ILD Flashcards
what are the physiologic properties of ILD?
- restriction - decreased compliance - abn gas exchange
in idiopathic pulmonary fibrosis what is issue?
the initial inflammation is unknown but TGF-beta is involved by activating fribroblasts leading to deposition of collagen thus fibrosis
what do see in PFTs in ILD?
-decreased TLV, FRC and RV - decreased FEV1, FVC and either normal or increased FEV1/FVC - decreased DLCO
what is the course of IPF?
it is pretty grim, not high survival rates however there is new tx. that slows down the decline in FEV1 and thus might be able to improve survival rates but thatis unknown
when you one use glucocorticoids in treating certain ILD?
if there are inflammatory (eosinophils, lymphocytes or granulomatous) or ground glass/alv infiltrates (sarcoidosis, COP, ceP) are present
what is there is extensive honeycombing?
no effective treatment, one should ocnsifer lung transplant
what do both ninetdanib and pirfenidone do?
slows down decline in function and increases exercise tolerance
inorganic dusts
pneumoconiosis
organic proteins
hypersensitivity pneumonitis
hallmark of silicosis
ineffective clearance
hallmark of HP
exaggerated immune response
amphiboles
asbestos fiber
worse asbestosis manifestation in pleura
mesothelioma
treatment of HP
removal of the causative agent, and steroids in symptomatic patients
silo filler’s lung and metal fume fever
are direct tissue injury
byssinosis
inhalation of unprocessed cotton dust causing direct airway tissue injury
RADS
irritant induced asthma where caustic fumes cause direct airway damage
when do we start to see asthma like symtpoms in RADS?
within 12 hr
what should we do about RADS?
treat it like asthma. most improve over time but may lead to permanent airway hyperresponsiveness
in immunologic occupational asthma we see ____ and we should expect what are two of the most common cause?
we see IgE mediated response and there is latency between exposure and symptoms - western red cedar and baker’s asthma
what can we do to determine if it is occupational asthma?
PEFR
treatment of occupational asthma
remove exposure, bronchodilators and anti-inflammatory (steroids)
true or false asbestosis can be seen quickly?
nope disease seen in many years after exposure but one can see pleural plaques with recent exposure
where are the silicosis particles mainly deposited? who are the main instigators? where can nodules be seen in?
deposited in upper lobes - macrophages release pro-inflammatory and pro-fibrotic mediators - seen in hilar lymph nodes
HP examples
- farmer’s lung - maple bark disease - bird-fancier’s lung -humidifier or air conditioner lung
HP hallmark in histology
lymphocytes
Inflammatory pattern characterized by the presence of collections of epithelioid histiocytes (macrophages), with or without multinucleated giant cells (these are also histiocytes) and necrosis
granuloma granulomatous
prototypical idipathic granulomatous disorder
sarcoidosis
sarcoidosis hallmark
non-necrotizing granulomas in different organs and increased serum ACE
Vasculitis and necrotizing granulomas in upper and lower respiratory tract and necrotizing or crescentic glomerulonephritis
GRANULOMATOSIS WITH POLYANGIITIS
GRANULOMATOSIS WITH POLYANGIItis has a c-ANCA
positive test
LANGERHANS CELL HISTIOCYTOSIS
proliferation of langerhan cells/APC and strongly associated with smoking
Organizing pneumonia histologic pattern without known cause
cryptogenic organizing pneumonia
dissuse alveolar damage
acute interstitial pneumonia
alveolar macrophages sfill alveolar spaces leading to mild interstitial fibrosis but there is good response to smoking ceddation and steroids
desquamative interstitial pneumonia
You are evaluating a patient with dyspnea, non-productive cough, and inspiratory crackles on exam. A chest CT shows diffuse ground glass opacities. Which of the following would NOT be expected? - decreased FEV1, decreasedFVC, nl FEV/FVC, decreasedTLC, decreasedDLCO - decreased compliance -Fibrosis on lung biopsy -Rapid shallow breathing - H/O tobacco smoking
-Fibrosis on lung bio-Fibrosis on lung biopsypsy
Do we see hypercapnia in ILD?
yea, they have a rapid shallow breathing, cystic airspaces and diffusion abnormality at later stages
asbestos (ferruginous) body
Birbeck granules are seen ultrastructurally within langerhans
birefringent silica particles under polarized ligh
Diffuse airspace filling by macrophages with light brown cytoplasmic pigment; In RB-ILD, the process is less extensive and has bronchiolocentric distribution
geographic necrosis
granulomatosis with polyangiitis the lung shows multiple cavitating mass-like lesions
HP- airway centered lymphocyte predominant inflammation extending into alveolar septa
interstitium infiltrated by lymphocytes
interstitium widened by fribrosis
LHC- Langerhans have a prominent nuclear grooves
microscopic cysts-honeycombing
normal interstitium
normal interstitium with elastic fibers
pathcy fibrosis- abestosis
sarcoidosis- notice the little background inflammation
sarcoidosis- packed histiocytes
silicotic nodule
what is this?and what do they suggest?
what is this- diffuse reticular infiltrates and what do they suggest- that fibrosis is presen
what is this? and what do they suggest?
what is this- ground glass opacities and what do they suggest- infection,blood and inflammation
