lupus Flashcards by Connie Zhou

the 4 autoimmune connective tissue disorder/multisystem autoimmune rheumatic diseases

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

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SLE where does it cause inflammation

multi-site inflammation- joints, kidney, skin

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

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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Typical triphasic colour changes in Raynaud’s phenomenon

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

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SLE clinical features

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

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Anti-nuclear antibodies (ANA) how to use results

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

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

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Antiphospholipid (APL) antibodies- where do they target

antibodies directed to phospholipids on cell membrane

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

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

2) Pregnancy loss (miscarriage)

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antiphospholipid antibody how to measure in lab

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

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Persistent presence of APL + a clinical event

“anti-phospholipid antibody syndrome”

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primary anti-phospholipid antibody syndrome is:

Anti-phospholipid antibody syndrome in absence of SLE

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

specific for SLE
Serum level of ab correlates with disease activity

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anti-Sm ab significance

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

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anti-Ro ab
anti-La ab
significance

secondary to sjogren’s syndrome
neonatal lupus syndrome

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what is seen in neonatal lupus syndrome

transient rash in neonate
permanent heart block

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Rheumatoid arthritis auto-antibody

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

SLE - immunopathogenesis
which are innate and which adaptive

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

adaptive immunity:
autoreactive B+T cells

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

worsen disease

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

anifrolumab

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

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

SLE investigations

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

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

low complement C3/C4
High anti-dsDNA antibodies

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

hydroxychloroquine- All pts
steroids-acute flare

mild-hydroxychloroquine
serious-immunomodulatory agent (methotrexate)

renal disease-mycophenolate+/-rituximab

steroid side effects

infection osteoporosis AVN

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

for persistently active disease, Bell target therapies- rituximab life-threatening, (ie.myocarditis) iv steroid+ IV cyclophosphamide SLE+ Antiphospholipid antibody syndrome anticoagulation (warfarin)

SLE and pregnancy what to do to get best outcome

pre-pregnancy planning and getting SLE into remission

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

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

SLE-pregnancy- Drug considerations: WHICH ARE teratogenic

MMF, cyclophosphamide, methotrexate, warfarin are teratogenic.

sjogren's syndrome what it is and effects *IGNORE

Autoimmune- lymphocytic infiltration of exocrine glands (lacrimal and salivary glands) Exocrine gland pathology: Dry eyes (xerophthalmia) Dry mouth (xerostomia) Parotid gland enlargement

sjogren's syndrome (other manifestation) *IGNORE

non-erosive arthritis Raynaud’s phenomenon autoimmune hepatitis

autoantibodies present in sjogren's syndrome *IGNORE

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

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

‘secondary’ Sjögren’s syndrome

what is the schirmer's test *IGNORE

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

inflammatory muscle disease 2 types *IGNORE

dermatomyositis =muscle and skin inflammation polymyositis =muscle only, no rashes

skin changes in dermatomyositis *IGNORE

-lilac colour rash around eyelids, malar, naso-labial folds -red/purple flat/raised lesions on knuckles (Gottron's papules)

inflammatory muscle disease investigation *IGNORE

1) bloods: high CK 2) electromyography- electrical activities of muscle 3) muscle biopsy polymyositis = CD8 T cell dermatomyositis = CD4 T cells+ B cells

what is inflammatory muscle disease associated with *IGNORE

malignancy and pulmonary fibrosis

systemic sclerosis signs *IGNORE

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

systemic sclerosis the 2 different types and elaborate *IGNORE

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

what does CREST describe and what it stands for

CREST describes a sub-type of limited systemic sclerosis. Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, Telangiectasia

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

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