The vasculitides comprise a heterogeneous group of disorders characterized by inflammation of blood vessels. Definitions of these conditions have been provided by the Chapel Hill Consensus Conference (updated in 2012). A large prospective study defining and validating diagnostic and classification criteria for the vasculitides (DCVAS, ClinicalTrials.gov NCT01066208) is underway.
In the ANCA-associated vasculitides morbidity and mortality is still very high, in the initial phase due to disease activity and infections, in the late phase due to cardiovascular disease and malignancies. Use of high dose corticosteroids and cyclophosphamide (CYC) plays a major role in morbidity and mortality. Recent studies have shown that CYC can be replaced by rituximab (RTX), both for induction and maintenance treatment. Phase 2 data suggests that prednisone can be replaced by an oral complement C5a-receptor inhibitor. Defining risk factors for relapse is important in order to select patients for long-term maintenance treatment. Some biomarkers for relapsing disease have been defined, such as PR3-ANCA vs. MPO-ANCA, rises in PR3-ANCA in renal disease and pulmonary hemorrhage, HLA-DPB1, and others, but prospective studies should demonstrate their clinical value with respect to level and duration of maintenance treatment.
In giant cell arteritis (GCA) aortitis has been recognized to occur in one out of three patients although being less severe in GCA than in isolated aortitis. Color duplex ultrasonography is valuable for a diagnosis of GCA, is comparable to MRI, but its use for selecting the site of biopsy did not improve the sensitivity of a temporal artery biopsy for a diagnosis of GCA. FDG-PET is useful as well for assessing active vascular inflammation in GCA and Takayasu arteritis but objective cut-off values for activity should still be defined. Levels of BAFF and Il-6 in serum are biomarkers for disease activity in GCA and PMR. In this respect, a phase 2 trial with tocilizumab in GCA has been succesful in showing that tocilizumab together with prednisone resulted in a higher rate of complete remission at a dose of 0.1 mg/kg/d at 12 weeks and a higher rate of relapse free remission at 52 weeks compared to prednisone alone. EULAR/ACR recommendations for the management of PMR have been established last year.
New developments in the other vasculitides will be discussed as well.
Disclosure of Interest None declared