ArticleArthritis research & therapy2021
Clinical characteristics and outcome of IgG4-related disease with hypocomplementemia: a prospective cohort study.
Article in Arthritis research & therapy, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers, 1 of them a synthesis that pooled it.
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Who cites it
12 citing papers in PubMed, 1 synthesis or guideline pooled it, 24 citations in OpenAlex.
- Risk factors for relapse of IgG4-related disease: a systematic review and meta‑analysis.Clinical rheumatology · 2025Pooled it
- Immunological Biomarkers to Assess Activity and Treatment Response in IgG4-Related Disease.Medicina (Kaunas, Lithuania) · 2026Article
- Comparison of Efficacy and Safety of Different Medication Protocols in Patients with Immunoglobulin G4-Related Disease Based on Follow-up Time: A Systematic Review and Network Meta-analysis.Archives of rheumatology · 2026Article
- Observational
- Immunoglobulin G4-related lung disease mistaken for pulmonary tuberculosis: A case report.World journal of clinical cases · 2025Article
- Elevated eosinophil-to-lymphocyte ratio (ELR) as a predictor of relapse for IgG4-related disease: a retrospective study across a decade.Clinical and experimental medicine · 2025Article
- Multiorgan involvement and circulating IgG1 predict hypocomplementaemia in IgG4-related disease.Annals of the rheumatic diseases · 2024Article
- Clinical features and relapse risks factors of IgG4 related disease: a single-center retrospective study.Clinical and experimental medicine · 2023Article
- Thoracic manifestations of IgG4-related disease.Respirology (Carlton, Vic.) · 2023Review
- Relapsing immunoglobulin G4-related sclerosing cholangitis during maintenance treatment with low-dose steroids: a case report.Translational gastroenterology and hepatology · 2023Article
- IgG4-Related Disease With Gastrointestinal Involvement: Case Reports and Literature Review.Frontiers in immunology · 2022Review
- Clinical Characteristics of Patients With IgG4-Related Disease Complicated by Hypocomplementemia.Frontiers in immunology · 2022Article
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Authors and funding
16 authors at 1 institution in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundImmunoglobulin G4-related disease (IgG4-RD) is a newly recognized systemic, immune-mediated, and fibro-inflammatory disease. Hypocomplementemia was found in part of IgG4-RD patients especially in the setting of active disease.
objectivesThis study aimed to clarify the clinical features, treatment efficacy, and outcome in IgG4-RD patients with hypocomplementemia.
methods312 IgG4-RD patients were recruited in our prospective cohort conducted in Peking Union Medical College Hospital. Patients were divided into hypocomplementemia group and normal complement group according to serum C3 and C4 levels measured at baseline before treatment. Low serum C3 levels (< 0.73 g/L) and/or C4 levels (< 0.10 g/L) were defined as hypocomplementemia. Demographic data, clinical characteristics, laboratory parameters, treatment, and outcome of two groups were analyzed and compared.
resultsHypocomplementemia was identified in 65 (20.8%) cases of untreated IgG4-RD patients at baseline. The average age of hypocomplementemia group was 55.85 ± 10.89 years, with male predominance (72.3%). Compared with normal complement group, patients with hypocomplementemia were likely to have more involved organs, higher IgG4-RD responder index (IgG4-RD RI), and higher laboratory parameters such as counts of eosinophils, inflammatory markers, immunoglobulin G (IgG), IgG1, IgG3, IgG4, and IgE. In addition, lymph nodes, lacrimal gland, submandibular gland, parotid gland, paranasal sinus, bile ducts, and prostate gland were more commonly affected (p < 0.05). Serum C3 and C4 showed a significant positively correlation with each other. Both C3 and C4 were negatively correlated with the number of involved organs, IgG, IgG3, IgG4, and IgG4-RD RI, as well as positively correlated with IgA and hypersensitive C reactive protein (hsCRP). 64 (98.5%) patients responded quickly to initial therapy at a 3-month follow-up. Fifteen (23.1%) patients relapsed during follow-up with mean recurrence time of 14.2 ± 13.8 months. Compared with normal complement group, there was no significant difference of relapse rate in two groups (P = 0.401).
conclusionsClinical characteristics of IgG4-related disease with hypocomplementemia differ from normal complement group. Serum C3 and C4 at baseline before treatment could be biological markers for disease activity. IgG4-RD with hypocomplementemia responded well to treatment and had no significant difference of relapse rate in IgG4-RD with normal complement.
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