What is Asherson’s Syndrome: Symptoms, Causes, Diagnosis, Treatment
The medical name of Asherson’s syndrome is Catastrophicantiphospholipid syndrome or CAPS. It is one of the rarest diseases that are found in the human body, which leads to the formation of blood clots in the smaller blood vessels of different organs of the body. This is a condition that was first explained by Ronald Asherson in the year 1992, giving the name to the condition. Asherson’s syndrome is a condition that leads to a thrombotic outbreak in multiple organs and also tissues necrosis in certain cases. It is considered to be one of the extreme cases of antiphospholipid syndrome.
The ailment of Asherson’s syndrome is known to have a 50% mortality rate. Investigations and researches are ongoing for more knowledge on the ailment; however, the causes and the trigger factors are still unknown. Some common conditions leading to ailment are those of trauma, infection, surgery, and medication.(1)
Asherson’s Syndrome: Brief Overview
Asherson’s syndrome is a really rare autoimmune disorder which can develop over a period of a week, days or even hours. In this condition, rapidly progressive clots of blood are formed in multiple organs of the human body. Asherson’s syndrome is common in patients who already have antiphospholipid syndrome. These people often experience an abnormal coagulation mechanism in the body. These patients often develop the serious problem of multi-organ failure that is a fatal situation, while the same symptoms in other patients may lead to just multi-organ thrombosis. Symptoms of the problem can differ from patient to patient and will depend on the organ that has been affected.(1)
The clots that are formed in Asherson’s Syndrome happen due to the antibodies in the human body. These antibodies are special proteins that are made by the immune systems. In this condition, the antibodies attack the healthy tissues of the body affecting the working of the cell membranes. In many situations, Asherson’s syndrome can also occur to people who do not have any other medical problems. The exact cause of Asherson’s syndrome is still unknown.(1, 2)
Symptoms of Asherson’s Syndrome
The symptom of Asherson’s syndrome is majorly thrombosis. Different symptoms that are developed during the course of this disease will depend on the organ that has been affected. Organs like the central nervous system, heart, lungs, kidney, and stomach often get affected. The symptoms specific to the organs are as follows:
- Symptoms of Asherson’s Syndrome When Affecting Kidneys: Involvement of the kidneys can lead to major problems and even failures in really serious cases. Symptoms like low urine production, renal hypertension, and other problems are common.
- Lungs: If the respiratory systems are affected then it can lead to respiratory distress. The patient can also develop dyspnea or an acute breathing disorder. The patient might also show signs like rapid and deep breathing and hypoxemia. The person may also show pulmonary embolism.
- Symptoms of Asherson’s Syndrome When Central Nervous System is Affected: If the central nervous system gets affected, then the person might experience stroke and seizure. In severe conditions, the person may also face encephalopathy, a disease where the brain function gets altered.
- Heart: In case the cardiac system is affected by Asherson’s syndrome, the patient may develop the problem of thickening and also an inflammation of the valves (valvular heart disease). This could lead to the critical complication of Mitral Valve Regurgitation. In this condition, there is a backflow of blood into the heart. Patients with this condition can experience chest pain leading to a heart attack.
- Stomach: Gastrointestinal problems can include acute stomach cramps. In other cases conditions like decreasing blood platelets, lower bone marrow and improper red blood cells are some common outcomes.
Causes of Asherson’s Syndrome
As mentioned in the opening of the discussion, the exact cause that leads to Asherson’s syndrome is still not known. However, it is seen that antibodies that are produced to fight against ailments start fighting against the healthy tissues of the body. The antibodies that are also found in antiphospholipid syndrome start to attack the phospholipid fatty molecules that are present in the cell membranes. This antibody targets the blood proteins all over the body. Two most common proteins that get targeted are the beta 2 glycoprotein and the prothrombin. However, further research into this domain is required so that more can be known about the trigger factors that can lead to Asherson’s Syndrome.(1)
Diagnosis of Asherson’s Syndrome
Generally, a history of thrombosis or that of pregnancy loss will lead to the requirement of making further investigations for the diagnosis of the condition. The diagnosis is generally done through a blood test, which exhibits whether the person has abnormal antibodies or not. In certain cases, it has been seen that harmless antiphospholipid antibodies can develop in a person for a limited time period. This could be due to the effect of some kind of medication or due to some type of infection. In such a situation often a second blood test is also done to confirm the status. In case there is some sort of abnormality in the antibodies present in the person, a quick investigation into the medical history of the person will be conducted to discern whether they have been caused by the antiphospholipid syndrome or not.
Treatment of Asherson’s Syndrome
Treatment for Asherson’s syndrome generally includes steps that will thin the blood. Medication is prescribed for this purpose so that the chances of forming a blood clot in the body can be minimized. Generally, this medicine will be needed by the patient throughout his or her life. Some of the possible medication combinations will include warfarin and aspirin, heparin and Coumadin, etc. However, the combination needs to be determined in a proper manner as improper combinations can lead to excessive bleeding and even chances of hemorrhage. Patients must seek immediate help if they see blood in their cough, vomit, urine, stool or has frequent nose bleeding that persists for more than ten minutes at a stretch.
Complications for Asherson’s Syndrome
Asherson’s Syndrome is a condition where the patient develops symptoms like that of ordinary or general thrombosis. This condition can also lead to a problem of Thrombotic thrombocytopenic purpura which is a blood disorder that leads to blood clots. Asherson’s syndrome is much more prevalent amongst women and develops around their mid-thirties or early forties. Findings reveal a tendency of lower blood platelets and also an inadequate number of red blood cells leading to anemia. Asherson’s syndrome also leads to small red or purple spots on the skin. Patients suffering from Asherson’s syndrome may also develop acute nervous disorders that could lead to paralysis and seizures.
More research is required into the domain so that proper treatment can be found for Asherson’s Syndrome. In the present times, the initial treatment comprises a dosage of anticoagulant and heparin that is delivered through an intravenous administration. Corticosteroids may also be needed along with heparin in certain cases. These steroids control the loss of tissues and help to contain the damages rendered to the body in this condition. Repeated plasma exchange might also be needed in this condition.
Plasmapheresis is also followed that removes the unwanted things in the bloodstream. Additionally, intensive antibiotics therapy is also continued to fight with the infections.