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