Interstitial lung disease Flashcards
course idiopathic pulmonary fibrosis
end stage lung, cor pulmonale
mesothelioma, arises from ____,
when?
arises from mesothelial cells of pleura 25-40 years after exposure
no relationship to smoking
bronchogenic carcinoma
in asbsetos related disease
additional risk with smoking
20 years after first exposure
complications asbestos related diseases
cor pulmonale
caplan syndrome
Caplan syndrome of complicated coal worker’s pneuoconiosis
CWP with rheymatoid nodules
histo coal worker’s pneuoconisis
antrascosis black pigment
fibrous opacity with pink fibrous tissue
types of hyeprsensitivity pnuemonitis
famers lung - themophilic actinomycetes bacteria saccharopolypora rectivirgula
silo fillers disease - plant material gases
Byssinosis - textile workers
clinical and laboratory findings intersitital lugn
dry cough
dypsnea
late inspiratory crackles, bibasilar
cor pulmonale
CXR - bilateral reticulondular infiltrates
____ pigment in coal worker’s pneumoconiosis
antrhacotic pigment
exposure progression in hypersensitivity pneumonitis
first exposure - IgG in serum
second - antibodies > immune complexes >interstital inflammation
chornic exposure: glanuloma formation (type IV response)
CXR and histo idiopathic pumonary fibrosis
increased interstitial markings (fibrosis) in lower zones
honeycomb cysts
fibroblasctic plugs
hypersensitivity pneumonitis
inhaled antigen producing granulomatous interstitial pneumonitis
pathology SLE interstitial lung disease
peleual effusion (young woman with effusion = sle)
presentation sarcoidosis
Skin: nodular granulomatous lesions, lupus pernio, erythema nodosum
**Eye: **uveitis
**liver: **granulomatous hepatitis
**other: **enlarged salivary and lacrimal glands, Diabetes insipidous, Bone marrow and spleen involvement
pathogenesis idiopathic pumonary fibrosis
repeated injury > alveolitis > cytokine release > interstitial fibrosis
pathogenesis sarcoidosis
disorder of immune regulation
antigen > CD4 interaction > cytokines > monocytes+histiocytes recruited > non-necrotizing granumloma formation
infiltrate of farmer’s lung hypersensitivitiy penumonitis
thermophilinic actinomycetes - saccharopolyspora rectivirgula
berylliosis tissue reaction
site of acquirement
granulomatous inflammation
nuclear and airspace industry (beryllium)
complications silicosis
cor pulmonale
caplan syndrome (rheumatoid nodules)
increased risk for TB (silicotuberculosis) and cancer
CXR sarcoidosis
bialteral hilar adenopathy
reticulonular shadows in lungs
interstitial granuloma distribution in sarcoidosis
other histo
lympathic distribution pattern
langhans giant cells
epitheliod histiocytes
simple coal workers pneumoconiosis vs complicated
progressive massive fibrosis
histo complicated coal worker’s penumoconiosis
fibrous opacities > 1cm
with/without central necrosis
massive fibrosis
caplan syndrome (rheumatoid nodules)
(no increased incidence of TB or cancer)
distribution of fibrous opacities in simple coal workers pneumoconiosis
upper lobes and upper-lower lobes
coal dust adjacent to respiratory bronchioles
aveolitis =
damage to pneumocytes and endothelial cells
in anthracosis, anthracotic pigment found in
intersitial compartment and lymph nodes
pathology systemic sclerosis (scleroderma) interstitial lung disease
interstitial fibrosis
pulmonary vascular hypertrophy
(commonest casue of death in these patients)
histo hypersensitivity pneumonitis
intersitital and alveolar inflammatory cell infiltrates
peri-bronchilar accentuation
ill-defined granulomas
(biopsy may be needed)
two forms of asbestos
serpentine
amphibole - straight and rigid
tissue appearance asbsestos related disease
deposits in respiratory bronchioles, ducts and alveoli
ferringinous boides - macrophages, fibers coated with ferritin (iron and protein)
benign pleural plaques
diffuse interstital fibrosis
collagen vascular diseases seen in interstitial lung disease
SLE (50% have interstiial lung)
RA
systemic sclerosis (scleroderma)
demographics sarcoidosis
AA:whites 10:1)
F:M 2:1
70%< 40 years
non smokers
most common occupational disease
silicosis
pathogenesis silicosis
quartz activates macrophages > cytokines > fibrogenesis
pathology RA interstitial lung disease
rhematoid nodules (caplan syndrome+pneumoconiosis)
interstitial fibrosis,
pleural effusion
distribution of particles in pneumoconiosis
1-5um particles reach bifurcation of bronchioles and alveolar ducts
lab findings sarcoidosis
increased ACE levels
hypercalcemia
polyclonal gammopathy
cutaneous anergy - no response to skin antigens(CD4 consumed)
pneumoconioses =
non-neoplastic lung disease in response to inhalation of mineral dust
types of interstitial lung disease
ARDS
Fibrosing lung disorders (pneumoconioses)
Granulomatous disorders (sarcoidosis)
idiopathic interstitital pneumonias
multisystem granulomatous disease of unknown etiology
sarcoidosis
end stage interstitial fibrosis (seen in idiopathic pulmoanry fibrosis)
hito
honeycomb lung (irregular dilatation of adjacent airwaus due to interstitial fibrosis)
