These details were requested from the author but have not been provided to date

These details were requested from the author but have not been provided to date. C.??? No difference in BVAS changes after three months After three months the benefit in terms of additional BVAS reductions in the IVIg group were lost compared with the placebo group. a therapeutic advantage over and above treatment with systemic corticosteroids in combination with immunosuppressants for the treatment of WG. Search methods For this update the Valsartan Cochrane Peripheral Vascular Diseases Group Trials Search Co\ordinator (TSC) Valsartan searched the Specialised Register (last searched November 2012) and CENTRAL (2012, Issue 11). Trial databases were searched by the TSC for details of ongoing and unpublished studies. No date or language restrictions were applied. Selection criteria Randomized controlled trials (RCTs), or quasi RCTs, or randomized cross\over trials. Participants had to be adults with a confirmed diagnosis of WG. Data collection and analysis Two authors independently extracted data and assessed trial quality. Relative risk was used to analyze dichotomous variables, and mean difference (MD) was used to analyze continuous variables. Main results We included one RCT with 34 participants who were randomly assigned to receive IVIg or placebo once daily in addition to azathioprine and prednisolone for remission maintenance. There were no significant differences between adjuvant IVIg and adjuvant placebo in mortality, serious adverse events, time to relapse, open\label rescue therapy, and infection rates. The fall in disease activity score, derived from patient\reported symptoms, was slightly greater in the IVIg group than in the placebo group at one month (MD 2.30; 95% Confidence interval (CI) 1.12 to 3.48, P 0.01) and three months (MD 1.80; 95% CI 0.35 to 3.25, P = 0.01). There was a significant increase in total F3 adverse events in the IVIg group (relative risk (RR) 3.50; 95% CI 1.44 to 8.48, P 0.01). Authors’ conclusions There Valsartan is insufficient evidence from one RCT that IVIg adjuvant therapy provides a therapeutic advantage compared with the combination of steroids and immunosuppressants for patients with WG. Given the high cost of IVIg (one dose at 2 g/kg for a 70 kg patient = $8,400), it should be limited to treat WG in the context of a well conducted RCT powered to detect patient\relevant outcomes. Plain language summary Intravenous immunoglobulin in addition to standard treatments for Wegener’s granulomatosis Wegener’s granulomatosis is a rare disorder that causes inflammation of the blood vessels. This inflammation restricts blood flow to various organs which can eventually damage the organs. Organs most affected by Wegener’s include the lungs, upper respiratory tract, kidneys, joints, skin and eyes. Wegener’s granulomatosis also produces a granuloma (a mass or nodule of inflammatory tissue) which is found around the blood vessels and which can also damage surrounding tissue. The cause of Wegener’s granulomatosis is unknown. Treatment is with corticosteroids and cytotoxic drugs which are often used for chemotherapy. Most patients get better with these drugs. However, the disorder returns in approximately half of patients. Intravenous immunoglobulin (IVIg) is an expensive and fairly rare blood product that has been used to treat Wegener’s granulomatosis but its effects on the disorder are unknown. We asked if IVIg provided an advantage as an additive to standard treatments. We found one small randomized trial in which 34 participants were randomized to receive IVIg or placebo once daily in addition to azathioprine and prednisolone for remission maintenance. This trial did not provide enough evidence to determine if IVIg has an advantage over corticosteroids and immunosuppressants for the treatment of Wegener’s granulomatosis. Background Description of the condition Wegener’s granulomatosis (WG) is a necrotizing small\vessel vasculitis that can affect any organ in the body but mainly affects the upper and lower respiratory tract, the kidneys, joints, skin and eyes. It is characterized by chronic tissue inflammation and the formation of granuloma (a mass or nodule of chronically inflamed tissue with granulations that is usually associated with an infective process). The annual incidence is low and diagnostic criteria vary, but it is estimated to be about 5 to 10 per million. (Scott 2000). Mild forms of the disease without renal involvement have been described and the course of the illness may vary from little activity to rapid progression. However, most patients with untreated generalized disease will experience a rapidly progressive fatal illness. Prior to the advent of immunosuppressive therapy, the five\month survival rate was 18% and two\year survival was 10% (Esper 1999). Definitive diagnosis is established by biopsy of the involved organs and the use of special stains that exclude mycobacterial and fungal infection. Clinical criteria for the diagnosis of WG were developed by the American College of Rheumatology and must.