Key Insights on Pediatric IgG4-Related Disease and Advances in Diagnosis and Treatment in 2024
1. New insights into pediatric IgG4-related disease: Eye involvement common, RCD criteria preferred
A multicenter study of 35 children with IgG4-related disease (IgG4-RD) identified three clinical clusters—isolated eye involvement, eye and neurological symptoms, and pancreato-hepatobiliary disease with lymph node involvement—highlighting the predominance of orbital disease (60%). The study found the 2020 revised comprehensive diagnostic criteria to be far more sensitive (88.5%) than the 2019 ACR/EULAR criteria (5.7%) in diagnosing pediatric IgG4-RD.
Read more here.
2. Treatment considerations and unmet needs for patients with IgG4-related disease
John Stone, MD, MPH, Director of Clinical Rheumatology at the Massachusetts General Hospital and Founder of the IgG4ward! organization, and Emma Culver, BSc, MBChB, DPhil, FRCP, Consultant Hepatologist and Hon Senior Lecturer in the Translational Gastroenterology and Liver Unit at John Radcliffe Hospital in Oxford, discuss the need for better treatment options for patients with IgG4-related disease, a potential FDA approval in 2025, and the unmet needs patients face, chiefly as it relates to education around the disease.
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3. Serum IgG4 levels linked to disease activity in IgG4-related sclerosing cholangitis
A study presented at the 2024 American College of Gastroenterology Annual Meeting found that elevated serum IgG4 levels in patients with IgG4-related sclerosing cholangitis are significantly associated with markers of liver dysfunction and systemic inflammation, suggesting IgG4 as an indicator of disease activity. Female patients had higher IgG4 levels than males, and those with elevated IgG4 also had higher levels of alkaline phosphatase, amylase, lipase, and inflammatory markers.
Read more here.
4. Prevalence of disease and procedure-associated damage in IgG4-RD highlighted at ACR Convergence 2024
A study presented at the 2024 ACR Convergence found that 92% of patients with IgG4-related disease experienced damage, particularly to the pancreas, kidneys, and salivary glands, often without symptoms during active disease. Invasive procedures, such as salivary gland resections and orbital biopsies, also significantly contributed to morbidity in these patients.
Read more here.
5. Multidisciplinary specialists discuss the diagnosis and management of IgG4-related disease
In this Roundtable discussion presented by Rare Disease 360, 3 clinicians from different subspecialty groups discuss their best practices for diagnosing and managing patients with IgG4-related disease.
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