Yes: not stated but confirmed on study assessment. a restorative advantage over and above treatment with systemic corticosteroids in combination with immunosuppressants for the treatment of WG. == Search methods == For this upgrade the Cochrane Peripheral Vascular Diseases Group Tests Search Coordinator (TSC) looked the Specialised Register (last looked November 2012) and CENTRAL (2012, Issue 11). Trial databases were searched from the TSC for details of ongoing and unpublished studies. No day or language restrictions were applied. == Selection criteria == Randomized controlled tests (RCTs), or quasi RCTs, or randomized crossover tests. Participants had to be adults having a confirmed analysis of WG. == Data collection and analysis == Two authors individually extracted data and assessed trial quality. TAK-632 Relative risk was used to analyze dichotomous variables, and imply 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 TAK-632 variations between adjuvant IVIg and adjuvant placebo in mortality, severe adverse events, time to relapse, openlabel save therapy, and illness rates. The fall in disease activity score, derived from patientreported symptoms, was slightly higher 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 adverse events in the IVIg group (relative risk (RR) 3.50; 95% CI 1.44 to 8.48, P < 0.01). == Authors' conclusions == There is insufficient evidence from one RCT that IVIg adjuvant therapy provides a restorative advantage compared with the combination of steroids and immunosuppressants for individuals with WG. Given the high cost of IVIg (one dose at 2 g/kg for any 70 kg patient = $8,400), it should be limited to treat WG in the context of a well carried out DKK1 RCT run to detect patientrelevant results. Keywords:Adult; Humans; Azathioprine; Azathioprine/restorative use; Chemotherapy, Adjuvant; Drug Therapy, Combination; Drug Therapy, Combination/methods; Glucocorticoids; Glucocorticoids/restorative use; Granulomatosis with Polyangiitis; Granulomatosis with Polyangiitis/therapy; Immunoglobulins, Intravenous; Immunoglobulins, Intravenous/restorative use; Immunologic Factors; Immunologic Factors/restorative use; Maintenance Chemotherapy; Maintenance Chemotherapy/methods; Prednisolone; Prednisolone/restorative use; Randomized Controlled Trials as Topic == Plain language summary == Intravenous immunoglobulin in addition to standard treatments for Wegener’s granulomatosis Wegener’s granulomatosis is definitely a rare disorder that causes inflammation of the blood vessels. This swelling restricts blood flow to numerous organs which can eventually damage the organs. Organs most affected by Wegener’s include the lungs, top respiratory tract, kidneys, joints, skin and eyes. Wegener’s granulomatosis also generates a granuloma (a mass or nodule of inflammatory cells) which is found around the blood vessels and which can also damage surrounding tissue. The cause of Wegener’s granulomatosis is definitely unfamiliar. Treatment is with corticosteroids and cytotoxic medicines which are often utilized for chemotherapy. Most individuals get better with these medicines. However, the disorder earnings in approximately half of individuals. Intravenous immunoglobulin (IVIg) is an expensive and fairly rare blood product that has been used to treat Wegener’s granulomatosis but its effects within the disorder are unfamiliar. We asked if IVIg offered an advantage as an additive to standard treatments. We found one small randomized trial in which 34 participants were randomized to receive IVIg TAK-632 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 definitely a necrotizing smallvessel vasculitis that can affect any organ in the body but mainly affects the top and lower respiratory tract, the kidneys, bones, skin and eyes. It is characterized by chronic tissue swelling and the formation of granuloma (a mass or nodule of chronically inflamed cells with granulations that is usually associated with an infective process). The annual incidence is definitely low and diagnostic criteria vary, but it is definitely estimated to be about 5 to 10 per million. (Scott 2000). Mild forms of the disease without renal involvement have been explained and the course of the illness may vary from little activity to quick progression. However, most individuals with untreated generalized disease will encounter TAK-632 a rapidly progressive fatal illness. Prior to the introduction of immunosuppressive TAK-632 therapy, the fivemonth.
