High‐Titer Antinuclear Antibody Defines a Distinct IgG4‐Related Disease Subset With Predilection for Lung and Kidney Involvement
- 1Department of Rheumatology and Immunology The First Medical Center of Chinese PLA General Hospital 28 Fuxing Road, Haidian District Beijing 100853 China 301hospital.com.cn
Abstract
Objective: This study investigated whether antinuclear antibody (ANA) positivity identifies a distinct clinical phenotype in IgG4‐related disease (IgG4‐RD), characterized by specific organ involvement patterns.
Methods: We conducted a cohort study of patients diagnosed with IgG4‐RD (2016–2024). Based on indirect immunofluorescence (IIF) results, patients were categorized as ANA‐negative (titer ≤ 1:100) or ANA‐positive, with the latter further stratified by titer: group 1 (moderate titer, 1:320) and group 2 (high titer, ≥1:1000). We comprehensively compared demographics, organ involvement, and laboratory profiles across groups. Multivariable logistic regression was used to quantify associations.
Results: Among 278 patients, 38 (13.7%) were ANA‐positive (n = 19 per titer‐based subgroup). ANA patterns differed: granular in group 1 versus homogeneous/granular homogeneous in group 2. Only patients in group 2 (high‐titer ANA) demonstrated a significantly elevated risk of lung and kidney initial onset and involvement compared to ANA‐negative patients. This group also exhibited marked hypocomplementemia. The adjusted odds ratios for lung and kidney involvement in group 2 were 10.18 (95% CI: 3.590–28.876) and 4.42 (95% CI: 1.605–12.151), respectively. No such associations were found in group 1.
Conclusion: IgG4‐RD combined with high‐titer ANA (≥1:1000), mainly presenting as homogeneous type or homogeneous granular type, characterized by a heightened propensity for lung and kidney, initial onset and involvement, and hypocomplementemia. Routine ANA testing and careful assessment of pulmonary and renal systems are warranted in these patients.
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BibTeX
@article{zhang2026hightiter,
title = {{High‐Titer Antinuclear Antibody Defines a Distinct IgG4‐Related Disease Subset With Predilection for Lung and Kidney Involvement}},
author = {Jie Zhang and Yurong Zhao and Yiwen Wang and Chao Xue and Lidong Hu and Jianglin Zhang and Jian Zhu},
journal = {Journal of Immunology Research},
year = {2026},
month = oct,
doi = {10.1155/jimr/5243624},
url = {https://doi.org/10.1155/jimr/5243624},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - High‐Titer Antinuclear Antibody Defines a Distinct IgG4‐Related Disease Subset With Predilection for Lung and Kidney Involvement
AU - Jie Zhang
AU - Yurong Zhao
AU - Yiwen Wang
AU - Chao Xue
AU - Lidong Hu
AU - Jianglin Zhang
AU - Jian Zhu
PY - 2026
DA - 2026/10/30
JO - Journal of Immunology Research
DO - 10.1155/jimr/5243624
UR - https://doi.org/10.1155/jimr/5243624
AB - Objective: This study investigated whether antinuclear antibody (ANA) positivity identifies a distinct clinical phenotype in IgG4‐related disease (IgG4‐RD), characterized by specific organ involvement patterns. Methods: We conducted a cohort study of patients diagnosed with IgG4‐RD (2016–2024). Based on indirect immunofluorescence (IIF) results, patients were categorized as ANA‐negative (titer ≤ 1:100) or ANA‐positive, with the latter further stratified by titer: group 1 (moderate titer, 1:320) and group 2 (high titer, ≥1:1000). We comprehensively compared demographics, organ involvement, and laboratory profiles across groups. Multivariable logistic regression was used to quantify associations. Results: Among 278 patients, 38 (13.7%) were ANA‐positive (n = 19 per titer‐based subgroup). ANA patterns differed: granular in group 1 versus homogeneous/granular homogeneous in group 2. Only patients in group 2 (high‐titer ANA) demonstrated a significantly elevated risk of lung and kidney initial onset and involvement compared to ANA‐negative patients. This group also exhibited marked hypocomplementemia. The adjusted odds ratios for lung and kidney involvement in group 2 were 10.18 (95% CI: 3.590–28.876) and 4.42 (95% CI: 1.605–12.151), respectively. No such associations were found in group 1. Conclusion: IgG4‐RD combined with high‐titer ANA (≥1:1000), mainly presenting as homogeneous type or homogeneous granular type, characterized by a heightened propensity for lung and kidney, initial onset and involvement, and hypocomplementemia. Routine ANA testing and careful assessment of pulmonary and renal systems are warranted in these patients.
SN - 2314-8861
SN - 2314-7156
ER -