5 things you need to know about IgG4-related disease


Delegates have been treated to a talk by expert Professor John Stone from Harvard on a newly described multi-organ condition known as IgG4-related disease.

We managed to catch up with Professor Stone before he headed home and asked him about the top 5 things you need to know about the disease.

Top 5 things you need to know about IgG4-related disease:

1. The disease is a fibro-inflammatory disease that has elements of both inflammation and fibrosis, which has ultimate implications for treatment.

2. It unifies a whole bunch of different disorders that have been regarded as single organ diseases.

3. It very often mimics cancer, in that it presents as mass lesions.

4. IgG4 is not a great marker for following disease activity — plasmablasts can be measured by flow cytometry and that’s probably the best biomarker for active disease.

5. A new T cell lymphocyte (discovered by Stone and colleagues) is potentially the driver of IgG4-related diseases but also other auto-inflammatory diseases.

What’s the treatment?

It’s one thing to recognise a new disease, it’s another to discover a treatment for it, says Stone.

While steroids work in most patients they don’t cure the disease and often patients become refractory to steroids, or they flare again, he explains.

Together with his research team Stone found that treatment with B-cell depletion targeting anti-CD20 therapy works very well for these patients.

“I think we’ve understood enough about the pathophysiology of this disease to think about several kinds of different targeted approaches to treatment,” he says.

Stone and his colleagues are just about to start a trial of an antibody that targets CD19 which is on the B-cell and Fc gamma receptor 2.

“The cross-linking of these receptors should lead to decreased B-cell activation and interference with its cross talk with the T cell and that we think will lead to very effective treatment which we’ll be testing soon,” he says.

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