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sarcoidosis Flashcards

1
Q

what is sarcoidosis

A

Systemic disorder of unknown
cause(s) characterised by formation
of noncaseating granulomas,
affecting the lung in >90% of cases

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2
Q

where can sarcoidosis affect

A 
any organ: 
nervous
cvs
ocular = blindness 
lung most common
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3
Q

what is the initial event that leads to granulomas

A

don’t know

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4
Q

features of sarcoidosis and what the suggest

A

 Accumulation of oligoclonal T cells at sites of granuloma formation
suggest antigen-specific cell-mediated immune response
 Kveim-Siltzbach spleen extract skin tests suggest specific immune
response
 Features resemble other antigen-induced disorders, including chronic
beryllium disease, hypersensitivity pneumonitis, and mycobacterialinduced granulomatous disease

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5
Q

what are the features that suggest infectious aetiology

A

other granulomatous diseases have infectious aetiology
• Epidemiological evidence suggesting clusters of disease in
environments linked with bioaerosol production
can be transmitted by transplant organs
difference in presence of microbe in sarcoid and control – organisms found most – P. acnes and micobacteria - however confidence intervals cross the non-significant line

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6
Q

results of high throughput microbe genetic typing

A

o Correct any microbe for the sample in the env ie contaminants – found that overall the burden of microbe in sarcoid seems larger than in healthy control
o Couldn’t find microbes that were consistently present across the tissue
o Found fungi
o Need more work

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7
Q

inconclusivity of finding microbes in sarcoid

A

heterogeneity between studies, especially the ones
on mycobacteria
only few international studies
unidentified organisms may be cause
Colonizing microbes may cause disease by their antigenic or
adjuvant properties rather than infection

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8
Q

RF for sarcoidosis

A

Agricultural exposures
Insecticides
Microbial bioaerosols (work environments with mould/damp exposure)

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9
Q

protective factors for sarcoid

A

cigarette smoking - develops when stop smoking

protective against granulomatous disorders

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10
Q

genetic predisposition of sarcoidosis

A

familial clustering in several populations
More common in monozygotic than dizygotic twins
g associations with major histocompatibility
complex region (MHC) class II alleles
• susceptibility alleles: HLA DR 11, 12, 14, 15, 17
• protective alleles: HLA DR1, DR4
Other non HLA genes: BTNL2, IL-23R, NOTCH4, 11q13 locus in common
with other immune mediated diseases (ulcerative colitis, ank spond, RA)

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11
Q

incidence of sarcoidosis

A

-Major geographical differences: prevalence highest in Northern Europe, USA, India
4x more severe and multi-organ in African-Americans than caucasians

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12
Q

patterns of organ involvement

A
  • Acute uveitis and erythema nodosum in Scandinavians, Irish, Puerto Ricans
  • in Japanese, eye and heart frequent, no Lofgren (Lung)
  • Lupus pernio more frequent in African-Americans

variation suggests gene involvement

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13
Q

severity

A

more severe lung impairment and extrathoracic involvement in US AfricanAmerican, Afro-Caribbean patients

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14
Q

could sarcoidosis be a family of disease

A

Lofgren’s disease has very distinct genetic associations (HLA DR3,
CCR2), demographics and clinical findings
• Different HLA associations with different organ involvement
(uveitis, cardiac)
• Different HLA associations with acute (HLA-DR3) and chronic
(HLA-DR14 and 15) disease

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15
Q

pathogenesis of sarcoidosis

A

• Unknown ag – presented by APC coupled with HLA class 2 = amplification = granuloma

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16
Q

explain the spread of sarcoidosis

A

Loosely-formed granulomas follow lymphatic pathways:
 bronchovascular bundles
 interlobular septa
 pleura

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17
Q

diagnosis

A

no test for sarcoid
need compatible findings across investigations: clinical, XR, histology
exclude alternative ie TB
histology = non caseating granulomas
histology needed, especially if immunosuppressive treatment being considered
and/or diagnostic uncertainty (tb, lymphoma, lymphangitis)
discuss whether to do bronchoscopic histological confirmation with pt
o Aim for diagnostic probability of >95%
o Symmetrical bilateral lymphadenopathy and erythema nodosum = is sarcoid
o Calcification around LN and on both sides, on TB unilaterally only

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18
Q

how many stages of sarcoid are there

A

4

done on XR

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19
Q

stage 1 sarcoid

A 
Bilateral hilar (+ Rt paratracheal) lymph nodes 
Symmetrical
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20
Q

stage 2

A 
Bilateral hilar (+ Rt paratracheal) nodes
 Parenchymal disease - ie white lung elargement
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21
Q

stage 3

A

Parenchymal disease

No BHL/mediastinal nodes

22
Q

stage 4

A

fibrosis and scarring

upper lobe/hilar pulled up, trachea pulled to R by upper lobe fibrosis

23
Q

why is staging important

A

gives broad cohort outcomes, however
Cannot distinguish between active inflammation, fibrosis and
inactive disease
• Large variability within scorers on stage IV; trivial vs end stage lung
fibrosis
• Some pts will present at stage 1 – most will stay here, some will present at stage ¾ - sometimes see switch – but can stick with the stage you present with

24
Q

broad cohort outcomes of the stages

A 
1 = good 
2 = progression in one third at 5
years
3 =  progression in two thirds at 5
years
4 =  overt fibrotic disease, significant mortality
25
Q

diagnosis with bronchoscopy and biopsy

A

in stage 1 or 2:
EBUS-TBNA/EUS-TBNA
• (with rapid onsite cytology if available) (80% diagnostic yield)
+- TBBx (90%)

stage 3/4 
BAL: 
Lymphocytosis: 25-30%
• +/- CD4/CD8 ratio (>4 highly specific)
• Microbiological sampling
• EBBx-TBBx
(90%)
26
Q

course of most pts

A

benign

27
Q

epidemiology of progressive pulmonary fibrosis

A 
significant minority of people have it 
Approximately 8-10% of sarcoidosis patients
will have shortened life expectancy; of
these roughly 70% due to pulmonary
involvement
28
Q

CT appearance of progressive pulmonary fibrosis

A

fibrotic changes with traction bronchiectesis

airway pulled apart by fibrosis and ground glass

29
Q

predictors of mortality in pulmonary sarcoidosis

A

age
fibrosis on HRCT >20% ie extensive disease
pul HTN

30
Q

what do you need to determine about the fibrosis and why

A 
Is the fibrotic disease stable or
progressive?
• Aim of treatment is to prevent
progression of fibrosis, but avoid
toxicity in stable fibrosis
• Ongoing active inflammation likely
to lead to further progression of
fibrosis
therefore need measures of disease activity
31
Q

what are the measures of disease activity

A 
HRCT patterns
• FDG-PET
• -Bronchoalveolar lavage: lymphocytosis
• -Serum markers: inflammatory markers, ACE, IL2R, chitotriosidase, 24-
hour urinary calcium
• -Serial Lung Function Tests
32
Q

can you assess reversibility with CT

A

o Nodular sarcoids – reversible, agglomerates of sarcoid glomeroma therefore active – reversible
o Ground glass – grey attenuation of parenchyma - mostly irreversible – cant determine if still inflamm
o Irreversible – UIP pattern with honeycombing
so CT cant always distinguish

33
Q

Use of FDG-PET to determine stable/progressive

A

FDG-PET/CT correlates with other markers of disease activity ie BAL and serum inflamm markers
• PET activity correlated with more severe lung function impairment and extensive
CT changes, including fibrosis
most have extra-thoracic PET positivity too
ie still inflammation in fibrotic changes
therefore treatment with anti-TNF (infliximab) = improvement

34
Q

monitoring pulmonary sarcoidosis

A

Change in symptoms of breathlessness/cough
Lung function: change in FVC, FEV1 by ≥10%; DLCO ≥15% (ATS/ERS criteria)
• (isolated drop in DLCO suggesting pulmonary vascular involvement)
chest Xray
• chest HRCT better correlated with lung function changes
• (FDG-PET to assess ongoing activity when disease not responding to treatment)
serum biomarker changes

worsening in >1 domain will increase the liklihood that the changes are real

35
Q

what is the major complication of sarcoidosis

A

chronic pulmonary aspergillosis
o 5-10% from chronic sarcoid suffering from CPA – all had advanced disease, high prev pul htn, treat with long term anti-fungal- death caused by advanced sarcoid, rather than massive haemoptysis – although concern in cavitating lesions full of fungus
o More common fungi exposure

36
Q

what are the indications for management of sarcoid

A

major loss of quality of life (fatigue that is life changing, skin disease, joint pains – may not danger from internal organ though)
danger from disease

37
Q

treatment options for QOL

A

discuss with pt
if dont treat - NSAIDs for joint pains as needed
if do:
Low dose prednisolone
• Hydroxycloroquine
• Less likely to use methotrexate or azathioprine (immunosuppressants), except for particular
cases

38
Q

death in sarcoid

A

<10% of patients have a shortened life expectancy
• Respiratory failure and cardiac failure/arrhythmias the most
common

39
Q

risk stratification in sarcoid

A

• Integration of clinical, functional and imaging features to
assess likelihood of progression of pulmonary sarcoidosis
• In cardiac disease, we need markers of accurate early
diagnosis to prevent loss of function and fatal arrhythmias

40
Q

how do you identify pts at risk of progression of fibrosis and so which to treat

A

• Severity of pulmonary fibrosis:
• Irreversible changes present
• Integration of symptoms, lung function and imaging
Which lung function thresholds ? Lung function:
• FVC <60%
• DLCO <50%
• Major airflow obstruction
Longitudinal behaviour - if function dropping and symptoms increasing need to treat

41
Q

describe cardiac sarcoid

A 
o	Should screen for involvement in all
o	Ask about presence of symptoms Palpations, disproportionate breathless, syncope, presyncope 
ECG abnormalities
Echo
if find things = furtehr imaging
42
Q

ECG abnormalities in cardiac sarcoid

A 
AV block, 
frequent premature ventricular ectopics,
non-sustained/sustained ventricular tachycardia, 
LBBx or RBBx, 
Q
waves, 
axis deviation
43
Q

echo abnormalities in cardiac sarcoid

A 
reduced LVEF, 
regional wall abnormalities, 
wall thinning or
thickening, 
ventricular aneurysms
44
Q

further investigations used if suspect cardiac sarcoidosis

A

cardiac MRI
FDG-PET alongside resting myocardial perfusion scan
PET tends to be more sensitive than MRI: however,
whether isolated PET positivity has prognostic significance
remains to be established

45
Q

treatment of sarcoidosis

A

o Toxicity of steroid very diff high dose compared to low dose
o Second line immunosuppression
o Corticosteroids – mainstay of treatment – moderates doses
o High dose long term = methylprednisolone
o Aim is to go as low as possible – maintain improvement and objective lung func improvement
o 2nd line – methotrexate, azathioprine +- hydroxychloroquine
 Used if low dose steroids not sufficient to allow control or need corticosteroid spearing – comorb (obesity, dm, osteoporosis), intolerance
o Biologics
 3rd agent – once pt has failed steroid and 2nd line
 Active and progressive disease despite non conventional or Toxicity or lack of tolerability to conventional
treatments

46
Q

steroid regieme in sarcoidosis

A

• Corticosteroids remain mainstay of treatment:
• Induction
• Moderate dose oral prednisolone (20-40 mg od)
• Or in selected cases with severe disease
• High dose iv methylprednisolone treatment (1 Gr x 3) followed by 20 mg od, then
tapered
• Maintenance
• Low dose 7.5-10 mg od

47
Q

PET

A

CT pick up radioactive glucose – injected into vein – goes to more active site ie inflammation or cancer- light up – wont tell sarcoid, just that there is positivity

48
Q

honeycomb pattern

A

parallel lines of cysts that have walls
end stage fibrosis
often seen in usual interstitial pneumonitis - pattern that defines idiopathic pul fibrosis
also in sarcoid
happens because disruption of architecture -dialtion of airways, formation of aberrent airways and cystic spaces

49
Q

why is the hilar pulled up in stage 4 sarcoid

A

all granulomatous disease go to apices because done with things inhaling eg hypersensitivity pneumonitis – fibrosis which retracts the lung and therefore pulls up the hilar

50
Q

presentation of sarcoid

A

o Non-specific
o Extra-pul involvemenht help – erythema nodosum – painfulnothces on shin
o Classic skin lesions elsewhere
o Young pts
o Fatigue – incredible
o Joint pain – ankles most commonly involved
o Cough, breathlessness – XR will show one of the stages

51
Q

what is TBNA

A

endobronchial aspirate – US guided – go with bronchoscopy and fine needle to biopsy LN of mediastinum

DPD (38 decks)

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