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Start free with EleplanCatastrophic antiphospholipid syndrome
ORPHA:464343Disease
Also called CAPS · Catastrophic APS
What it is
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 is often precipitated by infection, trauma, or surgery.
Key facts
- Age of onset
- Adult
- Inheritance
- Not applicable
- Classified as
- Disease
Signs and symptoms
Common30–79%
11Sometimes5–29%
26- Abnormal heart valve morphology
- Abnormal heart valve physiology
- Abnormality of the nervous system
- Amaurosis fugax
- Anti-annexin-V antibody positivity
- Anticardiolipin IgM antibody positivity
- Anti-phosphatidyl choline antibody positivity
- Anti-phosphatidyl ethanolamine antibody positivity
and 18 more in this range
Rare1–4%
13- Abnormality of the kidney
- Abnormal jugular vein morphology
- Acute encephalopathy
- Angina pectoris
- Avascular necrosis
- Chorea
- Dementia
- Eclampsia
and 5 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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