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Catastrophic antiphospholipid syndrome

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Disease 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 is often precipitated by infection, trauma, or surgery.

ORPHA:464343

Classification level: Disorder

Synonym(s):
  • CAPS
  • Catastrophic APS

Source: PubMed ID 27375916 22465135 22618528

Prevalence: Unknown

Inheritance: Not applicable

Age of onset: Adult

ICD-10: D68.6

ICD-11: 4A45

UMLS: C3662487

GARD: 9820

A summary on this disease is available in Français, Español, Deutsch, Italiano, Português, Nederlands
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

General public
Article for general public
Guidelines
Emergency guidelines
Français (2017.pdf) - Orphanet Urgences
Deutsch (2009.pdf) - Orphanet Urgences
Español (2009.pdf) - Orphanet Urgences
Italiano (2009.pdf) - Orphanet Urgences
Português (2009.pdf) - Orphanet Urgences
Anesthesia guidelines
English (2015) - Orphananesthesia
Español (2015) - Orphananesthesia
Čeština (2015) - Orphananesthesia
Clinical practice guidelines
English (2003) - Lupus
Disease review articles
Review article
English (2008) - Semin Thromb Hemost
The documents contained in this website are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.