| Objective:Takayasu arteritis(TA)in children is a chronic,non-specific granulomatous vasculitis with an insidious onset and heterogeneous clinical phenotype.Early diagnosis poses a challenge,but timely intervention can improve prognosis.This retrospective study summarizes the clinical characteristics of multiple aortitis in children to enhance pediatric rheumatologist’ understanding of the disease and provide valuable experience for its diagnosis and treatment.Method:Thirty-one patients diagnosed with Takayasu arteritis and received initial treatment at the Pediatric Rheumatology and Immunology Department of the First Hospital of Jilin University between February 2018 and October 2022 were included in this study.General conditions,clinical symptoms and signs,laboratory examinations,imaging examinations,therapeutic drugs,and follow-up information of the patients were collected and summarized.The Numano classification criteria were employed for clinical categorization,while the Kerr score was utilized to assess disease status.Results:Among the 31 children diagnosed with Takayasu arteritis,there were 24 cases in adolescence,4 cases in school age,2 cases in early school age,and 1 case in infancy.The male to female ratio was 1:2.88.The median age at onset was 12 years old;the minimum age was 58 days;the maximum age was16 years old;the median time from onset to definitive diagnosis was one month(0.48,2.62);and the median follow-up time was 24 months(10,33).In terms of clinical manifestations,all organs and systems may be affected,with highly heterogeneous symptoms primarily manifesting in the general and nervous systems.General symptoms include weakness(45.2%),fever(35.5%),and weight loss(35.5%),while nervous system symptoms include headache(48.4%)and dizziness(35.5%).Vascular lesions commonly present as hypertension(64.5%),vascular murmur(54.8%),anemic/weak pulse(35.5%)and abnormal blood pressure readings(35.5%).Complications arised in the form of ocular,cardiovascular,neurological and vascular damage.Retinal diseases were observed in all cases of eye lesions,with hypertensive retinopathy being the most prevalent(12.9%).For cardiac damdge,Cardiac dysfunction was a common occurrence(12.9%).Neurological complications included hypertensive encephalopathy(12.9%)and cerebral infarction(6.5%),while superficial thrombophlebitis was observed in 2 patients(6.5%).In laboratory examination,nearly half of the children(41.9%)exhibited elevated levels of white blood cells;The proportion of children with increased C-reactive protein and erythrocyte sedimentation rate was 87.1% and 93.5%,respectively.Among immunological indicators,abnormal levels of immunoglobulin G were observed in 18cases(58.1%),while abnormal levels of immunoglobulin A and immunoglobulin M were observed in 26 cases(83.9%)and 9 cases(29.0%),respectively.Additionally,elevated antinuclear antibody titers were detected in six cases(19.4%),although no specific antibodies were produced.In terms of etiology,mycobacterium tuberculosis infection was the main source of infection in 12 cases(38.7%),followed by hemolytic streptococcus infection(22.6%).The clinical classification at disease onset revealed that 20 cases(64.5%)were type V,5 cases(16.1%)were type I and type V were also 5 cases;only one case(3.2%)was classified as type IIb,with no instances of types IIa or III observed.The aortic arch and its branches were the most frequently affected vessels(64.3%),followed by the carotid artery(34.5%)and subclavian artery(18.1%).The abdominal aorta and its branches accounted for 32.1% of cases,with the abdominal aorta(10.4%),renal artery(7.2%),and superior mesenteric artery(6.8%)being more frequently affected,while other arterial lesions were relatively uncommon.Wall thickening and lumen stenosis were observed in 88.4% of these affected arteries.Occluded or nearly occluded arteries(6.4%),dilated arteries(2.4%),and aneurysms(2.8%)were uncommon.Among all the children,11 patients were first diagnosed in the departments outside the rheumatology department.4 cases were initially misdiagnosed as respiratory tract infection,2 cases had fever of unknown origin,and 2 cases had rash.The most extended delay in diagnosis was 16 months.In the medical treatment,glucocorticoids and non-biologic DMARDs were used in 31 children,of which 21(67.7%)received methylprednisolone pulses,and 10(32.3%)received oral glucocorticoids;non-biologic DMARDs were mainly methotrexate(90.3%)and Mycophenolate Mofetil(90.3%).Biologics were used in 21 of 31 children(67.7%),and tocilizumab was the most used in 20 cases(64.5%).In addition,a total of9 children underwent surgical treatment.After treatment,17(17/31,54.8%)of the 31 children were in remission at 3months,24(24/30,80%)at 6 months,and the remission rate decreased to 66.7%(18/27)at 12 months,but at 18 months and 24 months,more than 72% of the children were in remission.During the follow-up period,16 patients(51.6%)had 18 relapses,of which2 had 2 relapses.In the course of disease monitoring,16 cases had no progression of vascular lesions when inflammatory indexes were elevated,and 13 cases had no progression of vascular lesions accompanied by elevated inflammatory indexes.In the course of the disease,the recurrent clinical symptoms are mainly nerve and heart,and the carotid artery is the common progressing blood vessel.Conclusion:Takayasu arteritis was mainly seen in adolescent girls.The early heterogeneous clinical manifestations were mainly systemic symptoms and neurological symptoms,and hypertension was the most common sign.V-type was most common in clinical classification.Carotid artery,subclavian artery and abdominal aorta were the main affected arteries,and most of them presented wall thickening and lumen stenosis.In our center,intravenous methylprednisolone shock and tocilizumab were often used for treatment,but the children are prone to relapse,so it is necessary to further optimize the treatment plan. |
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