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1.
Semin Arthritis Rheum ; 66: 152446, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38669786

ABSTRACT

BACKGROUND: Immunosuppressive (IS) agents are recommended for the first-line treatment of patients with active Takayasu's arteritis (TAK) together with glucocorticoids (GCs). However, there is limited data comparing the efficacy and outcomes of different IS agents for this purpose. OBJECTIVES: In this study, we aimed to compare the outcomes of two most frequently used first-line IS agents, namely methotrexate (MTX) and azathioprine (AZA) in TAK patients. METHODS: TAK patients who received any IS agent in addition to GCs as the initial therapy were included in this multicentre, retrospective cohort study. Clinical, laboratory and imaging data of the patients were assessed. In addition, a matched analysis (cc match) using variables 'age', 'gender' and 'diffuse aortic involvement' was performed between patients who received MTX or AZA as the first-line IS treatment. RESULTS: We recruited 301 patients (F/M: 260/41, mean age: 42.2 ± 13.3 years) from 10 tertiary centres. As the first-line IS agent, 204 (67.8 %) patients received MTX, and 77 (25.6 %) received AZA. Less frequently used IS agents included cyclophosphamide in 17 (5.6 %), leflunomide in 2 (0.5 %) and mycophenolate mofetil in one patient. The remission, relapse, radiographic progression and adverse effect rates were similar between patients who received MTX and AZA as the first-line IS agent. Vascular surgery rate was significantly higher in the AZA group (23% vs. 9 %, p = 0.001), whereas the frequency of patients receiving ≤5 mg/day GCs at the end of the follow-up was significantly higher in the MTX group (76% vs 62 %, p = 0.034). Similarly, the rate of vascular surgery was higher in AZA group in matched analysis. Drug survival was similar between MTX and AZA groups (median 48 months, MTX vs AZA: 32% vs 42 %, p = 0.34). IS therapy was discontinued in 18 (12 MTX, 6 AZA) patients during the follow-up period due to remission. Among those patients, two patients had a relapse at 2 and 6 months, while 16 patients were still on remission at the end of a mean 69.4 (±50.9) months of follow-up. CONCLUSIONS: Remission, relapse, radiographic progression and drug survival rates of AZA and MTX were similar for patients with TAK receiving an IS agent as the first-line f therapy. The rate of vascular surgery was higher and the rate of GC dose reduction was lower with AZA compared to MTX at the end of the follow-up.


Subject(s)
Azathioprine , Immunosuppressive Agents , Methotrexate , Takayasu Arteritis , Humans , Takayasu Arteritis/drug therapy , Takayasu Arteritis/diagnostic imaging , Female , Male , Adult , Azathioprine/therapeutic use , Methotrexate/therapeutic use , Immunosuppressive Agents/therapeutic use , Retrospective Studies , Middle Aged , Treatment Outcome , Glucocorticoids/therapeutic use , Glucocorticoids/administration & dosage
2.
Clin Rheumatol ; 43(6): 1979-1987, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38598024

ABSTRACT

OBJECTIVE: The goal of the present study was to investigate the correlation between serum 25-hydroxyvitamin D [25(OH)D] levels and disease remission in Takayasu arteritis (TAK) patients. METHODS: This retrospective study included 59 patients in the study group and 80 patients in the validation cohort with TAK. After 6 months of therapy, patients were re-evaluated, and serum 25(OH)D levels were compared before and after treatment. Correlations between changes in 25(OH)D levels and changes in disease activity scores (NIH, ITAS2010, ITAS.A) were analyzed. Additionally, a predictive cut-off value for disease remission was determined. RESULTS: After 6 months of therapy, serum 25(OH)D levels in TAK patients significantly increased compared to baseline [(18.33 ± 7.25)µg/L vs (11.77 ± 4.14) µg/L] (P < 0.001). Positive correlations were observed between the increasing changes in the 25(OH)D level and the decreasing changes in the reduced NIH, ITAS2010, and ITAS.A scores (r = 0.455, P < 0.001; r = 0.495, P < 0.001; and r = 0.352 P = 0.006, respectively). A change of 8.45 µg/L in 25(OH)D level was identified as the predictive cut-off value for TAK remission (sensitivity 54.1%, specificity 90.9%, area under the curve = 0.741). Similarly for patients with normal baseline ESR, sensitivity is 68.0%, specificity is 92.3%, and area under the curve is 0.831, and for patients with normal baseline CRP, sensitivity is 58.3%, specificity is 90.0%, and area under the curve is 0.748. Validation in an additional 80 patients demonstrated a higher remission rate in those with a 25(OH)D level change > 8.45 µg/L. CONCLUSION: Serum 25(OH)D levels significantly increased after treatment in TAK patients, and an increase of ≥ 8.45 µg/L was predictive of disease remission, especially in individuals with normal baseline ESR and/or CRP levels. Key Points • Following treatment, there was a significant increase in serum 25(OH)D levels among TAK patients. • The elevated changes in 25(OH)D levels before and after treatment demonstrated a positive correlation with the reduction in disease activity scores. • In patients with TAK before and after treatment, an elevation in serum 25(OH)D levels exceeding 8.45 µg/L serves as an indicator for disease remission, particularly prominent in individuals with normal baseline ESR and/or CRP levels.


Subject(s)
Blood Sedimentation , C-Reactive Protein , Remission Induction , Takayasu Arteritis , Vitamin D , Humans , Female , Retrospective Studies , Male , Vitamin D/analogs & derivatives , Vitamin D/blood , Adult , Takayasu Arteritis/blood , Takayasu Arteritis/drug therapy , C-Reactive Protein/metabolism , C-Reactive Protein/analysis , Young Adult , Middle Aged , Severity of Illness Index , Adolescent , Biomarkers/blood
3.
Clin Exp Rheumatol ; 42(4): 843-851, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38607693

ABSTRACT

OBJECTIVES: Coronary artery calcification (CAC) is frequently observed in Takayasu's arteritis (TAK). Our objective is to calculate the prevalence and severity of CAC in TAK, while evaluating the influence of traditional cardiovascular risk factors, glucocorticoid exposure, and disease activity on CAC. METHODS: This retrospective study involved 155 TAK patients. We measured the Agatston score by coronary computed tomography angiography (CCTA) and categorised all patients into groups with or without CAC (41 vs. 114) to compare clinical characteristics and ancillary findings between the two groups. RESULTS: Among the TAK patients, a total of 41 TAK patients (26.45%) exhibited CAC. Age of onset, disease duration, history of hypertension, history of hyperlipidaemia, Numano V and glucocorticoid use emerged as the independent risk factors for developing CAC in TAK (OR [95% CI] 1.084[1.028-1.142], p=0.003; 1.005 [1.001-1.010], p=0.020; 4.792 [1.713-13.411], p=0.003; 4.199 [1.087-16.219], p=0.037; 3.287 [1.070-10.100], p=0.038; 3.558[1.269-9.977], p=0.016). Nonetheless, CAC was not associated with disease activity. Moreover, the extent of calcification score in TAK showed a positive correlation with the number of traditional cardiovascular risk factors. CONCLUSIONS: We recommend CCTA screening for Numano V classified TAK patients. Glucocorticoid usage significantly escalates the risk of CAC. Therefore, in cases of effectively controlled disease, the inclusion of immunosuppressants aimed at reducing glucocorticoid dosage is advisable.


Subject(s)
Computed Tomography Angiography , Coronary Angiography , Coronary Artery Disease , Takayasu Arteritis , Vascular Calcification , Humans , Takayasu Arteritis/diagnostic imaging , Takayasu Arteritis/epidemiology , Takayasu Arteritis/drug therapy , Takayasu Arteritis/complications , Female , Male , Retrospective Studies , Adult , Coronary Artery Disease/diagnostic imaging , Coronary Artery Disease/epidemiology , Coronary Artery Disease/etiology , Vascular Calcification/diagnostic imaging , Vascular Calcification/epidemiology , Middle Aged , Risk Factors , Prevalence , Severity of Illness Index , Glucocorticoids/therapeutic use , Glucocorticoids/adverse effects , Young Adult , Heart Disease Risk Factors
4.
RMD Open ; 10(1)2024 Mar 22.
Article in English | MEDLINE | ID: mdl-38519108

ABSTRACT

OBJECTIVE: To investigate the treatment efficacy and safety of baricitinib in patients with refractory Takayasu arteritis (TAK). METHODS: We performed a prospective cohort study in which baricitinib 4 mg daily was prescribed to patients with refractory TAK, combined with oral glucocorticoids (GCs). RESULTS: 10 patients with refractory TAK were enrolled with a median age of 28 (IQR=22-37) years, median disease duration of 50 (IQR=24-65) months. The median dose of GCs was 10 (IQR=8.1-22.5) mg prednisone or equivalence dosage at baseline. At 6 months of baricitinib treatment, 6/10 (60%) patients had an overall treatment response. During an average follow-up of 15.3 (range 4-31) months, 4/10 (40%) patients maintained overall treatment response. 8/10 (80%) patients tapered or maintained the same dose of GCs with no change of the combined classical synthetic disease-modifying antirheumatic drugs. Two patients discontinued GCs at 18 and 24 months and were in continuous remission till the end of the study. One patient withdrew baricitinib due to liver dysfunction. CONCLUSION: Baricitinib 4 mg daily is effective for refractory TAK and is well tolerated.


Subject(s)
Azetidines , Purines , Pyrazoles , Sulfonamides , Takayasu Arteritis , Humans , Infant , Child, Preschool , Prospective Studies , Takayasu Arteritis/diagnosis , Takayasu Arteritis/drug therapy , Tertiary Care Centers , Azetidines/adverse effects , Glucocorticoids/therapeutic use
5.
Medicina (Kaunas) ; 60(3)2024 Mar 09.
Article in English | MEDLINE | ID: mdl-38541182

ABSTRACT

Background: Takayasu's arteritis is a rare type of vasculitis with severe complications like stroke, ischemic heart disease, pulmonary hypertension, secondary hypertension, and aneurysms. Diagnosis is achieved using clinical and angiographic criteria. Treatment is medical and surgical, but unfortunately, the outcome is limited. Case presentation: A 34-year-old Caucasian woman had an ischemic stroke (2009). She was diagnosed with Takayasu's arteritis and received treatment with methotrexate, prednisolone, and antiplatelet agents, with a mild improvement in clinical state. After 6 years (2015), she experienced an ascending aorta aneurysm, pulmonary hypertension, and mild aortic regurgitation. Surgical treatment solved both the ascending aorta aneurysm and left carotid artery stenosis (ultrasound in 2009 and computed tomography angiogram in 2014). Morphopathology revealed a typical case of Takayasu's arteritis. Tumor necrosis factor inhibitors (TNF inhibitors) were prescribed with methotrexate. At 48 years old (2023), she developed coronary heart disease (angina, electrocardiogram); echocardiography revealed severe pulmonary hypertension, and angiography revealed normal coronary arteries, abdominal aorta pseudoaneurysm, and arterial-venous fistula originating in the right coronary artery with drainage in the medium pulmonary artery. The patient refused surgical/interventional treatment. She again received TNF inhibitors, methotrexate, antiplatelet agents, and statins. Conclusions: This case report presented a severe form of Takayasu's arteritis. Our patient had multiple arterial complications, as previously mentioned. She received immunosuppressive treatment, medication targeted to coronary heart disease, and surgical therapy.


Subject(s)
Aneurysm, Ascending Aorta , Coronary Disease , Hypertension, Pulmonary , Takayasu Arteritis , Adult , Female , Humans , Hypertension, Pulmonary/complications , Methotrexate , Platelet Aggregation Inhibitors , Takayasu Arteritis/complications , Takayasu Arteritis/diagnosis , Takayasu Arteritis/drug therapy , Tumor Necrosis Factor Inhibitors
6.
RMD Open ; 10(1)2024 Mar 04.
Article in English | MEDLINE | ID: mdl-38443091

ABSTRACT

OBJECTIVE: This study aims to evaluate the effectiveness and safety of adalimumab (ADA) compared with leflunomide (LEF) in patients with Takayasu arteritis (TAK). METHOD: A retrospective cohort study was performed with the following inclusion criteria: the fulfilment of the 2022 American College Classification/European Alliance of Associations for Rheumatology criteria for TAK, age ≥18 years, and written informed consent. Forty-four patients were treated with LEF (n=28) or ADA (n=16) therapy due to relapsing/refractory disease or toxicity from previous therapy. Patients were evaluated at baseline (T0), at a median of 7.0 months (T1) and at 15.0 months of follow-up (T2). Data regarding disease activity, daily dose of prednisone, side effects and angiographic progression were analysed. RESULTS: LEF and ADA groups had similar features on the baseline visit. However, intravenous methylprednisolone was more frequently prescribed for the ADA group (p=0.019). On T1 and T2 visits, complete response rates were similar for ADA and LEF groups (75.0% and 88.5%; p=0.397 and 62.5% vs 78.3%; p=0.307), respectively. The differences remained non-significant after adjusting for baseline variables by propensity score matching. Although the ADA group had a higher median daily prednisone on visit T1 (p=0.004), it was similar on visit T2 (p=0.595). Similar rates of angiographic progression were observed in ADA and LEF groups (40% vs 25%; p=0.467). Mild-to-moderate adverse events were observed only in the LEF group (17.9%). CONCLUSION: LEF and ADA had comparable outcomes after a median of 15.0 months of follow-up. However, withdrawal from therapy and mild-to-moderate adverse events were only observed in the LEF group.


Subject(s)
Takayasu Arteritis , Humans , Adolescent , Takayasu Arteritis/diagnosis , Takayasu Arteritis/drug therapy , Adalimumab/adverse effects , Leflunomide/adverse effects , Prednisone , Retrospective Studies
8.
Prague Med Rep ; 125(1): 62-68, 2024.
Article in English | MEDLINE | ID: mdl-38380455

ABSTRACT

Takayasu arteritis is a large vessel vasculitis, characterized by granulomatous inflammation of arterial vessels, that typically affects the aorta, its main branches and pulmonary arteries. Disease diagnosis is a challenge and requires awareness of the condition, as clinical signs can be not specific. We report a case of an adolescent with recurrent stroke diagnosed with Takayasu arteritis. A diagnosis of Takayasu arteritis was established due to angiographic findings in the magnetic resonance angiography in conjunction with systolic blood pressure discrepancy, arterial hypertension and increased acute phase reactants. Takayasu arteritis is a rare cause of ischemic stroke in children. However, stroke may be the first manifestation of the disease. Clinical experience and multidisciplinary approach, including aggressive treatment, is essential for the favourable outcome of the disease and the reduction of the associated morbidity and mortality.


Subject(s)
Hypertension , Takayasu Arteritis , Child , Humans , Adolescent , Takayasu Arteritis/complications , Takayasu Arteritis/diagnosis , Takayasu Arteritis/drug therapy , Magnetic Resonance Angiography , Cerebral Infarction , Pulmonary Artery
9.
Clin J Gastroenterol ; 17(2): 281-285, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38180710

ABSTRACT

A 23-year-old woman presented with fever, diarrhea, bloody stools, and arthralgia that did not improve despite previous treatments and was diagnosed with Crohn's disease. Remission was achieved after the introduction of infliximab, nutritional therapy, and 5-aminosalicylic acid treatment. However, the patient's blood sedimentation rate remained elevated without symptom recurrence, except for abdominal pain in the following year. Aortic wall thickening in the thoracic descending aorta was also observed on computed tomography. Accumulation in the thoracic descending aorta and abdominal aorta was confirmed using positron emission tomography-computed tomography. The patient was diagnosed with Takayasu's arteritis. The patient's abdominal symptoms resolved, and her blood sedimentation rate normalized after steroid administration.


Subject(s)
Crohn Disease , Takayasu Arteritis , Female , Humans , Young Adult , Adult , Crohn Disease/complications , Crohn Disease/drug therapy , Crohn Disease/diagnosis , Infliximab/therapeutic use , Takayasu Arteritis/complications , Takayasu Arteritis/diagnostic imaging , Takayasu Arteritis/drug therapy , Abdominal Pain , Diarrhea
11.
Rheumatol Int ; 44(3): 497-507, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38180499

ABSTRACT

The aim of this study is to present the current care situation of patients with giant cell arteritis (GCA), Takayasu arteritis (TAK), ANCA-associated vasculitis (AAV) and Behçet's disease (BD). Trends over the last 15 years will reflect improvements and remaining deficits in the management of vasculitides. Consecutive cross-sectional data from patients with vasculitides from the German National Database (NDB) of the Collaborative Arthritis Centres between 2007 and 2021 were included. Medication, physician- and patient-reported outcomes on disease activity and disease burden, inpatient stays and occupational participation are compared for different vasculitis entities and over time. Employment rates were compared to German population rates. Between 502 and 854 vasculitis patients were annually documented. GCA and AAV were the most common vasculitides. Median disease duration ranged from 2 to 16 years. Over the years, glucocorticoids decreased in proportion and dose, most markedly in GCA and TAK, while biologic therapies increased up to 27%. Physicians rated disease activity as low for the vast majority of patients, while patients-reported moderate outcomes in many dimensions. PROs remained largely unchanged. The proportion of employed patients (< 65 years) increased from 47 to 57%. In recent years, biologics are increasingly used in patients with vasculitides, while glucocorticoids decreased significantly. PRO's have not improved. Work participation increased but remains lower than that in the German population.


Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis , Behcet Syndrome , Giant Cell Arteritis , Takayasu Arteritis , Humans , Giant Cell Arteritis/drug therapy , Giant Cell Arteritis/epidemiology , Behcet Syndrome/diagnosis , Behcet Syndrome/drug therapy , Behcet Syndrome/epidemiology , Takayasu Arteritis/drug therapy , Takayasu Arteritis/epidemiology , Cross-Sectional Studies , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/epidemiology , Glucocorticoids/therapeutic use , Delivery of Health Care , Giant Cells
12.
Clin Rheumatol ; 43(3): 1171-1181, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38253779

ABSTRACT

OBJECTIVES: We aimed to determine the prevalence of cardiovascular involvement in our Blau syndrome (BS) cohort and provide detailed analysis of their cardiovascular manifestations and outcome. We also tried to find out the risk factors for developing cardiovascular involvement. METHODS: Clinical manifestations, laboratory findings, and treatments were reviewed. Clinical features were compared between children with cardiovascular involvement and those without angiocardiopathy. RESULTS: A total of 38 BS children were eligible for final analysis. Among them, 13 (34.2%) developed Takayasu-like vasculitis and/or cardiopathy. Compared with those without angiocardiopathy, recurrent fever was more frequent in BS patients with cardiovascular involvement (p < 0.001). What is more, tumor necrosis factor alpha antagonists (anti-TNF) were more urgently needed in children with cardiovascular involvement (p = 0.015). BS patients with cardiovascular involvement include 4 with Takayasu-like vasculitis and 9 with cardiopathy. The onset of cardiovascular manifestations ranged from 0.75 to 18.5 years of age, with most cases occurring before school period. Symptoms were elusive and lacked specificity, such as dizziness, short of breath, and edema. Some patients were even identified because of the unexpected hypertension during follow-up. Cardiopathy and vasculitis occurred in patients with different genotypes. Imaging changes were discovered before the presentation of the typical triad in 3/4 patients with Takayasu-like vasculitis. Three children developed left ventricular dysfunction with decreased left ventricular ejection fraction. Combination of glucocorticoids and methotrexate with anti-TNF agents is a common treatment option for these BS patients. In the cohort, BS-related cardiovascular involvement was controlled well, with cardiac structural and functional abnormalities completely recovered and slower progression of vasculitis lesions. CONCLUSION: Cardiovascular manifestations is not rare in BS patients. Because of its insidious onset, a systematic and comprehensive assessment of cardiovascular involvement should be performed in newly diagnosed patients with BS. Aggressive initiation of anti-TNF agents may be beneficial to improve the prognosis. Key Points • About 34.2% patients with Blau syndrome developed Takayasu-like vasculitis and/or cardiopathy. • Compared with those without angiocardiopathy, recurrent fever and application of anti-TNF agents were more frequent in BS patients with cardiovascular involvement (p < 0.001, p = 0.015) • Regular assessment of cardiovascular involvement is extremely necessary because of its insidious onset.


Subject(s)
Arthritis , Heart Diseases , Sarcoidosis , Synovitis , Takayasu Arteritis , Uveitis , Vasculitis , Child , Humans , Tumor Necrosis Factor Inhibitors , Stroke Volume , Ventricular Function, Left , Phenotype , Takayasu Arteritis/complications , Takayasu Arteritis/drug therapy , Takayasu Arteritis/diagnosis
13.
Emerg Microbes Infect ; 13(1): 2302099, 2024 Dec.
Article in English | MEDLINE | ID: mdl-38166581

ABSTRACT

There is limited evidence to support the association between tuberculosis (TB) and the occurrence of Takayasu arteritis (TAK). To investigate the incidence of active TB (ATB) in TAK and explore the impact of anti-rheumatic therapy on the occurrence of ATB or reactivation of Latent TB infection (LTBI) and their effect on interferon-γ release assay (IGRA) results, we conducted a prospective study based on the Chinese Registry for Systemic Vasculitis cohort. The standard incidence ratio (SIR) was calculated and stratified by age. Kaplan-Meier analysis was used to determine the effect of variables on ATB or LTBI reactivation in patients with TAK. Data from 825 patients with TAK in the registry were analysed. During a median follow-up of 5 years, 5 patients developed ATB with a crude incidence of 154 (95%CI:57-381) person-years/100,000. The SIR was 5.59 (95%CI:1.81-13.04). Glucocorticoids and conventional disease-modifying anti-rheumatic drugs (cDMARDs) did not increase the risk of ATB or LTBI reactivation (P > 0.05). However, the use of tumour necrosis factor inhibitor (TNFi) increased the risk of ATB in patients with LTBI (P < 0.001). Furthermore, the value of the IGRA assay decreased after treatment (P < 0.05). In conclusion, the incidence of TB infection is markedly increased in patients with TAK and patients with TAK are at high risk of developing ATB. Treatment with glucocorticoids and cDMARDs does not significantly increase the risk for ATB in patients with TAK. Moreover, IGRA may have limited effectiveness in monitoring ATB infection or LTBI reactivation in patients with TAK.


Subject(s)
Antirheumatic Agents , Latent Tuberculosis , Takayasu Arteritis , Tuberculosis , Humans , Interferon-gamma Release Tests/methods , Prospective Studies , Incidence , Takayasu Arteritis/complications , Takayasu Arteritis/drug therapy , Tuberculosis/complications , Tuberculosis/epidemiology , Tuberculosis/drug therapy , Risk Factors , Latent Tuberculosis/epidemiology , Antirheumatic Agents/therapeutic use
14.
Annu Rev Med ; 75: 427-442, 2024 Jan 29.
Article in English | MEDLINE | ID: mdl-37683286

ABSTRACT

Giant cell arteritis (GCA) and Takayasu arteritis (TAK) are large-vessel vasculitides affecting the aorta and its branches. Arterial damage from these diseases may result in ischemic complications, aneurysms, and dissections. Despite their similarities, the management of GCA and TAK differs. Glucocorticoids are used frequently but relapses are common, and glucocorticoid toxicity contributes to significant morbidity. Conventional immunosuppressive therapies can be beneficial in TAK, though their role in the management of GCA remains unclear. Tumor necrosis factor inhibitors improve remission rates and appear to limit vascular damage in TAK; these agents are not beneficial in GCA. Tocilizumab is the first biologic glucocorticoid-sparing agent approved for use in GCA and also appears to be effective in TAK. A better understanding of the pathogenesis of both conditions and the availability of targeted therapies hold much promise for future management.


Subject(s)
Giant Cell Arteritis , Takayasu Arteritis , Humans , Glucocorticoids/therapeutic use , Giant Cell Arteritis/drug therapy , Takayasu Arteritis/drug therapy
17.
Arthritis Care Res (Hoboken) ; 76(4): 531-540, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38059340

ABSTRACT

OBJECTIVE: Accurate clinical assessment of disease activity in Takayasu arteritis (TAK) can be challenging. 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET) can directly measure vascular inflammation. This study details the development of a new type of disease activity index called the Takayasu's Arteritis Integrated Disease Activity Index (TAIDAI). METHODS: Clinical symptoms for TAIDAI were identified from a literature review. Each symptom was paired with FDG-PET findings in corresponding arterial territories. Constitutional symptoms were paired with acute phase reactant levels. One point was given for each clinical symptom paired with supporting FDG-PET or laboratory abnormalities and summed into the TAIDAI score. A TAIDAI of ≥1 defined active disease. To assess performance of TAIDAI, face validity, content validity, and sensitivity to change were evaluated within a prospective observational cohort study of patients with TAK. RESULTS: Seventeen clinical symptoms were paired with imaging or laboratory abnormalities. In a cohort of 96 patients contributing 204 study visits, TAIDAI showed excellent sensitivity (96.3%) and good specificity (79.2%) compared to physician's clinical assessment. TAIDAI significantly correlated with physician global assessment, PET Vascular Activity Score, patient global assessment, and acute phase reactant levels. In patients treated with either tumor necrosis factor inhibitors or tocilizumab, a TAIDAI of 0 was achieved in 21 (91%) of 23 patients who met a predefined definition of clinical response. CONCLUSION: TAIDAI is new type of disease activity index in TAK in which clinical symptoms are integrated with specific laboratory and imaging findings. TAIDAI should be validated in future randomized controlled trials in TAK.


Subject(s)
Takayasu Arteritis , Humans , Takayasu Arteritis/diagnostic imaging , Takayasu Arteritis/drug therapy , Fluorodeoxyglucose F18/therapeutic use , Prospective Studies , Positron-Emission Tomography/methods , Acute-Phase Proteins/therapeutic use , Observational Studies as Topic
18.
J Rheumatol ; 51(3): 277-284, 2024 Mar 01.
Article in English | MEDLINE | ID: mdl-38101913

ABSTRACT

OBJECTIVE: Takayasu arteritis (TA) leads to stenotic disease. Aneurysmal lesions are rarer. This study assessed the main characteristics of aneurysmal disease in a Canadian cohort of patients with TA. METHODS: This monocentric retrospective study included patients with TA followed at the Mount Sinai Hospital Vasculitis Clinic in Toronto. Diagnosis of TA was based on clinical findings and/or satisfied the 1990 American College of Rheumatology classification criteria. RESULTS: Seventy-four patients were included. At any time, aneurysmal disease was found in 23 (31%) patients. Median disease duration was 9.0 (IQR 7.0-19.0) years. Prior hypertension (P = 0.02), fever (P = 0.04), and seizure disorders (P = 0.03) were more common. Limb claudication was less frequent (P = 0.01). Persistent and/or new aneurysms were demonstrated in 22/23 patients at follow-up. Thoracic aorta aneurysm (13/22) was most common, followed by abdominal aorta (8/22), subclavian (7/22), and carotid (6/22) artery disease. Aortic valve regurgitation was more frequent (9/23 vs 3/48; P = 0.001). Twenty-one patients had been treated with glucocorticoids (median 6.1 years [IQR 3.7-8.1]). Methotrexate, azathioprine, and leflunomide were repeatedly used. Infliximab (7/23) was used more often (P = 0.04), whereas tocilizumab was received by only 4 patients with aneurysmal disease (P = 0.01). Patients with aneurysms suffered more frequent relapses (2.0 [IQR 0.0-4.0] vs 1.0 [IQR 0.0-2.0], P = 0.04). CONCLUSION: Aneurysmal disease was found in a significant proportion of patients with TA. Given that aneurysms may carry a risk of rupture, and are associated with a higher rate of relapse, this finding should be reported systematically in TA studies.


Subject(s)
Aneurysm , Hypertension , Takayasu Arteritis , Humans , Takayasu Arteritis/complications , Takayasu Arteritis/drug therapy , Canada/epidemiology , Retrospective Studies , Aneurysm/complications , Aneurysm/diagnostic imaging
19.
Hosp Pract (1995) ; 51(5): 303-305, 2023 Dec.
Article in English | MEDLINE | ID: mdl-37964534

ABSTRACT

Takayasu arteritis (TA) is a chronic granulomatous large vessel arteritis. The renal arteries are affected in up to 60% of patients with TA, with renal artery stenosis (RAS) potentially leading to ischemic nephropathy, severe arterial hypertension, and heart failure. Bilateral RAS may rarely present with recurrent flash pulmonary edema, a life-threatening association which has been termed Pickering syndrome. In this report, we describe a 55-year-old woman with severe refractory arterial hypertension admitted for acute pulmonary edema, initially treated unsuccessfully with medical therapy with vasodilators and diuretics. Given the instrumental findings of bilateral RAS and suggestive signs and symptoms, the diagnosis of TA was made, resulting as the first described case of Pickering syndrome being the clinical presentation of TA. Interventional therapy with renal artery angioplasty procedure was performed with stenting of both right and left renal arteries, leading to the resolution of the clinical scenario and the successful discharge of the patient. At the 1 year follow-up visit the patient was asymptomatic and in good clinical conditions; a significant reduction in antihypertensive therapy was achieved while immunosuppressive therapy was continued. This case highlights that secondary causes of TA should always be sought in patients with refractory hypertension who do not respond to standard treatment; also, TA should be suspected in young patients with bilateral RAS, especially when other typical signs of TA are present; lastly, a thorough investigation is essential in complicated cases, as rare diseases like TA may manifest in unusual ways.


Subject(s)
Hypertension , Pulmonary Edema , Takayasu Arteritis , Female , Humans , Middle Aged , Takayasu Arteritis/complications , Takayasu Arteritis/diagnosis , Takayasu Arteritis/drug therapy , Pulmonary Edema/complications , Vasodilator Agents/therapeutic use
20.
Int J Rheum Dis ; 26(12): 2517-2525, 2023 Dec.
Article in English | MEDLINE | ID: mdl-37875306

ABSTRACT

OBJECTIVES: To determine the role of plateletcrit as a potential biomarker for disease activity and treatment response in Takayasu arteritis (TAK). METHODS: Totally, 215 newly diagnosed TAK patients were consecutively enrolled. Demographic data, clinical manifestations, laboratory and imaging examinations, and treatment strategy were recorded at baseline and at each visit during the 6-month treatment period. Normal plateletcrit (0.1%-0.4%) and hyper-plateletcrit (>0.4%) observed at baseline were used as group criteria. RESULTS: At baseline, the overall plateletcrit was 0.32 (0.24-0.38)%, with a normal and high level observed in 172 (80.00%) and 43 (20.00%) patients, respectively. Baseline plateletcrit was significantly higher in patients with active disease and associated with inflammatory biomarkers, including erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and interleukin (IL)-6 (all p < .01). At 6 months, complete remission was achieved in 171 (79.53%) patients, and a significant decrease in plateletcrit was observed in these cases (p < .01). Patients with a normal baseline plateletcrit were more likely to achieve complete remission compared to those with a high baseline plateletcrit (HR = 4.65, 95% CI: 2.38-19.08, p < .01). In addition, ESR (p = .01) and IL-6 (p = .02) levels were still higher in patients with a high baseline plateletcrit at 6 months. Progression of vascular lesions was indicated in 18 (8.37%) patients at 6 months, and these patients also had significantly higher baseline plateletcrit (p = .03). CONCLUSION: Plateletcrit levels were positively related to disease activity and inflammatory index in TAK. Importantly, patients with high baseline plateletcrit levels may show a worse treatment response at 6 months.


Subject(s)
Takayasu Arteritis , Humans , Takayasu Arteritis/diagnosis , Takayasu Arteritis/drug therapy , Biomarkers , C-Reactive Protein/analysis , Blood Sedimentation , Interleukin-6
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