Lip Biopsy of Minor Salivary Gland Histopathology for Sjogren's Syndrome (sjogren / sjogrens)

return to: Sjogren's Syndrome

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

Oral cavity, labial mucosa, biopsy:
       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.

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) patientshave 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 inhealthy individuals with a focus scopre >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 si suggested to be 4 (six if small) and should be surgically separated - with the minim 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-analysed by a single centre 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 DMRAD 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