VIDEO: ‘Highly treatable’ IgG4-related disease ‘rewarding’ to diagnose

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So, this is evolving very rapidly. There are several approaches to therapy. The cornerstone of treatment throughout the world still is glucocorticoids or steroids, prednisone or prednisolone. The great majority of patients are treated with steroids at the outset of their therapy, and many patients remain on steroids for a long period of time. This can be highly problematic because as I indicated, the disease is a disease that tends to affect middle-aged to elderly individuals, males more than females, and very often by the time patients are diagnosed, they have a couple of major problems.

Number one, they may have a lot of comorbidities already. They may already be diabetic, hypertensive, obese, hypercholesterolemic, et cetera. And putting these patients on steroids is a recipe for poor outcomes with regard to adverse effects. The second point that patients may have at the time of diagnosis is that they may already be diabetic because of the disease's tendency to involve the pancreas. So there again, having to treat these patients with steroids for prolonged periods of time is almost certain to cause problems. So that's steroids and a primary goal has been to find steroid sparing agents for this disease.

A number of conventional DMARDs, disease-modifying anti-rheumatic drugs have been tried. None has been particularly well studied, but we use medications such as mycophenolate mofetil and azathioprine (Imuran; Azasan) and methotrexate. And there is some evidence that these medications have at least a mild steroid sparing effect, but they really have not been subjected to rigorous clinical trials.

More recently and really a trend that goes back about 15 years ago, about 15 years now, is interest in treating patients with B-cell depletion. My group first used rituximab (Rituxan, Genentech) for the treatment of IgG-4 related disease back in 2009 or so, in a paper that was published in 2010.

Most recently, the first worldwide randomized double wide placebo-controlled trial of a B-cell depleting agent, namely inebilizumab (Uplizna; Amgen), has been completed. And those trial results were announced, top line results just a couple of months ago. It was an overwhelmingly positive trial confirming that B-cell depletion is in fact an effective therapy for this disease. These data will be published in greater detail shortly. So, there's been tremendous interest because of signals about the effectiveness of B-cell depletion and now the strongly positive mitigate trial in targeting B-cells. And I'm confident that other therapeutic options will emerge probably targeting B-cells as well.

So, I think that's where we are with medical therapies at the moment. Sometimes there are radiological or surgical interventions that need to happen, at least temporarily. Sometimes patients with retroperitoneal fibrosis need to have stents placed in the ureters or even nephrostomy tubes placed inside the kidneys. Stents are often also needed, at least temporarily, for biliary disease as well, but those stents can usually come out pretty quickly once medical therapy has been initiated.