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French National Diagnostic and Care Protocol for antiphospholipid syndrome in adults and children
Z Amoura1, B Bader-Meunier2, C Bal Dit Sollier3
1Department of Internal Medicine, Institut E3M, CIMI-Paris, Faculty of medicine, National Reference Centre of Systemic Lupus, antiphospholipid syndrome, and other autoimmune diseases, Sorbonne University, AP-HP, Pitié Salpêtrière, boulevard de l'Hôpital, 75013 Paris, France.
Insights
Antiphospholipid syndrome (APS) is a rare autoimmune disorder causing blood clots and pregnancy issues. Early diagnosis and specialist consultation are crucial for managing this condition, especially the severe catastrophic form (CAPS).
Area of Science:
- Rheumatology
- Immunology
- Hematology
Background:
- Antiphospholipid syndrome (APS) is a chronic autoimmune disease characterized by vascular thrombosis and/or obstetric complications.
- It involves persistent antibodies to phospholipids or associated proteins, presenting rarely in adults and even more rarely in children.
- APS can manifest as a rapidly progressive, life-threatening condition known as catastrophic antiphospholipid syndrome (CAPS).
Purpose of the Study:
- To highlight the diagnostic challenges and clinical features of Antiphospholipid Syndrome (APS).
- To emphasize the importance of early recognition and specialist referral for suspected APS cases.
- To outline the recommended treatment strategies for thrombotic events in APS.
Main Methods:
- Diagnosis relies on classification criteria combining clinical and biological features.
- Management involves prompt heparinization followed by long-term anticoagulation with Vitamin K antagonists (VKAs).
- Urgent consultation with APS specialists and autoimmune disease competence centers is advised for complex cases, particularly CAPS.
Main Results:
- APS diagnosis requires careful consideration due to its rarity and potential for overdiagnosis.
- Early anticoagulation is critical, with temporary discontinuation posing a high thrombosis risk.
- Specialized centers are essential for managing severe manifestations like CAPS.
Conclusions:
- Antiphospholipid syndrome (APS) necessitates rapid, expert diagnosis and management to prevent severe thrombotic or obstetric outcomes.
- Timely anticoagulation and avoidance of treatment interruptions are key to managing APS.
- Referral to specialized centers is vital for catastrophic antiphospholipid syndrome (CAPS) and complex APS cases.
Abstract:
Antiphospholipid syndrome (APS) is a chronic autoimmune disease involving vascular thrombosis and/or obstetric morbidity and persistent antibodies to phospholipids or certain phospholipid-associated proteins. It is a rare condition in adults and even rarer in children. The diagnosis of APS can be facilitated by the use of classification criteria based on a combination of clinical and biological features. APS may be rapidly progressive with multiple, often synchronous thromboses, resulting in life-threatening multiple organ failure. This form is known as "catastrophic antiphospholipid syndrome" (CAPS). It may be primary or associated with systemic lupus erythematosus (associated APS) and in very rare cases with other systemic autoimmune diseases. General practitioners and paediatricians may encounter APS in patients with one or more vascular thromboses. Because APS is so rare and difficult to diagnosis (risk of overdiagnosis) any suspected case should be confirmed rapidly and sometimes urgently by an APS specialist. First-line treatment of thrombotic events in APS includes heparin followed by long-term anticoagulation with a VKA, usually warfarin. Except in the specific case of stroke, anticoagulants should be started as early as possible. Any temporary discontinuation of anticoagulants is associated with a high risk of thrombosis in APS. A reference/competence centre specialised in autoimmune diseases must be urgently consulted for the therapeutic management of CAPS.
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