Catastrophic Anti Phospholipid Syndrome
Catastrophic antiphospholipid antibody syndrome caps caps is severe manifestation of antiphospholipid antibody syndrome that involves accelerated and widespread thrombosis which may lead to multi organ failure. Caps appears to involve a vicious spiral of progressive complement activation leading to microvascular thrombosis and tissue damage.
The cause of caps is unknown.
Catastrophic anti phospholipid syndrome. 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. Caps is a medical emergency as clots can cause life threatening multi organ failure. A catastrophic antiphospholipid syndrome occurred in 6 patients 5.
This rare syndrome is characterized by the development of multiple blood clots that block small blood vessels in several organs in the body. Anticoagulation corticosteroids and immunoglobulin. To confirm a diagnosis of antiphospholipid syndrome the antibodies must appear in your blood at least twice in tests conducted 12 or more weeks apart.
Secondary aps occurs in the context of an autoimmune disorder such as systemic lupus erythematosus. The use of oral anticoagulants in patients with thrombotic onset did not appear to be protective against recurrences p 0 26. Catastrophic antiphospholipid syndrome caps refers to an acute event when thrombosis is found in three different organs over the period of a week with biopsy evidence of microthrombi and the presence of apls or lupus anticoagulant in a titer higher than 40 u l.
The catastrophic antiphospholipid syndrome caps is a very rare complication encountered in a subset of patients with antiphospholipid syndrome. It was first described by ronald asherson in 1992. Catastrophic antiphospholipid syndrome is a rare disease with high mortality.
In caps multiple blood clots form throughout the body over a short period of time usually within a week. The antiphospholipid syndrome aps is an autoimmune disease characterized by arterial and venous thrombosis due to antiphospholipid antibodies. We have used it successfully for our patient.
Its early identification is crucial in order to establish an effective treatment. Catastrophic antiphospholipid syndrome caps is a rare form of antiphospholipid syndrome aps. A diagnosis of antiphospholipid syndrome is made only when these antibodies cause health problems.
Cyclophosphamide could be a promising treatment in cases of refractory caps. Antiphospholipid syndrome aps is a systemic autoimmune disorder in which the patient s immune system makes antibodies antiphospholipid antibodies apl that increase the risk of blood clots thickened blood and pregnancy complications. You can have antiphospholipid antibodies and never develop any signs or symptoms.
The disorder is referred to as primary when it occurs in the absence of another autoimmune disease. Fifty two women had 87 pregnancies successful in 78. Twenty nine percent of patients accrued functional damage.
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