
whole-lung lavage
prognosis good
hypersensitivity pneumonitis
hyperimmune respirartory syndrome
hypersensitivity pneumonitis Tx
avoid causative agent
corticosteroids
suspect hypersensitivty penumonitis
intermittent pulmonary and systemic symptoms
progressive pulmonary symptoms w/interstitial CXR changes
non-resolving pneumonia
agriculture and cattle farming
bird keeping
contaminated home
hot tubs
acute hypersensitivity pneumonitis
abrupt onset of cough, dyspnea, fever, chest pain
symtoms 4-6 hours after exposure
subside w/in 24 hours
subacute hypersensitivity pneumonitis
more gradual development
less severe
chronic hypersensitivity pneumonitis
insidious progressive dyspnea, cough, weight loss, fatigue
most progress toward pulmonary fibrosis w/respiratory failure
hypersensitivity pneumonitis CXR
acute- reticulonodular inflitrates
chronic- diffuse interstitial fibrosis
hypersensitivity pneumonitis HRCT
ground glass opacities
chronic w/traction bronchietctasis, honeycombing, and fibrosis
hypersensitivity pneumonitis PFTs
restriction
small lung volumes
decreased diffusion capactity