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Annals of the Rheumatic Diseases Dec 2022To develop and validate new classification criteria for Takayasu arteritis (TAK).
OBJECTIVE
To develop and validate new classification criteria for Takayasu arteritis (TAK).
METHODS
Patients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in six phases: (1) identification of candidate criteria items, (2) collection of candidate items present at diagnosis, (3) expert panel review of cases, (4) data-driven reduction of candidate items, (5) derivation of a points-based classification score in a development data set and (6) validation in an independent data set.
RESULTS
The development data set consisted of 316 cases of TAK and 323 comparators. The validation data set consisted of an additional 146 cases of TAK and 127 comparators. Age ≤60 years at diagnosis and imaging evidence of large-vessel vasculitis were absolute requirements to classify a patient as having TAK. The final criteria items and weights were as follows: female sex (+1), angina (+2), limb claudication (+2), arterial bruit (+2), reduced upper extremity pulse (+2), reduced pulse or tenderness of a carotid artery (+2), blood pressure difference between arms of ≥20 mm Hg (+1), number of affected arterial territories (+1 to +3), paired artery involvement (+1) and abdominal aorta plus renal or mesenteric involvement (+3). A patient could be classified as having TAK with a cumulative score of ≥5 points. When these criteria were tested in the validation data set, the model area under the curve was 0.97 (95% CI 0.94 to 0.99) with a sensitivity of 93.8% (95% CI 88.6% to 97.1%) and specificity of 99.2% (95% CI 96.7% to 100.0%).
CONCLUSION
The 2022 American College of Rheumatology/EULAR classification criteria for TAK are now validated for use in research.
Topics: Humans; Female; Middle Aged; Takayasu Arteritis; Rheumatology; Carotid Arteries; Cohort Studies; Intermittent Claudication
PubMed: 36351705
DOI: 10.1136/ard-2022-223482 -
Advances in Experimental Medicine and... 2017Atherosclerotic Renal Artery Stenosis is a form or peripheral arterial disease that tends to affect older subjects with hyperlipidemia, history of tobacco use, and who... (Review)
Review
Atherosclerotic Renal Artery Stenosis is a form or peripheral arterial disease that tends to affect older subjects with hyperlipidemia, history of tobacco use, and who have other coexistent forms of vascular insufficiency. An abdominal bruit on physical exam can be a helpful clue. Slowly progressive, it can lead to critical narrowing of the renal arteries which creates a cascade of events such as renin-angiotensin-aldosterone activation (RAAS), hypertension, acute pulmonary edema, and renal fibrosis. The hypertension is considered a secondary form and can even be resistant to multiple antihypertensives. The diagnosis can be made with imaging (duplex ultrasound CT scans, MRA, or angiography). Because of the unique circulation to the kidney, stenting and angioplasty are rarely curative. This was confirmed in three recent large clinical trials. Therapy consists of lipid and blood pressure control, and dual anti-platelet agents. Because the disease activates the RAAS system, ace inhibitors and angiotensin receptor blockers can be useful agents but carry the risk of ischemic nephropathy, a form of acute kidney injury related to reduced renal blood flow after challenge with these agents. As such these agents are used with caution. Little is known about optimal blood pressure agents or the effect of lifestyle modification.
Topics: Atherosclerosis; Blood Pressure; Humans; Hypertension, Renovascular; Predictive Value of Tests; Renal Artery Obstruction; Risk Factors; Treatment Outcome
PubMed: 27873231
DOI: 10.1007/5584_2016_89 -
Deutsche Medizinische Wochenschrift... Oct 2018Chronic mesenteric ischemia (CMI) is defined by stenoses or occlusions of the celiac artery, superior or inferior mesenteric artery. While it is mostly cause by...
Chronic mesenteric ischemia (CMI) is defined by stenoses or occlusions of the celiac artery, superior or inferior mesenteric artery. While it is mostly cause by atherosclerotic disease, less frequent cause in particular in younger patients are fibrowmuscular dysplasia or vasculitis. Risk factors include smoking, hypertension, dyslipidemia, higher age, and female sex. Symptomatic CMI only accounts for less than 5 % of intestinal ischemic events. The prevalence of asymptomatic CMI is unknown but may be as high as 15 % and is more frequent in peripheral artery disease (27 %) and abdominal aortic aneurysm (40 %). The celiac artery is mostly affected. Abdominal pain that is aggravated with food intake with preserved appetite, abdominal bruits, and severe malnutrition can be important clinical hints that raise the suspicion of CMI. The diagnostic modality of choice is duplex ultrasound that should be performed in specialized centers potentially together with functional test causally linking symptoms to relevant arterial stenosis or intestinal ischemia. Typically, a stenosis of only one artery is unlikely to cause CMI. The therapy of choice is revascularization. In most cases angioplasty potentially together with stenting is performed due to low post-interventional mortality in mostly multi-morbid patients with excellent technical and clinical success rates. Non-atherosclerotic CMI in particular in younger patients may require surgery. Similar to the treatment of patients with other cardiovascular diseases, atherosclerosis of mesenteric arteries requires life style modification and optimal medical therapy for risk reduction. Platelet inhibition is indicated after revascularization.
Topics: Chronic Disease; Humans; Mesenteric Ischemia
PubMed: 30286488
DOI: 10.1055/a-0585-8049 -
Urology Jul 2023
PubMed: 37076018
DOI: 10.1016/j.urology.2023.04.004 -
Pediatric Nephrology (Berlin, Germany) Dec 2021Renovascular hypertension (RVH) is defined as an elevated blood pressure caused by kidney hypoperfusion, generally as a result of anatomic stenosis of the renal artery... (Review)
Review
Renovascular hypertension (RVH) is defined as an elevated blood pressure caused by kidney hypoperfusion, generally as a result of anatomic stenosis of the renal artery with consequent activation of the Renin Angiotensin-Aldosterone System. The main causes include genetic and inflammatory disorders, extrinsic compression, and idiopathic alterations. RVH is often asymptomatic and should be suspected in any child with refractory hypertension, especially if other suggestive findings are present, including those with severe hypertension, abdominal bruit, and abrupt fall of glomerular filtration rate after administration of angiotensin-converting enzyme inhibitors or angiotensin-receptor blockers. There is a consensus that digital subtraction angiography is the gold standard method for the diagnosis of RVH. Nevertheless, the role of non-invasive imaging studies such as Doppler ultrasound, magnetic resonance angiography, or computed tomographic angiography remains controversial, especially due to limited pediatric evidence. The therapeutic approach should be individualized, and management options include non-surgical pharmacological therapy and revascularization with percutaneous transluminal renal angioplasty (PTRA) or surgery. The prognosis is related to the procedure performed, and PTRA has a higher restenosis rate compared to surgery, although a decreased risk of complications. This review summarizes the causes, physiopathology, diagnosis, treatment, and prognosis of RVH in pediatric patients. Further studies are required to define the best approach for RVH in children.
Topics: Angioplasty, Balloon; Child; Humans; Hypertension, Renovascular; Renal Artery; Renal Artery Obstruction
PubMed: 33851262
DOI: 10.1007/s00467-021-05063-2 -
Cureus Jul 2021Celiac artery compression syndrome (CACS), also known as median arcuate ligament syndrome, celiac axis syndrome, and Dunbar Syndrome, is a rare disorder that results...
Celiac artery compression syndrome (CACS), also known as median arcuate ligament syndrome, celiac axis syndrome, and Dunbar Syndrome, is a rare disorder that results from compression of the celiac artery by the median arcuate ligament. The following is a case that depicts an interesting presentation of a patient diagnosed with this rare condition. A 44-year-old male with a history of mutism was brought in by his family for weight loss of 100 lbs with intermittent abdominal pain, weakness and lethargy over a period of five years. His family reported that he had poor nutritional intake, and could only eat a small amount before he seemed to be in pain, and eventually refused to eat. He had no other prior medical history except for mutism, no family history of malignancy, no history of trauma, surgeries, smoking or substance use, and did not take any medications. Physical exam was largely unremarkable. Mesenteric vascular duplex demonstrated severe grade stenosis of the celiac trunk with post-stenotic velocity of 520 cm/sec. Contrast enhanced computed tomography angiography revealed acute angle J-configuration of the takeoff of the celiac axis, with stenosis at its origin and focal post-stenotic dilatation, confirming the diagnosis of CACS. CACS is an elusive diagnosis that should be considered in patients where other causes of abdominal pain and weight loss have been ruled out. The disease can present with the classic triad of post-prandial abdominal pain, weight loss, and an abdominal bruit. Imaging modalities including mesenteric vascular duplex, computed tomography abdominal angiography, magnetic resonance angiography and celiac artery angiography can help make the diagnosis. Treatment involves surgical decompression via division of the median arcuate ligament, with most patients experiencing significant and long-lasting relief from their symptoms.
PubMed: 34262830
DOI: 10.7759/cureus.16175 -
Seminars in Arthritis and Rheumatism Apr 2023To compare clinical and imaging characteristics in patients with Takayasu arteritis (TAK) and large vessel-giant cell arteritis (LV-GCA) in an Italian population.
OBJECTIVE
To compare clinical and imaging characteristics in patients with Takayasu arteritis (TAK) and large vessel-giant cell arteritis (LV-GCA) in an Italian population.
METHODS
We conducted a retrospective monocenter study comparing characteristics and outcomes of a cohort of 59 patients with TAK and a cohort of 127 patients with LV-GCA diagnosed between 1996 and 2016. Most of them (92%) were followed up for at least 24 months at Reggio Emilia Hospital (Italy). We also reviewed the literature discussing the results of the published manuscripts comparing LV-GCA to TAK RESULTS: LV-GCA patients had a higher prevalence of males (p = 0.003), and more frequently presented with cranial symptoms (p = 0.001), fever ≥38 °C (p = 0.007), polymyalgia rheumatica (p = 0.001), and hypertension (p = 0.001), and they had higher ESR levels at diagnosis (p = 0.0001). Differently, TAK patients had longer delay to diagnosis from the beginning of symptoms (p = 0.048), they presented more frequently with loss of pulses of large arteries (p = 0.0001), vascular bruits (p = 0.001), limb claudication (p = 0.003), myocardial infarction/angina (p = 0.03), and hypertension induced by renal artery stenosis (p = 0.001). Regarding treatment, TAK patients received a higher total and at 1 year cumulative prednisone doses (p = 0.0001 and p = 0.001, respectively), they had a longer duration of prednisone therapy (p = 0.008), and received during follow-up more frequently traditional immunosuppressants (p = 0.0001) and biological agents (p = 0.0001). Flares were more frequently observed in TAK patient (p = 0.001), while no differences were observed for long-term remission. New vascular procedures during the follow-up were more frequently performed in TAK patients (p = 0.0001). Regarding imaging at diagnosis, TAK patients had more frequently vascular stenosis/occlusion (p = 0.0001) and a higher number of vessels with structural damage per person (p = 0.0001), while LV-GCA patients had a higher number of inflamed vessels per person (p = 0.0001). Comparing the involved vascular districts at diagnosis for the presence of vessel inflammation and/or arterial damage, patients with LV-GCA had a more frequent involvement of thoracic and abdominal aorta (p = 0.024 and p = 0.007, respectively), and axillary, iliac and femoral arteries (p = 0.018, p = 0.002, and p = 0.0001. respectively), while in TAK patients, brachiocephalic, celiac, mesenteric and renal arteries were more frequently involved (p = 0.011, p = 0.019, p = 0.019, and p = 0.005, respectively). At imaging arterial damage at diagnosis was more frequently observed in TAK patients, specifically at common carotid, brachiocephalic, and subclavian arteries (p = 0.0001, p = 0.006, p = 0.0001, respectively) and descending aorta (p = 0.022). Regarding imaging during the follow-up, TAK patients developed more frequently new vascular stenosis/occlusion (p = 0.0001) and new vascular thickening (p = 0.002), no differences were observed for the development of new dilatation/aneurysm between the two vasculitides.
CONCLUSION
Patients with TAK and LV-GCA show a number of similarities and also differences. Indeed, it is unclear whether they are part of the same disease spectrum or they are different conditions. As more information regarding the pathogenesis and etiology becomes known, answers to these questions are like to be forthcoming.
Topics: Male; Humans; Female; Giant Cell Arteritis; Takayasu Arteritis; Retrospective Studies; Constriction, Pathologic; Prednisone; Carotid Arteries; Hypertension
PubMed: 36780709
DOI: 10.1016/j.semarthrit.2023.152173 -
Arthritis & Rheumatology (Hoboken, N.J.) Dec 2022To develop and validate new classification criteria for Takayasu arteritis (TAK).
OBJECTIVE
To develop and validate new classification criteria for Takayasu arteritis (TAK).
METHODS
Patients with vasculitis or comparator diseases were recruited into an international cohort. The study proceeded in 6 phases: 1) identification of candidate criteria items, 2) collection of candidate items present at diagnosis, 3) expert panel review of cases, 4) data-driven reduction of candidate items, 5) derivation of a points-based classification score in a development data set, and 6) validation in an independent data set.
RESULTS
The development data set consisted of 316 cases of TAK and 323 comparators. The validation data set consisted of an additional 146 cases of TAK and 127 comparators. Age ≤60 years at diagnosis and imaging evidence of large-vessel vasculitis were absolute requirements to classify a patient as having TAK. The final criteria items and weights were as follows: female sex (+1), angina (+2), limb claudication (+2), arterial bruit (+2), reduced upper extremity pulse (+2), reduced pulse or tenderness of a carotid artery (+2), blood pressure difference between arms of ≥20 mm Hg (+1), number of affected arterial territories (+1 to +3), paired artery involvement (+1), and abdominal aorta plus renal or mesenteric involvement (+3). A patient could be classified as having TAK with a cumulative score of ≥5 points. When these criteria were tested in the validation data set, the model area under the curve was 0.97 (95% confidence interval [95% CI] 0.94-0.99) with a sensitivity of 93.8% (95% CI 88.6-97.1%) and specificity of 99.2% (95% CI 96.7-100.0%).
CONCLUSION
The 2022 American College of Rheumatology/EULAR classification criteria for TAK are now validated for use in research.
Topics: Humans; Female; United States; Middle Aged; Takayasu Arteritis; Rheumatology; Carotid Arteries; Intermittent Claudication
PubMed: 36349501
DOI: 10.1002/art.42324 -
Revista Espanola de Enfermedades... Nov 2022A 74-year-old patient presented to our emergency department with melena for 24 hours, associated with postprandial abdominal pain, predominantly in the epigastrium, for...
A 74-year-old patient presented to our emergency department with melena for 24 hours, associated with postprandial abdominal pain, predominantly in the epigastrium, for one month. Urgent gastroscopy showed a pale mucosa with loss of vascular pattern in the gastric antrum, as well as several superficial ulcers, Forrest III, at that level and in the duodenal bulb highly suggestive of ischaemia. An abdominal computed tomography angiography (CTA) revealed a filiform celiac trunk, with calcified atherosclerotic plaques in the ostium, superior mesenteric artery and both renal arteries, with absence of enhancement in a large part of the intestinal wall, suggestive of ischaemia. An exploratory laparoscopy confirmed patchy ischaemia of the small intestine, as well as of the cecum, ascending colon, and portion of the transversus. Atherosclerosis is the most common cause of occlusive chronic mesenteric ischaemia. The 'classic triad' consisting of postprandial pain, weight loss and abdominal bruit, is found in only a minority of patients, with a significant percentage of paucisymptomatic patients due to abundant colateral circulation, making diagnosis difficult. The endoscopic finding of edema, erythema or signs of mucosal atrophy, as well as gastric or duodenal ulcers, not justified by other causes can guide us in the diagnosis. However, more distal sections of the digestive tract which are not accessible with the conventional endoscope can often be affected, and the absence of these findings, does not exclude the diagnosis. Abdominal CTA is the gold-standard imaging test. Early diagnosis of mesenteric ischaemia is a challenge in clinical practice. Recognizing its endoscopic signs can facilitate its early diagnosis and treatment.
Topics: Humans; Aged; Mesenteric Ischemia; Celiac Artery; Mesenteric Artery, Superior; Ischemia; Angiography; Acute Disease; Abdominal Pain
PubMed: 35545898
DOI: 10.17235/reed.2022.8857/2022 -
Journal of Investigative Medicine High... 2017Median arcuate ligament syndrome is a rare disorder that is clinically characterized by the triad of postprandial abdominal pain, weight loss, and often an abdominal...
Median arcuate ligament syndrome is a rare disorder that is clinically characterized by the triad of postprandial abdominal pain, weight loss, and often an abdominal bruit due to compression of the celiac artery by the median arcuate ligament. Given the nonspecific symptoms, this is a rare and difficult diagnosis to obtain. We present a patient with nonspecific abdominal pain in whom etiology was ultimately determined to be median arcuate ligament syndrome.
PubMed: 28904981
DOI: 10.1177/2324709617728750