IgG4-related Disease: 2013 Update

Having diagnosed a patient as having IgG4-related disease, I would have a low threshold for recommending immune-suppressive treatment, and would make that recommendation for any patient with vascular involvement. My initial approach would be prednisone at 40-60 mg/day with a plan to reduce the dose every two weeks, e.g., 40, 30, 20, 15, 10, 7.5, 5, and 2.5 mg for 2 weeks each. In the event of relapse, I would double the current prednisone dose, slow the taper, and add azathioprine, anticipating using that drug for one year if the patient were to remain in remission. In the event or subsequent relapse, I would stop azathioprine and use rituximab. In a patient with large artery involvement, I would consult a vascular surgeon soon after diagnosis, anticipating a need for surgical repair.