Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients.
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.


CONCLUSIONS
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.
Maksimowicz-McKinnon
Kathleen
K
Cleveland Clinic Foundation, Center for Vasculitis Care and Research, Cleveland, Ohio 44195, USA.
Clark
Tiffany M
TM
Hoffman
Gary S
GS
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D023903:Coronary Restenosis / Q000188:drug therapy / Q000208:ethnology / Q000517:prevention & control; D004359:Drug Therapy, Combination; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007194:India / Q000208:ethnology; D007558:Italy / Q000208:ethnology; D007564:Japan / Q000208:ethnology; D008137:Longitudinal Studies; D008297:Male; D008800:Mexico / Q000208:ethnology; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D013625:Takayasu Arteritis / Q000150:complications / Q000188:drug therapy* / Q000208:ethnology / Q000473:pathology*; D016896:Treatment Outcome; D014481:United States / Q000208:ethnology
D005938:Glucocorticoids; D007166:Immunosuppressive Agents