TY - JOUR
T1 - Catastrophic antiphospholipid syndrome in a patient with systemic sclerosis and hereditary angioedema
T2 - case report and literature review
AU - Liew, Jean
AU - Friedman, Marcia
AU - Desai, Sima
AU - Taute, Lindsay
AU - Neishaboori, Nastaran
AU - Stenzel, Peter
AU - Wanchu, Ajay
N1 - Publisher Copyright:
© 2017, © 2017 Japan College of Rheumatology.
PY - 2018/1/2
Y1 - 2018/1/2
N2 - Catastrophic antiphospholipid syndrome (CAPS) is a rare form of the antiphospholipid syndrome (APS) in which microvascular thrombotic events cause rapidly progressive multiorgan dysfunction. We describe a case of CAPS presenting in a patient with suspected systemic sclerosis (SSc) and hereditary angioedema (HAE), and conduct a literature review to examine the reported cases of CAPS in individuals with SSc. Two reported cases of APS occurring with HAE were also found. In CAPS, there may be multiple thrombi in the microvasculature of any organ, most commonly in the intra-abdominal viscera. Patients rapidly develop multiorgan dysfunction or failure. Diagnosis is partly based upon the presence of positive antiphospholipid antibodies. The recommended therapy is systemic anticoagulation, high-dose corticosteroids, and plasma exchange with or without the addition of intravenous immunoglobulin. Given the high mortality rate of CAPS, urgent diagnosis and treatment are necessary to attempt to halt the progression of multiorgan failure.
AB - Catastrophic antiphospholipid syndrome (CAPS) is a rare form of the antiphospholipid syndrome (APS) in which microvascular thrombotic events cause rapidly progressive multiorgan dysfunction. We describe a case of CAPS presenting in a patient with suspected systemic sclerosis (SSc) and hereditary angioedema (HAE), and conduct a literature review to examine the reported cases of CAPS in individuals with SSc. Two reported cases of APS occurring with HAE were also found. In CAPS, there may be multiple thrombi in the microvasculature of any organ, most commonly in the intra-abdominal viscera. Patients rapidly develop multiorgan dysfunction or failure. Diagnosis is partly based upon the presence of positive antiphospholipid antibodies. The recommended therapy is systemic anticoagulation, high-dose corticosteroids, and plasma exchange with or without the addition of intravenous immunoglobulin. Given the high mortality rate of CAPS, urgent diagnosis and treatment are necessary to attempt to halt the progression of multiorgan failure.
KW - Scleroderma
KW - antiphospholipid antibody syndrome
KW - catastrophic antiphospholipid antibody syndrome
KW - hereditary angioedema
KW - systemic sclerosis
UR - http://www.scopus.com/inward/record.url?scp=85135865131&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85135865131&partnerID=8YFLogxK
U2 - 10.1080/24725625.2017.1351049
DO - 10.1080/24725625.2017.1351049
M3 - Article
AN - SCOPUS:85135865131
SN - 2472-5625
VL - 2
SP - 33
EP - 38
JO - Modern Rheumatology Case Reports
JF - Modern Rheumatology Case Reports
IS - 1
ER -