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(or '''antiphospholipid
Antibody syndrome''') is a disorder of
Coagulation which causes
Thrombosis in both
Arteries and
Vein s, as well as recurrent
Miscarriage . It is due to the
Autoimmune production of
Antibodies against
Cell Membrane constituents. It is occasionally
Referred To as '''Hughes' syndrome''' after the
Rheumatologist Dr Graham R.V. Hughes (
St. Thomas' Hospital ,
London ,
UK ).
A very rare form is the
Catastrophic Antiphospholipid Syndrome , in which there is rapid organ dysfunction and failure. It carries a high
Mortality .
The presence of ''antiphospholipid antibodies'' (APLAs) is suggested by thrombosis (
Arterial or
Venous ) and recurrent miscarriage (especially in the second
Trimester , but often earlier). Other common findings, although not part of the classification, are
Thrombocytopenia (low
Platelet count) and
Livedo Reticularis (a
Skin condition). Many patients report
Headache s.
APLAs are present in the blood in the context of a number of
Disease s, most notably
Systemic Lupus Erythematosus (SLE). One can only speak of antiphospolipid syndrome when there are no other symptoms of one of these diseases (e.g.
Arthritis suggestive of SLE). A number of patients with the syndrome (about 10%) will eventually develop SLE, but most never get signs of this disease.
The diagnosis is often entertained in cases of
Thrombophilia (recurrent thrombosis) or recurrent miscarriage. Tests that are often performed at the same time are a
Full Blood Count ,
Liver Enzyme studies and
Renal Function studies.
Thrombophilia screening can consist of:
Antiphospholipid syndrome is tested for in the (DRVVT), the
Kaolin Clotting Time (KCT) or
Dilute Thromboplastin Time {TDT/DTT) are the prinicipal tests used for the detection of
Lupus Anticoagulant . A further antibody can be detected using an
Enzyme-linked Immunosorbant Assay (ELISA)
Immunological Test , which screens for the presence of antibodies to
Anticardiolipin .
Low Platelet Count and positivity for antibodies against β
2-glycoprotein or
Phosphotidylserine may also be observed in a positive diagnosis.
The diagnosis is made in case of a clinical event (thrombosis or recurrent miscarriage after 10 weeks gestation) and repeated positive tests of lupus anticoagulant and/or anticardiolipin antibodies performed 6-8 weeks apart. Repeat testing is necessary due to the naturally occurring presence of transient high levels of antiphospholipid antibodies following infection and inflammation. Other antibodies, although implicated, are not yet considered relevant for diagnosis.
Antiphospholipid syndrome is an
Autoimmune Disease , in which antibodies react against anionic
Phospholipid s on
Cell Membrane s. Being an
Autoimmune Disease , it is more common in women than in men. The exact
Cause is not known, but activation of the system of coagulation is evident.
Often, this disease is treated by giving
Aspirin to inhibit platelet activation, and/or
Warfarin as an
Anticoagulant . The goal of the
Prophylactic treatment is to maintain the patient's
INR between 2.0-3.0. It is not usually done in patients who have not had any thrombotic symptoms. During
Pregnancy ,
Heparin is used instead of warfarin because of warfarin's
Teratogen icity.
Women with recurrent miscarriage are often advised to take aspirin and to start
Heparin (or
Low Molecular Weight Heparin ) treatment after missing a
Period . This is the most effective treatment at the moment.
- Triona Holden. "Positive Options for Antiphospholipid Antibody Syndrome" ISBN 0897934091.
- Kay Thackray. ''Sticky Blood''. ISBN 1898030774. A personal account of dealing with the condition.
