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