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BRS Y2 > lupus > Flashcards

lupus Flashcards

1
Q

the 4 autoimmune connective tissue disorder/multisystem autoimmune rheumatic diseases

A

systemic lupus erythematosus
sjogren’s syndrome
systemic sclerosis (scleroderma)
autoimmune inflammatory muscle disease

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2
Q

SLE where does it cause inflammation

A

multi-site inflammation- joints, kidney, skin

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3
Q

autoimmune connective tissue disorders general points
Erosive, non-erosive?
What is typically seen in serum?
Common symptom/sign?

A

1)typically non-erosive (unlike Rheumatoid)

2)serum autoantibodies are characteristic

3) raynaud’s phenomenon
intermittent vasospasm of digits. triggered by cold exposures=triphasic colour change

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4
Q

Typical triphasic colour changes in Raynaud’s phenomenon

A

WHITE -> Vasospasm leads to blanching of digit
BLUE -> Cyanosis as static venous blood deoxygenates
RED -> Reactive hyperaemia

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5
Q

SLE clinical features

A

swan neck deformity/malar rash
raynaud’s
arthralgia
thrombocytopenia/haemolytic anaemia

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6
Q

Anti-nuclear antibodies (ANA) how to use results

A

Negative ANA effectively rules out SLE
However, ANA is not specific for lupus

1)+ve ANA

2)report strength (- max dilution which ab can still be detected) and pattern (autoantigen the ab are reacting to)

3) specific autoAb test to identify what ANA are reacting to:
Anti-ds-DNA ab
Anti-Ro
Anti-La
Anti-smith
Anti-RNP

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7
Q

which is stronger and by how much in dilution: ANA 1:320 and ANA 1:80

A

ANA 1:320 is three times stronger than ANA 1:80

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8
Q

Antiphospholipid (APL) antibodies- where do they target

A

antibodies directed to phospholipids on cell membrane

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9
Q

Antiphospholipid (APL) antibodies are associated with ↑ risk of:

A

1) Thrombosis
arterial (e.g. stroke)
venous (e.g. deep vein thrombosis, DVT)

2) Pregnancy loss (miscarriage)

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10
Q

antiphospholipid antibody how to measure in lab

A

measure in clinical lab: anticardiolipin antibodies, anti-beta2glycoprotein 1 antibodies

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11
Q

Persistent presence of APL + a clinical event

A

“anti-phospholipid antibody syndrome”

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12
Q

primary anti-phospholipid antibody syndrome is:

A

Anti-phospholipid antibody syndrome in absence of SLE

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13
Q

what is anti-double stranded DNA antibodies (anti-dsDNA) significant in

A

specific for SLE
Serum level of ab correlates with disease activity

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14
Q

anti-Sm ab significance

A

specific for SLE
serum level of ab does NOT correlate with disease activity

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15
Q

anti-Ro ab
anti-La ab
significance

A

secondary to sjogren’s syndrome
neonatal lupus syndrome

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16
Q

what is seen in neonatal lupus syndrome

A

transient rash in neonate
permanent heart block

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17
Q

Rheumatoid arthritis auto-antibody

A

RF
Anti-cyclic citrullinated peptide antibody
(Anti-CCP is a type of ACPA)

18
Q

SLE - immunopathogenesis
which are innate and which adaptive

A

innate immunity:
overactivity of type 1 interferon (ie.IFN-a) pathway/
complement pathway abnormalities

adaptive immunity:
autoreactive B+T cells

19
Q

Gene expression studies compared lupus patients to healthy controls found overexpression of genes activated downstream of the type 1 interferon receptor binding-> “interferon gene signature.” what is a high interferon gene signature associated with

A

worsen disease

20
Q

what does (not available in UK yet) can block type 1 interferon receptor to treat SLE

A

anifrolumab

21
Q

SLE- waste disposal hypothesis. how is the immune system generating a response to nuclear antigens? (should be hidden inside the cell)

A
  1. apoptosis leads to translocation of nuclear antigen to membrane surface
  2. impaired clearance of apoptotic cells= enhance nuclear antigen presentation to immune cells
  3. B cell autoimmunity
    4.- complement activation and Fc receptor engagement
22
Q

SLE investigations

A

high ESR
normal CRP

measure urine protein (uPCR)
creatinine
albumin
kidney biopsy if proteinuria

ANA
Anti dsDNA ab
complement consumption- low C3/C4
Antiphospholipid AB

23
Q

SLE-measuring disease activity
what is shown in unwell pt with active lupus
-what is their C3/C4/ anti-dsDNA ab levels

A

low complement C3/C4
High anti-dsDNA antibodies

24
Q

SLE management: for all pt? mild/serious disease? renal disease?

A

hydroxychloroquine- All pts
steroids-acute flare

mild-hydroxychloroquine
serious-immunomodulatory agent (methotrexate)

renal disease-mycophenolate+/-rituximab

25
Q

steroid side effects

A

infection
osteoporosis
AVN

26
Q

SLE management: for persistently active disease, life-threatening, SLE+ Antiphospholipid antibody syndrome

A

for persistently active disease,
Bell target therapies- rituximab

life-threatening, (ie.myocarditis)
iv steroid+ IV cyclophosphamide

SLE+ Antiphospholipid antibody syndrome
anticoagulation (warfarin)

27
Q

SLE and pregnancy what to do to get best outcome

A

pre-pregnancy planning and getting SLE into remission

28
Q

SLE and pregnancy- APL ab, renal, Ro ab?

A

1) Antiphospholipid antibodies associated with miscarriage.
Can reduce risk with aspirin or heparin

2) Pregnancy increases haemodynamic demands – will worsen renal dysfunction

3) Ro antibodies: can cause fetal heartblock

29
Q

SLE-pregnancy- Drug considerations: WHICH ARE teratogenic

A

MMF, cyclophosphamide, methotrexate, warfarin are teratogenic.

30
Q

sjogren’s syndrome what it is and effects *IGNORE

A

Autoimmune-
lymphocytic infiltration of exocrine glands (lacrimal and salivary glands)

Exocrine gland pathology:
Dry eyes (xerophthalmia)
Dry mouth (xerostomia)
Parotid gland enlargement

31
Q

sjogren’s syndrome (other manifestation) *IGNORE

A

non-erosive arthritis
Raynaud’s phenomenon
autoimmune hepatitis

32
Q

autoantibodies present in sjogren’s syndrome *IGNORE

A

Autoantibodies: Ro+, La+. RF often + but CCP -.

33
Q

what is it called Sjögren’s syndrome if occurs in context of another connective tissue disorder e.g. SLE *IGNORE

A

‘secondary’ Sjögren’s syndrome

34
Q

what is the schirmer’s test *IGNORE

A

assess tear production- under 5mm after 5 mins is abnormal

35
Q

inflammatory muscle disease 2 types *IGNORE

A

dermatomyositis
=muscle and skin inflammation

polymyositis
=muscle only, no rashes

36
Q

skin changes in dermatomyositis *IGNORE

A

-lilac colour rash around eyelids, malar, naso-labial folds

-red/purple flat/raised lesions on knuckles (Gottron’s papules)

37
Q

inflammatory muscle disease investigation *IGNORE

A

1) bloods: high CK
2) electromyography- electrical activities of muscle
3) muscle biopsy
polymyositis = CD8 T cell

dermatomyositis = CD4 T cells+ B cells

38
Q

what is inflammatory muscle disease associated with *IGNORE

A

malignancy and pulmonary fibrosis

39
Q

systemic sclerosis signs *IGNORE

A

Thickened skin with Raynaud’s phenomenon
-dermal fibrosis
-cutaneous calcinosis
-telangiectasia

40
Q

systemic sclerosis the 2 different types and elaborate *IGNORE

A

Limited systemic sclerosis
-Fibrotic skin limited to hands, forearms, feet, neck and face
-Anti-centromere antibodies
-Pulmonary hypertension
-Long history of Raynaud’s phenomenon

Diffuse systemic sclerosis
-Fibrotic skin proximal to elbows or knees (excluding face and neck)
-Anti-Scl-70 antibodies
-Pulmonary fibrosis, renal (thrombotic microangiopathy) involvement
-Short history of Raynaud’s phenomenon

41
Q

what does CREST describe and what it stands for

A

CREST describes a sub-type of limited systemic sclerosis.

Calcinosis,
Raynaud’s phenomenon,
Esophageal dysmotility,
Sclerodactyly,
Telangiectasia

42
Q

SLE-pregnancy- Drug considerations: WHICH ARE safe for pregnancy

A

Hydroxychloroquine, azathioprine, low molecular weight heparin (LMWH) safe

BRS Y2 (60 decks)

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