Background Primary Sjogren’s Syndrome (pSS) is a chronic inflammatory autoimmune disease characterized by lymphocytic infiltration into exocrine glands. Previous reports have demonstrated that a subgroup of anti-centromere antibody (ACA) positive pSS (ACA+/pSS) represents intermediate features between ACA negative pSS (ACA-/pSS) and limited cutaneous systemic sclerosis. ACA+/pSS patients had a higher prevalence of Raynoud’s phenomenon, primary biliary cirrhosis (PBC) and fibrotic change of minor salivary glands compared to ACA-/pSS patients.
Objectives We previously reported that submandibular gland (SG) ultrasonagraphy (US) is a useful noninvasive and inexpensive procedure for the evaluation of the morphological changes of salivary gland involvement in pSS patients (International Symposium on Sjogren’s Syndrome 2002, EULAR 2009). In the present study, we evaluated SG by using US in ACA+/pSS and ACA-/pSS patients.
Methods Twenty ACA+/pSS and 30 ACA-/pSS patients were studied. SG involvement was evaluated by a single blinded observer using US staging (range 1 to 4) that assigns points to the glandular size, different degree of glandular inhomogeneity and contrast of SG to diagastric muscle. The pulsatility index (PI) and resistance index (RI) were calculated by the pulsed wave traces by power Doppler US at the internal SG facial arteries. All pSS patients were evaluated for the presence of chronic thyroiditis, interstitial pneumonia, interstitial nephritis, Raynoud’s phenomenon, cutaneous manifestation, PBC and joint involvement. We also analyzed ACA, anti-SS-A antibodies, anti-SS-B antibodies, anti-M2 antibodies, rheumatoid factor (RF) and γ-globulin in serum from pSS patients. The diagnosis of PBC was based on liver function test, the presence of serum anti-M2 antibody and histopathological findings.
Results ACA+/pSS and ACA-/pSS subgroup did not differ in median age and the amount of whole saliva by gum test. The immunological parameters (anti-SS-A antibodies, anti-SS-B antibodies, RF and hyper γ-globulinemia) showed no significant differences between these subgroups. The prevalences of chronic thyroiditis, interstitial pneumonia, interstitial nephritis, cutaneous manifestation and joint involvement showed no differences between theses subgroups. However, the prevalence of PBC was significantly higher in ACA+/pSS patients than in ACA-/pSS patients (25.0% vs 3.3%, p=0.02). The prevalence of Raynoud’s phenomenon was tended to be higher in ACA+/pSS patients compared to ACA-/pSS patients (p=0.06). The US staging score was not significantly different between these subgroups (ACA+pSS; 2.45±1.1 vs ACA-pSS; 1.97±1.1), although the size of SG was significantly smaller in ACA+pSS than in ACA-pSS patients (203.0±76.0 vs 261.2±94.0 mm2, p<0.05). Furthermore, the prevalence of PI and RI measurement failure due to blood flow disappearance was higher in ACA+/pSS patients than in ACA-/pSS patients (75.0 vs 46.7%, p<0.05).
Conclusions By using US, we observed a higher prevalence of SG atrophy and low blood flow in ACA+/pSS patients as compared to ACA-/pSS patients. US assessment may be useful to evaluate the differences of histopathological changes of salivary glands between ACA+/pSS and ACA-/pSS patients.
Disclosure of Interest None Declared
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