Antiphospholipid and Sjogren's syndromes Flashcards Preview

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Flashcards in Antiphospholipid and Sjogren's syndromes Deck (24)
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1

Antiphospholipid syndrome

A syndrome of recurrent thrombi (venous or arterial) and/or recurrent miscarriages with positive test for antiphospholipid antibodies (aPL)
Can occur alone or in conjunction with other autoimmune conditions (lupus)

2

Test for antiphospholipid antibodies (aPL)

Anticardiolipin test
Lupus anticoagulant test
Anti-Beta-2-glycoprotein test

3

Anticardiolipin test

Detects IgG or IgM against cardiolipin (a negatively charged phospholipid)

4

Lupus anticoagulant test

aPL will have an anticoagulant effect in a test tube but a procoagulant effect in the body

5

Anti-Beta-2-glycoprotein test

detects abs against B2-glycoprotein, which interacts closely with phospholipids

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Criteria for APS

Positive results in two tests at least 6wks apart -- but not all people who are positive will develop APS

7

Pathogenesis of APS

Negatively charged phospholipids and B-2-glycoprotein are present of apoptic blebs --> similar mechanism as lupus
aPL binds to the N-terminus of B-2-glycoprotein on the membranes of endothium, platelets, monocytes and trophoblasts causing thombosis or miscarriage

8

The main Clinical Features of APS

Recurrent thrombosis and miscarriage are the most common presenting symptoms
Ischemic stroke occur in 20% and DVT in 40% of pts
Can be arterial or venous (but not in the same pt)

9

Treatment of patients with APS who have had 1 or more thrombus

Long term warfarin (used to aim for INR >3 but now thought that lower INR may be just as safe)

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APS and recurrent miscarriage

27% of women who have two or more miscarriages have APS

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Rarer features of APS (9)

Thrombocytopenia, chorea, migraine and epilepsy
Valvular heart disease, Renal impairment
Livedo reticularis
Positive Coombs test
May be involved in accelerated atheroma formation

12

Management of pregnant pts with APS

Pregnant pts should be given aspirin and subcut heparin from early in the pregnancy -- but pre-eclampsia and poor fetal growth is still common

13

Management of pts with APS who have never had a clot

Unclear - many are put on aspirin and clopidogrel prophylatically if there is a high IgG aPL level but there are no definite guidelines
They are rarely put on warfarin

14

Sjogren's syndrome

A syndrome of lymphocytic infiltration of exocrine glands causing dryness (eyes, vagina, mouth & skin)
Can occur with other autoimmune disorder (secondary) or alone (primary)

15

Genetic associations of sjogren's

HLA B8 and DR3

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Clinical features of Sjogren's

Dryness of eyes (also mouth, vagina, skin) - may have salivary and parotid gland enlargement
Can also have: arthralgia/static polyarthritis, Raynad's, pulmonary diffusion detects/fibrosis, vasculitis or other autoimmune conditions, polyneuropathy/fits/depression

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Rare features of sjogren's

Dysphagia/abnormal oesophageal motility as seen in scleroderma
Renal tubule defects causing nephrogenic diabetes insipidus or renal tubular acidosis

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Cancer risk with Sjogren's

Increased incidence of non-hodgkin's lymphoma

19

Eye Tests for Sjogren's

Schiermer tear test --> a strip of filter paper is placed under the lower eyelid - (15mm in 5mins
shows punctate or filamentary keratitis

20

Blood tests for Sjogren's

Raised immunoglobulin, circulating immune complexes and autoantibodies - Usually rheumatoid factor positive
ANA positive in 80% and anti-mitochondrial abs in 10%
Anti-Ro is positive in 60-90% of primary sjogren's (anti-La 35-85%)

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Impact of anti-Ro in pregnancy

Can cross the placenta and cause congenital heart block

22

Management of Sjogren's

Artificial tears and saliva is usually sufficient
Hydroxychloroquine may help any fatigue and arthralgia
Corticosteroids are rarely needed but can be used to treat persistent salivary gland swelling or neuropathy

23

Overlap syndromes

Patient shows characteristic features of more than one ARD -- treatment is usually the same
Undifferentiated ARD is when patients have positive autoantibodies and some clinical features (raynaud's and arthralgia etc) but not enough to make a clear diagnosis of any particular ARD -- may progress to a clear diagnosis or remain with a consistent mild disease

24

ARD

Autoimmune rheumatic diseases -- SLE, APS, sjogren's, scleroderma, myositis and dermatomyositis