ILD Flashcards
Ild definition
Progressive fibrosis interstitial pneumonitis of unknown cause
Prev 30,000 in uk
IPF histology
Alveolar injury
Fibroblastic foci with neutrophils
Mesenchymal cells
Architectural distortion
Interspersed normal lung
CT IPF
Def UIP
Subpleural basal predominance
Reticulation
Traction Bronchiectasis
Honeycombing
Architectural distortion and volume loss = propeller blade
No GG
Ix IPF
Ear and crp raised
RHF and anca raised
Restrictive pft with reduced TLCO
Incidence IPF
14 in 100,000
Men
55-70
Prognosis only worse in lung and pancreatic ca
Treatment evidence
Strong LTOT nintedanib and pirfenidone
Weak steroids acute IPF, gord treatment, PR
Treatment IPF
PR
LTOT
Cough suppressant
Pirfenidone
Antifibrotic stops collagen synthesis by targeting tgf a and b
Fvc 50-80
Se rash gi headache
CI if egfr below 30. Pregnancy or bf. Child Pugh C. Smoking
Stop fvc falls 10% in 12m
Nintedanib
TKI inhibits platelet derived gf and vegf
Fvc 50% and above
Se diarrhoea, weight loss and bleeding
CI mi 6m. Unstable angina. Smoking. Pregnant. Soy allergy
Increase survival 38%
Stopping fibrotic
Fvc increase 10% to 2nd
Still progress bsc
Benefit
Reduce FVC dropping
Nintedanib survival
No change symptoms
Drug induced ILD
Ild
NF hp, amiod pmeumonitis or cop, bleo fibrosis
Airway
Bb penicillamine
Acei cough
Pleural
Bb NF Mtx phenytoin sv
Ptx
Bloem
VTE phenytoin
Fibrosing mediastinitis ergot alkaloids
Haemorrhage Mtx NF penicillamine contrast
Radiation
Chronic Eo pneumonia few
OP
Migratory areas atypical consolidation beyond radiotherapy field
Mx pred
Pneumonitis
Radiological change without symptoms
Diffuse alveolar damage and vascular fibrosis
Pred 70mg
Worse pg with chemo fibrosis
NSIP
40s
CT
Ground glass predominant
Basal
Subpleural sparing ***
Homogenous
No honeycombing or traction Bronchiectasis or reticulation
1 Cellular
2 Inflammation and fibrotic
3 or fibrotic Hx
Bal lymphocytic
Bx except in CTD Ild
Restrictive reduced TLCO
Causes CTD drugs infection HIV bmt chemo
Worse prog
drop fvc 10% in 6m or TLCO 15% in 6m
Fibrotic
Pred then aza or MMf
5 year survival more than 50%
Cellular better pg
OP
Months onset
Alveoli plugged granulation tissue extends to bronchioles without architectural distortion
Cause infection drugs CTD cancer bmt pulmonary infarction
Drugs amio ifn minicycline ritux statin
Ct
Consolidation Subpleural and basal atol sign
Nodules
Reticulation poor prognosis
CTD basal finger like projection
Pft rest
Good prognosis
Pred 30-90mg and wean
Severe I’ve methylpred then cycle or aza
AIP
Acute diffuse alveolar damage
Few weeks onset after viral illness
CT
Diffuse patchy gg change +- consolidation.
Can develop fibrosis
Spares CT angles
Hx
Hyaline membrane /oedema / alveolar septal thickening
Honeycombing
Mx
Treat infection
I’ve methylpred
Itu
50% mortality
Hypersensitivity PNEUMONITIS
Organic substance inhaled then IR sensitised causes lung damage
Lots of antigen acute reversible
Low dose antigen chronic
Granuloma loose ass lymphocyte
Genetic pred
Dx symptoms inc systemic
Repeated inhalation 1-3 microns
Sensitised hyper responsive pt
Exaggerated response
JOBS: gardening mould**, farmers hay, hot tub, bird fancier
Type 3 and 4 immune mediated so TC mediated granuloma bronchi and alveoli
T3 subacute and T4 chronic
OE
-Squeaks ALVEOLITIS pathopnemonic HP
- clubbing
Inflammatory
Fibrotic
Hx
Bronchiolitis
non caseating granuloma
Ct
1) Acute micro nodules/GGo bronchocentric upper lobe/ mosaicism with apical sparing and med LAN; axial
2) Chronic I’ll defined centrilobular nodules /GGO /mosaicism /retic w traction BRe w honeycombing TOP lung/ CT MOSAICISM WITH THREE DENSITY SIGN (nodules w GG w air trap same lobe); UPPER LOBE
Thermophilic actinomycete hay
MOULD fungi
Asp clavitus bakery malt worker
MAC hot tub
Bird protein feather
Drugs amio mtx nf chemo
IgG to antigen exposure
BAL lymphocytes more than 25% (>50% diagnostic cellular). Normal fibrotic
Cd4 to cd8 less than 4
Gold standard BAL and TBB Bx necrotising loose granuloma
Mx
1) antigen avoidance esp inflam
2) Pred esp chronic indication significant impairment and symptoms or fibrosis. Pred 0.5mg/kg and taper 1m. Not evidence based
3) MMF or Aza ; indication worse lft on pred wean
4) RTX not licenced
Indication pred and ISS
TLCO <60% w symptoms
Any fibrosis
Pg poor chronic
UCSF non standardised questionnaire (exposures)
DIP
Pigmented macrophages in alveolar space
Progresses from RB ILd
Rare
Weeks to months
Clubbing
Smokers
30-50
CT
GGO lower peripheral
Mild retic and Honeycombing but mild
BAL pigmented macrophages alveolar spaces
Mx
Stop smoking
Pred
RB ILD
Pigmented machrophages in smokers
Years onset
Smokers
Over 30
Male 2x
CT
Centrilobular nodules
GGO
Thick walled airway
Ass centrilobular emphysema
Hx pigmented macrophages terminal bronchi
Pg good
Mx stopping smoking only evidence based
LIP
Lymphoid infiltrates and lymphoid hyperplasia
CTD sjogren***
HIV CVID
PCP hep b
AI
Phenytoin
Years onset with Resp sx and weight loss
Rare
Increased ig
CT
GGO
Reticulation
<3cm cysts throughout inc Subpleural
nodules centrilobular
BAL lymphocytes
Bx
Alveolar separation
Lymphoid hyperplasia
Mx steroids
Eosinophilia lung disease
Raised Eo with CT infiltrates
Asthma ABPA
Poorly controlled asthma
Positive RAST raised IgG prec
Central Bre
Steroid then itrac
Pulmonary Eo
Larvae cross to lungs cause allergy
Resp and constitutional sx
Pulmonary infiltrates
Eo sputum and stool
Albendazole then steroid
Tropical plum Eo
HS to wucheria cross into blood or lymph enter lungs
Weeks to months Resp and systemic relapse remit
Mid zone lung mottled. Cavitation. Effusion
Raised Eo blood.
BAL Eo and Eo Hx
Obs spiro
Filaricide mx
Chronic Eo pneumonia
Sob Haemoptysis asthma fever weeks to months
Middle aged women
Sputum Eo but normal bloods
Ct peripheral consolidation opposite pulm oedema
Bal over 60% Eo
Steroids 6m as 50% relapse
Acute Eo pneumonia Dx:
1 week unwell
Febrile hypoxia
Young men
Alveolar infiltrate GGO and effusion
Normal Eo
Bal Eo more than 25%
Steroids response
No relapse
Hyper Eo syndrome
Weeks to months itching /Resp systemic /cardiac /CNS /GIT /etoh intolerance - Eo tissue infiltration
Eosinophilia over 1.5, IgE high
Cxr infiltrates
CT interstitial infiltrates w effusions
Pred 50% response rest cyclo Aza
EGPA
Drug induced
Pulm vessel wall damage due to drug reaction
Rash and Resp
CT shadowing in hours lasts weeks after
Eo tissues on bx
Cause Mtx nsaid penicillin phenytoin NF cocaine carbamazapine
Mx stop and severe steroids
Alveolar microlithiasis
Diffuse calcified microlith in alveolar space
Mutation SLC34AZ
AR
Issue type 2 alveolar cells so caphos in alveoli
20-30s
Sob to Resp failure
Micro nodules bases
Restrictive
Lung tx
Amyloidosis
Extracellular deposition low molecular protein insoluble fibrils so crystal formation
AL cause MM, heart, kidney, GI, BM
AA chronic inflammation eg RA and infection
DA b2 microglobulin dialysis
Presentation
Laryngeal nodule
Trachea bronchial amyloid
Parenchymal nodule Subpleural and bilateral with cavity
Med LN
Pleural effusion
Diaphragm
TBB vs VATS
BMT
Restrictive reduced TLCO
Localised laster
Diffuse chemo
Hereditary haemorrhagic telengiectasia
1 in 8000
Epistaxis GI pulm haemorrhage
AD ENG or ACRL1 or SMAD4 gene
Risk phtn or VTE
Symptomatic treatment
If iron
VTE mx
AVM IR or surgeons
Pulmonary AVM
Abnormal blood vessels replace low resistance pulm capillaries
Abnormal connection so continuous RtL shunt, sv bypass pulmonary circulation
Ch 9
TGFB gene
Most due to HHT
Orthodoxia fall Sats sit up or platypnoea sob when sit up
Telengiectasia
Ct mass
Hypoxia on standing
Echo
Angiography
Risk 25% paradoxical emboli so stroke /MI /cerebral or peripheral abscess
10% pregnancy haemorrhage
CX
Pulmonary haemorrhage embolism IR reduce shunt and improve hypoxia or sx
Stroke aspirin and thrombolysis not advised no data
Cerebral abscess
Screen with CT diagnostic
HHT or first degree family pavm
Mx all refer embolisation unless CI (relative pregnancy or PH or renal impairment)
Re LTOT no alveolar hypoxia so only if symptomatic
Prophylactic Abx dental or endoscopic or surgical
Treatment pre pregnancy as 1% risk death haemorrhage
Pulmonary alveolar proteinosis
Macrophages cannot clear surfactant so fill space with protein
Rare middle aged smoking men
Defect GM CSF
Drugs dust lymphoma leukaemia
CX nocardia fungi NTM
Bx surfactant protein IHC
Alveoli PAS, cholesterol clefts and large foamy macrophages
Crazy paving
BAL milky cellular debris
Whole lung lavage under GA on bypass good ps
P g 1/3 respond same worse
Recurrent respiratory papilloma
HPV 6 and 11
Affects epithelium and mucus membrane
Nodules larynx trachea
Laser or debride vs ifn/mtx/aciclovir
Se laser thermal injury/ stricture/ spread
Idiopathic pulmonary haemosiderosis
Haemosiderin laden macrophages
RA hyperthyroid AIHA coeliac
Pulmonary haemorrhage
LAN
HSM
Clubbed
Ct diffuse pulmonary infiltrate
Restrictive raised kco
Bal haemksidderin laden mc
Mx
Iron
Cyclophos no change outcome
Lung tx
Ptx variable
Primary ciliary dyskinesia
Abnormal cilia so abnormal MC clearance so micro colonisation
Chronic infection
Bronchiectasis
Ar
Kids OM situs inversus Resp distress
Adult Bronchiectasis clubbing infertility
Saccharin test
Reduced feno
Abx
PT
Vaccine
Haemorrhage management
ARDS
Lung injury so increased vascular permeability
Fluid and protein in alveolar space + surfactant damage
Reduced compliance
VQ mismatch and increased LtoR shunt
Hypoxia
PHN
Dx
Within a week of insult
Bilateral infiltrates on CT
Echo no RHF
Pa02 over fio2 less than 300
Treat. Cause
Permissive Hypercapnia
To less than 6ml per kg
Increase peep
Plateau pressure less than 30
Paralyse
Negative fb
Ecmo
Sarcoid
Environmental trigger
Cd4 activated release il2 and ifn gamma so attract macrophages
Release ace
Granuloma fibroblast
Non caseating granuloma
Lofgrens EN BHL arthralgia — severe arthralgia steroid— 80% remits 2y
Chronic infiltrative lung disease
Stages lung
O nil
1 BHL —90% resolve
2 BHL and parenchymal change —70% resolve
3 parenchymal change —20% resolve
4 fibrosis —0% resolve
Other raised ace or ca
CT
Micro nodules perifissural
GGO
BHL
Fibrosis late
Ebus gold standard 93% sensitivity
Ebus Hx and afb
Bal cd4: cd8 over 4. Lymphocytes over 25%
Mx
Symptomatic stage 2 onwards pred then wean. Higher dose pred cardio and renal
Second line Mtx or aza or hq or MMf : indication progress/ SE pred/ not able taper below 10mg/ com/ pt wishes
3rd inflix + aza or Mtx
S4 lung tx
Pred indication
Severe symptom life threatening
Extrathoracic
When to treat lung
DLCO less than 65
Fev1 less than 70
Fvc drops 10% and TLCO drops 15%
Extra thoracic features Sarcoid
CNS
CN or neurosarcoid
CSF ace and raised wcc
Git cirrhosis
Calcium raised
Granuloma converts d3 to active vitamin d
Fluids and low dose pred
Skin en or lupus pernio
Topical pred then po
Eyes uveitis
Topical steroid then po pred or iss
Cardiac 5%
Cardiomyopathy with chb or pericarditis
Echo and ecg
Then holter with cmri
Steroid amio to ppm to heart transplant
Kidney
Hyperca or GN
Steroid then hq
Poor prognosis sarcoid
Black
Over 40
Neuropathy
Lupus pernio
Nephrocalcinosis
Lofgrens good prognosis
Langerhans cell histocytosis
Dendritic cell proliferation so Histocyte infiltrate lung and multi organ
MAPK pathway 85%
BRAVF 600 mutation 50%
Unknown antigen
BIRKBECK granules
Smokers
Pneumothorax 15%
Systemic langerhans
rib lesion
PH
DI
Skin lesion
HypoT
Lymphoma
Association. Phtn DI lymphoma lung cancer
CT
Nodules with cavity spare cpa
Upper and mid zones
Apical Thick walled cysts irregular
Normal lung in between
Bal pigmented macrophages
Hx birkbeck granules
Mixed defect pft
Mx
Don’t smoke
Pred not evidence based
Cladrabine
Transplant if PH
Pg variable
LAM
Abnormal proliferation smooth muscle and form cysts
TSC gene
Young
female
Non smoker
Ass epilepsy, LD, TS and kidney tumour angiomyolipoma
Cysts to Ptx
Chylous effusion
Obs spirometry or mixed
CT
Cyst various size with Normal lung between BUT MORE OVAL
Lower lobes Include CPA
Pleural effusion
Other
Lipomata kidney
LN abdominal
Skin swelling
Chylous ascites
Stop smoking
Avoid oestrogen
Sirolimus mTor inhx (IND poor lft)
Pleural pleurectomy or pleurodesis or thoracic duct ligation
Bad OCP increases symptoms
Birt Hogg
Defect follicular gene
Present cystic lung disease, skin tags, Ptx, renal tumour
Cystic lung disease
Lower zone
Oval lentiform septated cyst next to interlobular septa and vessel
No GGO
Other
Fibrofolliculoma
Kidney tumour
AD
FLCN gene
BAL
Cd4: cd8
High sarcoid
Low hsp
Neut (* more than 50%)
ARDS **
Dah
Aspiration pneumonia **
Bacterial infection **
IPF
Asbestosis
Lymph >25%
hsp
sarcoid
Chronic berylliosis
Cellular NSIP
Drug reaction
LIP
COP
Lymphocytes more than 50%
HSP
Cellular NSIP
Eo
Acute or chronic Eo pneumonia more than 25%
Drug induced
Asthma
Egpa
ABPA
PJP
Lymphoma
Specific
Proteinacious PAP
Haemosidderin haemorrhage
Macrophage foamy amiodatone
Erasmus
Scleroderma and silicosis
CT
Upper lobe nodules
Fibrocalcinosis
Anti synthetase
Auto ir myositis
Anti jo positive
Anti rna synthetase
Raynauds
Arthritis
Ild
Fever
PM or DM
Mechanics hands
CT
OP
NSIP
Ppfe
Cough and sob
Rf for Ptx
Ct
Apical caps
UL volume loss
Traction bre
Small vessel vasculitis
EGPA
Dx
Asthma
Eo more than 10% peripheral
Pulmonary infiltrates
Sinus disease
Mononeuritis
Bx eosinophils
PANCA MPO
—————————————————
Werner’s GPA
90% lung involvement
Dx
Subglottic stenosis, nasal ulcer
Nodules lung, pulmonary haemorrhage
Renal failure GN
CANCA positive PR3
Mx
End organ issue eg pulm haemorrhage or Renal failure :
methylpred then cyclophos then ritux/aza
Plex
Dialysis
Systemic sclerosis Ild
Rare 100 per million
Female middle aged
GI skin raynaud calcinosis telengiectasia microstomia
Anti scl 70
Pattern ILD
NSIP then UIP
Mx
A) Lower dose pred 10mg used
—Methylpred contra indicated risk Renal crisis
B) MMF or Cyclophosphamide/aza
C) Nintedanib in fibrotic NSIP no FVC cutoff. Reduce rate decline fvc
— not for ritux or aza
Cystic lung disease
Cyst
Thin walled
Normal lung
LAM
lCH
LIP
Birt Hogg dube
Secondary
UIP thick walled honeycomb
PJP prox UL bilateral
HSP
CTD ILD and Mx
Rheumatoid
Sle
Systemic sclerosis
PM/DM
Pred then cyclophos or MMf or aza or ritux. Avoid high dose MP risk renal crisis esp Syst sclerosis
Indication more than 20% lung or symptoms or reduced fvc
Nintedanib indication
- Worse FVC 10% 24m
- Reduce FVC 5-10% with worse fibrosis and symptoms
- Worsening symptoms and fibrosis
Prognosis IPF
3 years
Cause IPF
Gord
Wood
Metal
REPEATED ALVEOLAR EPITHELIAL INJURY
ABERRANT WOUND HEALING
UIP
IPF
CTD ILD esp RA
CHRONIC HP
SARCOID
NITROFURANTOIN ILD
ASBESTOSIS
Poor prognosis IPF
Low bmi
TLCO less than 40%
TLCO decline 15% or fvc 10% in 6-12m
PH
Fibroblasts on Bx
——————————-
Age
Gender
TLCO
Desat 6wt to less than 88%
TLCO less than 40%
TLCO fall 15% in a year
Fvc fall 10% in a year
Other bmi low, ph
ERS classification UIP
UIP
Probable
Indeterminate
Alternate diagnosis
Upper or mid lung
Fibrotic HP
Sarcoid
CTD ILD
Nodules
HP
Sarcoid
LCH
Pleural plaques
Asbestosis
Dilated oesophagus
Systemic sclerosis or ct Ild
Normal BAL
85% mc
10% lymphocytes
3% neutrophil
1% epithelial
Worse prognosis HP
Older
Clubbed
More exposure
Fibrotic foci
NSIP and UIP on histology
Unknown antigen
Heerfordt Waldenstrom syndrome
Subtype sarcoidosis
In Afro caribbeans
Enlarged parotid and salivary gland. Facial nerve paralysis. Anterior uveitis
Pulmonary htn in sarcoid
Reduced DLCO and restrictive
Increased PA
Sats less than 90%
Need for LTOT
Sarcoid CT
LN bilateral symmetrical homogenous icing sugar or eggshell
Nodules paraseptal with perifissural beading that coalesce
Fibrosis
Reticular opacity
Volume loss
Traction Bronchiectasis
Air trapping
Sarcoid no Bx
Lofgrens
Long standing pulmonary disease
Sarcoid and hydroxychloroquine
Fatigue
Joint swelling
Skin
Immune tox
PDL1 antagonist
Cough sob chest pain
Ct GGO hsp op
Bal exclude infection
Grade one mild dose reduce
Grade two hold and pred
Grade three ARDS - hold itu methylpred w Abx then post infection screen cyclo or mtx
IPF oe
Clubbing
Inspiration creps
ILD Ix
Ana
Anca
RHF anti ccp
IG
HIV
Avian prec
Myosotis panel
Honeycombing
Thick walled
3-10mm
Nintedanib fibrotic ILD
Transplant indication IPF
Fev1 falls 10% 6m
DLCO falls 15% 6m
DLCO <40%
Desat on 6WT
Sats < 88% on 8L
Po2<8 on 5L
Urgent
Po2 less than 8 despite 10L
Refractory RHf despite medication
Genetics IPF
TERT is short telomere syndrome
CP grey prem, blood abnormality, nail dystrophy, OP, liver disease
Muc5 is mutation in surfactant cells
CTD ILD epidemiology
ILD by disease
RA 11%
systemic sclerosis 50%
Myosotis 40%
Lupus 6%
Mixed connective 60%
Risk for ild ctd
Older male smoker
Increased duration disease
Severe disease
Positive serology
MTX and ild ctd
Reduces risk
RA CT
UIP
Then NSIP
Reticulation on CT
White lines on CT
Ritux and toci CTD ILD
Ritux
As good cyclophosphamide
Better se profile
Not licensed
Toci
Reduce fvc decline
Rheumatoid
Young women
Anti ccp
RhF
CT
UIP most common
NSIP
Mx
- Continue methotrexate
- Pred no evidence base
- MMF or cyclophos/aza - right thing in UIP RA retrospective study safe
- Nintedanib
MDA5
Rare
50-60
Female
10% risk cancers
MDA5
NSIP
OP
Pred
MMF then aza/mtx or ritux
Bad prognosis
Sjogren
Ro and La AB
LIP most common
NSIP
MPA
Small vessel vasculitis
MPO positive
CT
UIP
CT imaging HP
HRCT with expiratory hold
HP diagnostic steps
BAL and TBB diagnostic in non fibrotic
Fibrotic lung biopsy
Loose bronchocentric granulomatosis
Serum prec
IgG to precipins
Exposure
FNSIP vs fHP
HP upper lobe
NSIP basal
Sjogrens
Lymphocytic infiltration
Sicca
25% Resp pleuritic chest pain due to thickening / dry cough/ NSIP more than UIP or OP/ NHL 40x risk / pH rare
Ankylosing spondylitis
15% affects the lung
UL fibrosis
Pleural involvement
Cyst or cavity
Aspiration
Restrictive defect
Behcets
CP
Oral or genital ulcer
Skin lesion
Arthritis
Uveitis
Resp
Thrombosis
Pulmonary aneurysm
Effusion
Eo pneumonia
Rheumatoid
20% lung manifestation
Pleuritic pain
Effusion
Exudative or pseudochylo
Drain and steroid
Fibrosis
Sob and cough
Clubbing and crackles
UIP more than NSIP
Restrictive
Raised ana
Pred
Poor prognosis
Acute pneumonitis
Pred
Nodule Subpleural
CX Ptx
Pet less avid
OP
Fever cough sob
Ct Bx
Pred
OB
Proliferation terminal bronchioles
Sob and cough.
Squeak
Obs pft
Pred
Vasculitis
Association cryogenic/ arthritis/ Bronchiectasis/ Sjogrens/ Kaplan
SLE
Pleural
Bilateral Exudative
Raised lymph or neut
Bx non specific
Mx drain nsaid pred
Atelectasis
Ild
70% NSIP, 5% UIP
Restrictive
Good pg
Other
DAH
OP
Acute pneumonitis
50% mortality
Non specific alveolar injury on Bx
MP then cyclophos
PH
50% 5y mortality
Mx as PH
30% have antiphos
Shrinking lung
Sob and diaphragm weak
Ct Normal parenchyma
Restrictive with reduced TLCO
Pred
DM or PM
Ild
Sob cough arthralgia fever
Fine basal crackles
NSIP mostly
UIP
OP
dAH
Association
Anti synthetase
- polya fever mechanic hands myositis
- ANA neg/ RNA synthase JO post
MDA5
Anti pm scl DM
Pred then ritux
Poor prognosis
OP or DAH
pH
Pharyngeal weakness and aspiration - restrictive - t2 rf
Spontaneous pneumomed
Systemic sclerosis
Limited
Crest
Raynaud
PF or PH in 25%
Diffuse cutaneous
Rauynaud
PF or PH in 20%
Overlap
Systemic sclerosis
——————————-
Pulmonary fibrosis
Sob and raynauds
Crest and crackles chest
89% ANA, anti scl
Restrictive with reduced TLCO
NSIP then rarer OP
Pred then MMf or cyclophos
Prognosis better than IPF
PH
G1 or G3
Sob
CX RHF
Prostacyclin iv
Chest wall restriction due to skin thickening
Aspiration pneumonia
Bre
Percentage PE from DVT
70%
TAPSE
Tricuspid annular plane systolic excursion
Displacement of lateral tricuspid annulus towards apex during systole
measures RV function
Kaplan
Seropositive RA and
Low exposure pneumoconiosis
