Lip Biopsy Aids Neurologic Sjögren Disease Diagnosis

Labial gland biopsy (LGB) may help confirm a diagnosis when clinicians suspect Sjögren disease (SjD) with neurologic involvement, particularly when antibody results are negative or inconclusive, according to a retrospective study published in Immunity, Inflammation and Disease.¹

Neuro-Sjögren is the neurological subtype of SjD. It is difficult to diagnose because its manifestations vary from one patient to the next. LGB is an established part of the SjD work-up and is included in the 2016 American College of Rheumatology/European Alliance of Associations for Rheumatology (ACR/EULAR) classification criteria. However, the authors wrote that its role in patients with suspected neurologic disease has been unclear.

Diagnostic delays in SjD have been associated with worse outcomes. A recent US survey covered by AJMC^® found that the mean time from symptom onset to diagnosis was 2.3 years. Patients diagnosed more than 12 months after their symptoms began had 2.3 times the odds of moderate or greater disease activity compared with those diagnosed within 3 months. The Sjögren’s Foundation estimates that about 2.5 million Americans with the disease remain undiagnosed.²

How the Study Evaluated Labial Gland Biopsy in Suspected Neuro-Sjögren

Researchers at Xuanwu Hospital, Capital Medical University, in Beijing, China, reviewed records of 456 patients evaluated for suspected SjD between 2019 and 2023. Of these, 135 were classified as having suspected neuro-Sjögren and 277 as having suspected non-neuro-Sjögren. The center uses its own diagnostic pathway, which references the 2016 ACR/EULAR criteria. Based on this pathway, the investigators divided the suspected neuro-Sjögren group into patients who required LGB (n = 91) and those who did not (n = 44). They then compared clinical and laboratory features between patients whose biopsy was positive (n = 74) and those whose biopsy was negative (n = 17).¹

Biopsy Rates in Neurologic Sjögren Disease

Compared with patients with suspected non-neuro-Sjögren, patients with suspected neuro-Sjögren were:

• more likely to require LGB (67.4% vs 56.3%; P = .031)
• more likely to have a positive biopsy (81.3% vs 51.3%; P < .001)

Factors independently associated with needing a biopsy included younger age at disease onset and early onset of neurologic symptoms. Anti-SSB antibody positivity and antinuclear antibody (ANA) titers of 1:320 or higher were associated with a lower likelihood of needing LGB.

Among patients who had a biopsy, those with positive results more often showed nonspecific inflammatory or immunologic abnormalities. These included higher erythrocyte sedimentation rates, elevated serum IgG, and ANA titers of 1:320 or higher.

What the Findings Mean for Sjögren Disease Diagnosis

The study was a retrospective, cross-sectional review conducted at a single center in China, and the LGB-negative subgroup included 17 patients. The authors concluded that LGB may add supportive information in selected patients with suspected neuro-Sjögren, particularly when serologic findings are negative or inconclusive. However, they cautioned that the factors linked with needing a biopsy should be interpreted in the context of their center’s diagnostic pathway and “should not be regarded as universal indications for biopsy.”

References

1. Tian Z, Li X, Li X, Wang Y, Wang C, Zhao Y. The clinical utility of labial gland biopsy in patients with suspected neuro-Sjögren. Immun Inflamm Dis. 2026;14(9):e70507. doi:10.1002/iid3.70507
2. Atta H. Diagnostic delays linked to greater disease activity, worse quality of life in Sjögren disease. AJMC. October 9, 2026. Accessed October 13, 2026. https://www.ajmc.com/view/diagnostic-delays-linked-to-greater-disease-activity-worse-quality-of-life-in-sj-gren-disease