Primary Refractory Catastrophic Antiphospholipid Syndrome Masquerading as Buerger's Disease.
catastrophic antiphospholipid syndrome
eculizumab
thromboangiitis obliterans
Journal
Cureus
ISSN: 2168-8184
Titre abrégé: Cureus
Pays: United States
ID NLM: 101596737
Informations de publication
Date de publication:
07 Apr 2021
07 Apr 2021
Historique:
entrez:
11
5
2021
pubmed:
12
5
2021
medline:
12
5
2021
Statut:
epublish
Résumé
Catastrophic antiphospholipid syndrome (CAPS) is a lethal disease with up to 30% mortality rate. It can occur as a primary disease or secondary to an underlying autoimmune disease. Current treatment focuses on disease control with anticoagulation and steroids. Plasma exchange and intravenous immunoglobulin (IVIG) have shown some benefit when added. Monoclonal drugs such as rituximab have shown some benefit in refractory cases, and eculizumab, a drug approved for use in atypical hemolytic uremic syndrome, has demonstrated disease control in a few case reports. We describe a unique case of primary refractory CAPS with an unusual presentation that was treated with five lines of therapy before disease control was established.
Identifiants
pubmed: 33972907
doi: 10.7759/cureus.14350
pmc: PMC8104915
doi:
Types de publication
Case Reports
Langues
eng
Pagination
e14350Informations de copyright
Copyright © 2021, Tannous et al.
Déclaration de conflit d'intérêts
The authors have declared that no competing interests exist.
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