What Is Asherson’s Syndrome?
Asherson’s Syndrome is a severe variant of antiphospholipid syndrome. It is a rare, autoimmune disease that is characterized by the rapid formation of multiple blood clots progressively in the whole body thereby affecting multiple organs in the body. The duration of the blood clot formation varies from days to hours, or weeks.
Individuals suffering from antiphospholipid syndrome are most commonly affected by the disorder. The anticoagulation process fails to occur in these individuals thereby causing recurrent bleeding in their bodies. This ultimately leads to multiple organ failures in the patient's body, which is fatal.
What Are the Causes of Asherson’s Syndrome?
Asherson’s Syndrome is an autoimmune disorder where the phospholipid antibodies and lymphocytes self-attack the body’s healthy cells and tissues. that is caused due to self-attack of the body’s immune system. Environmental factors along with hereditary factors can also play a crucial role in the initiation of Asherson’s syndrome. Infected wounds and failed anticoagulation mechanisms inside the body of the patient are some of the predisposing factors.
What Is the Pathogenesis of Asherson’s Syndrome?
The disorder is mainly believed to occur as a result of wound infection, or autoimmune reaction in response to any trauma and failed anticoagulation process within the body. The blood clots occur with the antiphospholipid antibodies present in the body. Antibodies are specialized proteins or immunoglobulins that are produced by the body’s immune system to fight foreign germs and microbes. In Asherson’s syndrome, certain proteins that are attached to phospholipids (fat molecules involved in the proper functioning of cell membranes) are mistakenly attacked by the body’s antibodies. These phospholipids are present throughout the body. The mechanism of blood clot formation is unknown. It is suggested that an inflammatory response is caused by the antiphospholipid antibodies thereby activating the endothelial cells and platelets. This leads to progressive blood clot formation at a rapid rate.
What Are the Symptoms of Asherson’s Syndrome?
Asherson’s Syndrome is a life-threatening condition in an individual that mainly occurs due to the rapid formation of multiple blood clots in the body. They are formed progressively, usually within a few hours, days, or even weeks. The clinical features related to this disorder are very severe and include the following:
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Renal Dysfunction: Kidneys are severely affected causing conditions like raised blood pressure levels and decreased urine outflow.
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Lung Dysfunction: Lungs are severely affected leading to respiratory distress syndrome (breathing becomes difficult and fluid is leaked into the lungs) in adults. The oxygen levels are drastically reduced in circulating blood and difficulties in respiration are the important symptoms related to this condition. Pulmonary embolism (when a blood clot gets stuck in the artery of the lung) may also occur.
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Livedo Reticularis: It is an abnormal skin symptom characterized by a net-like pattern of bruising and reddish-blue discolored patches on the skin. The formation of gangrene (localized death of the body tissue) may also occur.
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Central Nervous System Dysfunction: Sudden seizures, stroke (brain attack due to sudden cessation of blood supply to the brain), and encephalopathy (brain malfunctioning due to viral infection) are a few common complications associated with the disorder.
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Cardiac Complications: Heart valves are inflamed and thickened. Heart complications like mitral valve regurgitation, chest pain, and heart attack may also occur.
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Abdominal Complications: Pain and cramps in the abdomen may also occur.
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Other Complications: Anemia, imbalance in the hormonal levels, and reduced blood pressure are other complications that may be associated with this rare autoimmune disease.
How Is Asherson's Syndrome Diagnosed?
The diagnosis of Asherson’s Syndrome should be done under the expert supervision of a healthcare professional. The line of treatment and prognosis is determined based on the accurate diagnosis of the disease. The steps involved in the diagnosis of the disease are:
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Clinical Examination: A thorough clinical evaluation is crucial for understanding the severity of the disease and the underlying cause. Based on the clinical data obtained, a proper treatment plan is determined for the patient.
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Identification of Symptoms: This step involves identifying typical classic features of the disease including widespread blood clots. The patient should be properly examined for multiple blood clots affecting simultaneously three or more organ systems in one week span.
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Investigations: Blood tests should be conducted. The blood tests will exhibit the presence of antiphospholipid antibodies in the serum of the patient.
How Is Asherson's Syndrome Treated?
Asherson’s Syndrome should be treated under the expert supervision of a doctor with a proper line of treatment. The following steps involved are:
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Antibiotic Application: This step involves the use of antibiotics against the risk of developing infections and parenteral anticoagulation in the affected individuals to prevent the risk of thromboembolism.
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Specific Drug Therapies: The application of heparin and corticosteroids is specifically done intravenously. Plasma therapy (plasma replacement) and immunoglobulins administration is also preferred for the treatment of the patient.
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Interdisciplinary Treatment Therapies: Different treatment modalities such as anticoagulation, plasma therapy, glucocorticoid administration, and antiplatelet drugs are also considered in the treatment therapy.
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Symptomatic Treatment Therapy: This involves the symptomatic treatment and management of other systemic conditions of the patient such as kidneys, abdomen, lungs, and heart-related problems.
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Monoclonal Antibodies Administration: Novel therapies are being developed to administer monoclonal antibodies such as Rituxan and Eculizumab to the affected individual in high doses.
How To Prevent Asherson’s Syndrome?
There is no definitive way to prevent the occurrence of Asherson’s Syndrome since it is an autoimmune disease. However, a few steps can be taken for the prevention and recurrence of the disease:
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Use of Antibiotics: To prevent the development of infections, the proper use of antibiotics is preferred.
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Anticoagulation Therapy: Parenteral anticoagulation (drugs that are used to prevent blood clotting) therapy is considered in the affected individuals.
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Proper Dietary Intake: Consumption of green leafy vegetables and a balanced diet helps to prevent the disease.
Conclusion
Asherson’s syndrome is a rare autoimmune disorder characterized by multiple organ involvement and malfunctioning of the body with progressive blood clot formation. A proper and accurate diagnosis and adequate treatment plan for the patient should be determined. The disease requires urgent medical care and attention from a health expert. One should visit the doctor immediately for consultation after observing the symptoms.
