Antiphospholipid Syndrome Presenting Research Articles

A rare case of antiphospholipid syndrome presenting as primary adrenal insufficiency

A rare case of antiphospholipid syndrome presenting as primary adrenal insufficiency

Vessel wall imaging in the diagnosis of antiphospholipid syndrome presenting as Moyamoya syndrome-A case report.

Objectives: We describe a case of anti-phospholipid syndrome (APLS) vasculopathy presenting with Moyamoya syndrome (MMS) and show the associated intracranial vessel wall MRI (VWI) findings. Methods: A 37-year-old-woman presented with acute onset dizziness and left-sided weakness. Neurologic exam revealed a left facial droop and left hemiparesis. She underwent a comprehensive laboratory work-up for stroke. Neuroimaging included a CT head, CT angiogram, VWI, and digital subtraction angiography. Results: Work-up revealed a triple-positive APLS antibody profile. CT of the head showed an acute right basal ganglia infarction and right frontal subarachnoid hemorrhage. CT angiogram revealed severe stenosis of the right internal carotid artery terminus in a Moyamoya pattern. Intracranial VWI showed long-segment concentric vessel wall thickening and homogeneous vessel wall enhancement and T2-hyperintense wall edema of the stenotic right ICA terminus, M1 middle cerebral artery, and A1 anterior cerebral artery. She was treated with long-term anticoagulation with warfarin and a right superficial temporal artery to middle cerebral artery bypass. Discussion: We present intracranial VWI features of vessel wall pathology in a patient with primary APLS presenting with MMS.

Blinded Epidural Blood Patch for Treating a Man with Antiphospholipid Syndrome and Autoimmune Thrombocytopenia Presenting with Spontaneous Intracranial Hypotension (P10-12.010)

Blinded Epidural Blood Patch for Treating a Man with Antiphospholipid Syndrome and Autoimmune Thrombocytopenia Presenting with Spontaneous Intracranial Hypotension (P10-12.010)

An Unusual Presentation of Primary Anti-phospholipid Antibody Syndrome as Acute Coronary Syndrome in Young – A Case Report

A case report of a 32-year-old female patient without any traditional risk factors presented with angina to our hospital within 6 h. Her electrocardiogram (ECG) showed extensive anterior wall -ST elevation myocardial infarction (MI). She was treated with thrombolytic therapy in the form of tenecteplase and was given standard treatment for acute coronary syndrome (ACS). Her history revealed three recurrent pregnancy losses and a history of preeclampsia. Echocardiography showed regional wall motion abnormalities of the mid, distal, apex, and anterior wall with severe left ventricular systolic dysfunction. Routine blood investigations showed elevated total leukocyte count with neutrophilic predominance. With a history of recurrent pregnancy loss, she was investigated for anti-phospholipid anti-phospholipid antibody syndrome (APS) and was found to have positive lupus anticoagulant 1. She underwent coronary angiography (CAG) which showed a left main shaft 40% stenosis with an ulcerative lesion containing thrombus in the ostioproximal to mid-left anterior descending artery (LAD), other coronary arteries were normal. She was managed conservatively with glycoprotein IIb/IIIa inhibitor (Tirofiban) and switched to oral anticoagulation with Vitamin K antagonist (VKA-Acenocoumarin) along with dual-antiplatelet therapy.

Acute Aortic Occlusion as an Initial Presentation of Antiphospholipid Syndrome.

Acute Aortic Occlusion as an Initial Presentation of Antiphospholipid Syndrome.

Case report: Antiphospholipid syndrome presenting as primary adrenal insufficiency

Case report: Antiphospholipid syndrome presenting as primary adrenal insufficiency

Paralytic Ileus as a Presentation of Antiphospholipid Syndrome in a Patient with Renal Angiomyolipoma: A Case Report

Antiphospholipid syndrome (APS) is one of the many causes of hypercoagulability which often presents with vascular thrombosis in the presence of antiphospholipid antibodies. Symptoms vary depending on the location of the thrombosis. Gastrointestinal symptoms such as paralytic ileus is one of the rare symptoms in APS. The main therapy for APS is use of anticoagulants, but this becomes difficult when there is ongoing bleeding. We report a successful but challenging treatment of a 42-year-old patient who presented with paralytic ileus due to APS with cofounded on-going renal bleeding caused by renal angiomyolipoma.

Acute limb ischemia as presentation of pediatric antiphospholipid syndrome: case report

Acute limb ischemia as presentation of pediatric antiphospholipid syndrome: case report

Special presentation of antiphospholipid syndrome

Antiphospholipid antibody syndrome (APAS) is a condition of acquired thrombophilia due to autoantibodies directed against membrane phospholipids and/or their cofactors. It may be primary or part of a systemic autoimmune disease, such as systemic lupus erythematosus (SLE). Dermatological lesions during APS are frequent, although non-specific, sometimes inaugural, and may be the only clinical manifestation. However, extensive cutaneous necrosis is rare and treatment is based on anticoagulants and appropriate local care. Herein, we report a case of multiple extensive cutaneous necroses in a female with SLE. The particularity of our case is the presence of two types of lesions, necrotizing plaques surmounted by hemorrhagic bullae surrounded by a purpuric border specific to antiphospholipid syndrome and ecchymotic plaques evolving according to the color of the biligenesis, which may be consistent with coagulopathy, in particular, a protein C or S deficiency, hence the interest in good knowledge and semiological analysis. Key words: Anti-phospholipid antibody syndrome; Extensive skin necrosis; Systemic lupus erythematosus

S3428 Catastrophic Antiphospholipid Syndrome Presenting as Hepatic Injury, Pancreatitis, and Rash

S3428 Catastrophic Antiphospholipid Syndrome Presenting as Hepatic Injury, Pancreatitis, and Rash

Cutaneous microvascular occlusion syndrome as the first manifestation of catastrophic lupus-associated antiphospholipid antibody syndrome: a case report

BackgroundAntiphospholipid syndrome (APS), defined by thrombotic events or obstetric complications in the presence of persistently high antiphospholipid antibodies, is characterized by a wide variety of clinical presentations and the effects of vascular occlusion can impact almost any organ system or tissue. Since adult-onset APS classification criteria are not well verified in pediatrics (where pregnancy-related problems are rare), estimating childhood prevalence is challenging. Stroke and pulmonary embolism are thromboembolic events occurring in children that can cause considerable long-term morbidity. Children with APS are more prone to recurrent thromboembolism than adults. Cutaneous symptoms are prominent and typically represent the first clue of APS. Although dermatologic findings are exceedingly heterogeneous, it is essential to consider which dermatological symptoms justify the investigation of antiphospholipid syndrome and the required further management.Case presentationWe describe a seven-year-old Iranian boy with retiform purpura and acral cutaneous ischemic lesions as the first clinical presentation of antiphospholipid syndrome in the setting of systemic lupus erythematous.ConclusionAPS in pediatrics, is associated with a variety of neurologic, dermatologic, and hematologic symptoms. Therefore, it is essential for pediatricians to be aware of the rare appearance of Catastrophic APS as an initial indication of APS.

Primary Antiphospholipid Syndrome Presenting with Cerebellar Stroke and Positive Titres of Anti-β2 Glycoprotein-1 Antibody Only: A Case Report (P9-5.026)

<h3>Objective:</h3> To describe a case of Primary Antiphospholipid Syndrome presenting with Cerebellar Stroke and positive titres of anti-β2 glycoprotein-1 antibody only. <h3>Background:</h3> Antiphospholipid syndrome (APS) is an autoimmune disease where the presence of antiphospholipid antibodies leads to the development of arterial and/or venous thromboses and obstetric complications. Amongst the myriad of presentations, APS can manifest in the form of cerebrovascular stroke. However, overlapping clinico-radiological findings and a lack of classic APS features can sometimes pose a diagnostic dilemma. <h3>Design/Methods:</h3> NA <h3>Results:</h3> A 31-year-old female presented with occipital headache and difficulty in walking. Neurological examination revealed ataxia, dysdiadochokinesia and intention tremor. Preliminary laboratory findings revealed raised ESR along with normal CRP, aPTT and PT levels. MRI brain showed an acute infarct in the right cerebellar hemisphere and right postero-lateral upper medulla. MRA Head &amp; Neck revealed an occluded intracranial right vertebral artery with an extracranial segment showing multifocal occlusions with aorta and its major branches unaffected. These features and the absence of fever, rash, joint pain or obstetric complications led to the consideration of stroke due to Takayasu Arteritis. Treatment was initiated with corticosteroids, anticoagulants and antiplatelet drugs. However, on review of other laboratory tests, notably the auto-immune profile showed the presence of anti-ß2 glycoprotein-I antibodies with negligible titres of lupus anticoagulant and anti-cardiolipin antibody. This pointed towards a diagnosis of Primary Antiphospholipid Syndrome, which was confirmed after 12 weeks when repeat laboratory tests indicated the presence of IgM anti-ß2 glycoprotein-I antibodies. The patient is now on lifelong Warfarin therapy and is leading a symptom-free life. <h3>Conclusions:</h3> This case illustrates the potential of anti-β2 glycoprotein-1 antibody to cause a cerebellar stroke in the absence of other commonly involved antibodies. It also highlights the value of a holistic stroke workup. Recognition of APS is imperative for the timely administration of treatment and to prevent a recurrence. <b>Disclosure:</b> Mr. Shah has nothing to disclose. Mr. Shah has nothing to disclose. Dr. Nagrani has nothing to disclose.

Abdominal pain secondary to bilateral adrenal haemorrhage as the first presentation of antiphospholipid syndrome

Medical Journal of AustraliaEarly View Medical education Abdominal pain secondary to bilateral adrenal haemorrhage as the first presentation of antiphospholipid syndrome Eugene Wang, Eugene Wang orcid.org/0000-0003-0268-447X Monash University, Melbourne, VIC Walter and Eliza Hall Institute of Medical Research, Melbourne, VICSearch for more papers by this authorRithin Nedumannil, Corresponding Author Rithin Nedumannil rithin.nedumannil@petermac.org Peter MacCallum Cancer Centre, Melbourne, VIC Cabrini Hospital, Melbourne, VICrithin.nedumannil@petermac.orgSearch for more papers by this authorMax Wolf, Max Wolf Peter MacCallum Cancer Centre, Melbourne, VIC Cabrini Hospital, Melbourne, VICSearch for more papers by this author Eugene Wang, Eugene Wang orcid.org/0000-0003-0268-447X Monash University, Melbourne, VIC Walter and Eliza Hall Institute of Medical Research, Melbourne, VICSearch for more papers by this authorRithin Nedumannil, Corresponding Author Rithin Nedumannil rithin.nedumannil@petermac.org Peter MacCallum Cancer Centre, Melbourne, VIC Cabrini Hospital, Melbourne, VICrithin.nedumannil@petermac.orgSearch for more papers by this authorMax Wolf, Max Wolf Peter MacCallum Cancer Centre, Melbourne, VIC Cabrini Hospital, Melbourne, VICSearch for more papers by this author First published: 07 March 2023 https://doi.org/10.5694/mja2.51874Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Early ViewOnline Version of Record before inclusion in an issue RelatedInformation

Painful unilateral facial swelling due to superficial temporal artery thrombosis: a rare presentation of Antiphospholipid Syndrome

Introduction Persistent painful unilateral temporal swelling is rarely seen in clinical practice. Antiphospholipid syndrome (APS) is an autoimmune and systemic disorder that causes changes in blood clotting homeostasis, marked by arterial or venous thrombosis, gestational morbidity, and high and persistently positive serum levels of antiphospholipid antibodies (aPL). APS is more common in young women and middle-aged adults, with no preference for race. Among its clinical features, temporal artery thrombosis, associated with headache and temporal and hemifacial edema is extremely rare, with few publications worldwide on this topic. Objective This report aims to present the case of a unilateral painful facial edema due to thrombosis of the superficial temporal artery as an unprecedented manifestation of Antiphospholipid Syndrome. Thus, the only record in the English-language literature surveyed in the Pubmed database in July 2022 highlights the rarity of this APS presentation and the consequent challenge in suspecting the correct diagnosis for adequate treatment. Methods Case report. Data disclosure was authorized by the patient through an informed consent form. Results A 32-year-old woman was presented with pain in the right temporal region of her face. The pain was intense, daily and continuous, pulsating, without irradiation, which worsened with physical activity and presented partial relief with common analgesics. After 20 days, she developed a right pale temporal edema (Figure 1) associated with a significant worsening of pain and intense right unilateral headache attacks triggered by chewing and speaking. She has a history of deep vein thrombosis in the left lower limb. Physical examination and imaging tests showed significant cold edema of the right temporal region with asymmetry of the temporal muscles, which was extremely painful on palpation and made it difficult to open the mouth. There were no other changes in the general physical... (Too see the complete abstract, please, check out the PDF.)

Concomitant Pulmonary Embolism and Anterior Myocardial Infarction as the Initial Presentation of Antiphospholipid Syndrome

Antiphospholipid antibody syndrome (APLS) is a systematic autoimmune disease characterized by thrombotic events, including arterial, venous, and microvascular complications. Often, patients initially present with either arterial or venous thromboembolism but rarely present simultaneously with arterial and venous thromboembolic events.1Garcia D. Erkan D. Diagnosis and management of the antiphospholipid syndrome.N Engl J Med. 2018; 378: 2010-2021https://doi.org/10.1056/NEJMra1705454Crossref PubMed Scopus (321) Google Scholar We report an unusual case of a 21-year-old woman who presented to our hospital with chest pressure secondary to a concomitant pulmonary embolism (PE) and anterior myocardial infarction. A 21-year-old woman with no significant past medical history, only on oral contraceptive pills, presented to the hospital with complaints of 4 days of progressively worsening substernal chest pressure associated with dizziness and lightheadedness. In the emergency department, she was mildly tachycardic but otherwise hemodynamically stable. An electrocardiogram revealed sinus rhythm with anterior T-wave abnormality (Figure 1A). Initial laboratory testing revealed mild thrombocytopenia and elevated troponin I levels of 9.63 ng/mL. A computed tomography angiography of the chest revealed small nonocclusive emboli in the right lower lobe pulmonary artery (Figure 1B); therefore, venous duplex ultrasound of the lower extremities was performed, which showed no evidence of deep vein thrombosis. Anticoagulation with intravenous heparin was started, and the patient was admitted to the floor with telemetry monitoring. Early morning on day 2 of hospitalization, the patient reported worsening chest pain, and the troponin I levels subsequently trended up to 20.08 ng/mL. A repeat electrocardiogram showed subtle ST-segment elevations, with QS complexes in the anterior leads. A bedside transthoracic echocardiogram revealed hypokinesis of the anterior wall and a severely reduced left ventricular ejection fraction. The patient was transferred to the cardiac catheterization laboratory for emergency coronary angiography. Coronary angiography showed 100% occlusion of the left anterior descending artery (LAD) at the ostium. The right coronary artery was patent with extensive right to left collaterals supplying the LAD. A filling defect was noted in the proximal LAD, consistent with a thrombus. Thrombus aspiration was performed using the Indigo System CAT RX (Penumbra, Inc), followed by the placement of a single 3.0 × 18 mm XIENCE Skypoint (Abbott) drug-eluting stent in the proximal LAD, with resultant TIMI-3 flow. A formal transthoracic echocardiogram demonstrated a left ventricular ejection fraction of 35%-40%, with a large left ventricular thrombus. Subsequent testing revealed antibody positivity for APLS, with elevated titers of anticardiolipin, lupus anticoagulant, and beta-2-glycoprotein antibodies. The patient was discharged on aspirin, ticagrelor, and warfarin along with high-intensity statin therapy, a low-dose angiotensin-converting enzyme inhibitor, and β-blocker therapy. The patient’s oral contraceptive pills were discontinued, and the patient was counseled about the use of an alternative nonhormonal mode of contraception. Approximately 12 weeks after discharge, the patient’s outpatient laboratory reports showed persistent elevation of all 3 antibodies, thus establishing the diagnosis of triple-positive APLS. Massive or submassive PEs can cause acute right heart strain and result in a small elevation of troponin levels. However, a high initial troponin level in patients with a nonmassive PE should raise suspicion of acute coronary syndrome, even with a low pretest probability of acute coronary syndrome.2Meyer T. Binder L. Hruska N. Luthe H. Buchwald A.B. Cardiac troponin I elevation in acute pulmonary embolism is associated with right ventricular dysfunction.J Am Coll Cardiol. 2000; 36: 1632-1636https://doi.org/10.1016/S0735-1097(00)00905-0Crossref PubMed Scopus (277) Google Scholar,3Kilinc G. Dogan O.T. Berk S. Epozturk K. Ozsahin S.L. Akkurt I. Significance of serum cardiac troponin I levels in pulmonary embolism.J Thorac Dis. 2012; 4: 588-593https://doi.org/10.3978/j.issn.2072-1439.2012.10.13Crossref PubMed Scopus (15) Google Scholar Therefore, the detection of a high initial troponin level should be followed by serial troponin level testing and electrocardiograms, an echocardiogram, and an ischemic evaluation. Patients with APLS may present with complications of arterial or venous thromboembolism; however, they rarely present with both simultaneously. Myocardial infarction and PE associated with APLS have been extensively reported in the literature; however, there are limited reports regarding the incidence of simultaneous events as the initial presentation of APLS.4Nazir S. Tachamo N. Lohani S. Hingorani R. Poudel D.R. Donato A. Acute myocardial infarction and antiphospholipid antibody syndrome: a systematic review.Coron Artery Dis. 2017; 28: 332-335https://doi.org/10.1097/MCA.0000000000000476Crossref PubMed Scopus (32) Google Scholar A high index of suspicion should be maintained for APLS in young patients presenting with concomitant arterial and venous thromboembolic events because the timely diagnosis and initiation of anticoagulation can prevent further thromboembolic complications. Further research is required regarding optimal anticoagulation strategies after percutaneous coronary interventions in patients with hypercoagulation disorders. The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Antiphospholipid antibody syndrome presenting as foot gangrene after bilateral total knee arthroplasty: A case report

Case: A 71 years old female who underwent bilateral total knee replacement, noted discolouration of toes and plantar aspect of right foot, along with cold right foot, 1 week after surgery due to thrombosis of dorsalis pedis artery in spite of adequate anticoagulation. She was eventually diagnosed with Anti phospholipid antibody (APLA) syndrome. Conclusion: APLA is not routinely investigated in preoperative workup for arthroplasty. It is also not a common condition in orthopaedic practice. Some patients may develop thrombosis after arthroplasty in spite of adequate thromboprophylaxis and a rare cause like APLA has to be kept in mind to help in ensuring appropriate treatment.

Primary Anti-Phospholipid Antibody Syndrome presenting as Multiple Cerebral Venous Sinus Thrombosis: An Arduous Manifestation

Lupus anticoagulants (LA) are circulating autoantibodies, primarily directed against phospholipids, that prolong the partial thromboplastin time. Antiphospholipid antibody syndrome (APS) is an autoimmune disorder, mainly found in young females, presenting with vascular thrombosis and/obstetric complications. Thrombosis at anatomically significant sites may lead to considerable morbidity and/or mortality. We here present a 22 years old lady with no prior rheumatological history, presented with sudden onset headache, vomiting, diplopia who later diagnosed with multiple cerebral venous sinus thrombosis (CVST) due to with primaryAPS.MR venography was instrumental in diagnosis. Except for mild headache, the other symptoms responded to anticoagulant. Such massive cerebral venous thrombosis is extremely rare in primary APS.

Primary Anti-Phospholipid Antibody Syndrome presenting as Multiple Cerebral Venous Sinus Thrombosis: An Arduous Manifestation

Lupus anticoagulants (LA) are circulating autoantibodies, primarily directed against phospholipids, that prolong the partial thromboplastin time. Antiphospholipid antibody syndrome (APS) is an autoimmune disorder, mainly found in young females, presenting with vascular thrombosis and/obstetric complications. Thrombosis at anatomically significant sites may lead to considerable morbidity and/or mortality. We here present a 22 years old lady with no prior rheumatological history, presented with sudden onset headache, vomiting, diplopia who later diagnosed with multiple cerebral venous sinus thrombosis (CVST) due to with primaryAPS.MR venography was instrumental in diagnosis. Except for mild headache, the other symptoms responded to anticoagulant. Such massive cerebral venous thrombosis is extremely rare in primary APS.

A case of completed course multifocal osteonecrosis (MFON) during pregnancy due to primary antiphospholipid syndrome

Osteonecrosis of both shoulders and hips is a rare presentation of primary antiphospholipid syndrome. A female patient aged 23 years old with no systemic diseases has her only complaint which was pain and limitations in both hips followed by both shoulders. Careful detailed history and clinical examination is essential for reaching optimum diagnosis thus good management. MRI for hip and shoulder joints is essential for the diagnosis of osteonecrosis. Exclusion of all causes of secondary osteonecrosis by history, clinical examination, and laboratory studies should be done before diagnosing the rare causes of osteonecrosis or the primary type. Pregnancy is an exacerbation factor for primary osteonecrosis. We recommend that the female patient with primary osteonecrosis should receive prophylactic antithrombotic during pregnancy. In conclusion, exclusion of all secondary causes of osteonecrosis is mandatory to reach an accurate diagnosis.

Antiphospholipid antibody syndrome presenting as pyoderma gangrenosum

Antiphospholipid antibody syndrome presenting as pyoderma gangrenosum