Overview
Catastrophic antiphospholipid syndrome (sometimes called CAPS or Asherson syndrome) is a rare and very serious form of antiphospholipid syndrome. In this condition, the immune system mistakenly makes antibodies called antiphospholipid antibodies that attack the body's own blood vessels. This causes widespread blood clots to form rapidly in multiple organs at the same time, usually over just a few days. The organs most commonly affected include the kidneys, lungs, brain, heart, and skin, but any organ can be involved. Because so many organs are damaged at once, CAPS is a life-threatening medical emergency. Symptoms depend on which organs are affected but can include kidney failure, difficulty breathing, confusion or stroke-like symptoms, skin changes, and abdominal pain. CAPS can occur in people who already have antiphospholipid syndrome or lupus, but it can also appear without warning in someone who has never been diagnosed with these conditions. Common triggers include infections, surgery, stopping blood-thinning medications, or other stressful events to the body. Treatment requires urgent hospitalization and typically involves a combination of blood thinners (anticoagulants like heparin), high-dose corticosteroids to reduce inflammation, and plasma exchange (a procedure that filters harmful antibodies from the blood). Intravenous immunoglobulin (IVIG) may also be used. In some cases, the biologic drug rituximab or eculizumab has been tried. Despite aggressive treatment, CAPS remains very dangerous, with a significant mortality rate, though outcomes have improved with modern combination therapy.
Also known as:
Key symptoms:
Sudden kidney failureDifficulty breathing or respiratory failureStroke or confusionSkin discoloration or livedo reticularis (net-like purple skin pattern)Heart problems including heart attackAbdominal pain from organ damageLow platelet count causing easy bruising or bleedingSeizuresVision changes or lossSwelling in the legsFatigue and general feeling of being very unwellGangrene of fingers or toesLiver problemsHigh blood pressure
Clinical phenotype terms (50)— hover any for plain English
Multifactorial
Caused by a mix of several genes and environmental factors
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
10 eventsBDH-Klinik Hessisch Oldendorf
Shanghai United Cell Biotechnology Co., Ltd — PHASE4
Shandong Suncadia Medicine Co., Ltd. — PHASE1
Xuanzhu Biopharmaceutical Co., Ltd. — PHASE4
Second Affiliated Hospital, School of Medicine, Zhejiang University — PHASE2
Shanghai Chia Tai Tianqing Pharmaceutical Technology Development Co., Ltd. — PHASE3
Minia University — NA
Pfizer — PHASE1
Sun Yat-sen University — PHASE2
Chipscreen Biosciences, Ltd. — PHASE1, PHASE2
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Catastrophic antiphospholipid syndrome.
11 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Rare Disease Specialist
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Catastrophic antiphospholipid syndrome.
Community
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Start the conversation →Latest news about Catastrophic antiphospholipid syndrome
Disease timeline:
New recruiting trial: Efficacy and Safety of HSK16149 Capsule in Chinese Patients With Diabetic Peripheral Neuropathic Pain Who Had an Inadequate Response to Pregabalin
A new clinical trial is recruiting patients for Catastrophic antiphospholipid syndrome
New recruiting trial: Relative Bioavailability of NX-5948 Tablets vs Capsules and the Effect of Covariates on the PK of NX-5948 Tablets
A new clinical trial is recruiting patients for Catastrophic antiphospholipid syndrome
New recruiting trial: A Phase Ia/Ib Clinical Study of GH56 Capsules in Subjects With MTAP-Deleted Advanced Solid Tumors
A new clinical trial is recruiting patients for Catastrophic antiphospholipid syndrome
New recruiting trial: A Phase Ib/II Clinical Study of GH21 Capsules Combined With Osimertinib Mesylate Tablets in Patients With NSCLC
A new clinical trial is recruiting patients for Catastrophic antiphospholipid syndrome
New recruiting trial: Clinical Trial Comparing TQB2868 Injection Combined With Anlotinib Hydrochloride Capsules With Placebo Combined With Chemotherapy as First-line Treatment for Metastatic Pancreatic Ductal Adenocarcinoma (mPDAC)
A new clinical trial is recruiting patients for Catastrophic antiphospholipid syndrome
New recruiting trial: Using a mHealth App to Improve Quality-of-Life Outcomes in Black Prostate Cancer Survivors
A new clinical trial is recruiting patients for Catastrophic antiphospholipid syndrome
New recruiting trial: Efficacy and Safety of Xiangjurupining Capsule for Hyperplasia of Mammary Glands
A new clinical trial is recruiting patients for Catastrophic antiphospholipid syndrome
New recruiting trial: Radiation Cystitis Treated With Pentosan Polysulfate Sodium Trial
A new clinical trial is recruiting patients for Catastrophic antiphospholipid syndrome
New recruiting trial: A Study to Evaluate the Efficacy and Safety of CS32582 in Participants With Moderate to Severe Plaque Psoriasis
A new clinical trial is recruiting patients for Catastrophic antiphospholipid syndrome
New recruiting trial: Zhibitai Capsules for the Treatment of Primary Hyperlipidemia
A new clinical trial is recruiting patients for Catastrophic antiphospholipid syndrome
Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.What organs have been affected, and what is the expected recovery for each?,How long will I need to take blood-thinning medication, and what are the risks?,What are the warning signs that CAPS could be coming back?,Are there activities or medications I should avoid while on blood thinners?,Should I be tested for lupus or other autoimmune conditions?,What should I do if I need surgery or a dental procedure in the future?,Are my family members at risk for antiphospholipid syndrome?
Common questions about Catastrophic antiphospholipid syndrome
What is Catastrophic antiphospholipid syndrome?
Catastrophic antiphospholipid syndrome (sometimes called CAPS or Asherson syndrome) is a rare and very serious form of antiphospholipid syndrome. In this condition, the immune system mistakenly makes antibodies called antiphospholipid antibodies that attack the body's own blood vessels. This causes widespread blood clots to form rapidly in multiple organs at the same time, usually over just a few days. The organs most commonly affected include the kidneys, lungs, brain, heart, and skin, but any organ can be involved. Because so many organs are damaged at once, CAPS is a life-threatening medica
How is Catastrophic antiphospholipid syndrome inherited?
Catastrophic antiphospholipid syndrome follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Catastrophic antiphospholipid syndrome typically begin?
Typical onset of Catastrophic antiphospholipid syndrome is adult. Age of onset can vary across affected individuals.
Are there clinical trials for Catastrophic antiphospholipid syndrome?
Yes — 11 recruiting clinical trials are currently listed for Catastrophic antiphospholipid syndrome on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Catastrophic antiphospholipid syndrome?
25 specialists and care centers treating Catastrophic antiphospholipid syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.