Figure - available from: Case Reports in Rheumatology
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Axial CT angiography sections show wall thickening in both common carotid arteries (arrows, (a)) and a small thrombus protruding into the lumen of the right common carotid artery (arrow, (b)).
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We present a case of a 34-year-old male who presented to the emergency ward with fever and abdominal pain. The diagnosis of Takayasu's arteritis and also antiphospholipid syndrome was made during an imaging workup of deep-vein thrombosis. A spontaneous coronary artery dissection was revealed in coronary CT angiography requested for chest pain and d...
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Background:
Increasing attention has been paid to acute myocardial infarction (AMI) in young female patients for whom secondary factors should be considered during the diagnostic process. Anti-phospholipid syndrome (APS), a rare autoimmune disease that is most common in young female patients, is reportedly related to AMI. To date, coronary interve...
Citations
... It is reasonable to think that the presence of antiphospholipid antibodies (aPL) may increase vascular damage in the course of a vasculitic disease such as TA due to their thrombotic effects. Although there were studies and case reports supporting the association of aPL and TA severity in the literature, the clinical importance of these antibodies in TA is still controversial [3][4][5][6][7]. This study was conducted to explore the frequency of aPL and its association with disease-related complications in TA. ...
... Although severe cases with TA and aPL had been reported, the thrombotic events in these patients were usually detected in the arterial circulation, and the number of cases with venous thrombosis or recurrent pregnancy losses, which are more typical for APL, were low. A venous thrombotic event (in 3 patients) or recurrent pregnancy (in 4 patients) loss was only present in 7 of the 19 reported cases in the literature [5,6,[16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32][33][34]. In studies where aPL were screened more systematically, it has been suggested that aPL in TA patients were associated with disease activity or an increased number of vascular interventions [3,4]. ...
Background:
The significance of antiphospholipid antibodies (aPL) is controversial in Takayasu arteritis (TA). This study was conducted to explore the frequency of aPL and their association with disease-related complications in TA.
Methods:
: This cross-sectional study was conducted to investigate the presence of anti-cardiolipin (aCL), anti-beta 2 glycoprotein- 1(aβ2G1) antibodies, and lupus anticoagulant (LA) in TA patients. TA patients admitted to the Department of Rheumatology of Hacettepe University Faculty of Medicine between December 2015 and September 2016 who fulfilled the American College of Rheumatology (ACR) classification criteria for TA were consecutively enrolled in the study. Patients were grouped according to aPL positivity and compared in terms of disease manifestations, type of vascular involvement at diagnosis, and vascular complications/interventions attributable to TA.
Results:
Fifty-three TA (49 female) patients were enrolled in the study. We detected 9 (16.9%) patients with IgM and/or IgG aβ2G1 and/or LA positivity. There were no patients with positive aCL. All aβ2G1 titers were low. There were no differences in terms of symptoms, signs, type of vascular involvement, the number of patients with disease-related complications or vascular interventions/surgery between aPL (+) and aPL(-) groups (p > 0.05 for all). The number of patients with thrombotic lesions was similar between the groups (p > 0.05). There were no patients with a history of venous thrombosis or on anticoagulant treatment in the aPL(+) group. Only 1 patient with IgM aβ2G1 (+) had a history of pregnancy loss.
Discussion:
Our results indicate that aPL positivity is not rare in TA. On the other hand, all aPL titers were low and no differences were found in the frequency of disease-related complications between aPL(+) and aPL(-) patient groups. Only TA patients with atypical manifestations with high suspicion of aPL-related complications should be considered to be investigated for aPL.
... A-B2GLP in 6 patients (31.5%) and aPE in 1 patient (5.2%). The different vessels involved included the internal carotid artery, aorta and coronary arteries in four patients each (21.05%), the vertebral artery and cerebral arteries in three patients (15.7%), and the superficial femoral artery in one patient (5.2%) [10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25] . In this review, we describe two cases of CVA resulting from dissection of the posterior cerebral artery. ...
Antiphospholipid syndrome (APS) is a multisystemic autoimmune disease which presents with thromboembolic disease, pregnancy complications and the presence of antiphospholipid antibodies. There are some reports of arterial dissections in different sites of the body associated with APS. We describe two patients with APS who developed ischaemic stroke as a result of vertebral artery dissection in the absence of acquired and genetic risk factors for arterial dissection. We also conducted a systematic review of the literature for cases of arterial dissection associated with APS. We suspect that APS may be a potential cause of vasculopathy and arterial dissection. Further research is needed to explore this possible association.
Learning points:
Antiphospholipid syndrome (APS) is a major risk factor for stroke in young patients.APS may be associated with vasculopathy and arterial dissection.Patients should meet both clinical and laboratory criteria for a diagnosis of APS.
... In particular, a question as to whether SCAD might have an inflammatory etiology arose in 1982 when a series of biopsies often showed focal infiltration of the adventitia with inflammatory cells, including eosinophilic granulocytes (5). Since then, numerous case reports have reported SCAD in the setting of lupus (6), antiphospholipid syndrome (7)(8)(9)(10)(11)(12), systemic vasculitis (11,(13)(14)(15)(16), and other autoimmune diseases (17,18). A case-control study also showed increased prevalence of hypothyroidism in patients with SCAD (19). ...
... In particular, a question as to whether SCAD might have an inflammatory etiology arose in 1982 when a series of biopsies often showed focal infiltration of the adventitia with inflammatory cells, including eosinophilic granulocytes (5). Since then, numerous case reports have reported SCAD in the setting of lupus (6), antiphospholipid syndrome (7)(8)(9)(10)(11)(12), systemic vasculitis (11,(13)(14)(15)(16), and other autoimmune diseases (17,18). A case-control study also showed increased prevalence of hypothyroidism in patients with SCAD (19). ...
... AUTOIMMUNE DISEASE. We included 25 validated autoimmune diseases (28), adding antiphospholipid syndrome due to its associations in case reports (7)(8)(9)(10)(11)(12). We defined each autoimmune disease as at least 1 inpatient encounter with a corresponding code as the primary diagnosis or at least 2 codes at least 30 days apart and within 2 years of the index date (31). ...
Background
Case reports and referral-based studies suggest spontaneous coronary artery dissection (SCAD) is associated with autoimmune diseases and causes 2% to 4% of acute coronary syndromes.
Objectives
This study determined the association of SCAD with autoimmune diseases, together with incidence and recurrence, in a population-based study.
Methods
This case–control study took place from 1995 to 2018 within the Rochester Epidemiology Project. The study identified cases with SCAD from diagnosis codes and verified them using coronary angiography images, matching each case to 3 control subjects on age, sex, county, and years of medical history. Autoimmune disease history came from a validated, code-based definition. A multivariable logistic regression model calculated the odds ratio (OR) for SCAD among patients with a history of autoimmune disease, adjusting for race and body mass index.
Results
The study identified 114 cases with SCAD (mean age 51 years and 90% women) and 342 matched control subjects. Autoimmune disease occurred in 13 (11%) cases with SCAD and 40 (12%) control subjects (p = 0.93). Even after adjustment, autoimmune diseases were not associated with SCAD (OR: 0.81; 95% confidence interval [CI]: 0.40 to 1.66). SCAD incidence between 2010 and 2018 (2.7 per 100,000; 95% CI: 1.7 to 3.7) was 10-fold higher than the incidence between 1995 and 2009 (0.3 per 100,000; 95% CI: 0.0 to 0.6). SCAD recurrence was 10% (95% CI: 3% to 16%) at 5 years.
Conclusions
These findings suggested SCAD pathogenesis is noninflammatory and screening for autoimmune diseases based on SCAD alone is not warranted. The code-based incidence of SCAD has increased over time, highlighting the importance of considering SCAD among patients with acute coronary syndromes.
... diagnosis (aortic dissection) was verified by CT angiography. Case series of APLAS patients with carotid artery dissection and coronary artery dissection have been reported [1,2] . Our literature search revealed our case is the fourth APLAS patient to be diagnosed with aortic dissection. ...
Systemic lupus erythematosus is a chronic autoimmune disease with a wide variety of clinical presentations induced by different immunocomplexes and autoantibodies. Antiphospholipid antibody syndrome (APLAS) is a life-threatening clinical condition characterized by venous and arterial thromboses or pregnancy morbidity in the presence of persistent moderate/high levels of antiphospholipid antibodies. Aortic dissection is rarely associated with APLAS and always requires prompt diagnosis and early treatment. We report a rare case with a striking presentation. The patient developed multi-organ failure due to lethal aortic dissection and the obstruction of abdominal and thoracic branch vessels.
Learning points:
Aortic dissection is a rare lethal clinical condition that always requires prompt diagnosis and early treatment.Signs of multi-organ ischaemia due to obstruction of abdominal and thoracic branch vessels should be kept in mind by clinicians.Venous thrombosis and medial wall necrosis in the aorta may be underlying complex pathophysiological mechanisms in patients with antiphospholipid antibody syndrome.
... Spontaneous coronary artery dissection (SCAD) is rare with a prevalence reportedly as low as 0.2% of all angiograms and 0.07% in men or 4% of coronary angiograms performed for acute coronary syndrome (ACS) cases. 1 2 There are few case reports on SCAD in association with systemic inflammatory disease but even fewer with antiphospholipid antibody or antiphospholipid syndrome. [3][4][5][6][7][8][9] SCAD almost always presents as ACS with elevated cardiac troponin and it predominantly occurs in women with reports of over 90% of patients being women. [10][11][12][13][14][15] We present a rare case of a male patient who had chronic SCAD of the right coronary artery (RCA) which presented as stable angina in association with a known diagnosis of antiphospholipid syndrome. ...
A 52-year-old man presented to our cardiology service for an elective diagnostic coronary angiogram for risk stratification in the context of stable angina. He was diagnosed with antiphospholipid syndrome 2 years prior and had three known thrombotic episodes in the form of a stroke, retinal artery occlusion and deep vein thrombosis. Our initial differential was atherosclerotic coronary artery disease, however, coronary angiography demonstrated a dominant right coronary artery with a long segment of chronic spontaneous dissection distally but with thrombolysis in myocardial infarction III flow. He was treated medically with antianginals which rendered him asymptomatic and is currently on regular follow-up in the cardiology outpatient department.
... Spontaneous coronary artery dissection has been associated with systemic inflammatory conditions (Supplementary material online, Table S2). 33,[68][69][70][71][72][73][74][75][76][77][117][118][119][120][121][122][123][124] While the prevalence of inflammatory systemic diseases was 8.9% in a Canadian series of 168 cases, 4 this is not yet widely corroborated in other series. 11 A clear mechanistic link between systemic inflammation and SCAD remains to be elucidated. ...
... Spontaneous coronary artery dissection has been associated with systemic inflammatory conditions (Supplementary material online, Table S2). 33,[68][69][70][71][72][73][74][75][76][77][117][118][119][120][121][122][123][124] While the prevalence of inflammatory systemic diseases was 8.9% in a Canadian series of 168 cases, 4 this is not yet widely corroborated in other series. 11 A clear mechanistic link between systemic inflammation and SCAD remains to be elucidated. ...
A new Study Group for this unusual and serious condition was announced at ESC Congress in Rome by the Acute Cardiovascular Care Association (ACCA) of the ESC.
Spontaneous Coronary Artery Dissection (SCAD) is an increasingly recognized cause of non-atherosclerotic acute coronary syndromes afflicting predominantly younger women. It is characterized by a separation of the layers of the coronary arterial wall by an accumulation of blood to form a false lumen. A build-up of pressure within the false lumen leads to external compression of the true coronary lumen restricting coronary blood flow and leading to myocardial ischaemia or infarction. SCAD should be distinguished from atherosclerotic dissections arising from plaque rupture events or erosions and from traumatic dissections or iatrogenic dissections arising during coronary procedures.
Historically SCAD was primarily considered to be a condition of pregnancy or associated with known connective tissue disorders. However, it is now clear these cases make up a small proportion of the prevalent population and most events occur unheralded in patients with minimal cardiovascular risk factors.1–3 In this conventionally low-risk group, diagnosis is frequently missed or significantly delayed. Furthermore, characteristic angiographic and intracoronary imaging appearances are not widely recognized, partly because of a common misconception that a visible dual lumen or linear dissection ‘flap’ will usually be present.2,4
Accurate diagnosis of SCAD is important because of key differences in management compared to atherosclerotic coronary disease. Success rates following revascularisation are lower in SCAD and if conservative management is possible (e.g. in haemodynamically stable patients with TIMI 3 flow in the infarct related artery), the dissection usually heals over a few weeks/months.5 Stenting may be essential to restore coronary blood flow but is complicated by the risk of proximal and distal migration of the mural haematoma such that long lengths of stenting may be required to restore coronary integrity and flow. Bypass surgery can be used as a bail out where percutaneous coronary intervention has failed or for high-risk left main stem or proximal dissections but longevity of grafts in the context of SCAD is reduced by healing of the native coronary and subsequent competitive flow leading to high-graft occlusion rates.
Management of SCAD-survivors is challenging with considerable uncertainty about the optimal approach. For example, antiplatelet therapy, whilst required in patients following coronary stenting, can precipitate menorrhagia and the indication in conservatively managed patients for a condition whose primary pathophysiological event is an intramural bleed, is less clear, especially after the acute phase. Likewise, the use of statins has been questioned for a condition whose primary pathophysiology appears distinct from atherosclerosis and unrelated to cholesterol. Furthermore, SCAD patients face particular questions unusual in an atherosclerotic population, such as about safe contraception and the risk of pregnancy. Of particular concern is recurrent SCAD which is well recognized and may affect as many as one in four patients over 5 years.4,6
To date research in Europe has been limited to a number of national registries, small clinical studies, and case series. Globally the largest reported series contain just a few hundred cases. As a result, SCAD represents a substantial area of unmet clinical need.
There is therefore an urgent necessity to coordinate research internationally to enable larger numbers of patients to be studied. This will advance our knowledge of the epidemiology, pathophysiology, and clinical management of SCAD. With this aim, an inaugural meeting was held to welcome the SCAD Study Group within the Acute Cardiovascular Care Association of the ESC at the recent ESC Congress in Rome. Support from the European Fibromuscular Dysplasia (FMD) Group (a condition which occurs in a significant proportion of SCAD patients1–3) was especially welcome. The aims of the Study Group are:
•To establish a collaborative partnership to advance research into SCAD
•To maintain a European registry of SCAD patients to advance understanding of epidemiology and variations in patient management and outcomes
•To coordinate and support clinical and pre-clinical research into SCAD
•To formulate and disseminate a European consensus on the diagnosis and management of SCAD
•To improve accurate diagnosis by raising awareness of SCAD
•To support patients with this condition
The Study Group welcomes any interested clinicians to make contact and hope by working together we can make an important contribution to better understanding SCAD and improving our care and support for SCAD-survivors.
... Faz III'te ise fibrozis ve stenozun neden olduğu distal bölgede kalan organ ve uzuvlarda iskemik bulgular görülür. 17,18 Özellikle aortik arkın etkilenmesi Faz II ve III'te ortaya çıkar. Serebral dalların da etkilendiği son dönemde vertigo, inme, baş dönmesi, nöbet gibi nörolojik semptomlar ön plandadır. ...
Takayasu Arteritis (TA) is a chronic major vein vasculitis mainly characterized by elastic artery involvement such as aorta, main branches of aorta and pulmonary arteries. Yearly incidence of the disease is 2.6/million and it is mostly seen in females between 20-30 years of age. Morbidity of the disease is related to organ ischemia development due to arterial stenosis. Systemic symptoms in acute period and different symptoms according to localization of the stiff veins in chronic period occur. Our aim in this compilation was to cover TA demonstrating itself as a back and shoulder pain based on our 20 year-old male case.
... The youngest was 9 years, and the oldest was 68 years (mean age, 41.4 ± 16.4 years); 6 patients (35.3%) were older than 50 years (Table 1). [8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27] The age range of our cohort was not significantly different from that of the reported cases worldwide (P = .94; Figure 1). ...
... Female Left internal carotid artery Gerede et al 27 34 ...
... Internal carotid artery and coronary artery involvement have also been reported. [25][26][27] Therefore, we postulate that the dissection can occur at any position of an affected vulnerable elastic vessel and is the result of an interaction of the vascular wall inflammation with hemodynamic turbulence. In this cohort, the location most susceptible to dissection was the carotid artery. ...
Objectives:
Takayasu arteritis is a relatively rare chronic nonspecific form of large-vessel vasculitis in which the involved vessels develop stenoses, occlusions, dilatations or aneurysms, and dissections. Dissections of the arteries in patients with Takayasu arteritis are not well understood. In this study, we explored the sonographic characterization of these rare complications secondary to Takayasu arteritis.
Methods:
We evaluated arterial dissections in 72 patients with a clinical diagnosis of Takayasu arteritis by vascular sonography and transthoracic echocardiography. We analyzed the dissection distribution and morphologic characteristics of the dissected intima/ layer and lumen of the involved vessels.
Results:
Twelve of 72 patients had arterial dissections, in whom 16 dissected segments were identified. The involved arteries included the carotid, subclavian, vertebral, brachial, celiac, and femoral arteries, aortic arch, and abdominal aorta. The dissection lesions occurred at any age and coexisted with the aforementioned injuries. Evaluation of the dissected intima/layer and the involved lumen by sonography was technically complicated. The morphologic characteristics of the dissected intimae and involved lumens were complex, with most of the dissected intimae/layers having the characteristic "macaroni" sign in the arterial wall. High-frequency and high-resolution sonography, color Doppler flow imaging, and other techniques were useful in elucidating greater lesion details.
Conclusions:
Arterial dissections in Takayasu arteritis can involve any anatomic vessel location and have complex morphologic characteristics. Sonography is the optimal technique for diagnosis and follow-up of patients with Takayasu arteritis and dissections.
