Unmet Needs in the Pathogenesis and Treatment of Vasculitides

Abstract

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.

Disease activity assessment of rheumatic diseases during pregnancy: a comprehensive review of indices used in clinical studies

“…Pregnancy does not seem to affect disease activity in TA patients [80], nevertheless, maternal complications such as sustained hypertension, PE, congestive heart failure and cerebrovascular accidents are not uncommon [3,84,[88][89][90][91][92][93][94][95][96].…”

The role of PET/CT in disease activity assessment in patients with large vessel vasculitis

“…GCA and Takayasu arteritis (TAK) are the two main forms of large vessel vasculitis (LVV) that share many clinical, pathological and radiographic features [ 1 , 2 ]. Despite many attempts to adopt standardized approaches for assessing disease activity in LVV, no single measure or set of measures have been accepted as valid and useful in clinical practice [ 3 ].…”

Higher Frequencies of Lymphocytes Expressing the Natural Killer Group 2D Receptor in Patients With Behçet Disease

“…Currently no laboratory assays or imaging approaches are available to support the clinical diagnosis. The classification criteria, introduced in 1990 and successively reviewed in 2006, are still a matter of discussion ( 4 ), however they help the physicians in identifying the patients with this condition. The BD Current Activity Form (BDCAF) is the most widely used index to assess disease activity in BD ( 5 ).…”