IgG4 related disease oder IgG4-RD ist a rare chronic fibroinflammatory disease that is
characterized by unpredictable disease flares.
I've been lucky to be involved in developing the description of IgG4-RD from the beginning
and I'm grateful to contribute to the growing field of IgG4-RD.
explore the need for proactive management approaches in Id particularly given the chronic
systemic nature of the disease which can progress undetected and lead to unpredictable
disease flares and potential.
Organ damage for patients Awareness of how organ damage manifests is critically important
to inform the timely diagnosis of ID to begin it's important to understand the overarching
goals of treatment for IGD.
Given the chronic nature of the disease there are two main aspects to the treatment induction
and maintenance. The goal of induction treatment is to reduce
inflammation in the short term.
This is particular important to gain initial Disease Control for newly diagnosed patients
patients presenting with disease flare following induction M patients may benefit from
maintenance of remission to help prevent disease flares over the long term.
Now let's look at the current tools in our toolbox induction and maintenance treatment in
IgG4-RD it's important to note that there are currently no FDA approved therapies for IgG4-RD
The Tand International Consensus Statement on management of IgG4-RD,
which was developed by forty-two Global Experts on the disease recommends glucocorticoid for
induction treatment.
Glucocorticoids are very effective for induction treatment with most patients showing
the response however the potential for side effects such as metabolic endocrine toxicities
and infections limit their use as a long term maintenance therapy.
A recent analysis on glucocorticoid use showed that nearly seventy five percent of patients
who were treated with a Pension equivalent dose of five milligram or greater a day developed
glucocorticoid related side effects the most common being infections,
metabolic and endocrine toxicities gastrointestinal and cardiovascular toxicities.
maintenance Conventional immunosuppressive therapies also known as Darts or biologics
specifically CD B cell depleting therapies have been used but many questions remain regarding
their effectiveness dosages and duration of treatment.
review the disease course of ID to better understand the need for both induction
maintenance treatment approaches Many patients disease that progresses undetected for months
and years before they experience clinical symptoms or a disease flare and receive a
diagnosis of IGD.
O Note IgG4-RD disease flares can occur in nearly any organ in the body and can also impact
multiple organs.
noted earlier symptoms typically respond to induction therapy with glucocorticoids but
unfortunately most patients relapse within three years following diagnosis often during
glucocorticoid tapering or after withdrawal of glucocorticoids Patients can also experience
low level disease activity between flares and continue to experience repeated flares.
Maintenance Therapy may help provide disease control in many patients.
It's also recommended in patients with risk factors for relapse or for those with severe
manifestations such as pancreatic or biliary involvement as shown on the figure.
currently there is no clear guidance on the optimal approach to maintenance therapy.
After some degree of remission is achieved with glucocorticoid induction therapy with or
without conventional immunosuppressive treatments biologics patients may receive
longer term maintenance therapy with conventional immunosuppressive treatments
biologics or low dose glucocorticoids.
in summary today we discussed the importance of proactive management in ID touching on the
following key points the disease course of ID is chronic
relapsing proactive ID Management includes both induction
maintenance therapy considerations.
Maintenance Therapy should be considered to help reduce the risk of relapse.
currently approved therapies as we continue to gain a better understanding of the systemic
nature of the disease and optimize long term disease Management we can continue to improve
the lives of patients with I Thank you for listening.
Please see the other videos in this series from my colleagues for more information on IgG4
RD including Disease Overview Pathophysiology of IgG4-RD and the multidisciplinary
approach to Diagnosis.