Diagnosis and Management of Catastrophic Antiphospholipid Syndrome and the Potential Impact of the 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria

Abstract

Catastrophic antiphospholipid syndrome (CAPS) is a rare and life-threatening condition characterized by the persistence of antiphospholipid antibodies and occurrence of multiple vascular occlusive events. CAPS currently remains a diagnostic challenge and requires urgent treatment. The diagnosis of CAPS is made difficult by classification criteria used as diagnostic criteria in clinical practice, knowledge derived from retrospective data and case reports, confounding clinical and biological features, and its rapid onset and mortality. The absence of prospective studies of CAPS limits the strength of evidence for guideline treatment protocols. This comprehensive review summarizes the current understanding of the disease, and discusses how the 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria impact the definition and therapeutic management of CAPS, which is considered the most severe form of APS. The correct integration of 2023 ACR/EULAR APS classification criteria is poised to facilitate CAPS diagnosis, particularly in critical situations, offering a promising avenue for improved outcomes.

Keywords: anticardiolipin; antiphospholipid antibodies; antiphospholipid syndrome; catastrophic antiphospholipid syndrome; lupus anticoagulant; sepsis; systemic lupus erythematosus; thrombotic microangiopathy.

Figure 1

Clinical manifestations of catastrophic antiphospholipid syndrome according to the literature [10,13]. Figure 1 was created with BioRender.com.

Figure 2

Overview of the available treatment options for catastrophic antiphospholipid syndrome. Treatment regimens are divided into first-line, add-on therapy, or treatment of refractory CAPS. CAPS: catastrophic antiphospholipid syndrome, IV: intravenous, SLE: systemic lupus erythematosus, TMA: thrombotic microangiopathy.