New insights into pediatric IgG4-related disease: Eye involvement common, RCD criteria preferred

A recent multicenter study investigated the characteristics of pediatric IgG4-related disease (IgG4-RD) across 13 rheumatology centers, aiming to identify disease clusters and evaluate the effectiveness of existing classification criteria.

The study enrolled 35 children diagnosed with IgG4-RD, with a median age of 13.3 years at diagnosis. The most frequently affected organ was the eye (60% of cases), followed by lymph nodes and the musculoskeletal system (34.3% each) and the neurological system (25.7%). Researchers identified 3 distinct clusters: those with isolated eye involvement, those with eye and neurological symptoms, and those with pancreato-hepatobiliary disease alongside lymph node involvement.

Serum IgG4 levels were elevated in 67.8% of patients tested. Almost all participants received corticosteroid treatment, with azathioprine being the most commonly used steroid-sparing agent. The study found that the 2020 revised comprehensive diagnostic (RCD) criteria demonstrated a sensitivity of 88.5%, significantly outperforming the 2019 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria, which had a sensitivity of just 5.7%.

Overall, the findings highlight the diverse clinical presentations of IgG4-RD in children, with orbital involvement being particularly prominent.

Reference
Kaya Akca U, Kose H, Kurt T, et al. A rare disease with many faces: A multicenter registry of IgG4-related disease in children. Rheumatology (Oxford). 2024;keae497. doi: 10.1093/rheumatology/keae497. Epub ahead of print. PMID: 39298509.