Antiphospholipid syndrome presenting as recurrent coronary thrombosis:A case report

BACKGROUND Antiphospholipid syndrome(APS)is a chronic autoimmune disease characterized by venous or arterial thrombosis,pregnancy morbidity and a variety of other autoimmune and inflammatory complications.Here,we report a case of APS associated with multiple coronary thromboses.CASE SUMMARY The patient,a 28-year-old male,suffered from recurrent coronary thromboses over a period of 31 months.Despite undergoing interventional coronary procedures,thrombolytic therapy,and anticoagulation treatment,the condition persisted intermittently.An extensive search for underlying thrombogenic factors revealed a diagnosis of APS.Accurate adjustment of the medication regimen led to the absence of further acute coronary syndrome(ACS)episodes during the subsequent 20-month follow-up.Although the patient occasionally experiences chest tightness,no further symptoms of distress have been reported.CONCLUSION APS can manifest as ACS.Screening for rheumatologic and immunological conditions is essential when encountering patients with multiple coronary thromboses.Treatment strategy should include symptomatic relief and a targeted and aggressive approach to address the underlying pathophysiology.

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：a survey of clinical characters in ten cases

Objective: To gain further understanding of the antiphospholipid syndr ome(APS). Methods: Analysing clinical and laboratory data on ten cases of APS. R esults: Thrombocytopenia appeared in all cases. Venous thrombi of limbs appeared in five cases and neurological abnormalities in two cases. Renal impairments we re found in three cases. One case manifested left renal venous thrombi and the o ther two cases thrombotic microangiopathy. Budd-Chiari syndrome was found in on e case. One of the ten cases was catastrophic APS(CAPS) presented as acute diffus e swel ling,cyanosis,pain,ischemia and necrosis in fingers and limbs,recurrent shoc k,ascites,hepatic and respiratory dysfunction. Anticoagulants and corticosteroids could be effective for dealing with APS. It was critical to trea t catastrophic APS with anticoagulants or plasmapheresis as early as possible. C onclusions: APS shows variable manifestations for good prognosis,but catastroph ic APS has fatal risk. The main treatment for APS is the use of anticoagulants a nd immunosuppressives.

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.

Prevalence and clinical significance of antiphospholipid antibodies among hospitalized COVID-19 patients

Objective:To describe the prevalence of antiphospholipid antibodies in coronavirus disease-19(COVID-19)and to find potential associations between antiphospholipid antibody positivity and clinical outcomes.Methods:From September to November 2020,clinical and laboratory data were collected from 50 COVID-19 patients hospitalized at Saiful Anwar General Hospital in Malang,Indonesia.Antiphospholipid antibodies were measured by finding Ig M anti-β2 glycoprotein,lupus anticoagulant,and Ig M/Ig G anticardiolipin.Clinical characteristics,thrombotic events,ICU admission,and mortality during hospitalization were recorded.Disease severity was defined by the Guidelines for the Prevention and Control of COVID-19,Indonesia.Results:Among 50 patients,5 patients(10.0%)were positive for antiphospholipid antibodies:4 patients(80.0%)had Ig M anti-β2 glycoprotein and 1 patient had Ig G anti-cardiolipin(20.0%)and Ig M anti-cardiolipin(20.0%),none of lupus anticoagulant was detected.Antiphospholipid antibodies were associated with anosmia(OR 8.1;95%CI 1.1-57.9;P=0.018),nausea and vomiting(OR 12.4;95%CI 1.2-122.6;P=0.010),diarrhea(OR 9.8;95%CI 1.3-70.9;P=0.010),cardiovascular disease(OR 1.4;95%CI 1.0-1.9;P=0.001),chronic kidney disease(OR 12.0;95%CI 1.6-90.1;P=0.05),acute coronary syndrome(OR 29.3;95%CI 2.0-423.7;P=0.001),moderate(OR 0.11;95%CI 0.01-1.10;P=0.031)and severe(OR 18.5;95%CI 1.8-188.4;P=0.002)disease severity,and in-hospital mortality(OR 8.1;95%CI 1.1-57.9;P=0.018).However,there is no correlation between the presence of antiphospholipid antibody and ICU admission.Conclusions:In summary,the prevalence of antiphospholipid antibodies in COVID-19 patients is low,mainly against Ig M anticardiolipin,and is associated with an acute coronary syndrome,gastrointestinal manifestations,moderate and severe disease severity,and increased risk of mortality.

Bilateral Choroidal Occlusion in Antiphospholipid Syndrome Associated with Systemic Lupus Erythematosus

This article reports a rare case of bilateral choroidal occlusion that occurred in a 24-year-old woman with antiphospholipid syndrome (APS) associated with systemic lupus erythematosus (SLE). This young lady concurred with aorta ventralis thrombosis and bilateral iliac artery occlusion when presented, and experienced a rapid deterioration of vision. She also has a history of recurrent miscarriage. Corticosteroid,immunosuppression and anticoagulation therapy were administered. Patients with APS associated with SLE are at risk for thrombotic phenomena, which may affect the ocular vessels of all sizes, including choroidal vessel.Our case alerts ophthalmologists and rheumatologists that bilateral choroidal occlusion may indeed be developed in patients with APS associated with SLE, and is a potential cause of visual morbidity.

Prevalence, significance and predictive value of antiphospholipid antibodies in Crohn's disease

AIM: To assess the prevalence and stability of different antiphospholipid antibodies(APLAs) and their association with disease phenotype and progression in inflammatory bowel diseases(IBD) patients.METHODS: About 458 consecutive patients [Crohn's disease(CD): 271 and ulcerative colitis(UC): 187] were enrolled into a follow-up cohort study in a tertiary IBD referral center in Hungary. Detailed clinical phenotypes were determined at enrollment by reviewing the patients' medical charts. Disease activity, medical treatment and data about evolvement of complications or surgical interventions were determined prospectively during the follow-up. Disease course(development f complicated disease phenotype and need for surgery),occurrence of thrombotic events, actual state of diseaseactivity according to clinical, laboratory and endoscopic scores and accurate treatment regime were recorded during the follow-up,(median, 57.4 and 61.6 mo for CD and UC). Sera of IBD patients and 103 healthy controls(HC) were tested on individual anti-β2-Glycoprotein-I(anti-β2-GPI IgA/M/G), anti-cardiolipin(ACA IgA/M/G)and anti-phosphatidylserine/prothrombin(anti-PS/PT IgA/M/G) antibodies and also anti-Saccharomyces cerevisiae antibodies(ASCA IgA/G) by enzyme-linked immunosorbent assay(ELISA). In a subgroup of CD(n = 198) and UC patients(n = 103), obtaining consecutive samples over various arbitrary timepoints during the disease course, we evaluated the intraindividual stability of the APLA status. Additionally,we provide an overview of studies, performed so far, in which significance of APLAs in IBD were assessed.RESULTS: Patients with CD had significantly higher prevalence of both ACA(23.4%) and anti-PS/PT(20.4%) antibodies than UC(4.8%, p < 0.0001 and10.2%, p = 0.004) and HC(2.9%, p < 0.0001 and15.5%, p = NS). No difference was found for the prevalence of anti-β2-GPI between different groups(7.2%-9.7%). In CD, no association was found between APLA and ASCA status of the patients.Occurrence of anti-β2-GPI, ACA and anti-PS/PT was not different between the group of patients with active vs inactive disease state according to appropriate clinical, laboratory and endoscopic scores in CD as well as in UC patients. All subtypes of anti-β2-GPI and ACA IgM status were found to be very stable over time, in contrast ACA IgG and even more ACA IgA status showed significant intraindividual changes.Changes in antibody status were more remarkable in CD than UC(ACA IgA: 49.9% vs 23.3% and ACA IgG:21.2% vs 5.8%). Interestingly, 59.1% and 30.1% of CD patients who received anti-TNF therapy showed significant negative to positive changes in ACA IgA and IgG antibody status respectively. APLA status was not associated with the clinical phenotype at diagnosis or during follow-up, medical therapy, or thrombotic events and it was not associated with the probability of developing complicated disease phenotype or surgery in a Kaplan-Meier analysis.CONCLUSION: The present study demonstrated enhanced formation of APLAs in CD patients. However,presence of different APLAs were not associated with the clinical phenotype or disease course.

THE STUDY OF PRODUCTION AND MECHANISM OF ANTIPHOSPHOLIPID ANTIBODIES IN PATIENTS WITH CORONARY HEART DISEASE

Objective To assess whether there was strong association between antiphospholipid antibodies and coronary heart disease, to study the environmental factors of APA production and APA pathogenic mechanism in patients with CHD.Methods Blood samples from 76 patients with CHD and 30 controls were tested for anticardiolipin antibodies IgG,human cytomegalovirus IgG,IgM by enzyme link immunosorbant assay and 6 keto PGF 1a ,endothelin by radioimmunoassay.Results A total of 27 patients were ACA positive in 76, as compared to 2 of 30 healthy individuals, P <0.05. There was no difference in ACA among acute myocardial infarction, old myocardial infarction, unstable angina pectoris, P >0.05. The number of ACA positive subjects was higher in HCMV infection patients with CHD than no HCMV infectious patients with CHD. There was no PGI 2 and ET level difference between ACA IgG positive and negative CHD.Conclusion There are strong association between APA and CHD. The HCMV infection may be an environmental factor of APA production in CHD patients with raised ACA. The alteration of PGI 2 and ET are not the pathogenic mechanism of ACA in patients with CHD.

Segmental small bowel necrosis associated with antiphospholipid syndrome:A case report

Antiphospholipid syndrome is a multi-system disease characterized by the formation of thromboembolic complications and/or pregnancy morbidity, and with persistently increased titers of antiphospholipid antibodies. We report the case of a 50-year-old, previously healthy man who presented with fever and new-onset, dull abdominal pain. A contrast-enhanced computed tomography scan showed segmental small bowel obstruction, for which an emergency laparotomy was performed. Histopathologic examination of resected tissues revealed multiple intestinal and mesenteric thromboses of small vessels. Laboratory tests for serum antiphospholipid(anticardiolipin Ig M) and anti-β2-glycoprotein I antibodies were positive. Despite proactive implementation of anticoagulation, steroid, and antibiotic therapies, the patient's condition rapidly deteriorated, and he died 22 d after admission. This case highlights that antiphospholipid syndrome should be suspected in patients with unexplainable ischemic bowel and intestinal necrosis presenting with insidious clinical features that may be secondary to the disease, as early diagnosis is critical to implement timely treatments in order to ameliorate the disease course.

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.

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.

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 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.

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.

Pathogenetic mechanisms of antiphospholipid antibody production in antiphospholipid syndrome

Antiphospholiipid syndrome(APS) is an autoimmune disease characterized by the pathological action of antiphospholipid antibodies(a PL),that leads to recurrent pregnancy loss and thrombosis.Despite limited evidence,it is clear that there are both inherited and acquired components of the ontogeny of these antibodies.Animal genetic studies and human familial and population studies highlight the influence of genetic factors in APS,particularly human leukocyte antigen associations.Similarly,both animal and human studies have reported the importance of acquired factors in APS development and infectious agents in particular have a great impact on a PL production.Bacterial and viral agents have been implicated in the induction of autoimmune responses by various mechanisms including molecular mimicry,cryptic autoantigens exposure and apoptosis.In this review we highlight the latest updates with regards to inherited and acquired factors leading to the manufacturing of pathogenic antibodies and APS.