Caring for someone with this diagnosis? Eleplan keeps diagnoses, medications, documents and every specialist in one plan.
Start free with EleplanAntiphospholipid syndrome
ORPHA:80Disease
Also called APLS · Antiphospholipid antibody syndrome · Classic APLS · Classic antiphospholipid syndrome · Hughes syndrome
What it is
A rare systemic autoimmune disease characterized by hypercoagulability with vascular thrombosis and, in women, pregnancy morbidity (miscarriages, severe pre-eclampsia, placental insufficiency) in the presence of serum antiphospholipid antibodies, such as lupus anticoagulant, anticardiolipin antibodies, and anti-beta2-glycoprotein 1 antibodies.
Key facts
- Prevalence
- 1-5 / 10 000
- Age of onset
- Adult
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Common30–79%
20- Anti-beta 2 glycoprotein I antibody positivity
- Anti-beta-2-Glycoprotein I IgG antibody positivityDiagnostic criterion
- Anti-beta-2-Glycoprotein I IgM antibody positivityDiagnostic criterion
- Anticardiolipin IgG antibody positivityDiagnostic criterion
- Anticardiolipin IgM antibody positivityDiagnostic criterion
- Antiphospholipid antibody positivityDiagnostic criterion
- Arterial thrombosisDiagnostic criterion
- Fatigue
- Female infertility
- Hypercoagulability
- Hypertension
- Ischemic stroke
- Livedo racemosa
- Livedo reticularis
- Lupus anticoagulantDiagnostic criterion
- Pain
- PreeclampsiaDiagnostic criterion
- Premature birthDiagnostic criterion
- Transient ischemic attack
- Venous thrombosisDiagnostic criterion
Sometimes5–29%
17- Anti-annexin-V antibody positivity
- Anti-phosphatidyl choline antibody positivity
- Anti-phosphatidyl ethanolamine antibody positivity
- Anti-phosphatidyl glycerol antibody positivity
- Anti-phosphatidyl inositol antibody positivity
- Anti-phosphatidyl serine antibody positivity
- Aortic regurgitation
- Aortic valve stenosis
and 9 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.
Powered by Eleplan
A rare diagnosis is just the start. Eleplan keeps the whole care plan in one place.
Diagnoses, medications, documents, appointments, and the whole care team — organized and always in sync, so you are not retelling the same story to every new specialist. With Ellie, your AI care assistant, on top of it. Free to start.