4 Patient-Care Centered Studies on IgG4-RD That You Need to Know About

Study That Examines Which IgG4-RD Patients Can Safely Stop Treatment and Maintain Remission

Factors Associated With Long-Term Remission After Drug Discontinuation in IgG4-Related Disease: A 3-Year Rule

Summary

This study explored which patients with IgG4-related disease (IgG4-RD) can safely stop treatment and maintain long-term remission without relapse. Since both continuing and discontinuing glucocorticoid (GC) or immunosuppressant (IM) therapy carry risks, identifying predictors of durable remission is critical. Researchers from Peking Union Medical College found that patients without salivary gland involvement and those who used an immunosuppressant early in treatment were significantly less likely to relapse after stopping medication. Additionally, remaining relapse-free for three years strongly predicted long-term remission.

Questions Answered by this Poster

• What factors predict relapse in IgG4-RD patients after treatment withdrawal?
• Does early use of immunosuppressants (IM) improve long-term outcomes?
• How long must patients remain relapse-free before remission is likely to last?
• Which organ involvements are associated with a higher risk of relapse?

Methods

• Study design: Retrospective cohort of IgG4-RD patients at Peking Union Medical College Hospital (China).
• Participants:
• 808 screened; 80 achieved remission before stopping treatment.
• Divided into: No-relapse group (n=37) and Relapse group (n=43).
• Criteria: All met 2019 ACR/EULAR classification standards for IgG4-RD.
• Data collected:
• Demographics, family history, organ involvement, lab markers, and treatment regimens.
• Laboratory values (IgG4 levels, eosinophil counts) before and after medication withdrawal.
• Statistical analyses:
• Univariate Cox regression for relapse predictors (HR, CI, p-values).
• Kaplan-Meier curve to estimate relapse-free survival over time.

Results

• Relapse group:
• More likely to have salivary gland involvement (submandibular and parotid).
• Less likely to have received immunosuppressant (IM) therapy at baseline.
• Higher baseline and pre-withdrawal IgG4 levels and eosinophil counts.
• Protective factors (lower relapse risk):
• Initial IM use → HR 0.41 (95% CI 0.20–0.84, p = 0.017).
• No salivary gland involvement → HR 3.26 (95% CI 1.32–8.06, p = 0.012).
• Time to relapse:
• Biphasic pattern with most relapses in first 36 months post-withdrawal.
• Patients relapse-free for 3 years had a 94.6% chance of long-term remission.

Conclusions

IgG4-RD patients without salivary gland involvement and those who begin treatment with immunosuppressants have a significantly lower risk of relapse after stopping therapy. The study suggests that three years of relapse-free status can serve as a practical clinical marker for durable remission, helping physicians tailor follow-up and maintenance strategies more effectively.

Key Data

• Sample: 80 remission patients (37 no-relapse, 43 relapse).
• Predictive markers:
• No salivary gland involvement (HR 3.26; p = 0.012).
• Initial IM therapy (HR 0.41; p = 0.017).
• Main relapse sites: Submandibular and parotid glands.
• Relapse-free 3 years = 94.6% probability of durable remission.

Authors

Yifei Wang, Yuxue Nie, Jingna Li, Jialei Zhang, Qinhuan Luo, Xinli Yang, Jialing Jiang, Nianyi Zhang, Linyi Peng, Yunyun Fei, Jiaxin Zhou, and Wen Zhang.

Affiliations:  Peking Union Medical College and Peking Union Medical College Hospital, Beijing, China.

Read the published abstract in ACR Meeting Abstracts

Study Highlights the Importance of Early Diagnosis and Treatment for IgG4-RD

Study Exploring When IgG4-RD Patients Should Start Treatment

Clinical Characteristics and Outcomes of IgG4-Related Disease Patients Who Chose Watchful Observation Without Treatment Intervention: A Single-Center Study

Summary

This study explored when treatment is needed for people newly diagnosed with IgG4-related disease (IgG4-RD) and when it may be safe to observe the disease without starting medication immediately. Researchers reviewed 80 cases and found that about one-third of patients were monitored without treatment. Those patients were typically younger, had lower inflammation markers, and had milder disease—often limited to glands or lymph nodes. Over time, most remained stable, though some required treatment later when new organ involvement appeared.

Questions Answered by this Poster

• Which IgG4-RD patients can be safely monitored without starting treatment?
• What clinical or lab factors predict who might eventually need therapy?
• How do organ involvement patterns differ between treated and observed patients?
• How long can untreated patients remain stable before needing intervention?

Methods

• Study period: 2005–2024
• Setting: Kagawa University Hospital, Japan
• Participants: 80 patients diagnosed using the 2020 Revised Comprehensive Diagnostic Criteria for IgG4-RD
• Groups:
• Treatment group: 54 patients (67.5%) started therapy at diagnosis
• Observation group: 26 patients (32.5%) were monitored without treatment
• Analysis: Compared demographics, labs, and organ involvement; tracked which observed patients later required treatment and why

Results

• At diagnosis:
• Observation group was younger and had lower IgG, CRP, sIL-2R, and IgG4-RD RI scores.
• Glandular and lymph node-only disease was more common in the observation group.
• Severe organ involvement (retroperitoneal, renal, pancreatic, pulmonary, or orbital) was more frequent in patients who began treatment.
• During follow-up:
• 7 of 26 observed patients (27%) eventually required therapy.
• New organ involvement, especially pancreatic disease, triggered treatment in 5 of these 7 patients.
• Average time to treatment: 30 ± 25 months after diagnosis.
• No significant baseline differences predicted who would later need treatment.
• 19 patients (73%) continued observation without needing medication, even with multiple organ involvement.

Conclusions

Many IgG4-RD patients—especially those with limited glandular or lymph node disease and low inflammation—can be safely monitored without immediate treatment. However, regular follow-up is essential, as some patients may develop new organ involvement that requires timely intervention.

Key Data

• Total patients: 80
• Treatment at diagnosis – 54 (67.5%)
• Observation only – 26 (32.5%)
• Observation group characteristics:
• Younger (average age ~63 years vs. 69 years in treatment group)
• Lower inflammation (CRP 0.1 vs. 1.4 mg/dL)
• Lower IgG levels (1789 vs. 2772 mg/dL)
• Lower disease activity (IgG4-RD RI 4.8 vs. 7.7)
• Commonly affected organs:
• Observation group – glands (lacrimal, salivary, lymph nodes)
• Treatment group – kidneys, lungs, pancreas, retroperitoneum, orbits
• Of the 26 observed:
• 7 required later treatment (27%)
• 19 remained stable without therapy (73%)

Authors

Kanako Chujo, Hiromi Shimada, Shusaku Nakashima, Taichi Miyagi, Koichi Sugihara, Yusuke Ushio, Mao Mizusaki, Naoto Manabe, Mayuko Wada, Risa Wakiya, Hiroki Ozaki, Hiroaki Dobashi

Affiliations: Kagawa University and Toho University School of Medicine, Japan

Read the published abstract in ACR Meeting Abstracts

Study Highlights the Challenges, Frequent Misdiagnoses, and Daily Impact that IgG4-RD Patients Face

Diagnostic Journey, Clinical Burden, and Quality of Life of Patients with IgG4-RD: Results of a Cross-Sectional Survey of Patients and Physicians in the United States

Summary

This study describes the real-world experiences of people living with IgG4-related disease (IgG4-RD) in the United States. It highlights how long it takes for patients to be diagnosed, which symptoms and organs are most commonly involved, what treatments are used, and how the disease affects quality of life. Overall, the findings show that IgG4-RD is often misdiagnosed at first, requires many tests and doctor visits, and has a meaningful impact on day-to-day function.

Questions Answered by this Poster

• What does the average diagnostic journey look like for IgG4-RD?
• Which symptoms and organs are most commonly affected?
• Who typically manages IgG4-RD care?
• What treatments are being used in real-world practice?
• How does IgG4-RD impact fatigue, daily activities, and work?

Methods

• Data came from the Adelphi IgG4-RD Disease Specific Programme™, a U.S. survey conducted in 2023–2024.
• Physicians submitted detailed information on 161 patients with IgG4-RD.
• 27 patients completed questionnaires on fatigue, quality of life, and work impact.
• Information collected included:
• Symptoms at first presentation
• Organ involvement
• Diagnostic tests and number of consultations
• Treatments used and timing
• Hospitalizations
• Patient-reported outcomes (FACIT-Fatigue, EQ-5D-5L, WPAI)

Results

• Participants: 161 physician-reported patients; 27 completed surveys. Average age 55; 62% male; 71% White.
• Before diagnosis: Symptoms began around age 50. Diagnosis took ~11.5 months. Most common initial symptoms were fatigue (42%), joint pain (23%), and weight loss (22%).
• Organ involvement: Pancreas (32%), parotid glands (22%), and retroperitoneal fibrosis (19%) were most affected.
• Diagnostic journey: First evaluations were usually by primary care (47%), gastroenterology (20%), or rheumatology (11%). Patients saw ~3 providers and underwent ~13 tests. 90% were initially misdiagnosed (often pancreatitis, chronic fatigue syndrome, or vasculitis).
• Treatment patterns: Treatment started ~9 months after diagnosis. Managed primarily by rheumatology (70%). Most common first therapies were prednisone (66%), rituximab (17%), and azathioprine (11%).
• Hospitalizations & impact: 14% were hospitalized in the prior year, mostly through the emergency room. Patient-reported outcomes showed notable impacts on fatigue, daily functioning, and quality of life.

Conclusions

This real-world survey highlights how challenging and time-consuming the diagnostic journey can be for people with IgG4-RD. Many patients face long delays, multiple tests, and frequent misdiagnoses before receiving proper care. Symptoms often affect quality of life, and treatment is highly variable. These findings underline the need for greater awareness, faster diagnosis, and more effective long-term management to improve daily functioning and overall health for patients living with IgG4-RD.

Key Data:

• 161 patients reported; 27 completed patient surveys.
• 11.5 months average time to diagnosis.
• 90% initially misdiagnosed.
• Most affected organs: pancreas (32%), parotid glands (22%), retroperitoneum (19%).
• Most common first symptoms: fatigue (42%), joint pain (23%), weight loss (22%).
• First-line treatments: prednisone (66%), rituximab (17%), azathioprine (11%).
• 14% hospitalized for IgG4-RD in prior year.
• Significant impact shown across fatigue, daily functioning, and work productivity.

Authors

Yasmin Hernandez-Barco (Massachusetts General Hospital; Harvard Medical School), Jenny Y. Park (Amgen Inc., Thousand Oaks, CA), Rajvi Wani (Amgen Canada Inc., Mississauga, ON), Andrea Meyers (Amgen Canada Inc., Thousand Oaks, CA), Matthew Charles Baker (Stanford University, Menlo Park, CA), Leonard Calabrese (Cleveland Clinic, Cleveland, OH), Kristina Patterson (Amgen Inc., Thousand Oaks, CA), Hannah Connolly (Adelphi Real World, United Kingdom), Shiva Lauretta Birija (Adelphi Real World, United Kingdom), Emma Culver (John Radcliffe Hospital; University of Oxford).

Read the published abstract in ACR Meeting Abstracts

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