Despite the progress in the last years on the field of vasculitides, there are 
several unmet needs regarding classification, disease activity assessment, 
predictors of flares and complications, and type of treatment for the different 
forms. The 1990 American College of Rheumatology (ACR) classification criteria 
currently used to define giant cell arteritis and Takayasu arteritis were 
designed to discriminate between different types of vasculitides but not to 
differentiate vasculitis from other disorders. Recently, efforts have been made 
to overcome the shortcomings of the ACR criteria. The lack of an accepted 
definition of disease activity in large-vessel vasculitides presents a major 
challenge in creating useful and valid outcome tools for the assessment of 
disease course. Identification of predictors of flares can aid in optimizing 
therapeutic strategies, minimizing disease flares, and reducing 
treatment-related side effects. It is furthermore important to recognize and 
characterize the risk factor that might predict the manifestations associated 
with poor outcome and prognosis. Two RCTs have evidenced the efficacy of 
tocilizumab in addition to glucocorticoids (GCs) in the treatment of giant cell 
arteritis (GCA). However, the role of tocilizumab or other biological agents 
without GCs needs to be investigated. Recent observational studies have 
suggested that rituximab is also effective in patients with eosinophilic 
granulomatosis with polyangiitis and in antineutrophil cytoplasmic antibodies 
(ANCA)-negative patients with granulomatosis with polyangiitis and microscopic 
polyangiitis. Rituximab or anti-TNF alfa may represent a possible alternative 
therapy in case of refractory or difficult to treat polyarteritis nodosa (PAN) 
patients. The new International Criteria for Behçet's Disease have shown a 
better sensitivity and a better accuracy compared to the older International 
Study Group on Behçet's Disease criteria. The EULAR recommendations for the 
management of Behçet's disease (BD) have been recently updated. However, the 
treatment of refractory disease is still a real challenge.