Management of Buerger's disease during pregnancy.

Buerger's disease (BD), also known as thromboangiitis obliterans, is a non-atherosclerotic inflammatory disorder of unknown aetiology that affects small-sized and medium-sized vessels of the extremities. It is usually observed in middle-aged adults, especially those who smoke or use tobacco products. This condition is more frequently observed in men, although recent findings indicate an increasing prevalence among women, potentially due to increased cigarette use. The association between pregnancy and BD is rare, with only a few published cases. Previous reports have indicated that BD may worsen during gestation due to the characteristic hypercoagulable state of pregnancy. In addition, it seems to be associated with intrauterine growth restriction secondary to infarction of placental vessels. Careful obstetric management of maternal and fetal status is mandatory in pregnancies complicated with BD. We report a successful case of a pregnancy in a patient with BD treated with low-molecular-weight heparin.

Diagnostic criteria for Buerger's disease: International Consensus of VAS - European Independent Foundation in Angiology/Vascular Medicine.

Buerger's disease (BD) remains a debilitating condition and early diagnosis is paramount for its effective management. Despite many published diagnostic criteria for BD, selective criteria have been utilized in different vascular centers to manage patients with BD worldwide. A recent international Delphi Consensus Study on the diagnostic criteria of BD showed that none of these published diagnostic criteria have been universally accepted as a gold standard. Apart from the presence of smoking, these published diagnostic criteria have distinct differences between them, rendering the direct comparison of patient outcomes difficult. Hence, the expert committees from the Working Group of the VAS-European Independent Foundation in Angiology/Vascular Medicine critically reviewed the findings from the Delphi study and provided practical recommendations on the diagnostic criteria for BD, facilitating its universal use. We recommend that the 'definitive' diagnosis of BD must require the presence of three features (history of smoking, typical angiographic features and typical histopathological features) and the use of a combination of major and minor criteria for the 'suspected' diagnosis of BD. The major criterion is the history of active tobacco smoking. The five minor criteria are disease onset at age less than 45 years, ischemic involvement of the lower limbs, ischemic involvement of one or both of the upper limbs, thrombophlebitis migrans and red-blue shade of purple discoloration on edematous toes or fingers. We recommend that a 'suspected' diagnosis of BD is confirmed in the presence of a major criterion plus four or more minor criteria. In the absence of the major criterion or in cases of fewer than four minor criteria, imaging and laboratory data could facilitate the diagnosis. Validation studies on the use of these major and minor criteria are underway.

Buerger's Disease - A Clinical Case.

Buerger's disease is a distal segmental nonatherosclerotic vasculopathy that involves the inferior and superior limbs of smoker males younger than 45 years old. This article aims to describe a clinical case and revise the literature about Buerger's disease. A 45-year-old smoker male repeatedly visited the emergency department for refractory pain and inflammatory signs in the right hallux. After developing ulcers in the right foot, Doppler ultrasonography revealed segmental occlusion of distal arteries of that limb. It was also observed in arteriography "corkscrew" collaterals. Autoimmune, thrombophilic and cardiovascular diseases were excluded. Analgesia, antibiotics and alprostadil were implemented. As a result, the patient stopped smoking and was submitted to minor amputation with complete healing, after which he remained asymptomatic. Buerger's disease is a diagnosis of exclusion. Therefore, smoking cessation is the most effective treatment and is crucial to prevent disease progression.

Cannabis arteritis presenting with Raynaud's and digital ulcerations: a case-based review of a controversial thromboangiitis obliterans-like condition.

Thromboangiitis obliterans (TAO), or Buerger's disease, is a non-atherosclerotic inflammatory disease of the small and medium-sized arteries, veins, and nerves of the legs and arms, strongly associated with the use of tobacco products in young adults. Cannabis arteritis (CA), an entity with similar clinical and pathological features, has been described in marijuana users as a subtype of TAO. Distinction between TAO and CA is challenging, given that most patients use tobacco and marijuana products concomitantly. Herein, we report the case of a male in his late forties who was referred to rheumatology with a 2-month history of hand swelling and bilateral painful digital ulcers with blue discoloration on his fingers and toes. The patient reported daily use of marijuana in blunt wraps and denied tobacco use. His laboratory work-up was negative for scleroderma and other connective tissue diseases. His angiogram confirmed the diagnosis of thromboangiitis obliterans, which was attributed to cannabis arteritis. The patient was started on aspirin and nifedipine daily and discontinued marijuana use. His symptoms resolved within 6 months and have not recurred for more than a year with continued avoidance of marijuana. Our case is one of the few that features primarily marijuana-driven CA and highlights the importance of not only considering marijuana use but also blunt wrap use in patients presenting with Raynaud's phenomenon and ulcerations as cannabis use rises globally.

Efficacy of Transpedal Retrograde Approach in Endovascular Treatment of Patients With Buerger's Disease.

BACKGROUND: Endovascular treatment of Buerger's disease is challenging, which usually impedes antegrade revascularization options. We aimed to evaluate the effectiveness of transpedal retrograde approach in patients with Buerger's disease with ambiguous proximal caps and/or previously failed endovascular intervention via antegrade approach. METHODS: Eighteen patients with the diagnosis of Buerger's disease who had previously failed antegrade endovascular interventions and/or ambiguous proximal caps were enrolled. Baseline demographic characteristics, severity of critical limb-threatening ischemia, wound scores, postprocedural pedal loop scores, and recovery or amputation rates were recorded. RESULTS: The patients presented with ischemic rest pain (n = 5, Rutherford stage 4), ischemic ulcers with minor tissue loss (n = 8, Rutherford stage 5), and severe ischemic ulcers or gangrene with major tissue loss (n = 5, Rutherford stage 6). Preprocedural wound score according to Saint Elian Wound Score System (SEWSS) was 15.72 ± 5.05. Retrograde transpedal puncture was achieved with an 89% success rate. Postintervention angiographic success rate was 100%. Postintervention Rutherford stage improved compared with preprocedural Rutherford stages (P<.01). In addition, the average SEWSS score decreased significantly after the interventions (P<.001). Postprocedural pedal loop score was found to be associated with SEWSS scores and amputation rates. CONCLUSIONS: Transpedal retrograde approach is a technically feasible and potentially effective treatment modality for Buerger's disease and may be considered as a first-line treatment option in the treatment of limb salvage, especially when proximal caps of target vessels are ambiguous or antegrade approach is unsuccessful.

A propósito de un caso: abordaje y manejo integral de la enfermedad de buerger en Atención Primaria / Regarding A Clinical Case: Comprehensive Approach And Management Buerger's Disease In Primary Care

Las enfermeras especialistas en atención comunitaria tenemos una misión con la salud pública para mejorar la salud de la comunidad. Desde nuestra consulta tenemos la gran responsabilidad de mejorar la salud de nuestros pacientes, desde la prevención y promoción de la salud y siempre con un enfoque centrado en la persona y su empoderamiento. A propósito de un caso clínico aborda la problemática que supone la pandemia del tabaquismo en nuestra sociedad actual y cómo desde nuestra consulta de atención primaria podemos abordar tanto la deshabituación como el seguimiento y tratamiento de las morbilidades asociadas. En este caso, la enfermedad de buerger. (AU)

Community care nurse specialists have a mission with public health to improve the health of the community. From our health center we have the great responsibility of improving the health of our patients, from the prevention and promotion of health and always with a personcentered approach and their empowerment. Regarding a clinical case, it addresses the problems that the smoking pandemic entails in our current society and from our primary care consultation we can address both cessation and follow-up and how to treat associated morbidities. In this case, Buerger's disease. (AU)

Comparative Analysis of Transcriptome Profiles in Patients with Thromboangiitis Obliterans.

BACKGROUND: Thromboangiitis obliterans (TAO) causes vascular insufficiency due to chronic inflammation and abrupt thrombosis of the medium and small arteries of the extremities. In our study, we aimed to determine biomarkers for the diagnosis of TAO by evaluating 15 male TAO patients with Shinoya diagnostic criteria and 5 healthy controls who did not have TAO-related symptoms in their family histories. METHODS: The Clariom D Affymetrix platform was used to conduct microarray analysis on total RNA extracted from whole blood. A total of 477 genes (FC ≤ 5 or >5) common to the fifteen patient and five control samples were selected using comparative microarray analysis; among them, 79 genes were upregulated and 398 genes were downregulated. RESULTS: According to FC ≤ 10 or >10, in the same TAO patient and control group, 13 genes out of 28 were upregulated, whereas 15 genes were downregulated. The 11 key genes identified according to their mean log2FC values were PLP2, RPL27A, CCL4, FMNL1, EGR1, EIF4A1, RPL9, LAMP2, RNF149, EIF4G2, and DGKZ. The genes were ranked according to their relative expression as follows: FMNL1 > RNF149 > RPL27A > EIF4G2 > EIF4A1 > LAMP2 > EGR1 > PLP2 > DGKZ > RPL9 > CCL4. Using protein-protein interaction network analysis, RPL9, RPL27A, and RPL32 were found to be closely related to EIF4G2 and EIF4A1. The Reactome pathway found pathways linked to 28 genes. These pathways included the immune system, cellular responses to stress, cytokine signaling in the immune system, and signaling by ROBO receptors. CONCLUSIONS: By figuring out the protein expression levels of the genes that have been found to explain how TAO disease works at the molecular level, it will be possible to figure out how well these chosen transcripts can diagnose and predict the disease.

Chronic management of pulmonary embolism in thromboangiitis obliterans.

Thromboangiitis obliterans (TAO), also known as Buerger's disease, is a rare small vessel vasculitis that is associated with an increased risk of arterial occlusion. Although venous thromboembolism has been described, the risk of its recurrence and the best long-term anticoagulation management is not known. Considering this, we would like to share our experience with two patients admitted to our hospital with recurrent pulmonary embolism and previous diagnosis of TAO, aiming to discuss the indication for indefinite anticoagulation in this population.

Endovascular treatment of Buerger's disease in patients with critical limb ischaemia.

OBJECTIVES: Thromboangitis obliteransis (TAO) is a nonatherosclerotic, inflammatory, occlusive arteritis that affects small and medium-sized arteries, veins and nerves. A large proportion of patients with TAO suffer from claudication, and the ultimate condition is gangrene and limb loss if there is no treatment or the cessation of smoking. Endovascular revascularisations are performed frequently and provide acceptable results in patients who are not suitable for surgery. In this study, we aimed to show our clinical experience in patients with TAO who were treated with endovascular revascularisation. METHODS: Between January 2014 and March 2020, 18 patients with lower-extremity critical limb ischaemia (CLI) underwent endovascular treatment (ET). Technical details and clinical success at follow up were documented. RESULTS: This study included 18 patients with lower-extremity TAO who presented with CLI and were treated with ET. The mean age of the patients was 38.8 ± 7.3 years. Fifty per cent of patients had pain at rest, 33.7% had minor tissue loss and non-healing ulcers, and 16.7% had major tissue loss on admission. The majority of lesions were located in the peroneal and tibial arteries (n = 13, 72.27percnt;). Two (11.1%) patients had distal superficial femoral artery occlusion concomitant with popliteal artery (PA) lesions, and three (16.7%) had PA occlusion concomitant with peroneal and tibial artery lesions. Re-establishment of antegrade flow in at least one vessel was achieved in 15 (83.3%) patients. Balloon angioplasty was performed in all patients. Plain old balloon angioplasty (POBA) was used in nine (60%) patients and drug-eluting balloon (DEB) angioplasty in six (40%). The mean duration of follow up in 15 patient who had undergone successful ET was 21.5 ± 8.1 months. The primary CLI-free rate at 12 and 24 months was 80% (66.7% in all patients). Secondary CLI-free rates at six, 12 and 24 months were 100, 93.3 and 53.3%, respectively. Patients who were active smokers during their follow up had a higher frequency of out-patient clinic consultations (p = 0.03). CONCLUSION: Controversy has continued on the role of ET in the treatment of TAO. This study shows that ET of TAO had promising primary and secondary patency rates with high technical success and limb-salvage rates.

An International Delphi Consensus on Diagnostic Criteria for Buerger's Disease.

BACKGROUND: Buerger's disease (BD) remains a debilitating condition. Despite multiple published diagnostic criteria for BD, none is universally accepted as a gold standard. METHODS: We conducted a 2-round modified Delphi consensus study to establish a consensus on the diagnostic. The questionnaire included statements from several commonly used diagnostic criteria for BD. Qualitative and quantitative analysis methods were performed. An agreement level of 70% was applied. RESULTS: Twenty nine experts from 18 countries participated in this study. Overall, 75 statements were circulated in Round 1. Of these, 28% of statements were accepted. Following comments, 21 statements were recirculated in Round 2 and 90% were accepted. Although more than 90% of the experts did not agree that the diagnosis of BD can be based only on clinical manifestation, none of the nonclinical manifestations of BD were agreed as a part of the diagnostic criteria. There was an agreement that a history of tobacco consumption in any form, not necessarily confined to the current use, should be a part of the diagnostic criteria of BD. The history of thrombophlebitis migrans, even if not present at presentation, was accepted as a clue for BD diagnosis. It was also agreed that discoloration of the toes or fingers could be included in the diagnostic criteria of BD. Experts agreed that histology results could differentiate BD from atherosclerosis obliterans and other types of vasculitis. The presence of corkscrew collaterals on imaging and burning pain reached the agreement at the first round but not at the second. There was no consensus regarding age cut-off, the requirement of normal lipid profile, and normal blood glucose for BD diagnosis. CONCLUSIONS: The present study demonstrated discrepancies in the various published diagnostic criteria for BD and their selective utilization in routine clinical practice worldwide. We propose that all published diagnostic criteria for BD be re-evaluated for harmonization and universal use.

Atypical Young-onset Dementia in Cerebral Thromboangiitis Obliterans: A Case Report.

Young-onset dementia (YOD, age at onset below 45 y) has a broad differential diagnosis. We describe a 41-year-old man with atypical manifestations of YOD syndrome in cerebral thromoboangiitis obliterans (CTAO). Extensive antemortem workup including clinical assessment, laboratory investigations, neuroimaging, and genetic testing did not elucidate a diagnosis. Postmortem neuropathologic examination revealed cortical sickle-shaped granular atrophy, resulting from numerous remote infarcts and cortical microinfarcts that mainly affected the bilateral frontal and parietal lobe, confirming CTAO. Although CTAO is a rare cause of vascular dementia, it should be considered as one of the differentials in patients with YOD with a history of heavy smoking and presence of symmetric damages of watershed-territory on neuroimaging.

Milestones in thromboangiitis obliterans: a position paper of the VAS-European independent foundation in angiology/vascular medicine.

Even today thromboangiitis obliterans has disease features that remain misunderstood or underappreciated. The epidemiology, etiology and pathophysiology of the disease are still unclear. Biomarkers and disease activity markers are lacking, thus clinical assessment is difficult. We are still struggling to establish unique diagnostic, staging and treatment criteria. This is an academic-collaborative effort to describe the pathophysiology, the clinical manifestations, the diagnostic approach, and the challenges of management of patients with TAO. A systematic search for relevant studies dating from 1900 to the end of 2020 was performed on the PubMed, SCOPUS, and Science Direct databases. Given the intriguing nature of presentation of TAO, its management, to some extent is not only different in different regions of the world but also varies within the same region. Following this project, we discovered ambiguity, overlap and lack of clear-cut criteria for management of TAO. An international group of experts however came to one conclusion. They all agree that management of TAO needs a call for action for a renewed global look with multi-center studies, to update the geographical distribution of the disease and to establish a unique set of diagnostic criteria and a consensus-based guideline for best treatment based on current evidence.

Intestinal thromboangiitis obliterans: a case report.

BACKGROUND: Thromboangiitis obliterans or Buerger's disease is a form of peripheral vascular disease in young male smokers. The involvement of the intestine occurs in only about 2% of the cases, when they may present as acute abdomen due to mesenteric ischemia. The uncommonness of the condition makes it a less suspected differential diagnosis, leading to a delay in appropriate management, thereby increasing chances of morbidity or mortality. Cessation of smoking is known to stall the disease progression including visceral involvement, but may not always be the case as happened in the case being presented. CASE PRESENTATION: Our Indian Hindu male patient, a known smoker, presented with diffuse abdominal pain along with bouts of vomiting and loose motions. He had a prior history of amputation of the right foot, 4 years before. At presentation he had abdominal distension with diffuse tenderness and guarding. An omental band attached to the tip of the appendix was discovered at the initial exploration along with dilated proximal bowel loops, for which a release of the omental band along with appendectomy was done. He developed an enterocutaneous fistula on the 6th postoperative day for which he had to be reexplored, and multiple jejunal perforations were found. Segmental jejunal resection and a Roux-en-Y gastrojejunostomy with distal ileostomy were done along with a feeding jejunostomy. The patient however again had feculent discharge from the wound for which a third exploration was done. The gastrojejunostomy and feeding jejunostomy sites were leaky, both of which were repaired primarily. The patient developed septicemia which progressed to refractory septic shock, and he ultimately succumbed to his illness on the 23rd postoperative day of the index surgery. CONCLUSION: Acute abdomen in a young man who is a chronic smoker and having an antecedent history of amputation of some part of an extremity for a nontraumatic cause should raise the suspicion of Buerger's disease of the intestine. Although it is a progressive disease and the situation has already progressed by the time intestinal symptoms manifest, early detection may give some scope of salvage and decrease the morbidity and mortality.

Women and Pregnancy in Thromboangiitis Obliterans.

Data regarding women and thromboangiitis obliterans (TAO) are conflicted, and a few cases of pregnancy have been described. We aimed to describe the interplay between TAO and pregnancies. Among 224 TAO patients, 22.8% were women. Demographic data, clinical manifestations, and outcomes were similar between men and women. Twenty-one (41.2%) women had 48 pregnancies. Thirty-six (75%) pregnancies with on term and complication free delivery occurred. None of the patients experienced a disease flare of TAO during pregnancy. TAO does not seem to affect pregnancy complications, and pregnancy does not seem to interfere with the course of TAO.

Thromboangiitis Obliterans (Buerger's Disease) in an Adolescent Male.

Arterial occlusive disease of the limb is very rare in children. Buerger's disease (BD) is a nonatherosclerotic, segmental inflammatory arteritis affecting the small and medium-sized vessels of the extremities. We report BD in a 16-year-old male presenting with arterial insufficiency of left foot and history of smoking cigarettes and cannabis for 2 years. BD was diagnosed based on history of smoking in combination with clinical, laboratory, and radiologic findings. Pediatric hemato-oncologists should consider BD in the differential diagnosis in adolescents who smoke cigarettes and/or cannabis and present with vascular insufficiency of the hands and/or feet.

Thromboangiitis Obliterans Biomarker Shifts in Different Acute Phase Stages: A Case Study.

Thromboangiitis obliterans (TAO) is a rare vasculopathy that is predominantly seen in young male smokers. Recently, new biomarkers have been shown to be useful in distinguishing TAO from acute phase TAO in an Asian study population. The present case study illustrates their application in a European patient during TAO exacerbation and their association with therapeutic performance.