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SP0077 Vasculitis: Year in Review
  1. C. Salvarani1
  1. 1Azienda Ospedaliera IRCCS di Reggio Emilia, Reggio Emilia, Italy


A major achievement in nosology has been the development of novel classification criteria for Behçet disease (BD), called “International criteria for Behçet’s disease” (ICBD) (1). Data on 2556 patients with BD diagnosed by experts and 1163 controls mimicking BD were collected from 27 countries, and the best discriminatory variables used to create a scoring scheme with a defined diagnostic cut-off. These criteria were tested in a validation set, yielding a sensitivity of 95% with a specificity of 91%, proving more sensitive in the same set than the International Study Group for Behçet disease (ISGB) criteria, although at the price of a somewhat reduced specificity.

In 2012, ANCA-associated vasculitis has been in the forefront of research. A genome-wide association study performed in a large UK cohort and replicated in a different cohort of patients with granulomatosis with polyangiitis and microscopic polyangiitis provided evidence for distinct genetic associations of these two disorders (2). Interestingly, the strongest genetic associations were found with the antigenic specificities of ANCA rather than with the clinical phenotypes.

A meta-analysis published last year aimed to investigate whether rising or persistently elevated ANCA titers might predict future relapses in patients with ANCA-associated vasculitis (3). The results derived from 18 studies demonstrated that ANCA titers were only modestly predictive of relapses.

A prospective, randomized controlled trial (MAINRITSAN) on 114 patients with ANCA-associated vasculitis has compared azathioprine (2 mg/kg/day) with rituximab (500 mg every 6 months) for remission maintenance (4). The primary end point of the study was the rate of major relapses at 28 months. 27% of patients in the azathioprine arm relapsed compared to 4% of those who received rituximab. Adverse events were overall comparable in the two groups. These findings suggest that rituximab might have a pivotal role not only in induction, but also in maintenance of remission in patients with ANCA-associated vasculitis.

With regard to giant cell arteritis (GCA), a study by our group re-assessed the histological alterations found in the temporal arteries from a large cohort of patients (5). In most cases, the histological pattern was that of a classical transmural infiltrate, but in some patients the inflammation appeared to be restricted to the vasa vasorum, to the periadventitial small vessels, or both. Visual loss was equally frequent in all groups of patients. These findings suggest that non-classical inflammatory patterns should be recognized as part of the spectrum of the histological abnormalities associated with GCA.

Last year also witnessed a significant progress in the treatment of large-vessel vasculitis, including GCA and Takayasu’s arteritis (TAK). Interleukin-6 has emerged as a pivotal cytokine driving inflammation in large-vessel vasculitis. On the basis of this rationale, 19 patients with GCA and 9 of those with TAK were treated with the interleukin-6 receptor inhibitor tocilizumab (6,7). Most patients had refractory disease, but a few had new onset of vasculitis. A favorable response was reported in virtually all patients treated, although some patients relapses after tocilizumab was withdrawn. Controlled studies are warranted to confirm these encouraging results and to define the precise role of tocilizumab in newly diagnosed and refractory GCA and Takayasu’s arteritis.

  1. Davatchi F et al, JEADV 2013 (in press)

  2. Lyons PA et al, New Engl J Med 2012; 367-214

  3. Tomasson G, Rheumatol 2012; 51:100

  4. Guillevin L, Arthritis Rheum 2012; 10 (Suppl):S706

  5. Restuccia G, Arthritis Rheum 2012; 64:549

  6. Unizony S et al, Curr Opin Rheumatol 2013; 25:3

  7. Salvarani C et al, Rheumatol 2012; 51:151

Disclosure of Interest None Declared

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