CATASTROPHIC

ANTIPHOSPHOLIPID
SYNDROME
JADE CLAWSON, SPTA
 What is it?

What causes it?

How is it diagnosed?
OBJECTIVES
How is it treated?

How does it relate to therapy?

References
 WHAT IS IT?
“Catastrophic Antiphospholipid Syndrome (CAPS) is a rare life-threatening
autoimmune disease characterized by disseminated intravascular thrombosis
that can result in multiorgan failure.” Most severe form of antiphospholipid
syndrome (APS).

APS is due to antiphospholipid antibodies directed against a diverse group

of proteins that are associated with phospholipids.

These antiphospholipid antibodies activate endothelial cells, platelets, and

immune cells which promotes a proinflammatory and prothrombotic
phenotype.
 Antiphospholipid •Primary
syndrome •Secondary

WHAT IS IT?
The most affected organs and tissues are
the kidneys, lungs, CNS, heart, skin, liver,
and GI tract
HOW IS IT DIAGNOSED?
Diagnostic Criteria Include:
A definite diagnosis of CAPS is made when the following 4 diagnostic criteria are present:
1. Involvement of 3+ organs/tissues
2. Development of manifestations in <1 week
3. Histological evidence of intravascular thrombosis
4. Presence of antiphospholipid antibodies on 2 occasions when tested 6 weeks apart

“The lupus anticoagulant is the antiphospholipid antibody test that best predicts thrombotic events
in classic APS, and 82% of patients in the CAPS registry have been reported to have a positive lupus
anticoagulant.”
WHAT CAUSES IT?
The exact cause of antiphospholipid syndrome is unknown. However, it is established that
about half of the diagnoses will occur with systemic lupus erythematosus (SLE). The
remaining cases are described as primary APS.

Triggers of APS:
Infection
Malignancy Pregnancy SLE (lupus)
(most common)
HOW IS IT TREATED?

A recent decline in the mortality rate in CAPS from

Medications 53% to 33% has been attributed to the use of the Many with CAPS also
following treatments in combination: present with SIRS (systemic
inflammatory response
Anticoagulation syndrome)
Glucocorticoids
Plasma exchange
Cyclophosphamide
Intravenous immunoglobulins
Anti-platelet agents
 NO Massage

HOW DOES IT
RELATE TO
THERAPY? “Acute exercise in patients with PAPS (primary
antiphospholipid syndrome) with exclusive
venous thrombosis was safe... This is an
important step to introduce regular exercise
training as a therapeutic tool in the management
of these patients.”
 REFERENCES
“Deep Vein Thrombosis.” UHS Health Topic - Deep Vein Thrombosis,
https://www.healthyhorns.utexas.edu/HT/HT_deepveinthrombosis.html

Garcia, C.B., Seguro, L.P.C., Perandini, L.A. et al. Rheumatol Int (2014) 34: 1737. https://doi.org/10.1007/s00296-014-3038-
7

Kazzaz, Nayef M et al. “Treatment of catastrophic antiphospholipid syndrome.” Current opinion in rheumatology vol. 28,3
(2016): 218-27. doi:10.1097/BOR.0000000000000269

Manzella, D., L. Vicente, A. Pérez de la Hoz, R. Zamora, G. De Rosa, and A. Pisarevsky. “Catastrophic Antiphospholipid
Syndrome As a Complication of Systemic Sclerosis”. Reumatismo, Vol. 71, no. 2, July 2019, pp. 92-98,
doi:10.4081/reumatismo.2019.1095.

Nayer, Ali, and Luis M Ortega. “Catastrophic antiphospholipid syndrome: a clinical review.” Journal of nephropathology vol.
3,1 (2014): 9-17. doi:10.12860/jnp.2014.03