The mean SD age was Nine patients 4.
All of them changed their place of residence and were known to be alive at the time of withdrawing from the cohort. Mean SD follow-up of this group was 6. At diagnosis, patients had antiphospholipid antibodies tested. Twelve patients fulfilled criteria for definite APS.
During follow-up, antiphospholipid antibodies were tested in patients and detected in 85 of them Table 1. Twenty-eight had APS according to Sapporo definitions. Twenty-four patients had arterial events 7 combined with venous thrombosis and 5 combined with small vessel thrombosis , 3 had venous events only, and 1 had isolated small vessel thrombosis renal thrombotic microangiopathy. Seven women in the group of patients with APS became pregnant after the diagnosis of SLE, 3 of them having obstetric complications 2 fetal deaths and 1 preeclampsia.
Two additional women with APS had fetal deaths before the diagnosis of lupus was met. Irreversible organ damage was accrued during disease course. The median range SDI scores were 0 at diagnosis, 1 at 10 years, and 2 at 15 years. Table 2 presents the number of patients with some permanent damage, which increased over the time.
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Damage was more severe in patients with APS. This effect of APS on irreversible organ damage was independent of other clinical variables. Mean SD age at the time of death was 54 19 years. Mean SD time of follow-up was 9. Given the small number of patients at risk beyond 15 years of follow-up, we decided to limit survival comparisons to that point. In 4 of these patients, thrombotic events could have been the direct cause or a major predisposing factor for death mesenteric ischemia, sudden death, atherosclerotic disease, and heart failure.
None of them were receiving anticoagulant treatment at the time of their death. Two patients died before prolonged oral anticoagulation was considered the standard treatment for patients with APS and thrombosis. Of the remaining 4 patients with APS, 2 died of cancer, 1 died of infection legionellosis , and 1 committed suicide.
Patients with some degree of irreversible organ damage also showed increased mortality Figure 2. Several variables were included in a Cox proportional hazards regression model to determine the independent predictors of survival at 15 years see "Methods" section. The final model included 3 explanatory variables: age at diagnosis OR, 1. The noninclusion of APS in the baseline model made thrombosis a significant final explanatory variable, which suggests that the influence of APS on survival is dependent of thrombotic manifestations. Increased mortality linked to APS is due to thrombosis, as a consequence of both acute events and irreversible organ damage.
Mortality rates and causes of death have been similar in our cohort and other recent series. Infection and cardiovascular diseases were the most frequent conditions that led to death. However, we have observed few patients dying of active lupus. A progressive increase of irreversible organ damage was seen in our cohort.
We have also shown that higher SDI scores lead to increased mortality, which is in agreement with the results reported by other authors. Indeed, APS was identified as an independent risk factor for mortality, together with age and lupus nephritis, 2 well-known adverse prognostic variables in lupus.
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Given the importance of thrombosis among the causes of death reported in recent prospective studies, 3 , 4 our results are not surprising. Indeed, 3 previous works also pointed in this direction. They found a higher incidence of deep vein thrombosis among patients with LA and increased mortality related to both deep vein thrombosis and LA positivity.
Gulko et al 10 studied patients with SLE to determine the impact of several variables on survival. They found that aCL positivity and thrombosis, together with renal failure, age, and infection, were major predictors of mortality.
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In our study, thrombosis, highly dependent on the presence of APS, was also identified as an adverse prognostic variable. However, thrombosis was the direct cause of only part of the deaths of lupus patients with APS. This observation has also been made by other authors. In our study, APS was a strong and independent cause of irreversible damage, which was also a predictor of death. It is probable that, apart from the inherent high risk of end-stage organ failure, permanent damage may make patients more prone to experience complications, such as infections and drug toxic effects and maybe cancer , leading to death.
This study has some limitations. The first is that arterial thromboses were the clinical manifestations associated with APS in most patients. Thus, patients in our group had a particularly severe form of APS. It is likely that the impact of APS on mortality and damage would be less in people with other clinical manifestations.
The second problem is the relatively small number of patients included in our study. We had to limit the extension of the survival analysis to 15 years due to the small group of patients followed up beyond that point. Finally, because this study was observational, no specific interventions in terms of therapy were planned. Some patients with APS and thrombosis did not receive prolonged oral anticoagulant treatment. This is especially true in patients treated before , when it became clearly established that vitamin K antagonists are the drugs of choice for this group of patients.
More Details Original Title. Other Editions 3. Friend Reviews. To see what your friends thought of this book, please sign up. To ask other readers questions about Understanding Hughes Syndrome , please sign up. Be the first to ask a question about Understanding Hughes Syndrome.
Lists with This Book. This book is not yet featured on Listopia. Community Reviews. Showing Average rating 4. Rating details. More filters. Sort order. Feb 05, Helen rated it it was amazing. Essential reading for anyone diagnosed with aps. April rated it it was amazing Apr 15, Anne Sigmon rated it it was amazing Jul 21, Ruth Buckwold rated it really liked it Oct 19, Thrombocytopenia associated with antiphospholipid antibodies is usually mild and only rarely causes easy or excessive bruising and prolong bleeding episodes.
Affected individuals are also at risk for autoimmune hemolytic anemia, a condition characterized by the premature destruction of red blood cells by the immune system. Some individuals have reported symptoms that resemble multiple sclerosis including numbness or a sensation of pins and needles, vision abnormalities such as double vision, and difficulty walking, but it is not known if these problems are related to APS. Some data show an association of APS with cognitive dysfunction, but the mechanism is not known.
In women, APS can cause complications during pregnancy including repeated miscarriages, fetal growth delays intrauterine growth retardation , and preeclampsia. Preeclampsia is a condition characterized by high blood pressure, swelling and protein in the urine. Symptoms associated with preeclampsia vary greatly, but may include headaches, changes in vision, abdominal pain, nausea and vomiting.
Antiphospholipid syndrome (APS) - NHS
The specific presentation, progression and organs involved vary from person to person. In some cases, infection, trauma, or surgery appears to trigger the condition. Antiphospholipid syndrome is an autoimmune disorder of unknown cause. Autoimmune disorders are caused when the body natural defenses antibodies, lymphocytes, etc. Researchers believe that multiple factors including genetic and environmental factors play a role in the development of APS. In rare cases, APS has run in families suggesting that a genetic predisposition to developing the disorder may exist.
The antibodies that are present in APS are known as antiphospholipid antibodies. These antibodies were originally thought to attack phospholipids, fatty molecules that are a normal part of cell membranes found throughout the body. However, researchers now know that these antibodies mostly target certain blood proteins that bind to phospholipids.
The two most common proteins affected are betaglycoprotein I and prothrombin. The exact mechanism by which these antiphospholipid antibodies eventually lead to the development of blood clots is not known. APS affects males and females, but a large percentage of primary APS patients are women with recurrent pregnancy loss. Some estimates suggest that 1 in 5 cases of recurrent miscarriages or deep vein thromboses are due to APS. As many as one-third of cases of stroke in people under 50 years of age may be due to APS.
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Symptoms of the following disorders can be similar to those of antiphospholipid syndrome. Comparisons may be useful for a differential diagnosis. Several rare genetic disorders are characterized by the formation of blood clots thromboses. These disorders may be collectively referred as the thrombophilias and include protein C deficiency, protein S deficiency, antithrombin III deficiency, and factor V Leiden.
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For more information on these disorders, contact the National Alliance for Thrombosis and Thrombophilia. Some individuals with APS may be misdiagnosed as having multiple sclerosis MS because of the development of similar neurological symptoms. Multiple sclerosis is a chronic disease of the brain and spinal cord central nervous system that may be progressive, relapsing and remitting, or stable.
The pathology of MS consists of small lesions called plaques that may form randomly throughout the brain and spinal cord. These patches prevent proper transmission of nervous system signals and thus result in a variety of symptoms including eye abnormalities, impairment of speech, and numbness or tingling sensation in the limbs and difficulty walking. The exact cause of multiple sclerosis is unknown. Lupus systemic lupus erythematosus is a chronic, inflammatory autoimmune disorder that can affect various organ systems.
In lupus, the organ systems most often involved include the skin, kidneys, blood and joints. Many different symptoms are associated with lupus, and most affected individuals do not experience all of the symptoms. The initial symptoms may include arthritis, skin rashes, fatigue, fever, pleurisy, and weight loss. In some cases, lupus may be a mild disorder affecting only a few organ systems. In other cases, it may result in serious complications. A diagnosis of antiphospholipid syndrome is made based upon a thorough clinical evaluation, a detailed patient history, identification of characteristic physical findings at least one blood clot or clinical finding , and a variety of tests including simple blood tests.