Catastrophic antiphospholipid syndrome: Difference between revisions
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{{Infobox medical condition | |||
| name = Catastrophic antiphospholipid syndrome | |||
| synonyms = CAPS, Asherson's syndrome | |||
| field = [[Rheumatology]], [[Hematology]] | |||
| symptoms = [[Thrombosis]], [[multi-organ failure]], [[skin rash]], [[neurological symptoms]] | |||
| complications = [[Organ failure]], [[death]] | |||
| onset = Acute | |||
| duration = Variable | |||
| causes = [[Antiphospholipid antibodies]] | |||
| risks = [[Autoimmune disorders]], [[infections]], [[surgery]], [[trauma]] | |||
| diagnosis = [[Clinical criteria]], [[laboratory tests]] | |||
| differential = [[Disseminated intravascular coagulation]], [[Thrombotic thrombocytopenic purpura]], [[Systemic lupus erythematosus]] | |||
| prevention = Management of [[antiphospholipid syndrome]], [[anticoagulation therapy]] | |||
| treatment = [[Anticoagulation]], [[corticosteroids]], [[plasma exchange]], [[intravenous immunoglobulin]] | |||
| prognosis = Poor without treatment, improved with aggressive therapy | |||
| frequency = Rare | |||
}} | |||
'''Catastrophic antiphospholipid syndrome''' ('''CAPS'''), also known as '''Asherson's syndrome''', is an [[Acute (medical)|acute]] and complex biological process that leads to [[Vascular occlusion|occlusion]] of small vessels of various organs. It was first described by [[Ronald Asherson]] in 1992. The syndrome exhibits [[thrombotic microangiopathy]], [[multiple organ thrombosis]], and in some cases [[tissue necrosis]] and is considered an [[extreme value|extreme]] or catastrophic variant of the [[antiphospholipid syndrome]]. | '''Catastrophic antiphospholipid syndrome''' ('''CAPS'''), also known as '''Asherson's syndrome''', is an [[Acute (medical)|acute]] and complex biological process that leads to [[Vascular occlusion|occlusion]] of small vessels of various organs. It was first described by [[Ronald Asherson]] in 1992. The syndrome exhibits [[thrombotic microangiopathy]], [[multiple organ thrombosis]], and in some cases [[tissue necrosis]] and is considered an [[extreme value|extreme]] or catastrophic variant of the [[antiphospholipid syndrome]]. | ||
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CAPS has a mortality rate of about 50%. With the establishment of a CAPS-Registry more has been learned about this syndrome, but its cause remains unknown. Infection, trauma, medication, and/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. A [[Thrombocytopenia|low platelet count]] is a common finding. | CAPS has a mortality rate of about 50%. With the establishment of a CAPS-Registry more has been learned about this syndrome, but its cause remains unknown. Infection, trauma, medication, and/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. A [[Thrombocytopenia|low platelet count]] is a common finding. | ||
==Presentation== | ==Presentation== | ||
Clinically, the syndrome affects at least three organs and may affect many organs systems. Peripheral thrombosis may be encountered affecting veins and arteries. Intra-abdominal thrombosis may lead to pain. Cardiovascular, nervous, kidney, and lung system complications are common. The affected individual may exhibit skin [[purpura]] and necrosis. Cerebral manifestations may lead to [[encephalopathy]] and [[seizure]]s. [[Myocardial infarction]]s may occur. [[Stroke]]s may occur due to the arterial clotting involvement. Death may result from multiple organ failure. | Clinically, the syndrome affects at least three organs and may affect many organs systems. Peripheral thrombosis may be encountered affecting veins and arteries. Intra-abdominal thrombosis may lead to pain. Cardiovascular, nervous, kidney, and lung system complications are common. The affected individual may exhibit skin [[purpura]] and necrosis. Cerebral manifestations may lead to [[encephalopathy]] and [[seizure]]s. [[Myocardial infarction]]s may occur. [[Stroke]]s may occur due to the arterial clotting involvement. Death may result from multiple organ failure. | ||
==Diagnosis== | ==Diagnosis== | ||
Individuals with CAPS often exhibit a positive test to antilipid antibodies, typically IgG, and may or may not have a history of [[lupus erythematosus|lupus]] or another [[connective tissue disease]]. Association with another disease such as lupus is called a secondary APS unless it includes the defining criteria for CAPS. | Individuals with CAPS often exhibit a positive test to antilipid antibodies, typically IgG, and may or may not have a history of [[lupus erythematosus|lupus]] or another [[connective tissue disease]]. Association with another disease such as lupus is called a secondary APS unless it includes the defining criteria for CAPS. | ||
==Treatment== | ==Treatment== | ||
Treatments may involve the following steps: | Treatments may involve the following steps: | ||
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* Additional steps may have to be taken to manage circulatory problems, [[kidney failure]], and [[respiratory distress]]. | * Additional steps may have to be taken to manage circulatory problems, [[kidney failure]], and [[respiratory distress]]. | ||
* When maintaining survival of the disease treatments also include high doses of Rituxan (Rituximab) to maintain stability. | * When maintaining survival of the disease treatments also include high doses of Rituxan (Rituximab) to maintain stability. | ||
[[Category:Rheumatology]] | [[Category:Rheumatology]] | ||
[[Category:Intensive care medicine]] | [[Category:Intensive care medicine]] | ||
[[Category:Syndromes]] | [[Category:Syndromes]] | ||
{{No image}} | {{No image}} | ||
Latest revision as of 01:33, 4 April 2025
| Catastrophic antiphospholipid syndrome | |
|---|---|
| Synonyms | CAPS, Asherson's syndrome |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Thrombosis, multi-organ failure, skin rash, neurological symptoms |
| Complications | Organ failure, death |
| Onset | Acute |
| Duration | Variable |
| Types | N/A |
| Causes | Antiphospholipid antibodies |
| Risks | Autoimmune disorders, infections, surgery, trauma |
| Diagnosis | Clinical criteria, laboratory tests |
| Differential diagnosis | Disseminated intravascular coagulation, Thrombotic thrombocytopenic purpura, Systemic lupus erythematosus |
| Prevention | Management of antiphospholipid syndrome, anticoagulation therapy |
| Treatment | Anticoagulation, corticosteroids, plasma exchange, intravenous immunoglobulin |
| Medication | N/A |
| Prognosis | Poor without treatment, improved with aggressive therapy |
| Frequency | Rare |
| Deaths | N/A |
Catastrophic antiphospholipid syndrome (CAPS), also known as 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 thrombosis, and in some cases tissue necrosis and is considered an extreme or catastrophic 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, but its cause remains unknown. Infection, trauma, medication, and/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. A low platelet count is a common finding.
Presentation[edit]
Clinically, the syndrome affects at least three organs and may affect many organs systems. Peripheral thrombosis may be encountered affecting veins and arteries. Intra-abdominal thrombosis may lead to pain. Cardiovascular, nervous, kidney, and lung system complications are common. The affected individual may exhibit skin purpura and necrosis. Cerebral manifestations may lead to encephalopathy and seizures. Myocardial infarctions may occur. Strokes may occur due to the arterial clotting involvement. Death may result from multiple organ failure.
Diagnosis[edit]
Individuals with CAPS often exhibit a positive test to antilipid antibodies, typically IgG, and may or may not have a history of lupus or another connective tissue disease. Association with another disease such as lupus is called a secondary APS unless it includes the defining criteria for CAPS.
Treatment[edit]
Treatments may involve the following steps:
- Prevention includes the use of antibiotics for infection and parenteral anticoagulation for susceptible patients.
- Specific therapy includes the use of intravenous heparin and corticosteroids, and possibly plasma exchanges, intravenous immunoglobulin.
- Additional steps may have to be taken to manage circulatory problems, kidney failure, and respiratory distress.
- When maintaining survival of the disease treatments also include high doses of Rituxan (Rituximab) to maintain stability.
