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.

Simultaneous intrahepatic and subgaleal hemorrhage in antiphospholipid syndrome following anticoagulation therapy

Warfarin is a widely used anticoagulant.Interindividual differences in drug response,a narrow therapeutic range and the risk of bleeding render warfarin difficult to use clinically.An 18-year-old woman with antiphospholipid syndrome received long-term warfarin therapy for a recurrent deep vein thrombosis.Six years later,she developed right flank pain.We diagnosed intrahepatic and subgaleal hemorrhages secondary to anticoagulation therapy.After stopping oral anticoagulation,a follow-up computed tomography showed improvement in the hemorrhage.After restarting warfarin because of a recurrent thrombosis,the intrahepatic hemorrhage recurred.We decided to start clopidogrel and hydroxychloroquine instead of warfarin.The patient has not developed further recurrent thrombotic or bleeding episodes.Intrahepatic hemorrhage is a very rare complication of warfarin,and our patient experienced intrahepatic and subgaleal hemorrhage although she did not have any risk factors for bleeding or instability of the international normalized ratio control.

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.

Liver transplantation in a patient with primary antiphospholipid syndrome and Budd-Chiari syndrome

The antiphospholipid syndrome(APS) is an acquired thrombophilic disorder in which autoantibodies are produced to a variety of phospholipids determinants of cell membranes or phospholipid binding proteins. There are few reports about association between antiphospholipid antibodies and development of BuddChiari syndrome(BCS). We report the case of BCS development in young Russian male with primary APS. The patient underwent orthotopic liver transplantation on August 26, 2012. At present time his state is good, the blood flow in the liver restored and its function is not impaired. We report about the first time the successful use of dabigatran etexilate for prolonged anticoagulation therapy in APS patient with BCS. In addition patient is managed with immunosuppressive drugs.

羟氯喹联合阿司匹林及低分子肝素治疗APS型复发性流产的临床疗效观察

目的:观察免疫抑制剂羟氯喹联合阿司匹林及低分子肝素在治疗抗磷脂综合征(APS)患者复发性流产(RSA)的临床疗效。方法:收集2022年1—12月就诊于广西壮族自治区玉林市妇幼保健院RSA门诊的APS型复发流产患者共65例,采用随机数字表法将患者分为治疗组和对照组。对照组30例常规予阿司匹林联合低分子肝素,治疗组35例在对照组治疗基础上加用羟氯喹。对比两组患者治疗前后的血清性激素水平[血清人绒毛膜促性腺激素(β-HCG)、孕酮(P)、雌二醇(E2)]、凝血功能[腺二磷(ADP)、D-二聚体]、子宫动脉指标[搏动指数(PI)、阻力指数(RI)]、血清抗心磷脂抗体(aCL)、抗-β2糖蛋白I抗体(anti-β2GPI Ab)、狼疮抗凝物(LAC)、治疗效果及不良反应的发生情况。结果:在妊娠12周后,治疗组患者血清E2、P和β-HCG水平较对照组升高(P<0.05);治疗组ADP、D-二聚体、子宫动脉PI和RI较对照组下降(P<0.05);治疗组患者aCL、anti-β2GPI Ab及LAC的转阴率高于对照组;治疗组治愈26例,好转8例,总有效率为97.14%;对照组治愈13例,好转11例,总有效率80.00%(P<0.05);治疗组的不良反应发生率较对照组显著降低(P<0.05)。结论:APS型RSA患者在低分子肝素联合阿司匹林治疗的基础上加用羟氯喹可以更有效的减少患者流产的发生率,值得进一步在临床推广应用。

Anticardiolipin Antibody Isotype Determination in Amniotic Fluid of Pregnant Patients with Systemic Lupus Erythematosus and/or Antiphospholipid Syndrome

Objective: To determine the levels and isotypes of aCl, as well as anti beta 2 glycoprotein 1 (antiβ2-GP1) antibodies in serum and amniotic fluid of pregnant patients with SLE and/or APLS, and healthy pregnant women serving as a control group. Material and Methods: We analyzed serum and amniotic fluid of pregnant patients with SLE and/or APLS, and of healthy pregnant women through ELISA. Results were compared using a Student’s T test. Results: 6 of 13 patients (46.1%), 5 with SLE and one with primary APLS had antiphospholipid antibodies in amniotic fluid. Two patients had IgG aCl and 4 patients had antiβ2-GP1 (one of them also showing IgM) in amniotic fluid. In serum, 4 patients (30%) had antiphospholipid antibodies present (one IgG aCl and three anti β2-GP1) as opposed to none in the control group having antiphospholipid antibodies in amniotic fluid. Only one control had IgM aCl in serum. Antiβ2-GP1 in the amniotic fluid of patients showed a statistically significant value when compared to controls. Conclusion: aCl and antiβ2-GP1 may be present in the amniotic fluid of patients with and without a history of fetal loss. The presence of IgM aCl and antiβ2-GP1 in amniotic fluid suggests its localized production.

Antiphospholipid syndrome：a survey of clinical characters in ten cases

Objective: To gain further understanding of the antiphospholipid syndrome(APS) . Methods : Analysing clinical and laboratory data on ten cases of APS. Results: Thrombocytopenia appeared in all cases.Venous thrombi of limbs appeared in five eases and neurological abnormalities in two cases. Renal impairments were found in three cases. One case manifested left renal venous thrombi and the other two cases thrombotic microangiopathy. Budd-Chiari syndrome was found in one case. One of the ten cases was catastrophic APS(CAPS) presented as acute diffuse swelling, cyanosis, pain, ischemia and necrosis in fingers and limbs,recurrem shock, ascites, hepatic and respiratory dysfunction. Anticoagulams and corticosteroids could be effective for dealing with APS. It was critical to treat catastrophic APS with anticoagulants or plasmapheresis as early as possible. Conclusions: APS shows variable manifestations for good prognosis, but catastrophic APS has fatal risk. The main treatment for APS is the use of anticoagulams and immunosuppressives.

The Value and the Variability of Magnetic Resonance Imaging in Patients with Systemic Lupus Erythematosus and Antiphospholipid Syndrome

Objectives: The objectives of this article are twofold: To review the value and the variability of MR imaging (MRI) findings in patients with systemic lupus erythematosus (SLE) and to reveal the frequency of central nervous system (CNS) involvement. SLE is a complex multisystem autoimmune connective tissue disorder with a broad spectrum of clinical presentations. Materials and methods: We present three cases, two with neurological symptoms and one case with musculoskeletal symptoms. Patients were imaged in the MRI department of the University Hospital of Patras. All patients had a previous history of antiphospholipid syndrome and known SLE. Results: None of the pts had a normal brain MRI. Abnormal lesions were typically high on FLAIR and T2-weighted images. One patient showed myositis of the tibial muscles bilateral and although she did not reveal any neurological symptoms, because of the presence of APS, she underwent brain MRI to detect any cerebrovascular involvement. The brain MRI was abnormal and showed extensive lesions and porencephalia. In all cases the differential diagnosis of the brain MRI included vasculitis, focal ischemia, multiple sclerosis or other entities. Parenchymal volume loss-cerebral atrophy, incompatible with their age, was obvious in all patients. Conclusion: Magnetic resonance imaging is the gold standard for the investigation of central nervous system in patients suffering from lupus. Lupus patients who also develop antiphospholipid syndrome must be submitted to brain MRI because central nervous involvement is very common and serious, although symptoms may be silent as in our case.

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.

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.

Recurrent pulmonary embolism in a boy with antiphospholipid syndrome

We report a 14-year-old patient with recurrent pulmonary embolism due to catastrophic antiphospholipid syndrome (APS) with severe pulmonary inflammation. We considered elevated antibodies against cardiolipin and anti-beta2-glycoprotein-1, but no clinical nor laboratory manifestations of systemic lupus erythematosus (SLE). Pulmonary embolism had been the first manifestation of catastrophic APS in this patient. We prescribed warfarin and systemic corticosteroids. A second embolism appeared during anticoagulation with warfarin. This event led to a marked decrease of his physical performance due to his obstructive and restrictive lung disease. Anticoagulation was changed from enteral warfarin to subcutaneous enoxaparine. We also prescribed inhaled corticosteroid which led to an improvement of his respiratory symptoms and overall poor physical condition.

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.

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.

Postpartum spontaneous colonic perforation due to antiphospholipid syndrome

The antiphospholipid syndrome(APS)is a multi-systemic disease being characterized by the presence of antiphospholipid antibodies that involves both arterial and venous systems resulting in arterial or venous thrombosis,fetal loss,thrombocytopenia,leg ulcers,livedo reticularis,chorea,and migraine.We document a previously unreported case of a 37-year-old female in whom APS was first manifested by infarction and cecal perforation following cesarean section.At laparotomy the underlying cause of colonic perforation was not clear and after resection of the affected bowel an ileo-colostomy was performed.The diagnosis of APS was established during post-operative hospital stay and the patient was commenced on warfarin.Eventually,she made a full recovery and had her stoma reversed after 4 mo.Pregnancy poses an increased risk of complications in women with APS and requires a more aggressive approach to the obstetric care.This should include full anticoagulation in the puerperium and frequent doppler ultrasound monitoring of uterine and umbilical arteries to detect complications such as preeclampsia and placental insufficiency.

Thrombotic microangiopathy involving the gallbladder as an unusual manifestation of systemic lupus erythematosus and antiphospholipid syndrome: Case report and review of the literature

Gallbladder disease is no more common in patients with systemic lupus erythematosus (SLE) than in the general population. We describe a 17-year-old patient with SLE, who developed nephritis that was well controlled with medications. Initial treatment consisted of azathioprine, aspirin and prednisone with stable control of her symptoms. Two years later she developed a right quadrant abdominal pain, and an abdominal ultrasound revealed microlithiasic cholecystitis. Open cholecystectomy was performed and the histopathological findings revealed vasculitis with thrombotic microangiopathy in the gallbladder. This case presentation illustrates that calculous or acalculous cholecystitis should be considered as a manifestation of active SLE and APS.

Systemic Lupus Erythematosus and Antiphospholipid Syndrome Related Retinal Vasculitis Mimicking Ocular Cysticercosis: a Case Report

MAKING accurate and timely diagnosis is often challenging when patients with a systemic disease first present with ocular manifestations.The possibility that vasculitis associated with systemic lupus erythematosus(SLE)and antiphospholipid syndrome(APS)can be misdiagnosed as cysticercosis has not been discussed in the literatures.

Antiphospholipid Antibody and Antiphospholipid Syndrome

Antiphospholipid antibodies(APA)APA is a big category for all kinds of negative charge phospholipid or lecithin- a protein complex autoantibodies or the same antibody,through its recognition of antigen(target protein) different,and

Significance of Laboratory and Clinical Manifestations of Antiphospholipid Syndrome

Clinical features of antiphospholipid syndrome Antiphospholipid syndrome(APS) is a form of immune mediated thrombophilia,characterized by thrombosis, recurrent fetal death and the presence of circulating antiphospholipid(aPL) antibodies directed against anionic phospholipids or protein-phospholipid complexes. Almost every vascular bed can be involved by