Background In Systemic lupus erythematosus (SLE), vasculitis is seen in 11% to 36% of patients. Small vessel vasculitis is seen in upto 86% and medium vessel vasculitis in the rest, and rarely large vessels are involved. We present a series of 4 such patients which is unique in that aortitis and lupus were diagnosed at the first presentation.

Objectives To study the clinical profile of SLE patients presenting with Large vessel vasculitis (LVV).

Methods Patients presenting to Rheumatology department between January 2012 and February 2015 fulfilling ACR criteria for SLE with evidence of large vessel involvement clinically and by Doppler ultrasound, CT angiography or MR angiography were included and followed prospectively. Demographic, clinical, laboratory and imagelogy data were studied.

Results All patients were females, between 21–37 years of age. All had clinical features suggestive of SLE with a positive serology for ANA and double stranded DNA. Mean age of the patients was 25.25±4 years, with median duration of disease of 34.5 (range 4 -60) months with mean SLEDAI of 22±7.

All patients had history of claudication of extremities. Imaging findings are described in table 1.

Patients 1, 2 and 4 had subclavian artery and aortic bruit. Patient 3, who presented with cerebrovascular accident had MR angiography showing multiple focal intracranial vessel stenosis along with large vessel involvement. Three patients had nephritis, one had CNS lupus. Three patients had high ESR 68,106,70 mm after one hour. One patient had a positive Anticardiolipin antibodies (1/3). Three had low complements (3/3). Patient 2 underwent renal biopsy, and found to have class V lupus nephritis.

Patient 1,2 and 3 received intravenous pulse cyclophosphamide therapy, Patient 4 was treated with azathioprine. Patient 1 underwent PTCA to aorta and subclavian artery in view of persistent claudication. After a mean follow up of 29±22 months all patients are stable.

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Conclusions Large vessel involvement in SLE predominantly involves aorta and subclavian arteries and is associated with high disease activity.

1. Barile-Fabris L, Hernández-Cabrera MF, Barragan-Garfias JA. Vasculitis in systemic lupus erythematosus. Current rheumatology reports. 2014 Sep 1;16(9):1–6.

2. Ramos-Casals M, Nardi N, Lagrutta M, Brito-Zerόn P, Bové A, Delgado G, Cervera R, Ingelmo M, Font J. Vasculitis in systemic lupus erythematosus: prevalence and clinical characteristics in 670 patients. Medicine. 2006 Mar 1;85(2):95–104.

Disclosure of Interest None declared