Related Experiment Video
Updated: May 18, 2026
08:25
Circumscribed Capsular Infarct Modeling Using a Photothrombotic Technique
Published on: June 2, 2016
Catastrophic antiphospholipid syndrome (CAPS)
Savino Sciascia1, Chary Lopez-Pedrera, Dario Roccatello
1Lupus Research Unit, The Rayne Institute, Division of Women's Health, King's College London, UK. savino.sciascia@unito.it
Best Practice & Research. Clinical Rheumatology
|October 9, 2012
Summary
Catastrophic antiphospholipid syndrome (CAPS) is a rare but life-threatening condition. This review covers the latest understanding of CAPS, including its causes, symptoms, diagnosis, and treatments.
Area of Science:
- Rheumatology
- Internal Medicine
- Pathology
Background:
- Catastrophic antiphospholipid syndrome (CAPS) is a severe variant of antiphospholipid syndrome (APS).
- It involves rapid multiple organ damage, small vessel occlusions, and high-titer antiphospholipid antibodies (aPL).
- CAPS accounts for less than 1% of APS cases but is often fatal.
Purpose of the Study:
- To review current knowledge on the pathogenesis of CAPS.
- To summarize the clinical manifestations and diagnostic criteria for CAPS.
- To discuss contemporary treatment strategies for CAPS.
Main Methods:
- Literature review of pathogenesis, clinical features, diagnosis, and treatment of CAPS.
- Synthesis of existing data on CAPS from various sources.
- Expert consensus on the state-of-the-art in CAPS management.
Main Results:
- CAPS is characterized by acute, widespread thrombotic events affecting multiple organs.
- Diagnosis relies on clinical presentation, histopathology, and serological evidence of aPL.
- Treatment often involves anticoagulation, corticosteroids, and plasma exchange or IVIg.
Conclusions:
- CAPS remains a critical medical emergency requiring prompt diagnosis and aggressive management.
- Further research into pathogenesis may reveal novel therapeutic targets.
- Multidisciplinary care is essential for improving outcomes in CAPS patients.
Related Concept Videos
01:19Acute Coronary Syndrome II: Pathophysiology and Clinical Manifestations
The pathophysiology of Acute Coronary Syndrome [ACD] involves several key processes:The main underlying cause of ACD is atherosclerosis, a chronic inflammatory disease characterized by the buildup of lipid-laden plaques within the coronary arteries.As the atherosclerotic plaque grows in the coronary artery, it may become unstable due to the formation of a lipid-rich core and a thin fibrous cap. Inflammatory cells within the plaque, such as macrophages, secrete enzymes that degrade the...
01:21Acute Pancreatitis II: Pathophysiology
The pathophysiology of acute pancreatitis centers on injury to pancreatic acinar cells, which initiates a cascade of harmful intracellular events.This injury leads to premature activation of trypsinogen to trypsin in the pancreas. Trypsin then activates other digestive enzymes, such as chymotrypsin, elastase, and phospholipase A2, which begin breaking down pancreatic tissue. The resulting autodigestion causes local inflammation, tissue swelling, hemorrhage, and fat necrosis.Injured acinar cells...
01:27Complement System
The complement system is a group of approximately 20 plasma proteins that strengthen the body's defenses against infections through opsonization, inflammation, and cell lysis. Opsonization involves coating pathogens with complement proteins, making them more recognizable and facilitating phagocyte engulfment. Certain complement proteins induce inflammation that attracts immune cells to the site of infection. Cell lysis involves the destruction of pathogens through the formation of a membrane...
01:30Peripheral Artery Disease I: Introduction
Peripheral artery disease (PAD) predominantly results from atherosclerosis, which involves the accumulation of fatty deposits, or plaques, within the walls of arteries. This causes them to narrow and harden, significantly reducing blood flow. PAD predominantly affects the legs, particularly the arteries supplying the thighs and calves. In rare cases, it may involve other arteries, including those in the arms.Etiology of PAD:The principal cause of PAD is atherosclerosis, which results from fatty...
01:22Portal Hypertension
Portal hypertension is an increase in blood pressure within the portal venous system. Normally, this pressure is less than 5 mmHg. It is considered clinically significant when it rises above 10 mmHg. At this threshold, complications from altered blood flow and venous congestion emerge.EtiologyPortal hypertension arises from conditions that impede blood flow through the liver. The most common cause is cirrhosis, in which chronic liver injury leads to fibrotic scarring. This fibrosis narrows or...
01:30Anticoagulant Drugs: Low-Molecular-Weight Heparins
Hemostasis is a crucial process that prevents excessive blood loss from damaged blood vessels. It involves various mechanisms such as vasoconstriction, platelet adhesion and activation, and fibrin formation. The importance of each mechanism depends on the type of vessel injury. In contrast, thrombosis is the abnormal formation of a blood clot within the blood vessels, leading to potential complications if the clot obstructs blood flow. Thrombosis can be caused by increased coagulability of the...