Search

Lung involvement, especially interstitial lung disease, is a potentially severe extra-glandular manifestation of Primary Sjogren's Syndrome (pSS-ILD). ILD can manifest either as a late complication of pSS or anticipate sicca symptoms, likely reflecting two different patho-physiological entities. Presence of lung involvement in pSS subjects can remain subclinical for a long time; therefore, patients should be actively screened, and lung ultrasound is currently being investigated as a potential low cost, radiation-free, easily repeatable screening tool for detection of ILD. In contrast, rheumatologic evaluation, serology testing, and minor salivary gland biopsy are crucial for the recognition of pSS in apparently idiopathic ILD patients. Whether the HRCT pattern influences prognosis and treatment response in pSS-ILD is not clear; a UIP pattern associated with a worse prognosis in some studies, but not in others. Many aspects of pSS-ILD, including its actual prevalence, association with specific clinical-serological characteristics, and prognosis, are still debated by the current literature, likely due to poor phenotypic stratification of patients in clinical studies. In the present review, we critically discuss these and other clinically relevant "hot topics" in pSS-ILD. More specifically, after a focused discussion, we compiled a list of questions regarding pSS-ILD that, in our opinion, are not easily answered by the available literature. We subsequently tried to formulate adequate answers on the basis of an extensive literature search and our clinical experience. At the same, we highlighted different issues that require further investigation.

Objectives: Ultra-high frequency ultrasonography (UHFUS) has been recently introduced in oral medicine due to its ability to image small anatomical structures including labial salivary glands (LSG). To date no ultrasonography morphological studies of labial salivary glands (LSG) have been carried out in SS. In this pilot study we aimed at analysing the distribution of UHFUS findings in LSG of patients with suspected SS, focusing in particular on the association with patients' oral dysfunction, antibody profiles and histopathology., Methods: Consecutive patients undergoing a LSG biopsy for clinically suspected SS were included in this study between January 2018 and January 2020. Intraoral UHFUS scan of the lip mucosa was performed with Vevo MD equipment, using a 70 MHz probe with a standardised protocol. LSG were assessed by using a four-grade semiquantitative scoring system (0-3), similar to the OMERACT scoring system used for major salivary glands. The distribution of UHFUS grades was compared in patients stratified according their final diagnosis, patients antibody profiles and LSG histopathology., Results: We included 128 patients with suspected SS: out of them, 54 (42.2%) received a final diagnosis of SS, made according to the ACR 2016 criteria and 74 (57.8%) were diagnosed as no-SS sicca controls. We found that LSG inhomogeneity was significantly greater in patients with SS than in no-SS subjects (p<0.0001). We also found that higher UHFUS pattern of inhomogeneity (i.e. grade 2 and 3) were significantly more frequent in both SSA+/SSB- and SSA+/SSB+ patients (p=0.001). A normal UHFUS pattern, by contrast, was significantly more common in SSA-/SSB- subjects (i.e. 15/83 (18.1%) vs. 1/33 (3%) vs. 0/12 (0%), p=0.001). Finally, LSG inhomogeneity was significantly associated with both the number of foci (p<0.001) and focus score (p<0.001). Particularly, we found that both the number of foci and the FS were significantly higher in patients presenting a UHFUS grading of 2 and 3 with respect to those presenting a UHFUS grading of 0 and 1 (p=0.01)., Conclusions: This preliminary study demonstrates the optimal feasibility of UHFUS and its high sensitivity in identifying negative patients on subsequent lip biopsy, thus avoiding invasive procedures in selected cases. Further studies are in progress to define the clinical and predictive role of the various patterns observed and their added value with respect to traditional salivary gland ultrasonography.