Catastrophic antiphospholipid syndrome (CAPS) is a rare and life threatening complication of antiphospholipid syndrome. Up to half of people with CAPS die, so timely treatment is essential.
Antiphospholipid syndrome (APS) is an autoimmune disease that increases your risk of blood clots.
As CAPS can affect multiple organ systems, symptoms can vary, and treatment can be challenging. CAPS is potentially life threatening and requires immediate medical attention.
Read on to learn how to recognize the symptoms of CAPS, how doctors work to diagnose and treat it quickly, and what the outlook may be for people who experience this complication.
What is the mortality rate for people with catastrophic antiphospholipid syndrome?
CAPS has a traditionally reported mortality rate of around 50%.
However, research over the past two decades suggests that survival rates have increased due to improvements in diagnosis and treatment. Some reports suggest that current mortality rates are closer to
CAPS causes blood clots to form throughout your body. Symptoms can vary significantly depending on which organs are affected.
Symptoms of CAPS may include:
- fingers that turn dark blue or black
- shortness of breath
- abdominal pain
- blood in your urine
- confusion
- seizures
- loss of consciousness, ranging from fainting to coma
CAPS symptoms come on suddenly and develop rapidly.
CAPS is a medical emergencyCAPS requires emergency medical care. If you or someone you know is experiencing sudden onset of the above symptoms, or has APS that suddenly gets worse, call 911 or your local emergency services right away.
For a doctor to diagnose CAPS, the International Congress on Antiphospholipid Antibodies proposes
- Three or more organs, systems, or tissues must be affected.
- The effects must have developed at the same time or over the course of a week or less.
- A histopathology report confirms a blood clot in small blood vessels in at least one organ or tissue.
- Blood tests confirm the presence of antiphospholipid antibodies.
While these criteria help guide doctors, they still rely on their clinical judgment. A doctor may:
- consider your symptoms
- consider whether you have a history of APS or lupus
- order several blood tests, including an enzyme-linked immunosorbent assay (ELISA)
- order imaging to rule out other conditions or view areas where you may have blood clots
Even though CAPS is a complication of APS, it may be the first presentation of the disease, especially in children and young adults. This can sometimes complicate diagnosis.
There’s no known optimal treatment for CAPS. However, treatment typically involves a type of combination treatment known as “triple therapy,” involving:
- anticoagulants (blood thinners)
- high dose glucocorticoids (steroid hormones to suppress the immune system and reduce inflammation)
- therapeutic plasmapheresis (TPE) or intravenous immunoglobulin (IVIG)
Doctors exercise caution when prescribing IVIG, as the risk of serious side effects, such as acute kidney failure and thrombosis, may be high for some people.
Your medical team may need to pause anticoagulation therapy if you’re about to undergo a medical procedure, such as a surgery, biopsy, or dental extraction.
If these treatments don’t work, a doctor may consider monoclonal antibodies like rituximab or eculizumab. These reduce the number of B cells, which help produce antiphospholipid antibodies.
The
- stopping anticoagulation therapy for APS
- infection
- surgery
CAPS may also be more common in people during the third trimester of pregnancy and the six weeks following delivery.
Since CAPS is a rare disease, it’s challenging for researchers to identify other risk factors. However, there may be a genetic component.
Even with treatment, up to half of people with CAPS may die. However, recent research suggests that
The most common causes of death from CAPS include:
- brain complications
- heart complications
- infections
- multiple organ failure
- lung complications
Some of the most common complications of CAPS include:
- kidney complications, like renal insufficiency and proteinuria
- lung complications, like acute respiratory distress syndrome and pulmonary embolism
- central nervous system complications, like encephalopathy and stroke
- heart complications, like high blood pressure, heart failure, and heart attack
- skin issues, like livedo reticularis and necrosis (skin cell death)
How common is catastrophic antiphospholipid syndrome?
CAPS occurs in fewer than 1% of people with antiphospholipid syndrome.
What is the difference between APS and CAPS?
CAPS is a rare complication of APS. In some cases, it may be the first presentation of APS symptoms.
Can children develop CAPS?
CAPS in children is very rare, with only 60 cases in the international CAPS registry.
How quickly does CAPS progress?
For a diagnosis of CAPS, symptoms typically progress
Can CAPS be prevented?
To prevent CAPS, experts recommend that people with APS keep taking anticoagulation therapy as prescribed by a doctor. They also recommend effective management and prevention of infections after operations.
Though rare, CAPS is a serious and potentially life threatening complication of APS. It can lead to blood clots in multiple organs and tissues, causing a wide range of symptoms. Even with treatment, many people experience fatal complications.
Still, timely treatment and diagnosis are essential to improving your chances of survival. If you have APS or another condition that puts you at risk of blood clots (like lupus), seek emergency help immediately if you experience rapidly progressing symptoms like blood in your urine or shortness of breath.
People with APS and similar conditions can reduce their risk of CAPS by following their recommended anticoagulation therapy and minimizing their risk of infections.