Antiphospholipid Syndrome (APLS) Part 1

1
Antiphospholipid Syndrome (APLS) Part 1

• CPD Presentation
• Haroon Amini
• 22/09/2010

2
What is APS or Hughes Syndrome?

• APS also known as stickyblood is a systemic
  autoimmune disease in which the body makes
  antibodies against its phospholipids.
• APS characterized by thrombophylic state
• And obstetrical complications.
• Primary APS is not associated with other
  diseases.
• Secondary APS occurs in association with a
  disease i.e. SLE

3
APSAs cause blood clots in arteries and veins.

• Arterial thrombosis resulting in ischemia and
  necrosis of the foot

4
History

• Early 1900, false positive test for syphilis.
• 1950, the APS was first considered as a
  complication of the disease lupus.
• 1950-1960, has something to do with clotting,
  pregnancy loss.
• The APS was discovered in 1983 by Professor
  Graham Hughes and his team at St Thomass
  Hospital in London.

5
History

• Professor Hughes realised APS could affect
  patients who were not suffering from Lupus.
• International colleagues re-named the syndrome as
  Hughes Syndrome to honour Professor Hughes.
• 1990, discovery of B2-Glycoprotein 1

6
Frequency

• The exact frequency of APS in the general
  population is unknown
• 1-5 of the general population is believed to
  have APS.
• It mostly affects females from puberty to 55
  years old

7

• 10-25 of women with recurrent miscarriage have
  APS.
• 30 of strokes occurring in younger (under 55
  years old) people are due to APS.
• aPL antibodies are found in approximately 30-40
  of patients with SLE, but only about 10 have APS

8
Pathophysiology

• APS is a systemic autoimmune disease in which
  APLA reacts against protein that binds on anionic
  phospholipids on cell membrane, in particular
  cardiolipin and ß2-glycoprotein I.
• aCLA react with the vascular endothelial
  structure of blood vessels. This disturbs the
  balance of Prostoglandin E2- Thromboxane
  production.

9
Pathophysiology

• aCLA binds ApoH, which in turn inhibits the
  function of Protein C over the Common Pathway of
  coagulation.
• In APS some ABs bind to Protein S which is a
  co-factor for Protein C.
• aCLA bind to platelet phospholipids, which
  increases the platelet aggregation.
• In primary APS, the presence of 3 isotopes aCL
  and LAC is believed to be associated with
  consecutive miscarriages.

10
http//www.uspharmacist.com/content/c/10167/?twom
en's_health,thrombosis
11
Etiology and research

• The etiology of APS remains unknown.
• The mechanism of excessive production of APLA and
  immune complex formation is not well understood.
• But some studies have shown evidence of the
  development of clinical manifestation of APS i.e.
  Foetal loss, thrombocytopenia and neurological
  changes.
• Deregulation of Complement system

12
Clinical symptoms

• DVT in lower leg
• Stroke
• Recurrent miscarriage can occur prior to 20 weeks
  gestation
• Placental infarction, early deliveries and still
  birth can occur in women with APS
• Thrombocytopenia, dementia, headache

13
Diagnosis

• Diagnosis requires clinical and laboratory
  findings with the clinical criteria being the
  primary method of diagnosis.
• Laboratory finding includes solid phase ELISA
  assay and Liquid phase coagulation assays Lupus
  Anticoagulant

14
Laboratory finding

• Normal ACL result IgG is below 23 ug/mL, while
  normal IgM and IgA is below 11 ug/mL.

15
Treatment

• The disease can be treated by Aspirin and
  Warfarin as anticoagulant.
• In pregnancy low molecular weight heparin and low
  dose of aspirin can be used.