Co-occurrence of Leriche syndrome and antiphospholipid syndrome in a man with refractory ulcers of the lower limbs

Antiphospholipid syndrome (APS) is an autoimmune disorder with characteristics of arterial and/or venous thrombosis due to hypercoagulation status. Although deep vein thrombosis is common, the involvement of arterial thrombosis is more dangerous and poses a high risk of complications. Acute aorto-iliac occlusive disease (AIOD, known as Leriche syndrome) is severe arterial thrombosis that is associated with high morbidity and mortality rates. Severe acute occlusion may cause spinal cord ischemia, leading to neurological defects, such as acute onset of paraplegia. Co-occurrence of acute aorto-iliac occlusive disease and antiphospholipid syndrome is rare and may present with atypical symptoms mimicking other diseases, including chronic ulcers, musculoskeletal events, and pulmonary diseases. In patients with weak femoral pulses and recurrent thrombotic events, co-occurrence of APS and AIOD should be taken into consideration. Here, we describe a rare case of co-occurrence of APS and AIOD presenting with acute lower leg weakness and numbness. Timely thrombectomies and bilateral common iliac artery stentings rescued distal blood flow. We highlight the clinical features and early diagnosis of co-occurrence of APS and AIOD in order to prevent catastrophic complications. The detailed mechanism and pathogenesis of antiphospholipid syndrome-induced acute aorto-iliac occlusive disease are also discussed.

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Lethal outcome of catastrophic antiphospholipid syndrome despite intensive triple therapy

Case Reports in Internal Medicine ◽

10.5430/crim.v6n1p13 ◽

2019 ◽

Vol 6 (1) ◽

pp. 13

Author(s):

Carlos Anjo ◽

Philip Fortuna ◽

Luís Reis ◽

Luís Bento

Keyword(s):

Mortality Rate ◽

Triple Therapy ◽

Antiphospholipid Syndrome ◽

Rare Complication ◽

Multiorgan Failure ◽

Lower Limbs ◽

Rapid Progression ◽

Muscular Tissue ◽

Lethal Outcome ◽

Catastrophic Antiphospholipid Syndrome

Catastrophic antiphospholipid syndrome (CAPS), an infrequent autoimmune disease, manifests as a widespread thrombotic disease with multiorgan failure with a 37% mortality rate. We report 39-year-old Caucasian women, medical history of Systemic Lupus Erythematous (SLE) complicated with APS and proliferative glomerulonephritis under dialysis. Patient was admitted at the ICU with septic shock with thoracotomy dehiscence origin. During the ICU stay, extensive lower limbs ulcers with polymorphonuclear infiltrate and steatonecrosis areas in adipose and muscular tissue. Lab studies: anti-β2-glycoprotein-Iantibody was detected. As probable CAPS diagnosis was made, triple therapy with methylprednisolone, immunoglobulin and anticoagulation immediately was started. Serious vasoplegic shock occurs with failure in vasopressor support increment and the patient died. The authors report an APS with rapid progression to catastrophic stage, rare complication that occurs only in 1% of catastrophic APS. In CAPS treatment, a statistical association between triple therapy and the decrease of mortality rate of patients with CAPS was observed, when compared with other combinations or with none of the treatments included in the triple therapy. Although the case had a lethal outcome, the authors emphasize the use of triple therapy in CAPS and the challenge of treating patients with CAPS and infection where the balance between the need for anticoagulation, aggressive immunosuppression and infection control is difficult to manage.

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WCC 2016-112: A case of varied presentation of thrombus

Indian Journal of Cardiovascular Disease in Women WINCARS ◽

10.1055/s-0038-1656419 ◽

2016 ◽

Vol 01 (S 01) ◽

pp. S13-S14

Author(s):

Ravi Kiran

Keyword(s):

Antiphospholipid Syndrome ◽

Pulmonary Thromboembolism ◽

Lower Limbs ◽

Histologic Examination ◽

Right Atrial ◽

Lv Function ◽

Shortness Of Breath ◽

Atrial Mass ◽

Right Atrial Mass ◽

History Of

AbstractAntiphospholipid syndrome is a well defined entity that is characterized by spontaneous abortion, thrombocytopenia, and recurrent arterial and venous thromboses. A right atrial thrombus mimicking myxoma with pulmonary thromboembolism in a patient with secondary antiphospholipid syndrome is rare.Herein, we describe the case of a 35 year old woman who was admitted to the hospital with shortness of breath, swelling of both lower limbs. Transthoracic and transesophageal echocardiography showed a right atrial mass suggestive of myxoma, and the patient subsequently underwent surgery. A histologic examination of the mass showed myxoma. In our patient, the preoperative investigations could not differentiate the thrombus from a myxoma, and she was operated in outside hospital. Later she presented with deep vein thrombosis and pulmonary thromboembolism to our hospital and was diagnosed as having secondary antiphospolipid syndrome and the review sides of the right atrial mass showed thrombus. Intracardiac thrombus has been rarely reported as a compication of antiphospholipid syndrome. Case report: In December 2015, a 35 year old woman was admitted to the hospital with swelling of both lower limbs since 15 days, shortness of breath since 15 days. She had a past history of acute left upper limb ischemia in 15/4/2013 for which catheter directed thrombolysis of brachial artery was done with steptokinase, coronary angiogram showed normal coronaries. History of cervical TB lymphadenitis for which she used ATT for 9 months in 2013. History of excision of right atrial myxoma was done on 3/11/15 at outside hospital. Histologic examination showed myxoma. Patient has pallor and bilateral pedal odema on general examination. At admission vitals were stable and systemic examination was normal except for raised jugular venous pressure. ECG showed T inversions in leads V 3-6, II, III, aVF. 2D ECHO showed dilated RA/RV severe TR, moderate PAH, RVSP 50 mm of Hg, TAPSE 1.3, good LV function, mild RV dysfunction, mobile thrombus at the junction of middle hepatic vein & IVC. CTPA showed saddle thrombus in PA extending into RPA, multiple collaterals in anterior & posterior chest wall with non visualisation of part of left subclavian vein.

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Leriche Syndrome as a Long-Term Continuum of Vascular Injury: from the Initial Clinical Manifestations to Possible Fatal Outcome (Significance of Interdisciplinary Cooperation Based on Own Clinical Observations)

Kardiologiia vid nauky do praktyky ◽

10.30702/card:sp.2020.10.039/043108125 ◽

2020 ◽

pp. 108-124

Author(s):

V Rudichenko ◽

A Kushneryk ◽

V Reizin

Keyword(s):

Diabetes Mellitus ◽

Risk Factors ◽

Erectile Dysfunction ◽

Strong Predictor ◽

Psychiatric Morbidity ◽

Fatal Outcome ◽

Clinical Manifestations ◽

Lower Limbs ◽

Clinical Observations ◽

Leriche Syndrome

Leriche syndrome typical signs include incapability for erection maintaining, fatigue feeling originating from both lower limbs, bilateral claudication with ischemic pain and lack or reduction of peripheral pulse (starting from femoral segment) combined with paleness or coldness of both lower limbs. The disease commonly affects men, and risk factors include hypertension, diabetes mellitus, hyperlipidemia and smoking. Currently the disorder is referred to type D aortoiliac injuries according to Trans-Atlantic Inter-Society Consensus for the Management of Peripheral Arterial Disease (TASC II). Data on psychiatric morbidity in Leriche syndrome is scarce. Some publications are dedicated in such a state to depressive disorder and erectile dysfunction, which were observed in psychiatric outpatient department. These may have several reasons, such as obesity, hypertension, diabetes mellitus, hypercholesterolemia and lower urinary tract symptoms. Moreover, erectile dysfunction is believed to be a strong predictor of general and coronary atherosclerosis. Leriche syndrome and penis arteries obstructive disease are considered to be two main reasons of impotence. Other reasons of Leriche syndrome may be lifestyle factors which are common with atherosclerosis: insufficient physical exercises, imbalanced diet and smoking. Statistics on psychiatric morbidity in Leriche syndrome is hard to receive because of multiple risk factors, partially because of atherosclerosis, which is the risk for vascular depression. The article represents historical data about prominent doctors in the history of vascular surgery who touched upon the problems of Leriche syndrome. The authors describe their own clinical observations of acute patient with prolonged development of full clinical manifestation with fatal outcome.

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Spontaneous regression of severe aortic stenosis after massive embolization in a patient with antiphospholipid syndrome

Seminars in Cardiovascular Medicine ◽

10.2478/semcard-2019-0002 ◽

2019 ◽

Vol 25 (1) ◽

pp. 4-8

Author(s):

Ali Youssef ◽

Mustafa Alrefae ◽

Sayed Abouelsoud

Keyword(s):

Aortic Valve ◽

Aortic Stenosis ◽

Antiphospholipid Syndrome ◽

Severe Aortic Stenosis ◽

Valve Surgery ◽

Lower Limbs ◽

Acute Ischemia ◽

Primary Antiphospholipid Syndrome ◽

Aortic Valve Surgery ◽

History Of

Summary We present a case of probably unreported aortic stenosis evolution in a patient with primary antiphospholipid syndrome (APS). A female patient, 54 years old, with a history of recurrent deep venous thrombosis, an episode of pulmonary embolism and was positive for APS antibodies. She was kept on warfarin and aspirin. The patient was admitted with acute pulmonary edema and severe aortic stenosis. While preparing for aortic valve surgery, the patient developed acute stroke, and a week later developed concurrent acute ischemia of both lower limbs. Emergency surgery salvaged the lower limbs and follow up transthoracic echocardiography showed marked regression of the thickening of the aortic valve leaflets, only mild aortic stenosis and moderate aortic regurgitation. Aortic valve surgery was cancelled, and the patient was kept on warfarin and aspirin. Conclusion Development of severe aortic stenosis is uncommon in primary APS, and this scenario of spontaneous improvement from a severe to a mild aortic stenosis, is probably unreported before.

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Sudden Onset Lower Limb Pain and Paraesthesia: A Case Report

10.33169/biomcase.bacroaoj-i-112 ◽

2020 ◽

Vol I (3) ◽

pp. 33-35

Author(s):

Gabriella Iannuzzo

Keyword(s):

Thrombus Formation ◽

Sudden Onset ◽

Lower Limbs ◽

Iliac Arteries ◽

Leriche Syndrome ◽

Ct Angiogram ◽

Increased Risk ◽

Lower Limb Pain ◽

Artery Disease ◽

Atherosclerosis Risk Factors

We describe the case of a woman with a metastatic pancreatic cancer and previous coronary artery disease who develops an acute Leriche syndrome. She arrived to emergency for onset orthopnea and dyspnoea on mild exertion. During the hospitalization is made a diagnosis of metastatic pancreatic adenocarcinoma. 10 days after admission suddenly the women reported severe pain in lower limbs and she was unable to maintain an upright position. Femoral, popliteal and posterior tibial pulses were not palpable bilaterally. At the Echo-doppler of the lower limbs limb pulses were barely appreciable with a tardus-parvus waveform. angiography showed critical thrombus formation in the sub-renal descending segment of the aorta and iliac arteries (which are CT commonly found in Leriche Syndrome); there was no perfusion of the inferior mesenteric artery; the internal and external iliac arteries as well as thigh and leg arteries showed slow and filiform flow with multiple sub-stenotic segments. Despite the immediate medical and surgical treatment woman died. Patients without known atherosclerosis risk factors should be investigated for hypercoagulable diseases or for co-morbidities which carry an increased risk of thrombo-embolism such as cancer. Considering that cancer and cardiovascular diseases are closely related, acute sub-renal aortic thrombosis should be considered when a patient presents with or develops unclear symptoms of sudden pain in the lower limbs with associated paralysis or paraesthesia and vascular investigations, such as ECHO-doppler and CT angiogram, must be performed.

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Approaching Venous Thromboembolism Disease (DVT) from Another Perspective: Better Management in a Multidisciplinary DVT Unit Improves Clinical Outcomes and Post Thrombotic Syndrome. One-Year Experience in Our Hospital

Blood ◽

10.1182/blood-2019-131327 ◽

2019 ◽

Vol 134 (Supplement_1) ◽

pp. 2431-2431

Author(s):

Milagros Suito ◽

Maria Cerdá ◽

Olga Benítez ◽

Alba Cabirta ◽

Francesc Bosch ◽

...

Keyword(s):

Pulmonary Embolism ◽

Board Of Directors ◽

Antiphospholipid Syndrome ◽

Research Funding ◽

Positive Impact ◽

Protein Deficiency ◽

Lower Limbs ◽

Advisory Committees ◽

Post Thrombotic Syndrome

INTRODUCTION: Deep vein thrombosis (DVT) is a frequent cause or morbidity and mortality, and multiple genetic and environmental factors are involved in its etiopathogenesis. Besides investigating about personal and familiar DVT history, thrombophilia testing is often asked by physicians in order to diagnose patients, even if current validated markers won't be decisive to set duration of treatment. DVT is a multidisciplinary management entity, so it`s important to unify the diagnosis and treatment circuit with the specialists involved, in order to get a positive impact in the prognostic of the disease. MATERIALS AND METHODS: We developed an observational and prospective study between January 2017 and December 2017 in our University Tertiary Hospital in order to register the patients with DVT that were treated in our DVT unit conformed by specialists of Hemostasis, Angiology and Intern Medicine. As secondary objectives, we wanted to know how a systematic circuit would improve the management and the complications of patients with DVT. After diagnosis in Emergency Service by Angiology -or Intern Medicine if pulmonary embolism (PE), they were sent to Hemostasis Unit within the first 7-10 days to optimize treatment according patient's necessities. After 3-6 months of starting treatment, patients were seen conjunctly by specialists of Angiology and Hemostasis to perform a doppler ultrasound and thrombophilia study in selected patients, in order to make the best decision about treatment. RESULTS: We included 172 patients with DVT, most of them were men (56.9%), the average age was 66.4 years old, and the incidence increased from 50 years old, on. Only 26.2% had a positive personal history of DVT and 18% familiar history. We found that 75 (43.6%) patients had idiopathic DVT. About anatomical localization, the majority presented in the lower limbs (83.1%) and 12.7% associated pulmonary embolism at diagnosis. All of them started treatment with LMWH, and after the first clinical visit, 26.7% continued with it, while 51.2% changed to VKA and 22.1% to DOACs. We tested only 69 patients for thrombophilia, according to guidelines (especially in incidental DVT): 23.8% were diagnosed of antiphospholipid syndrome, 19% of S protein deficiency, 14.3% of C protein deficiency and 14.3% of prothrombin mutation, as the most common findings. Patients with positive study or antiphospholipid syndrome and 21 of the 41 patients with negative study, received permanent anticoagulation. In the conjunct visit we found a 61.1% of residual/chronic DVT, 38.4% resolved, and 0.5% of recurrence (local extension). Only 18.6% of patients had post thrombotic syndrome and we didn`t see any hemorrhagic complications during the follow up period. We also identified a reduction of almost a 50% of clinical visits because of the systematic circuit. CONCLUSIONS: An ordered flow of patients diagnosed of DTV contributed in our clinical practice to avoid redundant hospital visits and complementary tests, and specially to release a better follow up of patients with a less percentage of bleeding (0%) and post thrombotic syndrome (18.6% vs 40% in world registries), as well as we found. It would be interesting to amplify the time of follow up, so that we would know if these benefits remain within time. Disclosures Bosch: Kyte: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding, Speakers Bureau; F. Hoffmann-La Roche Ltd/Genentech, Inc.: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding, Speakers Bureau; Takeda: Honoraria, Research Funding; AstraZeneca: Honoraria, Research Funding; AbbVie: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding, Speakers Bureau; Janssen: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding, Speakers Bureau; Celgene: Honoraria, Research Funding; Acerta: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding, Speakers Bureau; Novartis: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding, Speakers Bureau.

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Antiphospholipid Syndrome with Cutaneous Necrotic Lesion of the Lower Limbs

The Journal of Rheumatology ◽

10.3899/jrheum.101018 ◽

2011 ◽

Vol 38 (4) ◽

pp. 786-787

Author(s):

PENG-JIE WAN ◽

XIAO-QING ZHAO ◽

KAREN AU-YEUNG ◽

JIE ZHENG

Keyword(s):

Antiphospholipid Syndrome ◽

Necrotic Lesion ◽

Lower Limbs

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Leriche Syndrome Diagnosed Due to Polytrauma: a Case Report

10.21203/rs.3.rs-966846/v1 ◽

2021 ◽

Author(s):

Genki Yoshimura ◽

Ryo Kamidani ◽

Tomotaka Miura ◽

Hideaki Oiwa ◽

Yosuke Mizuno ◽

...

Keyword(s):

Small Bowel ◽

Lower Extremity ◽

Bowel Perforation ◽

Clavicle Fracture ◽

Lower Limbs ◽

Dump Truck ◽

Mandibular Fractures ◽

Bilateral Pneumothorax ◽

Thrombotic Occlusion ◽

Leriche Syndrome

Abstract Background: Leriche syndrome is caused by atherosclerosis and is often characterized by symptoms such as intermittent claudication as well as numbness and coldness of the lower limbs. Its exact prevalence and incidence are unknown because it is a rare disease. We report a case of Leriche syndrome diagnosed incidentally on a trauma pan-scan computed tomography (CT).Case Presentation: A 61-year-old Asian man was driving a passenger car when he had a head-on collision with a dump truck and received an emergency call. He was transported to our hospital in a doctor's helicopter. Physical examination revealed the following vital signs: respiratory rate, 23 breaths per minute; SpO2, 98% under a 10 L administration mask; pulse rate, 133 beats per minute; blood pressure, 142/128 mmHg; Focused Assessment with Sonography for Trauma, positive; Glasgow Coma Scale E3V5M6; and body temperature, 35.9℃.Trauma pan-scan CT showed bilateral mandibular fractures, bilateral multiple rib fractures, bilateral pneumothorax, sternal fractures, peri-thoracic hematoma, small bowel perforation, mesenteric injury, right clavicle fracture, right ankle debridement injury, and thrombotic occlusion of the bilateral common iliac arteries from just above the abdominal aortic bifurcation. Although thrombotic occlusion needed to be differentiated from traumatic aortic injury, the presence of collateral blood vessels led to the diagnosis of Leriche syndrome, and conservative treatment was performed.Damage control surgery was required for small bowel injuries. From the second day of admission, the patient was treated with continuous intravenous heparin and prostaglandin preparations. However, impaired blood flow and reperfusion injury in the right lower extremity progressed. On the fifth day of admission, right thigh amputation was performed. The patient required renal replacement therapy for approximately two weeks starting from the third day of admission.Conclusions: In this case, conservative therapy was initially chosen as the treatment for Leriche syndrome. However, the complex factors in the acute phase of trauma lead to hemorrhagic necrosis development, resulting in amputation of the lower extremity. Our findings making necessitate the importance of decision of treatment indication and choice.

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Comparison of the Serum Metabolic Signatures Based on 1 H NMR between Thrombotic Antiphospholipid Syndrome (APS) Patients and Healthy Individuals

Blood ◽

10.1182/blood-2019-131927 ◽

2019 ◽

Vol 134 (Supplement_1) ◽

pp. 5769-5769

Author(s):

Melissa Quintero ◽

Silmara Aparecida De Lima Montalvão ◽

Ljubica Tasic ◽

Stephany Cares Huber ◽

Joyce Maria Annichino- Bizzacchi

Keyword(s):

Venous Thrombosis ◽

Antiphospholipid Syndrome ◽

Conflicts Of Interest ◽

Lactate Production ◽

Immunosuppressive Drugs ◽

Lower Limbs ◽

Control Group ◽

Healthy Individuals ◽

Exclusion Criteria ◽

Metabolic Alterations

Antiphospholipid syndrome (APS) is an acquired immuno-mediated disorder, in which antiphospholipid antibodies (aPL) against a variety of phospholipids and phospholipid transport proteins are persistently produced. aPL include a heterogeneous group of circulating immunoglobulins: lupus anticoagulant (LA), anticardiolipin antibodies (aCL) and anti-β2glycoprotein-I (anti-β2GPI). Their presence is related to diverse clinical phenomena including arterial and venous thrombosis, which can be related to different pathogenic properties, such as its effect on the clotting pathways, endothelium and platelets. Metabolomic can be useful improving diagnosis, prognostic and therapeutic interventions, in vascular disease (Kirlikaya B. et al, 2019) The aim of this study was to identify metabolic alterations in patients with thrombotic APS characterized by deep venous thrombosis (DVT) compared to healthy individuals using liquid-state 1H-NMR data acquisition combined with chemometrics analysis. We evaluated ten serum samples of patients with APS (mean age 39 years; 6 female/4 male), who attended at the outpatient clinic of Hemocentro of Campinas, Unicamp-Brazil, from 2013 to 2017 (Table 1). The exclusion criteria were the presence of cancer and renal disease, smoking and alcoholism. The samples were collected up to 2 years the last thrombotic episode. All patients except one (suprahepatic vein) suffered DVT of the lower limbs and only two of them presented 2 thrombotic episodes. Six patients were classified as primary APS and four with secondary (three presented systemic lupus erythematosus (SLE) and one were diagnosed with psoriasis). All patients with secondary APS were using immunosuppressive drugs: prednisone and azathioprine, or methotrexate. None of the patients were triple positive. The control group were healthy individuals (mean age 41 years; 5 female/5male), from staff of the Hemocentro and the exclusion criteria were the same as for patients. Multivariate analysis pointed to slight differential metabolic profiles among APS and controls, and between primary and secondary APS. Different metabolites associated to energy metabolism, such as increased glucose consumption and lactate production, as well as differences at spectral regions corresponding to some aromatic amino acids were identified in APS group when compared to controls. Our results are promising, such as different profiles could be observed, both regarding patients vs. controls, such as primary vs. secondary APS. The use of immunosuppressive drugs in secondary APS may contribute to these changes. Although questions regarding the translatability of metabolomics findings into clinical practice, our results showed the high sensitivity and specificity of NMR spectroscopy to distinguish healthy and APS. To have a better understanding of the metabolic alterations potentially involved in the APS, additional analyses including mean spectra are being made. Disclosures No relevant conflicts of interest to declare.

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