Cookies on this website

We use cookies to ensure that we give you the best experience on our website. If you click 'Accept all cookies' we'll assume that you are happy to receive all cookies and you won't see this message again. If you click 'Reject all non-essential cookies' only necessary cookies providing core functionality such as security, network management, and accessibility will be enabled. Click 'Find out more' for information on how to change your cookie settings.


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).


Treatment options in IGG4-RD 
Agent Regimen

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

Mycophenolate mofetil

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.