The aims of IgG4-RD treatment are to alleviate symptoms, prevent long term organ dysfunction or damage and to maintain disease remission. An international consensus on treatment strategies based on current evidence has recently been published (Okazaki et al., 2017).
Initial treatment of first presentation is with high dose first line steroids and aims to achieve symptomatic and or biochemical remission. Treatment strategies to prevent relapse include the continuation of low dose steroid or the introduction of a second line immunosuppressive agent such as azathioprine, mycophenolate mofitil, methotrexate, cyclophosphamide or tacrolimus. A recent randomised controlled trial has shown maintenance low dose glucocorticoid over 3 years rather than 6 months reduces rates of relapse (Masamune et al., 2017). There is early evidence that rituximab is successful in treating first presentation or relapsing disease and is now funded by NHS England as a treatment for relapsing cases that have failed second line therapy (Carruthers et al., 2015; Hart et al., 2013; Khosroshahi et al., 2010, 2012; Wallace et al., 2014).
30-40mg for 2-4 week
Taper by 10mg every 2 weeks until 20mg then 5 mg every 2 weeks
2mg/kg per day in a single dose
750-1000 mg twice per day
|Mercaptopurine||2.5mg/kg per day in two divided doses|
|Methotrexate||10-25mg per week plus folic acid|
|Tacrolimus||Adjusted to a target blood level range of 4-11ng/mL|
|Cyclophosphamide||15mg/kg every 2 weeks for three doses and every 3 weeks thereafter by Intravenous infusions|
|Rituximab||1,000mg week 0 and week 2 by intravenous infusions. Further infusions at 3-6 months guided by response and relapse.|
Legend: *Prednisolone dose can be adjusted to body weight and disease aggressiveness.