Interstitial Lung Disease
what is it ?
big umbrella of these restrictive diseases
group of disorders affecting lung interstitium = hard to get air IN
what is IPF?
chronic, progressive, irreversible, diffuse {.....} tissue replacement of lung tissue --> causes restrictive lung diserase
what is fibrous
risk factors for OSA
occupation interstitial lung disease caused by inhalation of {{.....}}
inorganic particles
This is also called "......" syndrome
Pickwickian
major physical exam findings
bi-basilar velcro crackles
******** clubbing of nails ******
risk factors
no specific cause (idiopathic) primarily
genetics (AD)
elderly
male
*smoking can predispose*
what is sarcoidosis?
chronic, granulomatous inflammatory disease --> restrictive disorder
can have spontaneous remission
affects lungs & LNS
pneumoconiosis in rheumatoid patients working as coal miners & silica workers.
inflammation surrounds phagocytosed particle --> central necrosis surrounded by inflammation & fibrosis --> "nodules"
what is caplan noudles
aka caplan syndrome
OHS: obesity-related respiratory disorder characterized by daytime hypoventilation due to obesity. this hypoventilation leads to {.....} retention and low {....}
1st - CO2 retention
2nd - Low O2
diagnostics for ILD
Chest CT --> honeycombing (chronic fibrosis & if severe), ground-glass opacities (more so in a lobe)
IPF is not really inflammatory in nature (moa) like the other ILDs
this is more so associated with fibroblast hyperplasia and increased collagen deposition
MOA for sarcoidosis
type IV immune rxn (thelper1 cells) attack unidentified Ags --> activates immune cells & fibroblasts --> non-caseating sarcoid granuloma
if late stage --> further fibroblasts activation --> fibrosis
A specific type of pneumoconiosis:
Fibrogenic
Affects lower lobes
CT --> linear densities (thin white lines, scar)
High predisposition to cancer (mesothelioma)
what is asbestosis
due to asbestos
what are the 2 main characteristics/criteria of OHS?
1. increase daytime PaCO2 >45 mmHG
2. obesity (BMI > 30)
typical signs/sx of ILD patients
progressive SOB
ecxertional dyspnea
persistent dry cough
chest discomfort
fatigue w/ weight loss (good ddx --> cancer)
CT reveals what
honeycombing and ground glass opacities, specifically in lower lungs
acute sarcoidosis triad:
hilar lymphadenopathy
ereythema nodosum
polyarthalgia
this is also called .......
what is Lofgren syndrome
a type of granulomatous pneumonconiosis
affects upper lobes/apex
non-caseating granuloma
similar to sacroidosis (but here there is an identified trigger) --> similar in a sense that they are both type IV hypersensitvity responses and have non caseating granulomas
what is berylliosis
diagnostics for OHS include?
1. PFTS !!!!!
- have supportive findings of restrictive disease (reduced FEV1, FVC)
2. sleep study since 90% have OSA also
prognosis for any ILD (besides sarcoidosis if not severe)
bad. no treatment & progressive disease
sometimes sarcoidosis spontatneously resolves
(1) acute, unexplained worsening of resp. sx
(2) new/worsening bilateral ground glass opacities, or consolidation on CT
you can call this -->
what do diagnostics for sarcoidosis show most commonly?
CXR & CT
ground glass opacities
mosaic attenuation pattern
thickening of bronchovascular bundles & bronchial walls
parenchymal nodules, cysts, & cavities
honeycombing if severe (fibrosis)
if Lofgren syndrome --> bilateral hilar lymphadenopathy
This type of pneumoconiosis affects upper lobes, has focal nodules, and prob is the most common
Diagnostics:
CXR --> small nodules in upper lobes
CT --> eggshell calcifications (crazy paving) --> ground glass opacities with interlobular septal thickening
what is silicosis
1. inc wob due to excess fat
2. dec chest wall compliance (not as expansive)
3. leptin resistance alters resp drive (fat people are resistant= less drive for ventilation)
4. decreased ventilation drive (brain not as responsive to Co2)