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• Sjogren's Syndrome
• Lip biopsy for minor salivary gland (biopsy for Sjogren's syndrome)

Biopsy

Oral cavity, labial mucosa

• Lymphocytic sialadenitis with a positive focus score (2.25) consistent with Sjogren's syndrome.
• Sections show at least six lobules of minor salivary gland tissue that has a nodular lymphocytic infiltrate with 2.25 clusters of more than 50 lymphocytes per 4mm² of tissue (Focus score = 2.25).
• A focus score >= 1 (as seen in this case) can be used to support the diagnosis of Sjogren syndrome in the appropriate clinical context

Histopathology

As per Kroese et al (Kroese 2018): "A focus is defined as an aggregate of ≥50 lymphocytes and the focus score (FS) is the total number of foci per 4mm² salivary gland tissue" - with additional comment that "Not all pSS (primary Sjogren's syndrome) patients have a positive salivary gland biopsy" - reporting in 18 - 40% of cases the focus score is <1.0 - emphasizing the importance of the clinical diagnosis over a single study such as lip biopsy. They identify a false positive in healthy individuals with a focus score >1.0 in 6-9% with labial gland biopsy and 5% with parotid biopsy. Reporting from Fisher et al (Fisher 2017).

Kroese identify that the minimum number of minor salivary glands for analysis ia suggested to be 4 (six if small) and should be surgically separated - with the minimum surface area of gland resection to be 8 mm².

Vivino et al. in 2002 reported that a second expert evaluation of 58 labial salivary glands re-analyzed by a single center led to revision of the initial diagnosis in 53% of the patients (Vivino 2002).

References

Daniels et al.  Associations between salivary gland histopathologic diagnoses and phenotype features of Sjogren's syndrome among 1,726 registry participants.  Arthritis and Rheumatism 2011; 63(7): 2021-2030.

KROESE FGM, HAACKE EA, BOMBARDIERI M: The role of salivary gland histopathology in primary Sjögren’s syndrome: promises and pitfalls. Clin Exp Rheumatol 2018; 36 (Suppl. 112): S222-33. [excellent review article including information about response to DMARD to help SS pts with disease modifying antirheumatic drugs]

FISHER BA, JONSSON R, DANIELS T et al.: Standardisation of labial salivary gland histopathology in clinical trials in primary Sjögren’s syndrome. Ann Rheum Dis 2017; 76: 1161-8.

VIVINO FB, GALA I, HERMANN GA: Change in final diagnosis on second evaluation of labial minor salivary gland biopsies. J Rheumatol 2002; 29: 938-44