Interstitial Lung diseases, Part I, D Kinder, DSA Flashcards
Interstitial Lung disease affects what part of lungs
alveolar wall including epithelial cells, endothelial cells and interstitium
Clinical manifestations of interstitial lung disease
dyspnea, nonproductive cough, crackles prominent at lung bases
clubbing may be present
how is compliance and lung volume changed in interstitial lung disease
decreased compliance and decreased lung volumes
describe acid base state of interstitial lung disease
hyposemia without CO2 retention
interstitial lung disease commonly leads to what problem
pulm HTN
how will interstitial lung disease present on CXR
reticular with linear markings
reticulonodular with increased linear and small nodular markings
how do you distinguish between inflammation from fibrosis in interstitial lung disease
High res CT
What tissue specimens do you take for Dx of interstitial lung disease
thorascopic lung biopsy
transbronchial biopsy via flexible bronchoscopy
What will PFT show for interstitial lung disease
restrictive
decreased volumes
hypoxia
what are the known etiologies of interstitial lung disease
inhaled inorganic dusts
organic Ags
What are the unkonwn etiologies that can lead to interstitial lung disease
idiopathic interstitial pneumonias CT diseases (rheumatic) sarcoidosis pulm langerhans lymphangioleiomyomatosis Good pastures and wegeners chronic eos pneumonia pulm alveolar proteinosis
What is the pathologic process behind interstitial lung disease
inflammatory in alveolar spaces and scarring and fibrosis
What is a granuloma
localized collection of cells called epitheliod histocytes, generally with T lymphocytes, multinucleated giant cells
What are the idiopathic interstitial penumonias
usual interstitial pneumonia desquamative interstitial pneumonia respiratory bronchiolitis interstitial lung disease nonspecific interstitial pneumonia acute intersitital pneumonia cryptogenic organizing pneumonia lymphocytic interstitial pneumonia
Patient has patchy areas of parenchymal fibrosis and interstitial inflammation interspersed between areas of healthy lung
honey combin is present
Dx?
usual interstitial pneumonia
what is honey combing
cystic airspace from retratction of surrounding fibrotic tissue
signs of usual interstitial pneumonia
patchy areas of parencymal fibrosis and interstitial inflammation interspersed
fibrosis!!
hoenycombing
What characterizes desquamative interstitial pneumonia
large # intraalveolar monomuclear cells less inflammation, little fibrosis minimal architectrual distortion pigmented macrophages SMOKING
smoker has biopsy done of lungs
shows man intraalveolar mononuclear cells with little inflammation or fibrosis
there are some pigmented macrophages in respiratory bronchioles
Dx?
desquamative interstitial pneumonia
What is Respiratory bronchiolitis interstitial lung disease
associated with pigemented macrophages like DIP
NO interstitial inflammation
SMOKING
difference of desquamative vs respiratory bronchiolitis intersitial lung disease
the latter does not have any inflammation
What is characteristic of nonspecific interstitial pneumonia
mononuclear cell infiltration within alveolar walls
uniform process
fibrosis is variable, but less than Usual interstitial pneumonia
idiopathic or CT disorder
better prognosis
what is prognosis of nonspecific intersitial pneumonia
better than usual interstitial pneumonia
Characteristics of acute interstitial pneumonia
organizing or fibrotic stage of alveolar damage (like ARDS)
fibroblas proliferation and type II pneumocyte hyperplasia
no initial trigger identified
patient shows fibroblast proliferation and type II pneumocyte hyperplasia
Dx?
acute interstitial pneumonia
Characteristics of cryptogenic organizing pneumonia
organizing fibrosis (granulation) in small airways mild degree chronic interstitial inflammation intraluminal airway involvement idiopathic
When there is intraluminal airway involvement in an organizing fibrosis process in lungs what is top of DDx?
cyrptogenic organizing pneumonia
differential for cryptogenic organizing pneumonia
infections, toxic inhalants or CT disorder
characteristics of idiopathic pulmonary fibrosis
no recognizable causative agent dysregulated pattern of fibrosis in response to alveolar epithelial injury presents between ages 50 and 70 insidious onset dyspnea rales frequently have clubbin
patient is 55 years old, non smoker, complaining of dyspnea
you notice clubbing of fingers and rales on lung examination
upon biopsy results there is noted dysregulation of fibrosis in alveoli
Dx?
idiopathic pulmonary fibrosis
CXR idiopathic pulmonary fibrosis
interstitial pattern that is b/l and diffuse
more prominent at lung bases especially in peripheral and subpleural regions **
idiopathic pulm fibrosis on CT
intersitital densities are patchy, peripheral and subpleural
associated with small cycstic spaces
there is honeycombing
on CT what is indicative of irreversible fibrosis
honeycombing
how is Dx made of interstitial pulmonary fibrosis
surgical lung biopsy
if too frail– HRCT
prognosis idiopathic pulmonary fibrosis
poor with mean survival post Dx 2-5 yrs
no proven effective Tx
how will desquamative interstitial pattern show on CXR
ground glass
Tx for desquamative interstitial pneumonia
smoking cessation and maybe corticosteroids
CXR appearance of nonspecific interstitial pneumonia
ground glass
prognosis and Tx for nonspecific intersitial pneumonia
depends on degree of fibrosis
respond to corticosteroids
Tx cryptogenic organizing pneumonia
steroid response is dramatic in days-weeks
prolonged for mo to prevent relapse
What does CXR of cryptogenic organizing pneumonia look like
mimics pneumonia with one or more alveolar infiltrates
What will imaging look like with acute interstitial pneumonia
ground glass,alveolar filling
what is prognosis acute interstitial pneumonia
mortality high
What are the complicating CT diseases that cna cause interstitial lung disease
RA SLE progressive systemic sclerosis (scleroderma) polymyositis-dermatomyositis sjogrens syndrome overlap syndrome
what lobes are more commonly involved in CT disorders
lower lobe
most common site and manifestation of RA in thorax
pleura
pleurisy, pleural effusions
describe lung parenchymal involvement of RA
one or multiple nodules or development of interstitial lung disease
organ involvement of SLE
kidneys, lungs, nervous system, heart
SLE in thorax
pleuritic chest pain, pleural effusion
acute pneumonitis of alveolar spaces and walls
pulmonary involvement of sceroderma
severe with significat scarring of pulmonary parenchyma
pulmonary fibrosis strongly associated with autoAb to topoisomerase I, Scl70
what concurrent disease process affects the lungs in scleroderma (progressive systemic sclerosis)
pulmonary a HTN
small pulmonary vessel disease independent of fibrosis
what accounts for dyspnea in polymyositis-dermatomysositis
weakness of diaphragm
what accounts for dysphagia and recurrent aspiration pneumonia in polymyosistis-dermatomyositis
involvement of the striated mm in the proximal esophagus
histo shows APC “langerhans cell”
cytoplasmic rod like structures called X bodies
histiocytes, eos, lymphocytes, macrophages and plasma cells
Dx?
pulmonary langerhans histiocytosis
also called eosinophilic granuloma of lung or pulmonary histiocytosis X
how does pulmonary langerhans progress
starts as peribronchiolar and becomes diffuse
SMOKERS
CXR and CT apperance of pulmonary langerhans
CXR: nodular or reticulonodular disease with upper lobe involvement
CT: small cysts in addition to nodular and reticulonodular changes, cysts may rupture and cause pneumo
some cases there is extensive honeycombing
30 y.o woman with proliferation of atypical smooth mm cells around lymphatics, blood vessels and airways with numerous small cysts
dx?
most likley to have what genetic condition?
lymphangioleiomyomatosis
tuberous sclerosis complex
clinical manifestations lymphangioleiomyomatosis
dyspnea cough
vascular involvement– hemoptysis!
lymphatic obstruction– chylous pleural effusion
ariflow obstruction
rupture of cysts cal lead to spontaneous pneumothorax
CXR and HRCT of lymphangioleiomyomatosis
CXR: reticular pattern, cystic changes
lung volumes normal or inc
HRCT: cystic disease throughout parenchyma
PFT of lymphanioleiomyomatosis
Obstructive, restrictive or both
how does Goodpastures present in lungs
pulmonary hemorrhage and pulmonary fibrosis may develop
The anti GBM AB in goodpastures are against what
Collagen IV
Tx goodpastures
plasmapheresis
immunosuppressive Tx is given to dec formation of Ab
Lung involvement of wegeners
upper respiratory tract and lung have small necrotizing small vessel granulomatosis
CXR will show nodules, infiltrates and cavitationdiffuse
cANCA+
Tx wegeners
Cyclophosphamide
prednisone
What is characteristic of churg strauss syndrome
systemic nectrotizing vasculitis Affects upper and lower respiratory tracts preceded by allergic disorcers peripheral and lung eosinophilia increased IgE rashes
CXR Churg Strauss
bilateral patchy, fleeting infiltrates
diffuse nodular infiltrates
diffuse reticulonodular infiltrates
what will biopsy of churg strauss patient show
granulomatous angiitis or vasculitis
Tx of churg strauss
corticosteroids
What are clnical manifestations of chronic eos pneumonia
weeks-mo
fever, weight loss, dyspnea, productive cough
pulmonary infiltrates with peripheral distribution and more suggestive of alveolar than interstitial disease
increased eos in peripheral smear
BAL with increase eos
Tx chronic eos pneumonia
dramatic response to corticosteroids (days-weeks)
therapy prolonged for mo to preven recurrence)
alveolar spaces are filled with proteinaceous phospholipid material
Dx?
pulmonary alveolar proteinosis
Clinical manifestations of pulmonary alveolar proteinosis
dyspnea and cough
b/l alveolar infiltrates
superimposed to respiratory infections-Nocardia
Tx pulmonary alveolar proteinosis
Whole lung lavage
prognosis is good
HRCT pulmonary alveolar proteinosis
crazy paving pattern produced by thickening of interlobular septa accompanied by groun-glass alveolar filling
What cause hypersensitivity pneumonitis and what is it
bacteria, fungi, protozoa, animal proteins and reactive chemicals
hyperimmune respiratory syndrome caused by inhalation of a wide variety of allergic Ag that are usually organic
Tx of HS pneumonitis
identify causative Ag and avoid exposure
prevent progressive permanent lung damage
corticosteroids hasten resolution
When is HS pneumonitis higher on your differential
intermittent pulmonary ans sytemic Sx progressive Sx with interstitial CXR non-resolving pneumonia workplace exposure (cattle, ag, bird keeping) home exposure (ventialation, hot tubs)
acute presentation of HS pneumonitis
abrupt onset cough, dyspnea, fever, ches pain)
Sx 4-6 hrs post exposure
subacute presentation of HS pneumonitis
more gradual develoment of Sx
less severe intensity
chronic presentation HS penumonitis
insidious progressive dyspnea, cough, weight loss, fatigue
most progress to pulmonary fibrosis with resp failure
What professions and hobbies are affected by HS pneumonitis
farmers, bird keepers, woodworkders, office workers, chees makers, plastic industry workers, metal workers, painters, lifeguards, machine workers
Common etiology of faming caused HS pneumontiis
thermophilic actinomycetes in moldy hay or grain
what is common etiology of bird handlers with HS pneumonitis
animal proteins in droppings
what type of pathogens grow in ventialation areas or water-related contamination
thermophilic actinomycetes
mycobacterium avium intracellulare complex
CXR HS pneumonitis
acute- diffuse reticulonodular infiltrates
chronic- diffuse interstitial fibrosis
HRCT of HS pneumonitis
ground glass opacities early
chronic with traction bronchiectasis, honeycombing and fibrosis
PFT HS pneumonitis
restriction, small lung volumes, decreased diffusion capacity
What is sarcoidosis
systemic granulmatous disease known by non-caseating granulomas
What is classic population affected by sarcoidosis
age 40-60
peak exposure 20-39
3.5x higher in black americans
women>men
what is common initial presentation os sarcoidosis in lungs
abnormal CXR with mediastinal and hilar adenopathy
Lofgrens syndrome
acute manifestation of sarcoidosis
erythema nodosum (women)
arthritis (men)
b/l hilar lymphadenopathy
prognosis lofgrens syndrome
favorable
Sx sarcoidosis
general: fatigue, fever, night sweats and Lb loss
pulm: cough, DOE, wheeze, ches discomfort
skin: rash, macules, papules, nodules, hyperpigmentation or hypo, erythema nodosm
ocular: gritty or dry eyes, pain ,redness or blurred vision
cardiac: arryhthmia, palputations, near syncope, LE edema
NS: HA, blurred vision, numbnessm seizures
MSK: swelling and stiffness, pain
URT: nasal congestion, sinus pressure
Signs of sarcoidosis
pulm: rales, expiratory wheezing
skin: infiltration old scars and tattoos, maculopapular lesions, lupus pernio of nose cheeks ears and lips.
ocular: uveitis, iritis, scleral plaques
cardiac: irregular rhythm
NS: CN II VII VIII, gait instability
GI: HSM, jaundice
MSK: arthralgias, arthritis
lymph: adenopathy in cervical, supraclav, axillae and innguinal
What organ has 90% invovlment in sarcoidosis
lungs
mediastinal and hilar lymphadenopathy or parenchymal lung disease
What can be used in eyes to Dx sarcoidosis
biopsy of conjunctiva- yellow nodules
Why do patients with sarcoidosis have nephrolithiasis, nephrocalcinois or hypercalcuira
disorder of Vit D metabolism
What do you look for in bronchoalveolar lavage if suspect sarcoidosis
lymphocytosis
elevated CD4/CD8 ratio
look at ACE levels too in blood
What is staging of CXR sarcoidosis
stage 0 normal
1 b/l hilar adenopathy
2 b/l hilar adenopathy and parenchymal infiltrates
3 parenchymal infiltrates no lymphadenopathy
4 advanced parenchymal disease with fibrosis
PFT of sarcoidosis
restrictive ventilatory defect, concurrent obstruction, decreased DLCO
Tx sarcoidosis
ONLY Sx patients
prednisone 20-40 mg QD x 3 mo, then taper
cytotoxic and immunosuppressives if no response to prednisone
prognosis sarcoidosis
spontaneous remission in 50% patients at 3 yrs
2/3 remission after 10 yrs
less than 5% die from it
