Distinct clinical characteristics and relapse predictors identified in seronegative IgG4-RD study

Patients with seronegative IgG4-related disease (IgG4-RD) exhibit distinct clinical patterns compared to other patients with IgG4-RD, including lower serum IgG levels and eosinophil counts, and fewer affected organs, according to a study.

The study analyzed data from 698 treatment-naïve individuals categorized into 4 subgroups based on baseline serum IgG4 levels. The study found that patients with seronegative IgG4-RD, making up only 7.02% of the cohort, exhibited notably different clinical patterns compared to other patients with IgG4-RD. Specifically, these patients had lower serum IgG levels, eosinophil counts, and IgE levels, as well as fewer affected organs and lower responder index scores. They were more likely to present with fibrotic disease and specific organ involvement.

The study identified several risk factors for disease relapse in seronegative IgG4-RD patients, including younger age at onset, glucocorticoid monotherapy, and elevated levels of C-reactive protein and erythrocyte sedimentation rate. A nomogram model was developed to predict disease relapse, highlighting that patients with baseline scores over 84.65 were at increased risk.

Reference
Peng Y, Wang M, Sun R, et al. Revealing the distinct clinical patterns and relapse risk factors in seronegative IgG4-RD patients: A retrospective cohort study over a decade. J Intern Med. 2024;296(2):200-212. doi: 10.1111/joim.13814. Epub 2024 Jun 24. PMID: 38924246.