Background/Purpose:

Sicca syndrome is a clinical presentation that is common for several systemic diseases that may infiltrate the exocrine glands. The most frequent disease is Sjögren’s syndrome (SS), but other systemic diseases such as sarcoidosis, amyloidosis of IgG4-related disease may also infiltrate salivary glands. We analyzed the safety and usefulness of a new diagnostic tool for investigating minor salivary glands (minimally-invasive biopsy technique) in patients presenting with sicca syndrome.

Methods:

We present a prospective analysis of 200 patients presenting with sicca syndrome (defined by the presence of xerostomia and xerophthalmia, with positive results for ocular tests and/or parotid scintigraphy) in whom a minimally-invasive biopsy of minor salivary glands was carried out at a single center. For all biopsy samples, a cumulative focus score of lymphoplasmacytic infiltration was evaluated (Chisholm Mason score, CMs), together with investigation of other infiltrative processes caused by granuloma, amyloid, lipids or IgG4-positive cells.

Results:

All biopsies but two disclosed salivary gland tissue. Only 28 patients (14%) reported transient adverse events recovered completely during the first 24h. Histopathological diagnosis was available in 189 cases (158 women, 31 men, mean age 59 years): 44% of patients showed a non-specific chronic sialoadenitis (NSCS) (CMs=1-2), 21% focal lymphocytic sialoadenitis (FLS) suggestive of primary Sjögren syndrome (CMs=3-4), 12% chronic atrophic sialadenitis (CAS) (CMs unclassifiable), 18% normal glandular tissue (CMs=0) and other diagnosis in the remaining 4% (1 case of amyloidosis, 1 of sarcoidosis, 6 of lipoid infiltration and 1 salivary oncocytic cystadenoma); no patient had IgG4-related disease. The higher mean age was found in patients with CAS (69 years), followed by those with NSCS (59 years), those with FLS (57 years) and those with a normal result (54 years) (p<0.001). A higher frequency of FLS was found in patients with positive immunological markers including RF, anti-Ro, anti-La, hypocomplementemia and cryoglobulins (p<0.05). The percentage of patients diagnosed with Sjögren syndrome varied according to the concomitant systemic features: 13% of patients presenting with an isolated sicca syndrome had FLS in comparison with 32% of those who present with extraglandular involvement (p<0.05).

 Conclusion:

We found sialoadenitis in nearly 80% of patients presenting with sicca syndrome. One quarter of these patients were diagnosed with Sjögren syndrome (focal lymphocytic sialadenitis). An older age was associated with a higher frequency of atrophic and chronic histopathological findings, while the presence of systemic features was associated with a higher frequency of focal lymphocytic sialadenitis. Only 1% of patients showed other systemic infiltrative diseases (one case of sarcoidosis, one of amyloidosis); no patient showed infiltration by IgG4+ cells.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/usefulness-of-the-minimally-invasive-minor-salivary-gland-biopsy-in-patients-presenting-with-sicca-syndrome-prospective-evaluation-of-200-patients/