Catastrophic antiphospholipid syndrome: Lessons from the "CAPS Registry"

Area of Science:

• Rheumatology
• Hematology
• Critical Care Medicine

Background:

• Catastrophic antiphospholipid syndrome (CAPS) is a rare, severe manifestation of antiphospholipid syndrome (APS).
• First described in 1992, CAPS involves multiorgan dysfunction and microangiopathic features.
• The CAPS Registry, established in 2000, is a primary source of knowledge on this condition.

Purpose of the Study:

• To analyze data from the CAPS Registry to understand CAPS triggers, clinical presentation, and treatment efficacy.
• To evaluate the impact of specific therapeutic interventions on CAPS prognosis.
• To track changes in mortality rates associated with improved understanding and treatment of CAPS.

Main Methods:

• Analysis of case data collected in the dedicated CAPS Registry.
• Identification of common triggers, such as prothrombotic situations.
• Evaluation of treatment regimens, including triple therapy (anticoagulation, glucocorticoids, plasma exchange/IVIg).

Main Results:

• CAPS is frequently triggered by prothrombotic events leading to multiorgan thrombosis and cytokine storm.
• Triple therapy comprising anticoagulation, glucocorticoids, and plasma exchange and/or intravenous immunoglobulins is linked to better CAPS prognosis.
• Mortality rates have decreased from 50% in early reports to 25-30% in recent publications.

Conclusions:

• The CAPS Registry has been instrumental in advancing knowledge of this rare syndrome.
• Aggressive triple therapy is associated with improved survival in catastrophic antiphospholipid syndrome.
• Continued research and data collection are crucial for further reducing CAPS mortality.