Treatment
How IgG4-RD is managed and what patients can expect from therapy.
Glucocorticoids (steroids)
Glucocorticoids (steroids) are the cornerstone of initial treatment for IgG4-RD. They are effective in inducing remission in the majority of patients and are typically used as first-line therapy when active disease is present.
With timely recognition and treatment, many people do well on steroids or targeted therapies that calm the immune system and protect affected tissues.
B-cell depletion
B lymphocytes and plasma cells are central to IgG4-RD pathogenesis. B-cell depletion with available immunosuppressive drugs leads to rapid improvement in most cases, even in patients who have not responded fully to steroids.
This approach is particularly important in relapsing or refractory disease, and in patients for whom long-term steroid use is not appropriate.
Relapse is common
IgG4-RD follows a relapsing-remitting course. Even after successful initial treatment, relapse occurs in a significant proportion of patients. Regular monitoring is therefore important, even when the disease appears to be in remission.
The number of organs involved and the level of serum IgG4 correlate with the risk of relapse. Patients with multi-organ disease or persistently elevated serum IgG4 may require longer-term maintenance therapy.
Treatment decisions for IgG4-RD should always be made in close consultation with a specialist who has experience with this disease. Treatment approaches may differ based on the organs affected, disease severity, and individual patient factors.
Carsten Sørensen
Patient & Treasurer, Denmark
As a co-founder and chair of the federation, I am proud to lead a community advocating for better recognition and care for IgG4-RD.
About the European Federation IgG4-RD
We are the bridge between you, the patient, be it as a direct member or through your own national association and the medical world and wider community. We connect knowledge, compassion, and advocacy across Europe for those affected by IgG4-Related Disease.
