ArticleClinical rheumatology2025
Clinical and laboratory characteristics of IgG4-related disease involving multiple organs.
Article in Clinical rheumatology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
introductionIgG4-RD is a rare but increasingly recognized systemic condition characterized by complex clinical manifestations and multi-organ involvement, often leading to misdiagnosis or delayed diagnosis. This study aims to elucidate the clinical features of IgG4-RD and evaluate the diagnostic and therapeutic utility of key laboratory indicators.
methodWe conducted a retrospective analysis of IgG4-RD patients, comparing laboratory profiles across different organ involvement patterns. The diagnostic and therapeutic value of serum IgG4 levels in IgG4-RD was evaluated, with comparative analyses of serum IgG4 levels extended to non-IgG4-RD patients.
resultsDistinct patterns of laboratory markers were observed across affected organs, with elevated IgG and ESR levels consistently noted. The optimal diagnostic cutoff for serum IgG4 in IgG4-RD was 1.94 g/L, while a cutoff of 9.05 g/L effectively identified multi-organ involvement. Following 6 months of standardized treatment, serum IgG4 levels in IgG4-RD patients decreased significantly. Notably, ANCA-associated vasculitis patients exhibited markedly elevated serum IgG4 levels, which remained unchanged post-treatment.
conclusionsThe integration of serum IgG4 with other laboratory markers enhances diagnostic precision for IgG4-RD. Elevated serum IgG4 levels are associated with multi-organ involvement, underscoring their utility in assessing disease extent. Serum IgG4 is indispensable for monitoring therapeutic response. Given the overlapping clinical features between ANCA-associated vasculitis and IgG4-RD, accurate differentiation is essential for optimal clinical management. Key Points • IgG4-RD predominantly affects middle-aged and elderly males, yet its occurrence in younger individuals and less commonly involved organs (e.g., brain and heart) warrants attention. • Affected organs demonstrate distinct laboratory marker profiles, providing valuable insights for clinical diagnosis and therapeutic strategies. • Serum IgG4 levels are instrumental in diagnosing, assessing disease progression, and monitoring treatment response, though their interpretation should be integrated with other clinical indicators. • In ANCA-associated vasculitis, serum IgG4 levels are markedly elevated but remain stable post-treatment, offering a potential diagnostic criterion to differentiate it from IgG4-RD.
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