A thrombotic storm may also occur due to the following precipitating events: alterations in coagulation and fibrinolysis, which induce high mortality rates, and infections amongst pediatric patients where IgM and IgG anti-β2-GPI antibodies induce an endothelial signal, leading to a procoagulant state. It is also hypothesized that thrombotic storms occur due to prothrombotic genetic risk factors, which trigger a sped-up form of thrombosis after its first occurrence, rather than being caused solely by environmental factors. Cardiovascular, nervous, kidney, and lung system complications are common. More specifically with the heart, Ashersons syndrome can lead to complications such as mitral valve regurgitation (MVR) in which the mitral valve does not shut properly allowing backflow of blood into the heart as well as angina (chest pain) and myocardial infarction (heart attack). Furthermore, complications in the kidneys may occur, including low urine production and high blood pressure, while complications with the lungs can result in rapid breathing (hyperventilation) and low oxygen levels (hypoxemia). 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. Furthermore, the syndrome has been shown to manifest multi-organ failures and miscarriages in the context of pregnancies. 54% of pregnant mothers with the disease experienced fetal loss, and around 50% of pregnant mothers died from complications associated with the syndrome. Diagnosis
Two specific types of blood tests are used to aid in the diagnosis of Ashersons Syndrome.
Zafirlukast is an orally administered leukotriene receptor antagonist (LTRA) used for the chronic treatment of asthma. While zafirlukast is generally well tolerated, headache and stomach upset often occur. Some rare side effects can occur, which can be life-threatening, such as liver failure. Churg-Strauss syndrome has been associated with zafirlukast, but the relationship isnt thought to be causative in nature. Overdoses of zafirlukast tend to be self-limiting. Zafirlukast, like other LTRAs, works by inhibiting the immune system. Through its action on inflammatory cells in the lungs, zafirlukast reduces the production of inflammatory mediators that are implicated in the pathogenesis of asthma. Zafirlukast is extensively hepatically metabolized by an enzyme called CYP2C9. Zafirlukast inhibits the action of CYP3A4, leading to drug-drug interactions with other drugs that are metabolized by CYP3A4. Genetic differences in LTC4 synthase and CYP2C9 may predict how a person reacts to zafirlukast treatment. Zafirlukast (brand name Accolate) was the first cysteinyl leukotriene receptor antagonist approved in the United States. It is now approved in many other countries under other brand names. Medical uses
Zafirlukast is FDA-approved for the prevention and treatment of asthma in adults and children older than 5 years old. Like other leukotriene receptor antagonists, zafirlukast is thought to be useful for the long-term treatment of asthma, but it is generally less effective than inhaled glucocorticoids as monotherapy (which are the standard of care) or long-acting beta-2 agonists in combination therapy. Notably, zafirlukast is ineffective in the event of an acute asthma attack.