Limitations of therapy and a guarded prognosis in an american cohort of takayasu arteritis patients

Abstract

Objective. To describe the clinical, laboratory, and radiographic manifestations of Takayasu arteritis (TA) in a cohort from the US, evaluate the response to interventions, remission and relapse rates, and disease progression, and compare these observations with those from other cohorts in the US, Japan, India, Italy, and Mexico.Methods. Seventy-five patients were retrospectively studied using a uniform database that included clinical, laboratory, and imaging data. Vascular imaging studies were performed at least yearly to monitor disease progression.Results. Common manifestations at disease onset included loss or asymmetry of pulses (57%), limb blood pressure discrepancy (53%), and bruits (53%). Eleven percent of patients were asymptomatic prior to disease diagnosis. Initial angiographic studies showed aortic abnormalities in 79% of patients and frequent involvement of the subclavian (65%) and carotid (43%) arteries.Ninety-three percent of longitudinally followed patients attained disease remission of any duration, but only 28% sustained remission of at least 6 months' duration after prednisone was tapered to <10 mg daily. Both angioplasty and vascular surgery were initially successful, but recurrent stenosis occurred in 78% of angioplasty and 36% of bypass/reconstruction procedures. More than two-thirds of patients had difficulty performing routine daily activities and approximately one-fourth of all patients were unable to work.Our cohort was similar to the National Institutes of Health, Italian, Japanese, and Mexican cohorts in terms of the predominance of female subjects and disease manifestations, but differed from the Indian cohort in that the latter group had a higher frequency of male subjects, abdominal aorta and renal artery involvement, and hypertension.Conclusion. Although improvement of symptoms in TA usually follows glucocorticoid therapy, relapses usually occur with dosage reduction. Attempts to restore vascular patency are often initially successful, but restenosis occurs frequently. Chronic morbidity and disability occur in most patients with TA in the US.

Is 18F‐fluorodeoxyglucose positron emission tomography scanning a reliable way to assess disease activity in takayasu arteritis?

“…Assessing disease activity in TA patients is challenging because clinical, biologic, and radiologic data do not always correlate (1)(2)(3)(4). In the present study, we found a lack of correlation between FDG uptake and biologic or radiologic assessment of disease activity and a trend toward an association between FDG uptake and clinical activity.…”

“…Evaluation of disease activity in patients with TA is challenging because clinical features do not correlate with acute-phase reactants in ϳ50% of cases (1)(2)(3), and imaging modalities do not always correlate with clinical and laboratory parameters (4). The complexity of evaluating disease activity in TA is further underlined by the fact that up to 45% of patients in clinical remission have histologic evidence of active disease (1,5).…”

Tocilizumab for the Treatment of Patients With Refractory Takayasu Arteritis

“…1,3) Such patients require retreatment and high cumulative doses of GC, resulting in substantial toxicity and morbidity. Thus, immunosuppressive drugs such as methotrexate (MTX), azathioprine (AZA), cyclosporine A (CsA), cyclophosphamide (CYC), and mycophenolate mofetil (MMF) have been studied with an attempt to control the disease activity and lower doses of GC.…”

Single Stage Multiple Stenting in Takayasu's Arteritis

“…10,14,15,18) Severe hypertension, severe functional disability, and evidence of cardiac involvement are good predictors of either death or a major To manage active arteritis, an initial dose of corticosteroid administered at 1 mg/kg/day for one month, and then tapered down to less than 10 mg/day, is the initial mainstay of medical therapy for patients with Takayasu's arteritis. 4,9) However, studies report that only 20% of patients with Takayasu's arteritis maintain remission for 6 months on less than 10 mg prednisone. 4) Since no medical therapies can reverse established vascular stenosis, 4) the combination of medical therapy and subsequent revascularization is crucial in patients with Takayasu's arteritis.…”