Idiopathic sclerosing mesenteritis presenting with small bowel volvulus in a patient with antiphospholipid syndrome:A case report

BACKGROUND Sclerosing mesenteritis is a rare disorder involving inflammation of the mesentery.Its etiology remains unclear,but it is believed to be associated with previous abdominal surgery,trauma,autoimmune disorders,infection,or malignancy.Clinical manifestations of sclerosing mesenteritis are varied and include chronic abdominal pain,bloating,diarrhea,weight loss,formation of an intra-abdominal mass,bowel obstruction,and chylous ascites.Here,we present a case of idiopathic sclerosing mesenteritis with small bowel volvulus in a patient with antiphospholipid syndrome.CASE SUMMARY A 68-year-old female presented with recurrent small bowel obstruction.Imaging and pathological findings were consistent with sclerosing mesenteritis causing mesenteric and small bowel volvulus.Computed tomography scans also revealed pulmonary embolism,and the patient was started on a high dose of corticosteroid and a therapeutic dose of anticoagulants.The patient subsequently improved clinically and was discharged.The patient was also diagnosed with antiphospholipid syndrome after a hematological workup.CONCLUSION Sclerosing mesenteritis is a rare condition,and patients with no clear etiology should be considered for treatment with immunosuppressive therapy.

Thrombotic Events in Patients with Antiphospholipid Syndrome: A Single Center Study

Background: There is limited literature regarding risk factors for development of thrombosis and long-term thrombotic outcomes in Armenian APS patients. The aim of the study is to identify patients with APS with thrombotic complications and to evaluate the epidemiological statistics of thrombosis and thrombophilia and their complications in Armenia. Methods: We analyzed medical records of Patients with APS from January 2018 to December 2021 treated at the Armenian Thrombosis and Hemostasis Center was enrolled. Results: Both acquired and hereditary thrombophilia increase the risk of thrombosis. Thrombophilia was present in 61.5% of 123 patients. It was found that 38 pregnant women with thrombosis had a family history of VTE, myocardial infarction or stroke in the next of kin under 50 years of age. The prevalence of this history was 31.4% (11 patients) compared to 68.6% (27 patients), who did not have 41.6% of postpartum thrombotic events up to two months postpartum. Conclusion: Thrombosis in pregnancy is a redoubtable complication requiring an excellent cooperation between the obstetrician and hematologist. Clear detection of thrombosis in APS patients in all types allows to accurately predicting the method and duration of anticoagulant treatment and to prevent thrombotic complications.

Antiphospholipid syndrome and its role in pediatric cerebrovascular diseases: A literature review

Antiphospholipid syndrome(APS)or Hughes syndrome is an acquired thromboinflammatory disorder.Clinical criteria of APS diagnosis are large-and small-vessel thrombosis as well as obstetric problems;laboratory criteria are the presence of antiphospholipid antibodies(lupus anticoagulant,anticardiolipin antibodies and anti-β2-glycoprotein-1).The presence of at least 1 clinical and 1 laboratory criterion allows definitive diagnosis of APS.Primary APS is diagnosed in patients without features of connective tissue disease;secondary APS is diagnosed in patients with clinical signs of autoimmune disease.A high frequency of catastrophic APS as well as a high tendency to evolve from primary APS to secondary syndrome during the course of lupus and lupus-like disease is a feature of pediatric APS.The most characteristic clinical presentation of APS in the pediatric population is venous thrombosis,mainly in the lower limbs,and arterial thrombosis causing ischemic brain stroke.Currently,no diagnostic criteria for pediatric APS exist,which probably results in an underestimation of the problem.Similarly,no therapeutic procedures for APS specific for children have yet been established.In the present literature review,we discussed data concerning APS in children and its role in cerebrovascular diseases,including pediatric arterial ischemic stroke,migraine and cerebral venous thrombosis.

Antiphospholipid syndrome with renal and splenic infarction after blunt trauma:A case report

BACKGROUND In trauma patients,bleeding is an immediate major concern.At the same time,there are few cases of acute vascular occlusion after blunt trauma,and it is unclear what assessment and diagnosis should be considered for these cases.Herein,we describe a patient diagnosed with antiphospholipid syndrome after a hypercoagulable workup for acute renal and splenic vascular occlusion due to blunt trauma.CASE SUMMARY A 20-year-old man was admitted to the emergency department with abdominal pain after hitting a tree while riding a sled 10 h ago.He had no medical history.Radiological investigations revealed occlusion of the left renal artery with global infarction of the left kidney and occlusion of branches of the splenic artery with infarction of the central portion of the spleen.Attempted revascularization of the left renal artery occlusion through percutaneous transluminal angioplasty failed due to difficulty in passing the wire through the total occlusion.Considering the presence of acute multivascular occlusions in a young man with low cardiovascular risk,additional laboratory tests were performed to evaluate hypercoagulability.The results suggested a high possibility of antiphospholipid syndrome.Treatment with a subcutaneous injection of enoxaparin was started and changed to oral warfarin after two weeks.The diagnosis was confirmed,and he continued to visit the rheumatology outpatient clinic while taking warfarin.CONCLUSION A hypercoagulable workup can be considered in trauma patients with acute multivascular occlusion,especially in young patients with low cardiovascular risk.

Immunoadsorption therapy for Klinefelter syndrome with antiphospholipid syndrome in a patient:A case report

BACKGROUND Klinefelter syndrome(KS) is a genetic disease of male sex chromosome malformations that affects sperm production and reduces testosterone production. It has been reported that there is currently more than 10 cases of KS combined with antiphospholipid syndrome(APS).CASE SUMMARY Here, we describe a 31-year-old male patient with chromosome 47, XXY type, who suffered deep vein thrombosis of the lower limbs accompanied by abnormal antiphospholipid antibody, lupus anticoagulant and factor VⅢ. After treatment with immunoadsorption therapy, glucocorticoids, cyclophosphamide, intravenous immunoglobulin and anticoagulant therapy, the patient showed dramatic symptomatic improvement. During the follow-up, the patient did not develop any new thrombotic events.CONCLUSION Immunoadsorption combined with glucocorticoid and cyclophosphamide shock comprehensive treatment has achieved significant results for patients with KS combined with antiphospholipid syndrome.

Severe digital ischemia coexists with thrombocytopenia in malignancy-associated antiphospholipid syndrome: A case report and review of literature

BACKGROUND Paraneoplastic syndromes are characterized by atypical clinical manifestations.Several reports of hepatocellular carcinoma(HCC)paraneoplastic phenomena have been reported.They usually manifest as one type in an individual,but it is not common for the two clinical manifestations to occur simultaneously.CASE SUMMARY A 52-year-old female patient was admitted to hospital with pale skin and numbness of the second to fifth fingers in the left hand,which rapidly developed into severe digital ischemia.Computed tomography angiography revealed uneven thickness of the left ulnar artery with severe local luminal stenosis.Blood analysis during hospitalization showed persistent mild to medium thrombocytopenia and insensitive to hormonal therapy.Antiphospholipid antibody testing showed high titer of IgG anticardiolipin antibodies(aCLs),IgA aCLs,IgG anti-β2-glycoprotein-I(anti-β2 GPI),and IgA anti-β2 GPI.The exact diagnosis was HCC when the high a-fetoprotein levels,computed tomography findings,and the history of chronic hepatitis B came together.This was a rare case of coexisting manifestations as presenting symptoms of malignancy-associated antiphospholipid syndrome.The patient underwent several operations,antithrombotic treatments and hormonal therapy.However,the patient refused chemotherapy and died 8 wk after diagnosis.CONCLUSION This report highlights the importance of atypical clinical changes that could alert the physicians to vigilance for a concomitant underlying malignancy.

Antiphospholipid Syndrome: Nephrologists’ Perspective

Introduction: Antiphospholipid antibody syndrome is a complex autoimmune disease that can affect all systems of the body and characterized by presence of antiphospholipid antibodies that cause vascular thrombosis and pregnancy complications, kidney involvement is very common in patients with antiphos- pholipid syndrome and leads to many serious complications. Objectives: study the effect of antiphospholipid syndrome on kidneys. Data Sources: By searching and reviewing Medline databases (Pub Med and Medscape) and all renal involvement in antiphospholipid syndrome materials available till 2019. Study Selection: All studies were independently assessed for inclusion. They were included if they fulfilled the following criteria: 1) published in English language;2) published in peer-reviewed journals;3) discussed the involvement of kidney in antiphospholipid syndrome. Data Extraction: Data from each eligible study were independently abstracted using a data collection form to capture information related to our concerned study outcomes. If the studies did not fulfill the inclusion criteria, they were excluded. Study quality assessment includes if ethical approval is gained, eligibility criteria specified, adequate information and defined assessment measured. Data Synthesis: Structured review with the results tabulated was made for comparison. Conclusions: We can conclude that kidney affection in patients with antiphos- pholipid syndrome has a wide spectrum and variation.

Bilateral Carotid Aneurysms Secondary to Catastrophic Antiphospholipid Syndrome in a Patient with Differential Diagnosis of Polyarteritis Nodosa

Background: Extracranial Carotid Artery Aneurysm is considered a therapeutic and diagnostic challenge. In an unprecedented way in the literature, we describe an aneurysm originating from the Catastrophic Antiphospholipid Syndrome. Case Presentation: A 25-year-old male patient came to the Emergency Room of the ABC University Hospital in Sao Bernardo do Campo referring to bilateral neck pain for 1 month, associated with carotid aneurysms. Due to the severity and urgency of the clinical condition, immediate surgical therapy was performed without a definitive etiological diagnosis. The initial morphological analysis of the carotid artery suggested a diagnosis of Polyarteritis Nodosa. After anamnesis, physical examination, the use of a specific primary vasculitis algorithm, and a review of the pathological anatomy was requested, which showed bilateral carotid aneurysms secondary to catastrophic antiphospholipid syndrome. Conclusion: It remains evident that Extracranial Carotid Artery Aneurysm-related morbidity and mortality caused by Catastrophic Antiphospholipid Syndrome are influenced by a quick and correct diagnosis.

Successful Management of a Complicated Ulcer Caused by Autoimmune Vasculitis, with Sildenafil Citrate in a Patient with Primary Antiphospholipid Syndrome and Transient Steroid-Induced Diabetes Mellitus: A Case Report and Literature Review

Antiphospholipid syndrome (APS) is a prothrombotic condition that can affect both the venous circulation and the arterial system. The deep veins of the lower extremities and cerebral blood flow are the most common sites of venous and arterial thrombosis, respectively. Skin ulceration and gangrene may be associated with an active vasculitis in patients with APS. These kinds of ulcers are considered intractable because healing is difficult to achieve. Using steroids as immunesuppressant therapy is still a basic part of managing APS and vasculitis. Nevertheless, in the presence of steroid-induced diabetes mellitus, the priority is to achieve faster wound healing, because of the potential complications that can develop due to metabolic dysfunction and augmented vulnerability to infection. Until recently, there were few reports demonstrating the benefits of the use of adjuvant phosphodiesterase-5 inhibitors like sildenafil citrate in different clinical entities with courses like Raynaud’s phenomenon, scleroderma, and resistance to vasodilator therapy. We present the first report demonstrating the additional benefit of sildenafil citrate for the integration of grafts in a patient with steroid-induced diabetes and APS who had ulcers due to vasculitis resistant to the usual vasodilator therapy.

Evaluation of materno-fetal antibody transfer in patients with antiphospholipid syndrome

Introduction:Antiphospholipid syndrome(APS)is characterized by thrombotic events and recurrent pregnancy losses and is considered the most common acquired thrombophilia.Objective:To carry out a narrative review of the transplacental passage and antibodies in patients with APS.Methods:A narrative literature review Results:When it is not associated with any connective tissue disease,it is said to be primary,and when in association with systemic lupus erythematosus,it is said to be secondary.Gestational morbidity is frequent,and it is crucial to evaluate the passage of these antibodies transplacentally since there are animal models of the syndrome with passive transfer of these antibodies.The transplacental passage of specific antibodies has already been determined in studies,which demonstrated low levels of these antibodies in the maternal serum,but an efficient transplacental passage for the newborn.Conclusions:There are few studies on this maternal-infant passage in patients with APS reviewed here.

Efficacy of telitacicept in patients with lupus-and antiphospholipid syndrome-associated refractory thrombocytopenia

Introduction:Thrombocytopenia,a common noncriteria manifestation of antiphospholipid syndrome(APS),is severe in approximately one-third of patients with APS.However,there are no guidelines for treating such thrombocytopenia.B-cell-targeting therapy may be an option in refractory cases;however,its efficacy has not been firmly established.Here,we report on two patients with refractory antiphospholipid antibodies(aPLs)-associated thrombocytopenia treated with telitacicept.Case Description:Case 1,a 39-year-old woman,presented with systemic lupus erythematosus(SLE)and APS with diffuse alveolar hemorrhage,persistent thrombocytopenia,and recurrent miscarriages.The thrombocytopenia had been refractory to multiple lines of treatments,the most recent being mycophenolate mofetil and prednisone(5 mg/day).After receiving telitacicept(160 mg/week)for 3 months,she had presented with decreased titers of aPLs and a slight increase in platelet counts(14×10^(9)to 35×10^(9)/L).Case 2,a 51-year-old woman,presented with SLE and APS with refractory thrombocytopenia.She had been diagnosed with pulmonary tuberculosis 4.5 years ago and received antituberculosis therapy for 2 years.She had also undergone pulmonary lobectomy 4 years ago for lung adenocarcinoma.Her thrombocytopenia relapsed(lowest 14×10^(9)/L)when prednisone was tapered to<10mg/day.After adding telitacicept(160 mg/week)to cyclosporin A(150 mg/day)and hydroxychloroquine(400 mg/day)for 5 months,aPLs decreased and platelet counts increased(78×10^(9)to 183×10^(9)/L),enabling halving of her dose of prednisone from 15 to 7.5 mg/day.Neither patient had any adverse effects.Conclusion:Telitacicept can safely reduce aPLs titers and improve refractory thrombocytopenia in patients with secondary APS.Randomized-controlled trials to assess its effectiveness are urgently required.

Role of β2-glycoprotein I in the pathogenesis of the antiphospholipid syndrome

Antiphospholipid syndrome(APS)is typically characterized by increased levels of three classes of antiphospholipid antibodies,namely lupus anticoagulant,anti-β2-glycoprotein I(anti-β2GPI),and anticardiolipin antibodies.β2-Glycoprotein(β2GPI)is a phospholipid-binding protein composed of five domains(DI-V)and a major antigen in APS.β2GPI is expressed on the surfaces of several cell types,including endothelial cells,monocytes,trophoblast cells,and platelets.Its binding to the anti-β2GPI antibody triggers downstream signaling events and ultimately exerts a variety of cellular effects.β2GPI modulates hemostasis and the complement system,as well as playing an important role in APS-associated vascular injury.Therefore,studying β2GPI will help elucidate the pathogenesis of APS and improve the treatment of patients with this condition.This review will mainly focus on the structure and function of β2GPI,as well as its implication in the pathogenesis of APS.

The mTOR pathway in the antiphospholipid syndrome

This perspective discussed the available evidence on the involvement of mTOR pathway in antiphospholipid syndrome(APS),from the aspects of endothelial cells,platelets,monocytes and anti-phospholipid antibodies(PLs),which may lead to future therapeutic applications of mTOR inhibition in APS.

Artemisinin analogue SM934 protects against lupus-associated antiphospholipid syndrome via activation of Nrf2 and its targets

Kidney is a major target organ in both antiphospholipid syndrome(APS)and systemic lupus erythematosus(SLE).The etiology of antiphospholipid syndrome nephropathy associated lupus nephritis(APSN-LN)is intricate and remains largely unrevealed.We proposed in present work,that generation of antiphospholipid antibodies(aPLs),especially those directed towards the oxidized neoepitopes,are largely linked with the redox status along with disease progression.Moreover,we observed that compromised antioxidative capacity coincided with turbulence of inflammatory cytokine profile in the kidney of male NZW×BXSB F1 mice suffered from APSN-LN.SM934 is an artemisinin derivative that has been proved to have potent immunosuppressive properties.In current study,we elaborated the therapeutic benefits of SM934 in male NZW×BXSB F1 mice,a murine model develops syndrome resembled human APS associated with SLE,for the first time.SM934 treatment comprehensively impeded autoantibodies production,inflammatory cytokine accumulation and excessive oxidative stress in kidney.Among others,we interpreted in present work that both anti-inflammatory and antioxidative effects of SM934 is closely correlated with the enhancement of Nrf2 signaling and expression of its targets.Collectively,our finding confirmed that therapeutic strategy simultaneously exerting antioxidant and anti-inflammatory efficacy provide a novel feasible remedy for treating APSN-LN.

Antiphospholipid syndrome:a clinical perspective

Antiphospholipid syndrome (APS) is a thromboinflammatory disease with a variety of clinical phenotypes. Primary thrombosis prophylaxis should take an individualized risk stratification approach. Moderate-intensity vitamin K antagonist such as warfarin remains the primary strategy for secondary thrombosis prophylaxis among APS patients, especially for patients with predominantly venous disease. For now, direct oral anti-coagulants should be avoided in most APS patients, especially those with history of arterial manifestations. Obstetric APS management should be tailored based on an individual patient’s antiphospholipid antibody profile, and obstetric and thrombotic history. Pharmacological agents beyond anticoagulants may be considered for the management of microthrombotic and nonthrombotic manifestations of APS, although more data are needed. A relatively recent discovery in the area of APS pathogenesis is the implication of neutrophil extracellular traps in thrombin generation and initiation of inflammatory cascades. APS is a complex thromboinflammatory disease with a broad clinical spectrum. Personalized therapy according to an individual’s unique thrombosis and obstetric risk should be advocated.

Patient with antiphospholipid syndrome accompanied by pre-eclampsia who developed hellp syndrome and eclampsia after abortion

Antiphospholipid syndrome （APS） refers to a group of clinical symptoms and signs caused by antiphospholipid antibody （aPLA）. We reported a rare case of poor outcome of a pregnant woman with APS. The pregnant woman had APS, hemolytic anemia, elevated liver function and low platelet count （HELLP） syndrome, and eclampsia and had a poor outcome from a second pregnancy. She was treated with antispasmodics, sedatives, and anti-hypertensive agents, along with anticoagulant therapy and infusion of immunoglobulin. APS during pregnancy often makes pregnancy even more complex and risky. Obstetricians should carry out anticoagulation treatment throughout the perinatal period.

Development of cerebral venous sinus thrombosis in an aplastic anemia patient with antiphospholipid syndrome

Aplastic anemia （AA） is an acquired disorder in which .bone marrow fails to produce or release sufficient amounts of blood cell. Antiphospholipid syndrome （APS） is an autoimmune disease characterised by recurrent arterial or venous thrombosis, pregnancy morbidity and the persistence of positive antiphospholipid antibodies （aPL）, including anticardiolipin antibody （ACL） and lupus anticoagulant （LA）. 1 The most prevalent neurologic manifestation of APS is cerebrovascular ischemic events due to arterial thromboses. The deep veins of lower extremities are commonly involved in venous thrombosis of APS, but cerebral venous sinus thrombosis is rare with only several cases reported.2-11 AA together with APS is extremely rare.12 Here we report a case of cerebral venous sinus thrombosis in an AA patient with APS.

Possible effects of chemokine-like factor-like MARVEL transmembrane domain-containing family on antiphospholipid syndrome

Antiphospholipid syndrome(APS)is a systemic autoimmune disease defined by thrombotic or obstetrical events and persistent antiphospholipid antibodies(aPLs).Chemokine-like factor-like MARVEL transmembrane domain-containing family(CMTM)is widely expressed in the immune system and may closely related to APS.This review aimed to systematically summarize the possible effects of CMTM on APS.Publications were collected from PubMed and Web of Science databases up to August 2020.CKLF,CKLFSF,CMTM,antiphospholipid syndrome,immune cells,and immune molecules were used as search criteria.Immune cells,including neutrophil,dendritic cells(DCs),T-cells,B-cells,and inflammatory cytokines,play an important role in the development of APS.Chemokine-like factor 1(CKLF1)has a chemotactic effect on many cells and can affect the expression of inflammatory cytokines and adhesion molecules through the nuclear factor-kB(NF-kB)pathway or mitogen-activated protein kinase(MARK)pathway.CKLF1 can participate in the maturation of DCs,T lymphocyte activation,and the activation of neutrophils through the MAPK pathway.CMTM1 may act on Annexin A2 by regulating Ca^(2+) signaling.CMTM2 and CMTM6 are up-regulated in neutrophils of APS patients.Some CMTM family members influence the activation and accumulation of platelets.CMTM3 and CMTM7 are binding partners of B-cell linker protein(BLNK),thereby linking B cell receptor(BCR)and activating BLNK-mediated signal transduction in B cells.Moreover,CMTM3 and CMTM7 can act on DCs and B-1a cell development,respectively.CMTM may have potential effects on the development of APS by acting on immune cells and immune molecules.Thus,CMTM may act as a novel prognostic factor or immunomodulatory treatment option of APS.

Myocardial infarction in a patient with systemic lupus erythematosus and antiphospholipid syndrome

This case report we presented aims to report a-31-year-old man with systemic lupus erythematosus （SLE） and antiphospholipid syndrome （APS） who developed myocardial infarction （MI） and also aims to discuss the possible mechanisms. The results showed that traditional risk factors alone do not cause coronary heart disease with SLE, and SLE-related factors influence the atherogenic process. We found that although SLE patients with acute MI benefit from percutaneous coronary intervention （PCI） therapy, it is very important to choose the reasonable antithrombotic strategies in patients with SLE and APS undergoing PCI who require oral anticoagulant therapy.

Myocardial infarction in antiphospholipid antibody syndrome

A 52-year-old man was admitted to hospital with chest pain after physical activity. Emergency coronary angiography showed multiple throm-boembolic occlusions in the anterior descen-ding coronary artery and in the right coronary artery. Further testing revealed anticardiolipin and ?2-glicoprotein antibodies (the patient had been diagnosed for ulcerative colitis and poly-myalgia rheumatica). Heparin and nitrate were administered intravenously in addition to oral aspirin and metoprolol. Soon after, the patient referred a withdrawal of chest oppression, and his general clinical condition rapidly stabilised. A follow-up examination was performed 9 months later the discharge: he had resumed most of his activities and sieric concentration of lupus anticoagulant antibodies and anticardiolipin an- tibodies, IgM isotype, were decreased.