Systemic Autoimmune Rheumatic Diseases Flashcards
What is antiphospholipid syndrome characterised by?
1) Thrombosis (clots) e.g. VTE
2) Pregnancy morbidity (miscarriage and neonatal death)
What are the three autoantibodies that are drivers of aPL syndrome?
1) Anti-cardiolipin (aCL)
2) Lupus anticoagulant (LA)
3) Anti-beta2 glycoprotein 1
What are the 3 Sydney classification criteria for aPL syndrome?
1) One or more arterial, venous or small vessel thrombosis
2) ≥1 fetal death ≥ 10 weeks
≥ 1 premature birth < 34 weeks
≥ 3 spontaneous abortions < 10 weeks
3) aCL, beta2GP1 or LA positive at least twice > 12 weeks apart
What increases the risk of having an aPL event?
If you are triple positive for all 3 antibodies
Why should all new lupus patients have a test for the aPL antibodies?
To figure out the risk of thrombosis in the future
What % of lupus patients have aPL?
30%
What are the risk factors for aPL and thrombosis?
- Surgery
- Immobilisation
- Hyperlipidaemia
- Hypertension
- Obesity
- Oestrogens
- Pregnancy/puerperium
- Cox-2 selective inhibitors
- Long distance travels
- 50% of APS + thrombosis ≥ 1 risk factor
What can occur in the brain in aPL?
- Multiple strokes
- Haemorrhage
- Death
How is cerebral APS different from cerebral lupus (difficult to distinguish)?
Cerebral APS mainly consists of cerebral events whereas cerebral lupus is more hallucinations, seizures etc
What are the clinical features of cerebral APS?
- Headache
- Cognitive dysfunction
- Ischaemic stroke
- TIA
- Acute ischaemic encephalopathy
- Amaurosis fugax
- Cerebral venous thrombosis
- Chorea
- Transverse myelitis
- Seizures
How do you treat CNS lupus with aPL?
Anticoagulation for strokes/TIAs
What are consequences of aPL in pregnancy?
- Uteroplacental insufficiency and IUGR - fetal death and still birth
- Pre-eclampsia
- Abruption
- Premature delivery
- Thrombosis
How would you treat someone who is aPL positive and pregnant but hasn’t had any thrombosis or pregnancy loss?
- Careful monitoring
- Aspirin?
How would you treat someone who is aPL positive and pregnant who has had previous maternal thrombosis, recurrent embryonic loss < 10 weeks, late fetal death, IUGR or severe pre-eclampsia?
Heparin + aspirin
How do you treat someone with aPL postpartum?
Heparin for 6 weeks bc pregnancy is a high thrombotic state
How do you treat aPL patients who have had thrombosis and what is the target INR?
- Long term warfarin
- Target INR for venous thrombosis (DVT/PE) = 2-3
- Target INR for arterial thrombosis (stroke, MI, recurrent events) = 3-4
What are the features of primary Raynaud’s?
- Common
- Young women affected
- Symmetrical
- Doesn’t involve thumbs
- Doesn’t involve antibodies (can be discharged)
- Due to spasm of arteries causing reduced blood flow
What are features of secondary Raynaud’s?
- Asymmetrical
- Thumbs implicated
- Macroscopic nailfold capillary loops
- Digital infarcts
- Autoantibodies
- 95% predictive of CTD (SLE, systemic sclerosis)
- Need more investigation and follow up
What feature is pathognomonic of autoimmune Raynaud’s (disease)?
Nailfold capillaries
Describe nailfold capillaries
- Regular distribution of capillaries without pathological enlargements
- Giant capillaries
- Loss of capillaries
- Haemorrhages
- Neoangiogenesis
How is (secondary) Raynaud’s treated?
- Keep warm e.g. heated gloves
- Calcium channel blockers e.g. nifedipine
- Sildenafil
- Prostacyclin infusions
- Bosentan
- (Sympathectomy)
What antibodies are present in diffuse cutaneous systemic sclerosis?
- Anti-Scl 70 - DNA topoisomerase I
- Anti-RNA polymerase I, III
What antibodies are present in cutaneous systemic sclerosis?
Anti-centromere
What are features of systemic sclerosis?
1) Early diffuse disease < 2 years
2) Rapid cutaneous progression
3) Renal crisis
4) GI involvement (diverticular disease, peritonitis due to perforation, fibrosis)
5) Early interstitial lung disease (pulmonary fibrosis)
6) Pulmonary hypertension
7) Microscopic nail folds (nailfold capillaroscopy)
8) Systemic (sclero)dactyly (shiny, fibrosed, tight skin)
9) Raynaud’s
10) Telangiectasia
11) Digital ulceration bc blood vessels are not supplying the digits with sufficient blood leading to areas of ischaemia
