Catastrophic antiphospholipid syndrome

Orpha code: 464343OMIM code:

Definition

A rare systemic autoimmune disease characterized by acute onset of life-threatening thromboses in three or more organs either simultaneously or within less than a week, in the presence of serum antiphospholipid antibodies (such as lupus anticoagulant, anticardiolipin antibodies, and anti-beta2-glycoprotein 1 antibodies), and with histopathological confirmation of small-vessel occlusion in at least one affected organ. The condition occurs in a small subset of patients with antiphospholipid syndrome, often precipitated by infection, trauma, or surgery.

Disease data
Classification

Disease

Synonyms
CAPS
CAPS
Katasrofalny APS
Catastrophic APS
ORPHA code
464343
OMIM code
-
ICD10 code
D68.6
ICD11 code
-

No additional description.

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