ArticleCureus2025
Challenges in Diagnosing and Differentiating IgG4-Related Disease From Sjögren's Disease: A Case Report and Literature Review.
Article in Cureus, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers, 1 of them a synthesis that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
2 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Beyond anti-SSA and anti-SSB. The clinical significance of new antibodies in the diagnosis of Sjögren disease.Rheumatology international · 2026Pooled it
- From inflammatory initiation to fibrotic remodeling: mechanisms and precision therapeutic strategies in otorhinolaryngologic involvement of IgG4-related disease.Frontiers in medicine · 2026Review
Corrections and comments
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Authors and funding
10 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
IgG4-related disease (IgG4-RD) is a multi-organ fibroinflammatory condition often misdiagnosed due to its clinical similarities with other rheumatologic diseases such as Sjögren's syndrome (SS) and malignancy. This case highlights a 76-year-old woman with a complex medical history who presented with symptoms initially suggestive of SS but was ultimately diagnosed with IgG4-RD. The patient in our case report presented with bilateral, large, nontender submandibular lymphadenopathy. Initial serological tests were negative for SSA and SSB markers but revealed elevated immunoglobulin G levels of 2811 mg/dL. Imaging showed enlarged submandibular glands and lymphadenopathy. A core biopsy revealed a dense nodular lymphocytic infiltrate with variable parenchymal fibrosis and salivary gland acinar atrophy. The specimen was sent for further immunohistochemistry (IHC) testing as extended lab workup showed IgG4 levels of 843 mg/dL, raising concern for underlying IgG4-RD. IHC showed an increased number of IgG4 plasma cells, up to 50% in some areas, confirming the diagnosis of IgG4-RD. Treatment with prednisone led to rapid symptomatic improvement. Differentiating IgG4-RD from SS is challenging due to overlapping clinical and histological features. Elevated serum IgG4 levels (>135 mg/dL) are suggestive but not definitive for IgG4-RD; biopsy remains the gold standard for diagnosis. This case underscores the importance of considering IgG4-RD in patients presenting with SS-like symptoms, especially in the absence of anti-Ro/SSA and anti-La/SSB antibodies, and of initiating an extensive workup to fully delineate the extent of the disease in order to initiate timely treatment and prevent organ damage. This case report emphasizes the need for thorough evaluation in patients with SS-like symptoms to differentiate IgG4-RD, ensuring accurate diagnosis and effective treatment. Prompt recognition and management of IgG4-RD can improve patient outcomes and prevent long-term morbidity.
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