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Learn on APLS
Classification criteria for APLS
Clinical presentation / manifestations
Other specific subtypes
Management guidelines
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APLS present in 15 – 20% off all DVT patients
Major cause of recurrent abortion after excluding
other causes
Ix is important to determine Rx to prevent
recurrent Sx
Mainstay of Rx is anticoagulants
Identify risk factors for thrombosis
Synonyms;
 Anticardiolipin antibody syndrome
 Hughes syndrome
 lupus anticoagulant syndrome……..
Antiphospholipid syndrome
OBSTETRICIAN
OPTHALMOLOGIST
NEUROLOGIST
RHEUMATOLOGIST
GASTROENTERO
LOGIST
Antiphospholipid
syndrome
DERMATOLOGIST
NEPHROLOGIST
HEMATOLOGIST
VASCULAR SURGEON
CARDIOLOGIST
Psychiatry – Mad cow disease,
Obstetrics - Recurrent abortion, pre eclampsia
Neurology – Migraine, epilepsy, movement disorder, BSE, Parkonsonism
Transplant Surgery – Cardiac, renal
Gastroenterology – Coeliac artery stenosis
Hypertension- Renal artery stenosis
Metatarsal #, AVN
Orthopaedics
Angina, CAD
Cardiology
Tinnitus, Minierre’s dis
ENT
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APS is more common in women (5:1 )
Females -more frequently -arthritis, livedo
reticularis, and migraine
Males -myocardial infarction, epilepsy and lower
extremity arterial thrombosis .
Mean age of onset -31 years
AcA-associated thrombosis- more common than
LA- associated thrombosis, with a ratio of 5:1
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Arterial or venous thrombosis
Pregnancy morbidity
LA or aCL X2 (6 weeks apart)
- For classification NOT for diagnosis
1. PAPS
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Exclude other underlying
diseases or conditions
2. SAPS
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List of causes
Autoimmune conditions
e.g. SLE, RA, SS etc….
Drugs like hydralazine,
quinidine, phenytoin and
amoxycillin
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SLE - 25-50% •
Sjogren’s syndrome – 42%
Rheumatoid arthritis – 33%
Autoimmune thrombocytopenic purpura - 30%
Autoimmune hemolytic anemia – Unknown
Psoriatic arthritis – 28%
Systemic sclerosis – 25%
Mixed connective-tissue disease - 22%
Polymyalgia rheumatica or Giant cell arteritis - 20%
Behcet syndrome - 20%
3. CAPS
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Most severe form of APLS
Preseentation with
multiple organ
involvement developed
over short period of time
( < a week )
HPE : small vessels
occlusion
4. SNAPS
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Significant no of patients
with livedo reticularis are
aPL -ve
66% pregnancy-related
morbidity
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Malar or discoid rash
Oral, pharyngeal or nasal ulceration
Frank arthritis
Pleurisy / pericarditis
Persistent proteinuria > 0.5 gm/day, biopsy proven
immune complex related GN
Lymphopenia , 1000 cells / ul
Anti DsDNA or ENA
ANA > 1 : 320
Treatment with drugs known to cause APLS Follow up
< 5 years from the initial manifestations
1960 – “sero-negative RA”
1970 – “sero-negative SLE”
2000 – “sero-negative APS”
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Become pregnant
Are immobile for a time, such as being on bed rest
or sitting during a long flight
Have surgery
Smoke cigarettes
Take oral contraceptives or estrogen therapy for
menopause
Have high cholesterol and triglycerides levels
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Hypercoagulable state ( malignancy, OCP, HRT, Protein C
& S deficiency, anti thrombin III deficiency etc.. )
Atherosclerotic vascular disease with cholesterol
emboli
Systemic necrotising vasculitis
DIVC
Infective endocarditis
TTP
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Lupus anticoagulant ( 2 or more occasions at 12 weeks
interval )
Anticardiolipin – IgG / IgM ( > 40 IgG antiphospholipid unit
GPL/ml or IgM antiphospholipid – MPL/ml at least for 2 occasions at 12
)
Presence of anti B2 glycoprotein ( IgM/IgG )
antibodies on 2 occasions at 12 weeks interval )
VDRL
PT / APTT
FBC ( thrombocytopenia and evidence of hemolysis )
weeks interval
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Imaging
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HRCT
MRI
Echo
Angio / venogram
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Anticoagulants- Heparin ( conventional / LMWH )
Aspirin ( may consider clopodegrel if patient allergic to
aspirin )
Warfarin
NOAC ( Rivaroxaban )
Surgery
Condition
Women with previous
thrombosis
Pregnancy
Unfractionated or LMWH in
therapeutic dossage
Past pregnancy
Return to warfarin
Women with antiphspholipid
Low dose aspirin
antibodies but history pregnancy
loss or tromboembolism
Low dose heparin
Women with median to high
anticardiolipin anti-ß2
glycoproteinl or lupus
anticoagulant or 2-3 (0r more)
1sttrimester losses or 1 or more
fetal death or 1 or more very
early preterm births due to
placental insufficiency
Continue
unfractionated or
LMWH for 6/52
postpartum
Continue life long
aspirin
Low dose aspirin or
fractionated or low
molecular weight heparin
Algorithm for Antithrombotic Treatment of Patients With Antiphospholipid Antibodies
INR indicates international normalized ratio. Circled capital letters indicate strength of evidence supporting
treatment recommendations.
*Importance of transient antiphospholipid antibodies is uncertain.
JAMA. 2006;295:1050-1057
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Hydroxychloroquine ( for SLE )
Statins ( for hyperlipidaemia )
Plasma exchange / cyclophosphamide for CAPs )
Rituximab for recurrent thrombosis
3.5 : Some cases, e.g., movement disorder
3.0 – 3.5 : Focal cerebral lesion
2.5 – 3.0 : Most cases
2.0 – 2.5 : DVT
1.5 – 2.0 : Prophylaxis