Chronic Interstitial Lung Dz Flashcards
CXR
- small lungs
* fine lines to peripher (don’t branch like vessels)
What causes x ray lines?
Inflamed septal area (connective tissue)
Thickening it
Rule out Heart Failure/Renal failure
Diffuse Parenchymal Lung Disease
Known causes
- drugs
- inhaled particles (hypersensitivity pneumonitis)
- immune complexes
- associated w/ collegen vascular dz (immune complexes)
DPLD classification
- known cause
- idopathic
- Granulomatous DPLD (sarcoidosis = mostly AA)
- other (rare) = LAM, HX, etc.
DPLD
Etiology
*occupational/inhalant
(Inorganic, organic (HyperPneum), fumes)
- Drugs
- radiation
- infectious (TB, P carinii)
- Pulm edema, uremia
- Collegen vascular dz
DPLD
Unknown etiology
*Idiopathic Interstitial
HUGE LIST
*sarcoidosis
Pathogenesis
- injury to alveolar epithelial cells
- inflammation at MEMBRANE-LEVEL
- scarring/structural (honeycombing)
- repair = pro-inflammatory /pro-fibrotic cytokines from inflammatory/epithelial/fibroblast cells
BAL
Neutrophilic inflammatory profile?
UIP/IPF
Inorganic dust
BAL
Lymphatic inflammatory?
Sarcoidosis
Hypersens Pneumonitis
BAL
CD4/CD8 ratio
High : sarcoidosis
Low:HSP/HIV
Elasticity Measurement
Restrictive - stiffer lung, takes more work, so person accepts lower max inspiration
Spirometry
Restrictive
All volumes reduce
Spirometry
Obstructive
RV up (trapped air) Encroaches on other volumes
W/ exercise
O2 fine at rest, terrible with exercise, membrane too thick
Exercise Desaturation
V/Q
Low V because alveoli not elastic
CO2 up
- Dyspnea
- Dry cough
CXR =
- infiltrates
- restriction
- resting desaturation
- exercise desaturation
DOWN D L CO2 (CO2 diffusion)
*RULE OUTpulmonary HTN
Dx
DPLD
Areas of DX
Clinical
- History
- PE
- Lab
- PFTs
Radiology
- CXR
- HRCT
Pathology
- FOB w/ BAL/TBB
- Surgical lung biopsy
Ground glass opacities
Inflammation, water in lung
AA?
Sarcoid
Fever/HIV?
Pneumocystis
DX questions
- Specific Dx?
- Active dz?
- Need biopsy?
- Therapy?
PE
- crackles
- clubbing
- lesions, eye, lymphadenopathy, cardio, hepatosplenomegaly, MSK
- Labs
- images
- PFTs
Sarcoid in
Upper lobes
IPF in
Lower lobes
lympadenopathy + infiltrate
Sarcoid
Silicosis
Infxn (TB, Histoplasm)
Malignacy
HRCT
- early detection
- ground glass opacities
- determine biopsy site
- progression/assessment
PFTs
- restrictive
- small airway dz
- reduced DLCO2
- hypoxemia at rest
- exercise desaturation
Infiltrates + Pleural dz
- infxn (TB, Fungal)
- Malignancy
- Collogen vascular dz : SLE, RA
- Drug Hypersensitivity
- Cardio dz
- Asbestos-related Dz
PE
Watch for
- small lung volumes
- inspiratory crackles
- position dependent crackles
- Evidence for pulmonary HTN
- clubbing
- extrapulmonary signs (skin, cardio, etc.)
Idiopathic Pulmonary Fibrosis (IPF)
Also called
Cryptogenic Fibrosing Alveolitis (IPF)
Must distinguish from NSIP (nonspecific interstitial pneumonia)
Tobacco Smoker’s dz
- Respiratory Bronchiolitis Interstitial Lung Dz (RB-ILD)
- from small airway to big
*Desquamative Interstitial Pnuemonia (DIP) = macrophage cells crowd airways
Cryptogenic Organizing Pneumonia (formerly BOOP)
Pneumonia like shadows on CXR (not normal infiltrate)
- progressive dyspnea
- clubbing
- old
- progressive cough, non-productive
- mid-late inspiratory crackle
- restrictive
- DOWN DLCO
- WIDE P(A-a)DO2
- exercise desaturaton
IPF
IPF
Tx
Transplant
1/2 dead in 5 years (more deadly than lung cancer)
IPF
Biopsy?
Yes, MUST HAVE
Fibroblastic foci
- younger 40
- no association cig smoking
- dyspnea, cough, fatiuge
- weight loss
- basilar crackles
- less clubbing
Nonspecific Intersitial Pneumonitis NSIP
Classic NSIP
CXR
Histology
CXR = ground glass opacities
Histology = uniform, no fibroblastic foci
NSIP
Tx
Steroids
- SOB
- PFT = mixed obstructive/ restrictive
- DLCO down
- Pigmented intraluminal macrophages in 1st and 2nd order respiratory bronchioles
- smoker
Respiratory Bronchiolitis-Associated Lung Dz RB-ILD
Linked to DIP (more severe)
RB-ILD
Tx
Steroids
Smoking cessation
*male
*smoker
*clubbing
*40s
*insidious onset dyspnea + dry cough
*
Desquamative Interstitial Pneumonitis (DIP)
DIP
Tx
Steroids
Smoking cessation
*complete recovery possible
- young/middle age
- CXR: BILATERAL HILAR LYMPHANDOPATHY
- Pulmonary infiltration
- low fever
- skin lesions: ERYTHEMA NODOSUM/LUPUS PERNIO/MACUOPAPULAR RASH
- EYE: anterior uveitis, conjuctivitis
- non-caseating granulomas = liver, spleen, eyes, salivary ,
- CARDIO : arrhythmias
- LABS: UP ACE / Ca2+ /eosinophils
- dry cough
- dyspnea
- fatigue malaise weight loss
- AA
*NO clubbing
*NO lung noise
*
Sarcoidosis
Sarcoidosis
Biopsy
Non-caseating epithelioid cell granuloma
NEW WAY = ultrasound to find lymph node, then fine needle aspirate node
Rule out TB w/ culture
Sarcoidosis
Tx
STEROIDS
Methotrexate
Skin: *Hydroxychloroquine
Sarcoidosis
Path
*CD4+ T cells - antigens/self-antigens
HIV can eliminate!
- central immune activation, peripheral immune depression
- Granulomas (–>fibrosis) = eyes, lungs, liver
Sarciodosis
Sequalae
Large, calcified lymph nodes
Sarcoidosis
Prognosis
Self-limited course
Relentless, fibrosis of lungs/eye etc.
Asymptomatic w/ Bilat Hilar lymph nodes?
Biopsy
Sarcoidosis
Cause
Unknown
Some think microbacterial antigen
CXR
Huge potato like bilat hilar lymph nodes
Diffuse nodules
Sarcoidosis Stage 1
Get biopsy
CXR
Infiltrates more in upper lobes
Hilar lymph can push trachea
Sarcoidosis stage 2
If horrible chest x ray but patient looks well
Sarcoidosi
HRCT
Nodularity
(Edges subplural, along airways, in lung)
Beading pattern
Sarcoidosis
Sarcoidosis
Dx approach
- BIOPSY
- Extent/severity of organ involvment
- stable? Active? (NO TEST, must check function tests)
- Therapy will help?
- 4-8 hours after exposure
- fever, malaise
- cough, dyspnea
- s/s 12-48 hours
- farmer’s lung (mold in hay)
- Bird fancier’s lung
Hypersensitivity Pneumonitis
Extrinsic Allergic Alveolitis
Bird fancier’s lung
Antigens from
Feathers + droppings
More complex than farmers
Hypersensitivity Pneumonitis
Path
Inflammation in small airways
Asbestos exposure leads to
Pleural thickening = mesothelioma
Pleural plaques
Shaggy ??? In bottom of lungs
Hot tub lung
Hypersensitivity Pneumonitis
Accumulation of dust in lung
Asbestos, etc.
Pneumoconiosis
Asbestos in
Elevator break pads
Auto break pads
Pipes in building
Asbestos + cig smoker
Highest possible risk
Sarcoid
PFT
Restriction
Low DLCO
Common work exposures
- asbestos, shipyard work
- Silica, mining, quarrying, sandblasting
- Solvents, degreasers
- Two-part glue, paint, urethane
Questions about work experience
- # hours worked
- employment duration
- “mist in air?”
Labs
Up ACE?
Sarcoidosis
CXR
- Honeycombing
- more in bases
- NO ground glass really (no inflammation)
UIP