Catastrophic antiphospholipid syndrome
Encyclopedia
Catastrophic antiphospholipid syndrome, CAPS, also Asherson’s syndrome, is an acute and complex biological process that leads to occlusion
of small vessels of various organs. It was first described by Ronald Asherson in 1992. The syndrome exhibits thrombotic
microangiopathy, multiple organ failure, and tissue necrosis and is considered an extreme variant of the antiphospholipid syndrome
.
CAPS has a mortality rate of about 50 %. With the establishment of a CAPS-Registry more has been learned about this syndrome, yet the cause remains elusive. Infection, trauma, medication, or surgery can be identified in about half the cases as a “trigger”. It is thought that cytokines are activated leading to a cytokine storm
with the potentially fatal consequences of organ failure. Patients exhibit a positive test to antilipid antibodies, typically IgG, and often have a history of lupus
or another connective tissue disease
. Thrombocytopenia
is a common finding.
Clinically, the syndrome can affect many organs systems. Peripheral thrombosis may be encountered affecting veins or arteries. Intraabdominal thrombosis may lead to pain. Renal and pulmonary complications are common. The patient may exhibit skin purpura and necrosis. Cerebral manifestations may lead to encephalopathy and seizures. Myocardial infarctions may occur. Death may result from multiple organ failure.
Treatments may involve the following steps:
Vascular occlusion
Vascular occlusion is a sudden blockage of a blood vessel, usually with a clot. It differs from thrombosis in that it can be used to describe any form of blockage, not just one formed by a clot. When it occurs in a major vein, it can, in some cases, cause deep vein thrombosis. The condition is...
of small vessels of various organs. It was first described by Ronald Asherson in 1992. The syndrome exhibits thrombotic
Thrombosis
Thrombosis is the formation of a blood clot inside a blood vessel, obstructing the flow of blood through the circulatory system. When a blood vessel is injured, the body uses platelets and fibrin to form a blood clot to prevent blood loss...
microangiopathy, multiple organ failure, and tissue necrosis and is considered an extreme variant of the antiphospholipid syndrome
Antiphospholipid syndrome
Antiphospholipid syndrome or antiphospholipid antibody syndrome , often also Hughes syndrome, is an autoimmune, hypercoagulable state caused by antibodies against cell-membrane phospholipids that provokes blood clots in both arteries and veins as well as pregnancy-related complications such as...
.
CAPS has a mortality rate of about 50 %. With the establishment of a CAPS-Registry more has been learned about this syndrome, yet the cause remains elusive. Infection, trauma, medication, or surgery can be identified in about half the cases as a “trigger”. It is thought that cytokines are activated leading to a cytokine storm
Cytokine storm
A cytokine storm, or hypercytokinemia is a potentially fatal immune reaction consisting of a positive feedback loop between cytokines and immune cells, with highly elevated levels of various cytokines.-Symptoms:...
with the potentially fatal consequences of organ failure. Patients exhibit a positive test to antilipid antibodies, typically IgG, and often have a history of lupus
Lupus erythematosus
Lupus erythematosus is a category for a collection of diseases with similar underlying problems with immunity . Symptoms of these diseases can affect many different body systems, including joints, skin, kidneys, blood cells, heart, and lungs...
or another connective tissue disease
Connective tissue disease
A connective tissue disease is any disease that has the connective tissues of the body as a target of pathology. Connective tissue is any type of biological tissue with an extensive extracellular matrix that supports, binds together, and protects organs...
. Thrombocytopenia
Thrombocytopenia
Thrombocytopenia is a relative decrease of platelets in blood.A normal human platelet count ranges from 150,000 to 450,000 platelets per microliter of blood. These limits are determined by the 2.5th lower and upper percentile, so values outside this range do not necessarily indicate disease...
is a common finding.
Clinically, the syndrome can affect many organs systems. Peripheral thrombosis may be encountered affecting veins or arteries. Intraabdominal thrombosis may lead to pain. Renal and pulmonary complications are common. The patient may exhibit skin purpura and necrosis. Cerebral manifestations may lead to encephalopathy and seizures. Myocardial infarctions may occur. Death may result from multiple organ failure.
Treatments may involve the following steps:
- Prevention includes the use of antibioticAntibioticAn antibacterial is a compound or substance that kills or slows down the growth of bacteria.The term is often used synonymously with the term antibiotic; today, however, with increased knowledge of the causative agents of various infectious diseases, antibiotic has come to denote a broader range of...
s for infection and parenteral anticoagulation for susceptible patients. - Specific therapy includes the use of intravenous heparinHeparinHeparin , also known as unfractionated heparin, a highly sulfated glycosaminoglycan, is widely used as an injectable anticoagulant, and has the highest negative charge density of any known biological molecule...
and corticosteroidCorticosteroidCorticosteroids are a class of steroid hormones that are produced in the adrenal cortex. Corticosteroids are involved in a wide range of physiologic systems such as stress response, immune response and regulation of inflammation, carbohydrate metabolism, protein catabolism, blood electrolyte...
s, and possibly plasma exchanges, intravenous immunoglobulin. - Additional steps may have to be taken to manage circulatory problems, renal failureRenal failureRenal failure or kidney failure describes a medical condition in which the kidneys fail to adequately filter toxins and waste products from the blood...
, and respiratory distressRespiratory distressRespiratory distress may refer to either/both:* Labored breathing, the physical presentation of respiratory distress*Shortness of breath or dyspnea - a sensation of respiratory distress-See also:*List of terms of lung size and activity...
.