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[Antiphospholipid antibody syndrome]

F Moccia¹, G Greco

¹Dipartimento d'Emergenza, Ospedale S. Martino, Genova.

La Clinica Terapeutica

|July 1, 1994

Summary

Antiphospholipid antibody syndrome (APS) can occur with Systemic Lupus Erythematosus or as a primary condition. It involves thrombosis, pregnancy loss, or low platelets, diagnosed by specific antibodies and leading to endothelial damage.

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Area of Science:

Immunology
Rheumatology
Hematology

Background:

Antiphospholipid antibody syndrome (APS) is a condition that can arise secondary to Systemic Lupus Erythematosus (SLE) or as a primary autoimmune disorder.
Primary APS lacks the clinical and serological markers of SLE, presenting uniquely.

Purpose of the Study:

To outline the characteristics and diagnostic criteria of primary APS.
To review the underlying mechanisms of thrombosis in APS.
To summarize current and alternative therapeutic strategies for APS.

Main Methods:

Review of existing literature on antiphospholipid antibody syndrome.
Analysis of clinical manifestations and laboratory findings associated with APS.
Evaluation of proposed pathogenic mechanisms for thrombosis in APS.
Compilation of data on therapeutic interventions for APS.

Main Results:

Primary APS is defined by clinical events (thromboembolism, pregnancy loss, thrombocytopenia) and laboratory evidence of antiphospholipid antibodies (aPLs), including anticardiolipin antibodies and lupus anticoagulant.
Thrombosis in APS is attributed to endothelial damage, not vasculitis, with multiple proposed mechanisms.
Standard therapy involves anticoagulants (dicumarol, heparin) combined with prednisone.

Conclusions:

APS presents with distinct clinical and laboratory features, requiring specific diagnostic approaches.
Understanding the thrombotic mechanisms is crucial for effective management.
Treatment options range from conventional anticoagulation and corticosteroids to more intensive therapies like immunoglobulins, plasmapheresis, and cyclophosphamide for severe cases.

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