Early recognition of IgG4-RD: Red flags identified by ERN-ReCONNET task force

Raising awareness of specific early warning signs for IgG4-related disease (IgG4-RD) among primary and secondary care providers can significantly improve early diagnosis and facilitate timely referral to specialized centers, ultimately preventing irreversible organ damage, according to a task force from the European Reference Network for Rare Connective Tissue Diseases.

The group, comprising experts, primary care physicians, and patient representatives, worked collaboratively to identify early indicators that could lead to improved diagnosis and patient outcomes. This initiative aims to increase disease awareness and facilitate early intervention, ultimately improving patient outcomes in IgG4-RD.

Five key indicators reached 100% consensus among experts:

• • Swelling in ≥1 organ systems.
  • Involvement of the pancreas and biliary tree.
  • Elevated serum IgG4 levels.
  • Tissue infiltration by IgG4-positive plasma cells.
  • Presence of obliterative phlebitis (vein inflammation leading to blockage).

These red flags are designed for use in primary and secondary care settings to enhance the referral process and ensure timely diagnosis at specialized centers.

Reference
Della-Torre E, Talarico R, Ballarin J, et al. Identification of red flags for IgG4-related disease: an international European Reference Network for Rare Connective Tissue Diseases framework. Lancet Rheumatol. 2024;S2665-9913(24)00192-9. doi: 10.1016/S2665-9913(24)00192-9. Epub ahead of print. PMID: 39486422.