Renal artery involvement is associated with increased morbidity but not mortality in Takayasu arteritis: a matched cohort study of 215 patients.

BACKGROUND: We analyzed differences in presentation and survival of Takayasu arteritis (TAK) with or without renal artery involvement (RAI) from a large monocentric cohort of patients with TAK. METHODS: Clinical and angiographic features were compared between TAK with versus without RAI, with bilateral versus unilateral RAI, and with bilateral RAI versus without RAI using multivariable-adjusted logistic regression. Inter-group differences in survival were analyzed [hazard ratios (HR) with 95% confidence intervals (95%CI)] adjusted for gender, age at disease onset, diagnostic delay, baseline disease activity, and significant clinical/angiographic inter-group differences after multivariable-adjustment/propensity score matching (PSM). RESULTS: Of 215 TAK, 117(54.42%) had RAI [66(56.41%) bilateral]. TAK with RAI or with bilateral RAI had earlier disease onset than without RAI (p < 0.001). Chronic renal failure (CRF) was exclusively seen in TAK with RAI. TAK with RAI (vs without RAI) had more frequent hypertension (p = 0.001), heart failure (p = 0.047), abdominal aorta (p = 0.001) or superior mesenteric artery involvement (p = 0.018). TAK with bilateral RAI (vs unilateral RAI) more often had hypertension (p = 0.011) and blurring of vision (p = 0.049). TAK with bilateral RAI (vs without RAI) more frequently had hypertension (p = 0.002), heart failure (p = 0.036), abdominal aorta (p < 0.001), superior mesenteric artery (p = 0.002), or left subclavian artery involvement (p = 0.041). Despite higher morbidity (hypertension, CRF), mortality risk was not increased with RAI vs without RAI (HR 2.32, 95%CI 0.61-8.78), with bilateral RAI vs unilateral RAI (HR 2.65, 95%CI 0.52-13.42) or without RAI (HR 3.16, 95%CI 0.79-12.70) even after multivariable adjustment or PSM. CONCLUSION: RAI is associated with increased morbidity (CRF, hypertension, heart failure) but does not adversely affect survival in TAK. Key Points •Renal artery involvement in TAK is associated with chronic renal failure. •TAK with renal artery involvement more often have heart failure and hypertension. •Bilateral renal artery involvement (compared with unilateral) is more often associated with hypertension and visual symptoms. •Renal artery involvement is not associated with an increased risk of mortality in TAK.

Validation of the 2022 American College of Rheumatology/EULAR classification criteria for Takayasu arteritis.

OBJECTIVES: The present study validates the 2022 ACR/European Alliance of Associations for Rheumatology (EULAR) classification criteria for Takayasu's arteritis (TAK), compared with the 1990 ACR TAK classification criteria. METHODS: The fulfilment of 2022 ACR/EULAR and 1990 ACR TAK criteria from four referral centres was assessed for TAK compared with extracranial giant cell arteritis (EC-GCA) and other controls. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), likelihood ratio of a positive test (LR+) or negative test (LR-), and area under receiver operating characteristics curve (AUC) were calculated. RESULTS: Among 504 patients with TAK (404 females) and 222 controls (151 females, 144 patients with EC-GCA), the 2022 ACR/EULAR criteria had better sensitivity (95.83% vs 82.94%) and NPV, but poorer specificity (63.51% vs 90.54%), PPV, LR+, LR- and AUC at the pre-determined cut-offs than the 1990 ACR criteria. The 2022 ACR/EULAR criteria had greater specificity (76.06% vs 57.62%) and AUC (0.845 vs 0.771), with similar sensitivity (93% vs 96.53%) in males as in females. The 2022 ACR/EULAR criteria performed similarly with only EC-GCA as controls (sensitivity 95.83%, specificity 60.42%, AUC 0.781). Sensitivity remained similar, whereas specificity was higher for 40-60 years vs <40 years. Cut-offs of ≥6 (sensitivity 91.87%, specificity 82.88%) and ≥7 (sensitivity 86.71%, specificity 86.49%), or removing the point for female sex (sensitivity 92.64%, specificity 81.08%) greatly improved the balance between sensitivity and specificity. CONCLUSION: The poor specificity of the 2022 ACR/EULAR TAK criteria in real-life settings was improved by increasing the cut-off to 6 or 7, or removing the point for female sex.

Presentation and clinical course of pediatric-onset versus adult-onset Takayasu arteritis-a systematic review and meta-analysis.

Takayasu arteritis (TAK) is a less common large-vessel vasculitis which can occur in either children or adults. However, differences between pediatric-onset and adult-onset TAK have not been systematically analyzed. We undertook a systematic review (pre-registered on PROSPERO, identifier CRD42022300238) to analyze differences in clinical presentation, angiographic involvement, treatments, and outcomes between pediatric-onset and adult-onset TAK. We searched PubMed (MEDLINE and PubMed Central), Scopus, major recent international rheumatology conference abstracts, Cochrane database, and clinicaltrials.gov, and identified seven studies of moderate to high quality comparing pediatric-onset and adult-onset TAK. Meta-analysis of 263 pediatric-onset and 981 adult-onset TAK suggested that constitutional features (fever, and in subgroup analyses, weight loss), hypertension, headache, and sinister features of cardiomyopathy, elevated serum creatinine, and abdominal pain were more frequent in pediatric-onset TAK, whereas pulse loss/pulse deficit and claudication (particularly upper limb claudication) were more frequent in adult-onset TAK. Hata's type IV TAK was more common in pediatric-onset TAK, and Hata's type I TAK in adult-onset TAK. Children with TAK also appeared to require more intense immunosuppression with more frequent use of cyclophosphamide, biologic DMARDs, tumor necrosis factor alpha inhibitors, and, in subgroup analyses, tocilizumab in pediatric-onset TAK than in adult-onset TAK. Surgical or endovascular procedures, remission, and risk of mortality were similar in both children and adults with TAK. No studies had compared patient-reported outcome measures between pediatric-onset and adult-onset TAK. Distinct clinical features and angiographic extent prevail between pediatric-onset and adult-onset TAK. Clinical outcomes in these subgroups require further study in multicentric cohorts.

A Case of Acute Ischemic Stroke Treated with Endovascular Treatment for Tandem Occlusion of the Common Carotid Artery and Internal Carotid Artery Terminal Portion Related to Takayasu Arteritis.

Objective: This report presents a case of mechanical thrombectomy for left internal carotid artery (ICA) terminal portion occlusion performed by left common carotid artery recanalization during hospitalization after diagnosing bilateral common carotid artery occlusion due to Takayasu arteritis. Case Presentation: A 25-year-old woman with fever and cervix pain a few months ago visited our department after repeated transient aphasic attacks. Magnetic resonance imaging (MRI) demonstrated diffuse infarction in the left middle cerebral artery (MCA) area, and she was diagnosed with Takayasu arteritis due to bilateral common carotid artery occlusion and left subclavian artery stenosis. On the 20th day of hospitalization, the terminal portion of the left ICA was occluded and thrombectomy was performed after balloon dilation of the left common carotid artery. Lastly, left common carotid artery stenting was performed. Aphasia and sensory disturbance remained, but she was transferred to a rehabilitation hospital with a modified Rankin Scale (mRS) of 2 on the 65th day of hospitalization. Antithrombotic and immunosuppressive therapy were performed, and restenosis did not develop. Conclusion: Angioplasty and stenting of common carotid artery occlusion can be effective treatments in thrombectomy for intracranial occlusion due to Takayasu disease.

Successful Hybrid Neurovascular Surgery for a Ruptured Basilar Artery Dissecting Aneurysm in a Patient with Jeopardized Collateral Circulation Associated with Aortitis Syndrome.

BACKGROUND: Dissecting aneurysms of the basilar artery (BA) are rare lesions, and the management of them has been controversial and challenging. The treatment becomes much more difficult when the patient has a disorder such as aortitis syndrome, which makes cerebral arteries occluded and tortuous. We describe a case of a ruptured BA dissecting aneurysm associated with aortitis syndrome, successfully treated with hybrid neurovascular surgery. CASE DESCRIPTION: A 64-year-old woman with a medical history of aortitis syndrome developed a severe headache and was diagnosed with a subarachnoid hemorrhage from a BA dissecting aneurysm. Her right common carotid artery and right vertebral artery (VA) were occluded because of aortitis syndrome. Blood flow in the right middle cerebral artery (MCA) was mainly supplied by the right posterior communicating artery. It was essential to preserve blood flow in the BA to prevent a cerebral infarction in the area of the right MCA. Stent-assisted coil embolization was considered the most appropriate treatment. The occluded right VA was thought to be more appropriate for introduction of an intravascular stent than the left VA. The patient underwent open surgery to expose the right VA. An intravascular stent was placed through the exposed right VA, and intra-aneurysmal coils were introduced from the left VA. The aneurysm was successfully treated with a hybrid technique. CONCLUSIONS: To our knowledge, this is the first report of hybrid neurovascular surgery for a BA dissecting aneurysm. A ruptured BA dissecting aneurysm in a patient with aortitis syndrome was successfully treated with hybrid neurovascular surgery.

Axillobifemoral Bypass for Aortitis Syndrome in a Living-Donor Liver Transplant Patient.

A 64-year-old female patient with aortitis syndrome presented with progressive intermittent claudication for 6 months. Her medical history was notable for living-donor liver transplantation for primary biliary cirrhosis 4-years prior and chronic immunosuppressive therapy. Evaluation included normal laboratory examination, and contrast-enhanced computed tomography angiography which demonstrated severely calcified descending aorta with high-grade stenosis below the diaphragm. The patient was treated by axillobifemoral bypass using an 8-mm ringed expanded polytetrafluoroethylene graft under general anesthesia. Medical management included decreased preoperative doses of immunosuppressants and predonisolone, which were resumed after the operation, and chronic anticoagulation. There were no postoperative complications.

A Case of Membranous Glomerulonephropathy Associated with Takayasu's Arteritis.

Glomerulonephropathy is a rare complication of Takayasu's arteritis (TA). To date, most glomerulonephropathies associated with TA show the histological feature of mesangial proliferation. Membranous glomerulonephropathy (MG) is a form of glomerulonephropathy in which the mesangial proliferation is not conspicuous and its association with TA is extremely rare. A 54-year-old man was referred to our hospital due to progressive edema in the lower limbs and nephrotic range proteinuria. Five years previously, he underwent percutaneous angioplasty for left subclavian artery stenosis. Kidney biopsy revealed stage II MG. General examination including enhanced CT scan confirmed the presence of TA. He started oral prednisolone therapy at a dose of 40 mg daily. The C-reactive protein level normalized 7 days after the prednisolone therapy. Three months later, proteinuria had remitted. Though the true relationship between MG and TA was not revealed in present case, considering the fact that complete remission of nephrotic syndrome occurred following the improvement of C-reactive protein level in response to steroid therapy, TA might be the secondary cause of MG. To our best knowledge, only two case reports described the association of MG and TA previously. Those two patients, however, also demonstrated the feature of systemic lupus erythematosus in addition to TA. This is the first case report that describes a patient who presented as MG associated with TA, but not complicated by systemic lupus erythematosus.

Imaging diagnosis and interventional radiology in systemic vasculitis with particular emphasis on Takayasu arteritis: Part 2 / 中国介入影像与治疗学

In part 1 of this article, clinical features and imaging findings of Takayasu arteritis (also known as aortitis syndrome) were described in detail. In part 2, treatment of Takayasu arteritis will be first described and discussed. This will be followed by description of clinical features and imaging findings of other systemic vasculitis. Comments on interventional radiology for systemic vasculitis will also be made.

Imaging diagnosis and interventional radiology in systemic vasculitis with particular emphasis on Takayasu arteritis: Part 1 / 中国介入影像与治疗学

This is a review article describing some new and interesting aspects in the diagnosis and treatment in systemic vasculitis and demonstrating several cases that we have encountered. Particular emphasis will be put on Takayasu arteritis (also known as aortitis syndrome) most commonly observed in Japan and other oriental countries. CT and MRI better demonstrate the vessel wall abnormality of Takayasu arteritis than angiography. Importance of plain chest radiograph should also be kept in mind; it could provide a clue in the diagnosis of Takayasu arteritis in its early stage.

Successful Emergency Operation for a Ruptured Anastomotic False Aneurysm of Atypical Coarctation Due to Aortitis Syndrome: Report of a Case / 日本心臓血管外科学会雑誌

A 37-year-old female was admitted to our hospital because of haemoptysis. She had undergone descending thoracic aorta-abdominal aorta bypass grafting 11 years previously. Then the diagnosis was atypical coarctation due to aortitis syndrome. No follow up had been continued. Angiogram and CTscan disclosed a false aneurysm at the anastomotic site of the descending thoracic aorta, which was ruptured into the left lung. An emergency operation was performed. A new extra-anatomical ascending aorta abdominal aorta bypass was constructed using 16mm Dacron prosthesis, and three permanent clamps were employed for thromboexclusion of the descending aorta, previous bypass graft and the ruptured aneurysm. At present, three years after the operation, she is leading normal life with medication of hypotensive drugs. Pathogenesis, surgical approach and long-term postoperative care were discussed.

Rare complications for aortitis syndrome / 日本心臓血管外科学会雑誌

Rare complications-1) Sarcoidosis, 2) Amyloidosis, 3) Phycomycosis-following surgical therapy for aortitis syndrome are reported. Sarcoidosis occurred in 39 y.o. female following Bentall operation for AAE and AR was diagnosed by biopsy and was controlled with drug completely 1 year after the onset. Amyloidosis found in 56 y. o. male after AVR and AAo plication for AAE and AR started with severe diarrhea and the diagnosis was made by autopsy. Phycomycosis was diagnosed by necropsy in 49 y. o. female after CABG and thoraco-abdominal bypass operation. Poor control of inflammation and administration of gluco-corticoid are the common problems for the 3 cases. Aortitis syndrome is autoimmune disease and some immunological factor has a role for the cause of the three complications. We must be very strict about the administration of the gludo-corticoid and the control of the inflammation.