Autoinflammatory & rare systemic diseases
Identification of red flags for IgG4-related disease: an international European Reference Network for Rare Connective Tissue Diseases framework
Authors' conclusion
Red flags for IgG4-related disease are intended for use in primary and secondary care to improve referral to centres of expertise and prompt early diagnosis.
Study context
- Population
- Task force of IgG4-RD experts from ERN-ReCONNET, patient representatives and primary care physicians
- Outcome
- Red flags that might increase the suspicion of IgG4-RD in primary and secondary care
Methodological detailssystematic literature search and expert agreement exercise, international, task force of experts, patient representatives and primary care physicians, level of agreement exercise
- Systematic literature search and expert agreement exercise
- International
- Task force of experts, patient representatives and primary care physicians
- Level of agreement exercise
Recommendations
As reported in the abstract
Red flags reaching 100% agreement among experts
- Swelling in one or more organ system
- 100% agreement
- Pancreatic and biliary tree involvement
- 100% agreement
- Increased serum IgG4
- 100% agreement
- IgG4+ plasma cell tissue infiltration
- 100% agreement
- Obliterative phlebitis
- 100% agreement
Original publication
Identification of red flags for IgG4-related disease: an international European Reference Network for Rare Connective Tissue Diseases framework. Lancet Rheumatol. Published 29 October 2024. doi:10. 1016/ s2665-9913(24)00192-9
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