Neurological manifestations of antiphospholipid antibody syndrome

Lupus ◽  
1998 ◽  
Vol 7 (2_suppl) ◽  
pp. 67-74 ◽  
Author(s):  
R.L. Brey ◽  
A Escalante

Thrombosis, thrombocytopenia, recurrent fetal loss and a variety of non-thrombotic neurological disorders have all been associated with antiphospholipid antibodies (aPL). Cerebral ischemia associated with aPL is the most common arterial thrombotic manifestation. Depression, cognitive dysfunction, depression and psychosis have all been associated with aPL. The presumed pathophysiologic mechanism underlying these manifestations is thought to be a result of cerebral ischemia in some, but not all cases. Seizures, chorea and transverse myelitis all appear to be associated with aPL. An interaction between aPL and central nervous system cellular elements rather than aPL-associated thrombosis seems to be a more plausible mechanism for these clinical manifestations. Migraine on the other hand, does not appear to be associated with aPL in either lupus or non-lupus populations. Neuroimaging studies show an increased frequency of brain abnormalities in patients with aPL, but none appear to be specific. The best treatment strategy for preventing neurological manifestations of aPL is not fully defined. For thrombotic manifestations, both antiplatelet and anticoagulant therapies have been suggested. In some patients, immunosuppressant therapy has been used. For non-thrombotic manifestations, some combination of immunosuppressant therapy and symptomatic treatment may be warranted.

Lupus ◽  
1996 ◽  
Vol 5 (5) ◽  
pp. 456-457 ◽  
Author(s):  
LR Espinoza

Of the many clinical manifestations seen in the antiphospholipid antibody syndrome (APAS), two deserve major therapeutic consideration: recurrent fetal loss and vascular thromboses. Treatment of these two major complications remain empirical, although recent studies appear to indicate the beneficial use of multiple therapeutic options including low dose aspirin, alone or in combination with a moderate amount of prednisone, heparin and intravenous gammaglobulin for the prevention of fetal loss, and longterm anticoagulation with maintenance of an international normalized ratio (INR) of 3 to 4 as an effective measure in the prevention of vascular thrombosis. The use of interleukin-3 in animal models of the syndrome has been shown to be effective in the prevention of fetal loss, and this therapeutic modality appears promising, particularly because of its recognized low frequency of side effects in therapeutic trials in humans.


2021 ◽  
Vol 14 (1) ◽  
pp. e236592
Author(s):  
Ying Ling ◽  
Mary Jane Bell ◽  
Lisa Chodirker ◽  
Shirley Lake

A high functioning 74-year-old man with systemic lupus erythematosus presented to the emergency department with acute anxiety. He was found to have elevated cardiac enzymes and admitted to the cardiology service for investigation. In hospital, he developed an erythematous papular rash, and deteriorated to being somnolent and bedridden. He was found to have new multiterritory ischaemic strokes. It was eventually noted that he had persistent eosinophilia, present even on admission, which had been overlooked as the total leucocyte count was normal. Serology for antiphospholipid antibody syndrome (APS) was positive. He was diagnosed with hypereosinophilic syndrome (HES) secondary to new APS, and responded to high-dose steroids. This case highlights the importance of fully evaluating a leucocyte differential to make a diagnosis of HES. We discuss the definition, clinical manifestations, diagnostic approach and management of this important condition.


1998 ◽  
Vol 79 (02) ◽  
pp. 282-285 ◽  
Author(s):  
Josep Ordi-Ros ◽  
Francesc Monegal-Ferran ◽  
Nuria Martinez ◽  
Fina Cortes-Hernandez ◽  
Miquel Vilardell-Tarres ◽  
...  

SummaryObjective: To evaluate the usefulness of IgA antiphospholipid antibodies as markers of thrombosis and/or antiphospholipid antibody syndrome. Patients and Methods: A cross-sectional study design in a tertiary, university-based, autoimmune reference hospital. Seven-hundred ninety-five patients classified into five different groups – autoimmune diseases (255), deep vein thrombosis (153), transitory ischemic attacks (108), obstetric complications (196), infectious diseases (83) and controls (81) – were tested for IgA, IgG and IgM aPL, and lupus anticoagulant. Plasma and serum samples were drawn for detection of aPL using an internationally standardized ELISA method and LA was carried out using coagulometric assays. Results: True IgA aPL were found only in two patients with systemic lupus erythematosus; these patients were also positive to IgG aPL. Conclusion: The incidence of true positivity to IgA anticardiolipin antibodies is extremely low. Their determination was not helpful in diagnosing the antiphospholipid syndrome or in explaining thrombotic events or aPL related manifestations – fetal loss – in the groups studied.


2019 ◽  
Vol 11 (2S) ◽  
pp. 83-88
Author(s):  
O. E. Zinovyeva ◽  
N. V. Vashchenko ◽  
O. E. Mozgovaya ◽  
T. A. Yanakaeva ◽  
A. Yu. Emelyanova

The paper considers various variants of nervous system injury in alcoholic disease. It discusses the epidemiology, pathogenesis, diagnosis, and clinical manifestations of central and peripheral nervous system lesions in the presence of acute and chronic alcohol intoxication. Attention is paid to the issues of etiotropic, pathogenetic, and symptomatic treatment for neurological manifestations of alcoholic disease and to the role of neurotropic B vitamins in the treatment of alcohol-induced deficiency and non-deficiency states.


2012 ◽  
Vol 39 (8) ◽  
pp. 1628-1631 ◽  
Author(s):  
ANAT SCHEIMAN ELAZARY ◽  
MATAN J. COHEN ◽  
SUHAIL AAMAR ◽  
ZVI DRANITZKI ◽  
OSHRAT TAYER-SHIFMAN ◽  
...  

Objective.To characterize the clinical manifestations of patients with antiphospholipid antibody syndrome (APS) and pulmonary hemorrhage (PH).Methods.We performed a retrospective, single-center analysis of patients with APS who were followed up from 1980 to 2011. Of these patients, only those who fulfilled the Sydney criteria for APS were included. Patients with APS that manifested with PH were called the PHAPS group. The rest of the patients with APS served as controls. Clinical manifestations were compared between the PHAPS group and controls.Results.Sixty-three patients fulfilled the criteria for APS. Thirteen experienced PH and were included in the PHAPS group. Seventy-five percent of the patients with PHAPS and 22% of the controls had mitral valve disease (p = 0.001). Central nervous system (CNS) involvement (cerebrovascular accident, seizures) was present in 61% and 16% of the patients with PHAPS and controls, respectively (p = 0.001). Skin involvement (livedo reticularis, chronic leg ulcers) was present in 54% and 8% of the patients with PHAPS and controls (p = 0.001). Pregnancy morbidity occurred in 87.5% and 32.5% of the patients with PHAPS and controls (p = 0.005). Ninety-two percent and 83% of the patients with PHAPS had high-titer immunoglobulin γ (IgG) anticardiolipin and β2-glycoprotein I IgG antibodies compared to 43% and 30% of the controls (p = 0.002, p < 0.001, respectively).Conclusion.Patients with PHAPS were more likely than controls to have mitral valve disease, skin disease, CNS involvement, and pregnancy morbidity as well as high-titer APS. PHAPS seems to be a unique subgroup of all patients with APS.


Author(s):  
Vivian de Oliveira Rodrigues ◽  
Adriana de Góes e Silva Soligo ◽  
Gabriel Duque Pannain

AbstractAntiphospholipid antibody syndrome (APS) is a systemic, autoimmune, prothrombotic disease characterized by persistent antiphospholipid antibodies (aPLs), thrombosis, recurrent abortion, complications during pregnancy, and occasionally thrombocytopenia. The objective of the present study was to review the pathophysiology of APS and its association with female infertility. A bibliographic review of articles of the past 20 years was performed at the PubMed, Scielo, and Bireme databases. Antiphospholipid antibody syndrome may be associated with primary infertility, interfering with endometrial decidualization and with decreased ovarian reserve. Antiphospholipid antibodies also have direct negative effects on placentation, when they bind to the trophoblast, reducing their capacity for invasion, and proinflammatory effects, such as complement activation and neutrophil recruitment, contributing to placental insufficiency, restricted intrauterine growth, and fetal loss. In relation to thrombosis, APS results in a diffuse thrombotic diathesis, with global and diffuse dysregulation of the homeostatic balance. Knowing the pathophysiology of APS, which is closely linked to female infertility, is essential for new therapeutic approaches, specialized in immunomodulation and inflammatory signaling pathways, to provide important advances in its treatment.


2020 ◽  
pp. 107815522093549
Author(s):  
Gizem Yassa ◽  
Abdur R Shakir ◽  
Kuppuswamy Jagarlamudi ◽  
Ahmet E Yassa

Introduction Immune thrombocytopenia is an autoimmune disorder associated with increased thrombocyte destruction and impaired production in the bone marrow. Proposed mechanisms include an antibody or autoreactive T-cell-associated autoimmunity and thrombopoietin deficiency among others. Clinical manifestations are predominantly mucocutaneous hemorrhages including petechiae, purpura, mucosal bleeding in the urinary or the gastrointestinal tracts, menorrhagia, and epistaxis. The purpose of the treatment is to prevent bleeding rather than normalizing the platelet counts. First-line treatments include corticosteroids ± intravenous immunoglobulin and Anti-D which mainly decrease antibody-mediated platelet destruction and increase the number of peripheral Tregs. Second-line and subsequent therapies include splenectomy, chimeric anti-CD20 antibody (rituximab), which eliminates B cells and act as an immunomodulatory agent, and Thrombopoietin receptor agonists (romiplostim), which promote platelet production. Case report We describe a 40-year-old male patient diagnosed with immune thrombocytopenia that was refractory to first-line corticosteroid and intravenous immunoglobulin and second-line romiplostim monotherapy treatments. Management and outcome: The patient was given the romiplostim and rituximab combination which not only successfully treated thrombocytopenia but also resulted in grade 3 bone pains and the patient’s subsequent refusal to continue therapy. Discussion Common adverse effects of rituximab are infusion reactions and prolonged immunosuppression; those of romiplostim include thrombosis, headaches, arthralgia–myalgia, and gastrointestinal symptoms. This case shows that romiplostim has not caused any discernible side effects when given alone, while combination with rituximab resulted in severe bone and joint pains. We hypothesize that this combination regimen shows a synergistic effect both in terms of efficacy and adverse-effect probability and/or severity.


Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 5496-5496
Author(s):  
Jessica D. Emed ◽  
Tanya Z. Martens ◽  
Susan R. Kahn

Abstract Background and Objectives: Pregnancy may be adversely affected by thrombophilia via venous thromboembolism-induced morbidity and mortality or via gestational vascular complications such as fetal loss or preeclampsia. Women with thrombophilia are often prescribed heparin for daily self-injection during pregnancy. The purpose of this study was to explore the unique experiences, challenges, and coping strategies of pregnant women with thrombophilia who are on daily heparin injections. Study Methods: A qualitative, descriptive approach with semi-structured, individual interviews was used. Women with thrombophilia followed at the Thrombosis Clinic of a large university affiliated hospital in Quebec, Canada who were prescribed heparin during pregnancy and who were pregnant at the time of the study or within the last 12 months were eligible. Interviews lasting 45 minutes performed by a trained graduate nursing student were conducted with each participant. Thematic analysis was used throughout the processes of interviewing, transcribing verbatim, and categorizing the data. Results: Nine women (age range 30–36) participated in the study. Four participants had antiphospholipid antibody syndrome, three had the lupus anticoagulant, one had Factor V Leiden and one had Protein S deficiency. For all participants, the diagnosis of thrombophilia was made via blood testing after referral to Clinic for evaluation of multiple early pregnancy losses, late pregnancy losses, or other serious pregnancy complications. None had previous venous thromboembolic events. Four main themes were identified based on analysis of the interview transcripts: the emotional impact of diagnosis and treatment of thrombophilia, which was influenced by past pregnancy experiences as well as the experiences of others; the need for professional support, particularly concerning medical decision-making regarding whether to choose treatment in certain cases, but also with regards to information, injection technique, and emotional needs; uncertainty vis-à-vis treatment outcomes; and successful coping strategies such as taking control and maintaining perspective. Conclusions: This qualitative research shows that, during pregnancy, coping with thrombophilia and daily heparin injections can be a stressful experience. However, the ensuing challenges are perceived as manageable discomforts in light of the outcome of a healthy baby. Implications for health professionals include providing written information to complement explanations given during appointments, standardization of self-injection teaching, establishing peer networks among women who have had similar experiences, and follow-up with a nurse, who can provide ongoing support around coping with uncertainty and the stress of self-injection, revisit the information given, and provide emotional support and referral, if needed. Greater standardization of physician decision making regarding the need for thrombophilia screening and anticoagulation may also be beneficial, since differences in physicians’ approaches across institutions was reported by participants as a source of stress. Nurses, in collaboration with physicians, are in a key position to enhance the quality of care to this population. Further research should evaluate a standardized program based on interventions suggested by this study.


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