Regarding catastrophic antiphospholipid syndrome (CAPS.)
a) Outline the pathophysiology, clinical and diagnostic features. (70% marks)
b) Outline the specific treatment options for CAPS. (30% marks)
This question was poorly answered, and a lot of this was due to the nuance and context of the question. Most candidates answered the question with reference to APS or the presence of AP antibodies, and not CAPS, which is a severe manifestation of antiphospholipid syndrome that involves accelerated and widespread thrombosis, which may lead to multi-organ failure. CAPS appears to involve a vicious spiral of progressive complement activation, leading to microvascular thrombosis and tissue damage. There was a poor understanding of the pathophysiology in the answers, and many candidates gave answers pertaining to the diagnosis of the organ damage caused by CAPS, rather than the diagnosis of the syndrome itself.
Garcia, David, and Doruk Erkan. "Diagnosis and management of the antiphospholipid syndrome." New England Journal of Medicine 378.21 (2018): 2010-2021.
Cervera, Ricard. "Antiphospholipid syndrome." Thrombosis research 151 (2017): S43-S47.
Miyakis, Spyridon, et al. "International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS)." Journal of Thrombosis and Haemostasis 4.2 (2006): 295-306.
Cervera, Ricard, and Gerard Espinosa. "Update on the catastrophic antiphospholipid syndrome and the “CAPS Registry”." Seminars in thrombosis and hemostasis. Vol. 38. No. 4. 2012.
Belmont, H. Michael. "Catastrophic antiphospholipid syndrome." Hughes Syndrome. Springer London, 2006. 171-180.
Mehta, Trupti P., Maureen A. Smythe, and Joan C. Mattson. "Strategies for managing heparin therapy in patients with antiphospholipid antibody syndrome." Pharmacotherapy: The Journal of Human Pharmacology and Drug Therapy 31.12 (2011): 1221-1231.