Clinical characteristics and biopsy accuracy in suspected cases of Sjögren’s syndrome referred to labial salivary gland biopsy

Abstract

BackgroundLabial salivary gland biopsy (LSGB) is the most important diagnostic tool for the diagnosis of Sjögren’s syndrome (SS), but its diagnostic value is rarely studied. This study assessed the sensibility and specificity of LSGB, and the clinical profiles of patients who were referred for biopsy.MethodsRetrospective analysis of the histopathological reports from LSGB and medical report data from patients who underwent LSGB between 2008 and 2011 was conducted.ResultsAbout 290 biopsies were performed and 74 were excluded due to insufficient clinical data. Of the 216 patients, 0.46% was carrier of hepatitis C virus, 30.1% had primary SS (pSS), and 8.8% had secondary SS (sSS). Of the samples, 94.3% presented dryness symptoms, 51.6% experienced dryness only, 42.7% had systemic manifestations, and 66.9% presented low unstimulated salivary flow and/or Schirmer’s test. LSGB was necessary in 67.6% to confirm the presence of SS based on the American-European Consensus Group 2002 criteria (AECG). Based on specialist’s opinion, sensibility level was 86.57%, and specificity was 97.43%. Positive predictive value (PPV) was 95%, and negative predictive value (NPV) was 92.6%. Determined accuracy was 93.3%. Concordance (kappa coefficient) of LSGB and specialist’s opinion was 0.851, and LSGB with AECG criteria was 0.806. Of the 98 patients referred with fibromyalgia and dryness, 36.7% had SS and LSBG focus score of ≥ 1. Patients with SS were older, and showed more severe lachrymal and salivary dysfunctions, greater frequency of fibromyalgia, anti-nuclear antibodies (ANA), anti-SSA-Ro, and anti-SSB-La.ConclusionsLabial salivary gland biopsy has high sensibility, specificity, positive and negative predictive values for diagnosis of pSS. In the clinical practice, it is useful, especially for those patients with glandular dysfunctions and negative antibodies.

Oral yeast colonization in patients with primary and secondary Sjögren's syndrome

“…The SS pathogenesis is yet to be completely understood. However, several immune‐related processes, as well as possible predisposing factors have been described, supporting the idea of a multifactorial autoimmune disorder (Giovelli, Santos, & Serrano, ; Tzioufas, Kapsogeorgou, & Moutsopoulos, ). The most related symptoms of the disease are ocular dryness and hyposalivation.…”

Adipose tissue is prominent in salivary glands of Sjögren’s syndrome patients and appears to influence the microenvironment in these organs

“…Although only a few published studies have evaluated the diagnostic usefulness of SG biopsies [15], the diagnostic role of SG histology remains widely accepted and a central part of both AECG and ACR classification criteria for SS [9,10]. This routine biopsy assessment not only aids in the diagnosis of SS patients with no autoantibody production [18], but can also help in predicting adverse disease outcomes, such as lymphoma and extraglandular manifestations, which may complicate the course of disease [17]. Interestingly, the destruction of glandular tissue in SS is commonly accompanied by the presence of both adipose and fibrotic tissue [43], where adipocytes can occupy a large part of the gland.…”

“…This is why routine assessment of SG tissue may be useful in order to predict adverse outcomes, such as lymphoma and extraglandular manifestations, which may complicate the course of SS [17]. Moreover, it is particularly useful for those patients with glandular dysfunctions who do not show any autoantibody production [18]. Focus scoring is also essential for the prognosis of SS by providing information about different subsets of patients [19].…”

The Potential Role for Early Biomarker Testing as Part of a Modern, Multidisciplinary Approach to Sjögren’s Syndrome Diagnosis

“…Although invasive, biopsy of minor salivary glands has been traditionally considered the gold standard for making the diagnosis of SS [7,47], and is one of the objective criteria cited by the ACR [36]. While labial salivary gland histopathologic findings offer a high degree of specificity for SS [36], experience suggests that biopsy findings identify SS at more advanced stages of disease when gland damage has already occurred.…”