Salvageability of renal function following renal revascularization in children with Takayasu arteritis-induced renal artery stenosis

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Salvageability of renal function following renal revascularization in children with Takayasu arteritis-induced renal artery stenosis Obiagwu P 1, Gajjar P 1, Scott C 1, Numanoglu A 2, McCulloch M, 1 Nourse P 1 1. Departments of Paediatric Medicine 2. Department of Paediatric Surgery

Introduction Takayasu s arteritis (TA) is a chronic granulomatous periarteritis affecting mainly the aorta and it s large vessel branches. Renal arteries involvement 78%-86% malignant hypertension and chronic kidney disease. Revascularization option to control hypertension and preserve renal function. Literature mainly in adults and only limited to a few case series in children.

Objective To evaluate the impact of renal artery revascularization on renal function of children with renal artery stenosis (RAS) secondary to TA in our centre

Materials and Methods: Study centre and population A 22 year retrospective study of all children diagnosed with Takayasu s arteritis at the Red Cross TA was diagnosed based on the EULAR/PReS endorsed consensus criteria Renal artery stenosis (RAS), confirmed by angiography was considered significant if >50% of the lumen was occluded.

Medical therapy Antituberculous medications Steroids Immunosuppressive agents cyclophosphamide, methotrexate Antihypertensives

Indications for revascularization procedures Refractory hypertension Deterioration in renal function

Revascularization Procedures Percutaneous transluminal angioplasty (PTA), Kidney auto-transplantation Arterial bypass surgery (ABP) either using autologous or prosthetic vascular grafts revascularizations using splenic, hepatic and other vessels.

Criteria for success of renal preservation following revascularization American Heart Association guidelines for Reporting of Renal Artery Revascularization in Clinical Trials (2002): Outcome assessed at: 2 weeks 3 months, 3 6 months and 6 12 months Creatinine was measured and GFR calculated using Schwartz formula 1)Improvement: greater than 20 % increase in e-gfr from pre-surgery value 2)Stabilization: e-gfr within 20% of pre-surgery value 3)Failure: Greater than 20% deterioration in e-gfr from pre-surgery value Benefit improvement or stabilazation. Associations between outcome and variables were determined. Significance was set at p< 0.05.

Results: Baseline characteristics of 59 children diagnosed with Takayasu s Sex Male Female No(%) 25 (42.4) 34 (57.6) Age (years) 9.98 (range 1.10-14.65 ) Race No(%) Black 31 (52.5) Mixed race 27 (45.8) White 1 (1.69) Mantoux test Positive Negative No(%) 55(93.2%) 4(6.8%) Erythrocyte sedimentation rate 52.9 (range 5-143mm/hr )

Results 59 children with TA during the study period RAS in 45 patients (76.3.2%) bilateral in 30 patients (50.8%). 20 children had renal artery revascularization The mean duration of follow up was 31.8 ± 34.1 months (range: 1 141 months)

Demographic and laboratory variables of 20 children who had revascularization surgeries for renal artery stenosis Variable Age (years) Mean Range 9.1 ± 3.1 2-14 Gender Male (%) Female (%) 9 (45) 11 (55) Hypertension (%) 20 (100) Baseline egfr in µmol/l range, mean (SD) 53 150, 88.6 (25.4) Side of renal artery stenosis Right (%) Left (%) 5 (25) 2 (10)

Renal function outcomes 20 children had 27 revascularization procedures 6 children had bilateral revasc Most common procedure was PTA which was done singly or in combination with other procedures in 10 patients.

Outcome Outcome Period 3 months 6 months 1 year 2 years Improvement 8/17 (47%) 9/17 (53%) 6/14 (43%) 5/13 (38%) Stabilization 9/17 (53%) 8/17 (47%) 8/14 (57%) 7/13 (54%) Failure 0 0 0 1/13 (8%) 92% of the patients had stable or improved renal function at the long term follow up (p< 0.05).

Outcome Association between renal outcome after revascularization and Age p = 0.977 Gender p = 0.257 Type of revascularization p = 0.526 Nephrectomy p = 0.452 at the various follow up periods. Bilateral revascularization significantly associated with an improvement in renal function in the early 3-month postoperative period (OR 0.03; 95% CI 0.001 0.851; p = 0.04). This significance was not observed at further follow up periods.

Limitations Retrospective review over many years Small number of patients No control group Patients varied considerably in site and degree of stenosis Effect of collaterals

Conclusion Revascularization may have a beneficial effect on preservation of renal function in patients with RAS secondary to Takayasu s Arteritis