SLE & Sjogren's Flashcards

1
Q

What HLA is associated with SLE?

A

HLA B8, DR2 & DR3

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2
Q

In what population is SLE commonly seen in?

A

Women of child bearing age

Asians and Afro-Caribbeans

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3
Q

What are the clinical features of SLE?

A

‘MD SOAP BRAIN’
Malar rash
Discoid rash

Serositis (pericarditis/pleuritis)
Oral ulcers
Arthritis
Photosensitivity

Blood disturbance
Renal
ANA+
Immunological investigations
Neurological (seizures)
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4
Q

What do immunological tests in SLE show?

A

> 95% are ANA+. ANA encompasses all of the following immunological markers:
Anti-dsDNA is the most specific and found in 60%
ENA is found in 40% (anti-Ro, anti-La, anti-Sm, anti-RNP)
40% are RF+
Antiphospholipid antibodies may be present (anticardiolipin or lupus anticoagulant)

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5
Q

What are the three best tests to diagnose SLE?

A

1) Anti-dsDNA antibody
2) C3 and C4 levels (will be decreased)
3) Raised ESR but CRP normal

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6
Q

What are the causes of drug induced lupus?

A
'SHIPP'
Sulfonamide
Hydralazine
Isoniazid
Phenytoin
Procainamide 

The disease remits if the drug is stopped.
Anti-histone antibodies are present in most people with drug induced lupus

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7
Q

What are the general management measures and maintenance management of someone with SLE?

A

Heavy sunblock
Hydroxychloroquine unless CI

Maintenance: NSAIDs and hydroxychloroquine
Azathioprine, methotrexate as steroid sparing agents

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8
Q

What do you use for mild, moderate and severe flares of SLE?

A

Mild: hydroxychloroquine
Moderate: mycophenolate or DMARDs
Severe: High dose steroids, mycophenolate, rituximab, cyclophosphamide

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9
Q

What can antiphospholipid antibodies cause?

A

Antiphospholipid antibodies are the anticardiolipin and lupus anticoagulant antibodies

Can cause CLOTs
Coagulation defect
Livedo reticularis
Obstetric (recurrent miscarriage)
Thrombocytopenia
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10
Q

What is the laboratory criteria to have a diagnosis of SLE?

A
Positive ANA
Anti-dsDNA
Anti-Smith antibodies
Antiphospholipid antibodies present
Low complement levels
Positive direct Coombs test
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11
Q

What is scleroderma?

A

Tightening and fibrosis of the skin
May manifest as plaques
ANA + in 90%

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12
Q

What is myositis?

A

Autoimmune mediated striated muscle inflammation

Two types: polymyositis or dermatomyositis

Polymyositis: Insidious onset of progressive symmetrical muscle weakness. Dysphagia, dysphonia or respiratory weakness.

Dermatomyositis: Myositis + skin signs. Shawl sign positive (macular rash). Heliotrope rash on eyelids. ANA antibodies will be present.

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13
Q

What do tests show in myositis?

A

Muscle enzymes increased in plasma
EMG and biopsy confirm diagnosis
MRI shows muscle oedema

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14
Q

What is the management of myositis?

A

Start prednisolone

Use hydroxychloroquine for skin disease

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15
Q

What are the features of Sjogren’s syndrome?

A
Dry mucosal surfaces
Keratoconjunctivitis sicca 
Vaginal dryness
Arthralgia
Raynaud's 
Parotitis
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16
Q

What do investigations show in Sjogren’s syndrome?

A

RF+ in 100% of patients
ANA+ in 70%
Anti-Ro and Anti-La present in 70%

Schirmer’s test: measures tear formation

17
Q

What antibodies are present in limited systemic sclerosis?

A

Anti-centromere antibodies

18
Q

What antibodies are present in diffuse systemic sclerosis?

A

Anti-Scl-70

19
Q

What are the features of CREST syndrome?

A
Calcinosis
Raynaud's phenomenon
Dysphagia
Sclerodactyly 
Telangiecstasia