Advances in the Treatments of Systemic Vasculitides

Abstract

Necrotizing systemic vasculitides are severe diseases in which prognosis improved since steroids and cytotoxic agents have been prescribed. The outcome is now better, and remission is obtained in more than 80% of patients. In this review paper, we will cover the indications of conventional treatments and new biotherapies and other therapies. The strategies that improve patient's outcome are discussed in the light of the prospective trials organized by international research groups.

High-dose intravenous immunoglobulin therapy induces and maintains complete remission for polyarteritis nodosa

“…It was suggested that IVIG could prevent vasculitis by binding to the Fcγ receptor, thus preventing immune complex deposition in the vessels, or by neutralization of anti-endothelial antibodies that might cause inflamed vasculitis in PAN through antiidiotypic antibodies. Thus, IVIG likely interacts with different components of the immune system, including cytokines, complement with Fc receptors and several cell surface molecules [2,8] .…”

Clinical Features And Prognostic Factors Of Churg-strauss Syndrome

“…However, not all patients had the same prognosis. Stage of diagnosis and organ involvement features have been reported to inf luence CSS prognosis [29]. Five factors-cardiomyopathy, severe gastrointestinal involvement, central nervous system involvement, 24-hour urine protein over 1 g, and serum creatinine over 1.4 mmol/L-have been associated with CSS prognosis.…”

Intravenous immunoglobulins as treatment of severe cutaneous polyarteritis nodosa

“…Furthermore, IVIgs have a marked impact on different effector cells of the immune system (e.g. B and T lymphocytes, dendritic cells) and regulate a wide range of genes 10,17 . Additionally, a relationship between cutaneous polyarteritis nodosa and infectious agents has been speculated, mainly including Streptococcus (group A β hemolytic Streptococcus ) and more rarely viruses and Mycobacterium tuberculosis 1,3,6–8 .…”

“…10,17 Thus, IVIgs interact with the different components of the immune system, including cytokines, complement with Fc receptors and several cell surface molecules. 10,17 Furthermore, IVIgs have a marked impact on different effector cells of the immune system (e.g. B and T lymphocytes, dendritic cells) and regulate a wide range of genes.…”

“…B and T lymphocytes, dendritic cells) and regulate a wide range of genes. 10,17 Additionally, a relationship between cutaneous polyarteritis nodosa and infectious agents has been speculated, mainly including Streptococcus (group A b hemolytic Streptococcus) and more rarely viruses and Mycobacterium tuberculosis. 1,3,[6][7][8] In fact, our results suggest that IVIgs may be useful in some patients with cutaneous polyarteritis nodosa through toxin or microbial neutralising leading to dampening of inflammatory response.…”