Restrictive Lung Disease - Parks and Baker Flashcards
what are the following values in Restrictive lung disease TLC DLCO FVC FEV1
dec. TLC
dec. DLCO
dec. FVC
normal/inc FEV1
what are the chest wall mediated causes of RLD?
- neuromuscular disorders
- obesity
- pleural disorders
what are the fibrosing causes of RLD?
- idiopathic pulmonary fibrosis
- non-specific intersitial pneumonia
- cytogenic organizing pneumonia
- connective tissue disroders
- pneumoconiosis
- drug rxns
- radiation pneumonitis
what two autoimmune dz can cause fibrosis of the lung?
RA
SLE
describe the process of forming a honeycomb lung.
- lung is injured
- lung reacts with inflammation and wound healing
- the distal airway ends up being “amputated” by the fibrosis and becomes a dead air space not connected to anything
what type of inflammation causes honeycombing?
interstitial and alveolar inflammation
What are the three general categories of things needed to Dx IPF?
- clinical picture
- CXR
- pathologic changes
What are the pathologic changes in IPF known as?
usual intersitial pneumonia UIP
What other diseases show UIP?
connective tissue disorders
chronic hypersensitivity reactions
pneumonia
asbestosis
You need to have (UIP/IPF), but you don’t necessarily have (UIP/IPF) if you have (UIP/IPF)
you need UIP to have IPF; but having UIP doesn’t mean you automatically have IPF
which lobe of the lung is most affected by IPF?
lower lobes; most fibrosed
Honeycombing is also called what pathologically?
cystic spaces within fibrosis
how soon does IPF kill you?
3-5 yyears
What kind of fibrosis takes place in IPF?
subpleural
histologically, what are the earliest lesions seen in IPF?
fibroblastic foci
What are the three histologic findings that are characteristic for IPf?
- fibroblastic focus
- subpleural fibrosis
- temporal heterogeneity
Subpleural fibrosis leads to…
collapse and obliteration of alveolar air spaces
What is the MOA hypothesis for the formation of IPF?
- repeated stimulus
- sequential lung injury
- ABERRANT WOUND HEALING
a. inflmmation/Th1/2balance / genetics - fibrosis
What are the environmental factors that lead to IPF?
- smoking
- exposure to metal fumes and wood dust
- hair dressers
the genetic predispositions that affect IPF are associated with what cell type?
pneumocytes
What age is associated with IPF?
> 50
Nintedanib inhibits (blank) for growth factors for fibroblasts in IPF
tyrosine kinase receptors
how does IPF first present?
dry cough and dyspnea
when does IPF normally onset?
40-70
when does clubbing occur in IPF?
late; indicates hypoxemia
What are the Tx available for IPF?
steroids
immunosuppressants
lung transplant
what is the big difference between NSIP (nonspecific interstitial pneumonia) and IPF?
NSIP Biopsies fail to show diagnostic features of any of the other well-characterized ILDs
aka
looks like IPF, but LACKS UIP PATTERN
what are the two types of NSIP?
cellular
fibrosing
which type of NSIP happens in younger people and has better outcomes?
cellular
which NSIP has NO temporal heterogeneity or honeycombing
fibrosing
which NSIP is characterized by moderate chronic interstitial INFLAMMATION
cellular
which NSIP is characterized by diffuse or pathy FIBROSIS
fibrosing
When UIP and NSIP are both present, prognosis is only as good as the (blank) lesion
worst
how does NSIP present?
40-55; dyspnea with cough for a few months
How does Cryptogenic organizing pneumonia (BOOP) present?
cough with dypsnea
what do you see on CXR in BOOP
pathy airspace opacities
what does the organizing mean in cryptogenic organizing pneumonia?
long term changes of fibrinous exudates
Polyploid plugs of loose organizing connective tissue, aka (blank), are seen in BOOP
Masson bodies
does BOOP present with temporal hetergeneity, honeycombing, or interstitial fibrosis?
nope
T/F: the lung architecture in BOOP is normal
true
Masson bodies are massive of what type of tissue?
granulation tissue
what is the reason that Masson bodies form?
When you get a regular strep pneumo, you have to heal.
But these people come in with a cryptogenic (aka idiopathic) pneumonia that forms granulation tissue to heal
Organizing pneumonia with intra-alveolar fibrosis can be secondary to….
infections inflamm/injury inhaled toxins drugs connective tissue disease GVHD
what should your history really check for with organizing pneumonia
workplace exposure to chemicals
arthralgias
malar rash
RA or SLE
Describe the lung involvement in RA?
- chronic pleuritis
- diffuse interstitial pneumonitis and fibrosis
- intrapulm. rheumatoid nodules
- pHTN
Describe the lung involvement in systemic sclerosis?
diffuse interstitial fibrosis; UIP or NSIP
describe the lung involvement in lupus?
patchy, transient parenchymal infiltrates
what are pneumoconioses?
Diseases induced by inhalation of organic and inorganic particulates, chemical fumes and vapors.
what particle size is most dangerous to the lungs?
1-5um; settles in distal airways
Small particles can reach toxic levels and cause acute lung injury while large particles stay in the lung parenchyma and evoke what ?
fibrosing collagenous pneumoconioses
T/F: pneumoconioses cannot travel by blood or lymph
false!
Describe the pathogenesis of injury in pneumoconioses?
- Dust activates mac’s
- mac’s release IL1, TNF-a, and ROS
- mediators lead to continual cell injury
- Fibroblast proliferation, collagen deposition
- interstitial fibrosis
what color do the lymph nodes become with continual particulate exposure?
anthracotic (black)
describe a silicotic nodule
collagen core surrounded by fibroblasts and lymphs
describe an asbestotic alveolus
asbestos bodies within the alveolus with interstitial fibrosis
which pleura becomes thickened in asbestosis?
visceral pleura
which lobes of the lung are most affected in asbestosis?
lower
smoking reduces (blank) clearance
mucociliary
Smoking worsens the effects of all inhaled dusts, but particularly…
asbestos
What are the three severities of coal worker’s lung?
- anthracosis
- simple CWP
- complicated CWP
t/f; CWP has little to no decrease in lung function
true
a minority of patients with CWP will progress to..
progressive massive fibrosis (PMF)
T/F: coal worker’s lung has no increased risk of TB or cancer
true
CWP is associated with what other lung diseases?
COPD
what is the most common occupational disease in the world?
silicosis
Describe the lesions in silicosis?
nodular and fibrosing; seen in progressive exposure
what do you see in actue silicosis?
Abundant lipoproteinaceous material in alveoli
actue silicosis is identical to what other disease?
alveolar proteinosis
what element causes silicosis?
quartz
T/F: pure quartz is worse for your lungs than quartz mixed with other shit
true
Early in silicosis, the tiny nodules will later become…
hard collagenous scars
what technique will show silica crystals in a biopsy?
polarized microscopy
which lobes of the lung are most affected by silicosis?
upper zone nodularity
When do you get SOB in silicosis
late, when you get massive fibrosis
Describe the hilar calcification in silicosis
EGGSHELL calcification
T/F: silicosis makes you more susceptible to TB
true
T/F: silicosis makes you more susceptible to cancer
true; possibly carcinogenic
What are the effects on the pleura of asbestos?
Pleural Effusions
Fibrous plaques
Diffuse pleural fibrosis
Mesothelioma
what are the effects on the lung parenchyma of asbestos?
lung carcinoma
interstitial fibrosis aka Asbestosis
mesothelioma is cancer of the…
pleura
what is the most common type of asbestos used in industry?
serpentine (curly)
why is serpentine asbestos less likely to cause pathology?
gets stuck higher up in the lung
what is the only type of asbestos associated with mesothelioma?
amphibole (needle like)
T/F: smoking increases the risk of having mesothelioma
FALSE
What is asbestosis in pathology terms?
Diffuse pulmonary interstitial fibrosis
how to asbestos bodies form?
macs try to phagocytize the fibers
what is another name for asbestos bodies?
feruginous bodies: aka coated in iron (Fe)
asbestosis has a similar appearance histologically to…
UIP
What parts of the lung are fist affected in asbestosis?
lower lung and subpleura areas
Describe the presentation of asbestosis?
DOE first, then Dyspnea with rest, productive cough
How common is asbestosis after:
10 years exposure
>20 years exposure
rare at 10
common at 20 plus
what does the CXR look like in asbestosis?
fine reticular pattern with greater dominance in the lower lobes
Pleural plaques from asbestos are well circumscribed and dense with what two things?
collagen and Ca
Where do the plaques form in asbestosis?
anterior and posterolateral parietal pleura;
DOMES OF THE DIAPHRAGM
what type of pleural effusion happens in asbestosis?
serous
T/F: pleural fibrosis is common later stages of asbestosis
false
What is the catch phrase for sarcoidosis?
NONCASEATING GRANULOMAS in many tissues and organs
What is the cause of sarcoid?
unknown
Which race and which gender tend to get sarcoid?
females, blacks (10x more than whites)
in which race is sarcoid rare?
asians
Which cell mediates the immune response to sarcoid?
CD4+ T cells
The genetic predisposition has familial and (blank) clustering
racial
What are the possible environmental exposures that may cause sarcoid
Possibly a microbe (Rikettsia; Propionibacterium; mycobacteria)
when looking at a lymph node with granulomas, how do you know if they are caseating or non-caseating?
the lesions that have red/darker in the middle are caseating; the singled colored/completely light lesions are non-caseating
what type of biopsy can be used to Dx sarcoid?
transbronchial biopsy
T/F: sarcoid causes honeycombing
true
T/F: asbestosis causes honeycombing
true
Explain how sarcoid leads to interstitial fibrosis
The granulomas, which are present in the interstitial space, result in fibrosis. So if granulomas are diffuse then possibly diffuse interstitial fibrosis can result.
Which lymph nodes in the lung does sarcoid affect?
hilar and peribronchial;
what will you see on CXR of sarcoid?
hilar and peribronchial LAD
What are the pulmonary Sx that people have with sarcoid?
SOB
cough
chest pain
hemoptysis
What are the two lab values that help indicate sarcoid?
- elevated ACE
2. Hypercalcemia
why do pts with sarcoid have hypercalcemia?
granulomas release vit. D which increases Ca absorption
in what percent of pts have a histologically affected spleen with sarcoid?
75%; 20% actually are enlarged
after the spleen, what is the next organ to be affected in sarcoid?
liver
What other disease may sarcoid mimmic?
Sjogrens; problems with salivary glands and tear production
What is hypersensitivity pneumonitis?
Immune mediated, mostly interstitial lung disorders caused by abnormal sensitivity/reactivity to an inhaled organic antigen
while asthma affects the bronchioles, hypersensitivity pneumonitis affects the….
alveoli
What are the layman’s names for hypersensitivity pneumonitis?
farmer’s lung
pigeon breeder’s lung
Humidider/AC lung
hypersensitivity pneumonitis is centered around which structure?
bronchiole, although it affects the alveoli
The interstitial pneumonitis caused by hypersensitivity pneumonitis has an infiltrate of what three cell types?
lymphs
plasma cells
macrophages
What type of granulomas does hypersensitivity pneumonitis show?
noncaseating!!
T/F: hypersensitivity pneumonitis causes interstitial fibrosis
true
T/F: hypersensitivity pneumonitis causes honeycombing
true
T/F: hypersensitivity pneumonitis causes obliterative bronchiolitis
true; late though
What is the difference between pneumonia and pneumonitis?
Pneumonia= inflammation within alveoli Pneumonitis = inflammation in the interstitium
Describe the acute presentation of hypersensitivity pneumonitis?
FEVER
dyspnea, cough, leukocytosi 4-6 hours after exposure
What are the Sx of chronic hypersensitivity pneumonitis?
respiratory failure
dyspnea
cyanoiss
What part of the the exam is most important when thinking about hypersensitivity pneumonitis?
the history; symptoms get worse after cleaning the bird cage, or some such shit
T/F: removal of the stimulus can prevent hypersensitivity pneumonitis from becoming a fibrotic disease
true
what is the key feature in pulmonary eosinophilia?
RAPID ONSET FEVER
dsypnea, hypoxemia, resp failure
what do you find in bronchio-alveolar lavage fluid in pulmonary eosinophilia?
25% eos
Simple pulm. eosinophilia has (transient/perm.) lesions
transient
T/F: simple pulm. eosinophilia is benign in its course
true
besides in the BAL fluid, where else do you find eos in pulm. eosinophilia?
peripheral blood
what are the lesions like in chronic eosinophilic pneumonia?
focal areas of consolidation with lymphs and eos
What are the systemic symptoms of pulm. eosinophilia?
fever, night sweats, dsypnea
T/F: pulmonary eosinophilia responds well to steroids
true
Secondary eosinophilia can be caused by…
Usually due to infection, drug rxn, asthma, vasculitis, aspergillosis
What is Pulmonary Alveolar Proteinosis?
Accumulation of acellular surfactant in the intra-alveolar and bronchiolar spaces
Pulmonary Alveolar Proteinosis is identical to what other dz?
silicosis
What are the three types of Pulmonary Alveolar Proteinosis?
Acquired
congenital
secondary
T/F: Pulmonary Alveolar Proteinosis is fatal
true; 3-6 mo w/o transplant
What are the causes of secondary Pulmonary Alveolar Proteinosis?
malignancies, immunodef, or SILICOSIS
90% of all cases of Pulmonary Alveolar Proteinosis are what type?
acquired
What is the MOA of the pathology of Pulmonary Alveolar Proteinosis?
Ab to GM-CSF; it is an autoimmune disorder
WHat is the Tx for Pulmonary Alveolar Proteinosis?
GM-CSF supplementation: works in 50% of the cases
2. Whole lung lavage is standard of care.
