A Young Disease: A Holistic Approach to the Treatment of Antiphospholipid Syndrome

CAPS

Catastrophic antiphospholipid syndrome (CAPS) is a highly lethal variant of APS, causing multi-organ failure due to microcirculation thrombosis. The good news is that it’s relatively uncommon. According to data from the European Forum on aPL CAPS Registry, only 1% of patients with APS develop CAPS.¹⁰ The CAPS Registry was created in the year 2000 and now includes about 1,000 patients worldwide.

“Initially, the mortality rate from CAPS was 50%,” Dr. Cervera said. “So the 50% who recovered—what therapies did they receive? If they received the combination of anticoagulation, steroids and plasma exchange [PLEX] or intravenous immunoglobulin [IVIG], the survival rate was as high as 70%.”  This finding was a statistically significant difference compared with other treatment combinations.¹¹

These data ultimately led to the proposal of triple therapy for CAPS, which includes anticoagulation, high-dose intravenous glucocorticoids and PLEX with or without IVIG.^12,13 Glucocorticoids are included to treat the cytokine storm and systemic inflammatory response that occur in CAPS.  PLEX and IVIG help remove the aPL and cytokines from the body as quickly as possible.

“We are very proud to share that with the use of triple therapy, the mortality rate from CAPS has decreased from 75% (if no drugs are used) to 26%. In other words, the risk of death is nearly 10 times higher if you don’t use triple therapy. The importance of this cannot be stressed enough,” Dr. Cervera said.¹⁴

Despite the success of triple therapy, some patients with CAPS relapse or don’t respond to triple therapy. In these cases, adding rituximab is an option.¹⁵ Because complement is involved in APS pathogenesis, adding eculizumab, a monoclonal antibody targeted against complement C5, may be another option.¹⁶ 

We are very proud to share that with the use of triple therapy, the mortality rate from CAPS has decreased from 75% (if no drugs are used) to 26%. —Dr. Cervera

Ask the Expert

Dr. Cervera was kind enough to field questions from the audience at the end of his talk.

Question: What about the risk of thrombosis with IVIG? 

Dr. Cervera: Be aware that [thrombosis] is a potential risk, but it’s a small risk as compared with the benefits in this very fatal condition [CAPS].

Question: What about heparin-induced thrombocytopenia?

Dr. Cervera: In this case, use fondaparinux.

Question: Do you discuss the risk of CAPS with all of your patients with APS?

Dr. Cervera: Patients with triple aPL positivity are at higher risk of developing CAPS, but there are single aPL-positive patients who can get it too. The most important thing is to avoid triggers, such as infection, surgical procedures and lupus flares. Even a simple upper respiratory infection or minor procedure, such as a dental extraction or renal biopsy, can trigger CAPS. Pregnancy can, too. So discuss potential triggers with your patients, and try to prevent those that you can.

Question: What do you recommend for patients with APS and low platelets?