Antiphospholipid Syndrome

Question 1

Who gets Antiphospholipid Syndrome?

Answer 1

Females > Male
Middle Aged

More common in patients with SLE

Question 2

True of False

One in five women with reccurent miscarriages are diagnoses with APLS

Answer 2

False

One in 10 women are.

Question 3

Antiphospholipid Syndrome is associated with what disease?

Answer 3

SLE

Other AI disease
- RA
- Srojrens
- Systemic sclerosis

Syphilus (rook)
Hepatitis C (rook)

Question 4

What are the key features of APLS on history?

Answer 4

History:

Of arterial or venous thrombosis
–> DVT
–> PE
–> CVA - TIA or stroke
–> MI
–> any blood clot

Of pregnancy complications / miscarriages:
–> 3 or more unexplained miscarriages before 10 weeks
–> 1 or more miscarriages after 10 weeks (in a normal fetus)
–> 1 or more preterm birth of a normal fetus before 34 weeks because of (i) eclampsia OR (ii) features of placental insuffiency

Question 5

What are the key features of APLS on examination?

Answer 5

Purpura
Ecchymosis
Livedo reticularis, with or wihtout retiform purpura
Acral livedo reticularis
Leg ulcers (PG-like ulcers)
Cutaneous necrosis
Gangrene
Subungal splinter haemorrhages
Anetoderma
Nail fold ulceration
Thrombophlebitis
Raynaud phenomenon
Distal oedema

Question 6

DDx of APLS?

Answer 6

Heparin induced thrombocytopaenia

Thrombotic thrombocytopaenic purpura

Disseminated intravascular coagulation

Disorders of cryoprecipitation

Sneddon syndrome

Question 7

How do you treat APLS?

Answer 7

First Line:
- Anticoagulation: Heparin or warfarin in the NON-pregnant patient (target INR of 2.5)

• heparin +/- low dose aspirin in the pregnant patient

Second line:
- Oral direct thrombin and anti-factor Xa inhibitor
- combination antiaggregant therapy (aspirin + clopidogrel)
- low dose aspirin and HCQ in patients with SLE

Third line:
- plasmapheresis, IVIG, rituximab

Question 8

What are the antiphospholipid antbodies?

Answer 8

Lupus Anticoagulant
B2 Glycoprotein
Anti-cardiolipin

Question 9

What is catastrophic APLS?

Answer 9

uncommon severe phenotype of APLS
- multi-organ failure
- renal involvement
- acute
respiratory distress syndrome

precipitating factors:
- infection (often younger patients), - surgical procedures,
- drugs (e.g. sulfur-containing diuretics, captopril, oral contraceptives),
- discontinu-
ation of anticoagulant therapy
- malignancy