Antiphospholipid Antibody Syndrome (APLA)

I. DIAGNOSIS
This complex syndrome actually is described as primary
(not associated with another illness) and secondary (associated
with SLE or another rheumatic disease). The antiphospholipid
antibody (APLA) is detected in more than
one-third of patients with lupus.
This syndrome is defined as the presence of either
APLA or a lupus anticoagulant accompanied by this list
of clinical manifestations:
1. Recurrent thrombosis of veins or arteries that are
unexplained, that is, the usual causes of thrombosis
such as immobilization or postsurgical are lacking
2. Frequent second- or third-trimester miscarriages
3. Thrombocytopenia and livedo reticularis commonly
occur; patients with SLE commonly show positive blood
serology for APLA, but the majority of these patients do
not show clinical manifestations of the syndrome
4. Heart valves may become involved with thrombotic
masses that may embolize and cause stroke
5. Ulcers of the lower legs around the ankle may resemble
ulcers caused by obstruction to the venous system and
stasis ulcers, but ulcers caused by this syndrome are
exquisitely painful and may resemble pyoderma gangrenosum
The principal autoantigen of antiphospholipid antibody
syndrome has been shown to be beta-2-glycoprotein 1
(apolipoprotein H), a protein that binds cardiolipin and
exposes an antigenic epitope. The antibody is called
cardiolipin, lupus anticoagulant, or anti-beta-2 glycoprotein
1 depending on the test used. Two inexpensive
tests, a standardized enzyme-linked immunosorbent assay
for anticardiolipin antibodies and a clotting test for
lupus anticoagulant, rapidly rule out antiphospholipid
syndrome.
The antiphospholipid syndrome and factor V Leiden are
the most common causes of thrombophilia accounting
for greater than 20% of cases of recurrent thrombosis in
individuals under age 40. Approximately 15% of women
with recurrent pregnancy loss have this syndrome.

II. MANAGEMENT
Thrombosis of veins or arteries should be treated with
full-dose oral anticoagulation with warfarin. A Canadian
study indicated that using warfarin to keep the target
INR at 2–3 provides beneficial results that are equal to
that observed with high-dose anticoagulation with an
International Normalize Ratio (INR) of 3.1–4.0. A
retrospective study in women in whom antiphospholipid
antibody syndrome was diagnosed because of pregnancy
loss (none of whom had a previous thrombotic event)
suggests that treatment with low-dose aspirin provides
protection from thrombosis. An ongoing clinical trial in
the United States is comparing aspirin with placebo
and a study in the UK is comparing aspirin with low-dose
warfarin plus aspirin.
Fortunately, this syndrome is rare. Individuals who
have lupus and other autoimmune diseases may test positive
for the antiphospholipid antibody syndrome, but the
majority usually do not manifest the symptoms, signs, and
complications outlined above.

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