We have created definitions for ultrasonographic abnormalities of Giant Cell Arteritis. The ‘halo’ sign is a ‘homogenous, hypoechoic wall thickening, well delineated towards the luminal side, visible both in longitudinal and transverse planes, most commonly concentric in transverse scans.’ At the superficial temporal artery, the interobserver reliability in acquired and dynamic images has a k = 0.87 and 0.60 respectively; the intraobserver reliability in acquired images and live exercises has a k = 0.88 and 0.71 respectively. Ultrasonography is more reliable (k = 0.8) than temporal artery biopsy (k = 0.4) when compared against physician verified diagnosis at 100-week follow-up. Ultrasonography of 25 patients may be enough for service validation if audited against biopsy and long-term outcomes.
Activity and Damage form the twin sides of vasculitis assessment. We have validated the Birmingham Vasculitis Activity Score v3 in two separate studies with convergent validity against treatment decision (r = 0.54) and excellent interobserver reliability (ICC = 0.996). A new Combined Damage Assessment index had lower interobserver (ICC = 0.78) and intraobserver reliability (ICC = 0.87) vs the Vasculitis Damage Index (ICC = 0.94 and 0.92 respectively).
Granulomatosis with Polyangiitis, Microscopic Polyangiitis and Eosinophilic Granulomatosis with Polyangiitis have remission rates of 30%-93%, 75%-89% and 81%-91% respectively. The 5-year survival is 74%-91%, 45%-76% and 60%-97% respectively. At diagnosis, the quality of life as measured by the Short Form – 36 is worse than normative data. Older age and neurologic involvement at baseline are associated with lower physical composite scores.
My work has resulted in improvements in the diagnosis of Giant Cell Arteritis, assessment of primary systemic vasculitis and understanding outcomes in Antineutrophil Cytoplasm Antibody associated vasculitis. They have also informed the research agenda for further developments in the field
