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SSc is an auto-immune disease characterized by life-threatening manifestations such as lung fibrosis or pulmonary arterial hypertension. Symptoms with a detrimental impact on quality of life are also reported and sicca syndrome (xerostomia, xeropthalmia) is present in up to 80% of patients with SSc. Sicca syndrome can occur in the absence of overlap with Sjögren’s disease and recent studies highlight that fibrosis of minor and major salivary glands, directly linked to the pathogenesis of SSc, could be a major contributor of xerostomia in SSc. This narrative review provides an overview of the clinical presentation, diagnostic strategies, management and future perspectives on sicca syndrome in patients with SSc.

Objective The aim of this study was to assess intraobserver and interobserver reliability of minor salivary gland biopsy (MSGB) in SS. Methods All MSGBs available from the Tolerance and Efficacy of Rituximab in Primary Sjogren's Syndrome (TEARS) study were subjected to a standardized blinded assessment by a single specifically trained pathologist twice at a 2 month interval; both the Chisholm-Mason (CM) grade and the focus score (FS) were determined. Baseline histopathological reports by local pathologists at each study centre were compared with the first standardized blinded assessment. Agreement was assessed for the dichotomized FS (dFS) and dichotomized CM (dCM) grade, as well as for nine other histopathological features. Results Eighty-nine MSGBs were studied. Intraobserver κ values were 1 for dFS, 0.80 for dCM, 0.67 for germinal centre-like structures, 0.44 for fibrosis and 0.29 for confluent foci. Most of the local histopathological reports based their diagnosis on the CM grade, although the FS was often reported or the data needed to determine it were provided. Interobserver agreement κ values were 0.71 for dFS, 0.64 for dCM, 0.46 for focal lymphocytic sialadenitis, 0.42 for non-specific chronic inflammation and 0.16 for fibrosis. Conclusion Although FS reliability was good, disparities were noted in the assessment methods used by local pathologists. The protocol for FS determination was not followed routinely, with the result that the FS was often overestimated. Germinal centre-like structures, which predict lymphoma, showed good reliability but were inconsistently reported.

International audience; OBJECTIVE: The aim of this study was to evaluate whether salivary gland ultrasonography (SGUS) improves the diagnostic performance of the 2012 ACR classification criteria for SS. METHODS: We studied a cohort of 101 patients with suspected SS seen at a single centre in Brittany, France. An SGUS echostructure score ≥2 was considered abnormal. The reference standard was a clinical diagnosis of SS made by a group of experts blinded to SGUS findings. RESULTS: SS was diagnosed in 45 patients. Similar proportions of patients with and without SS had an ocular staining score ≥3. Adding RF positivity and ANA titre ≥1:320 as an alternative to anti-SSA/SSB positivity increased the sensitivity of the serological item without modifying specificity compared with using anti-SSA/SSB alone. SGUS was 60.0% sensitive and 87.5% specific for SS. Adding the SGUS score to the ACR criteria increased sensitivity from 64.4% to 84.4% and only slightly decreased specificity, from 91.1% to 89.3%. CONCLUSION: The diagnostic performance of the ACR classification criteria for SS is notably improved by adding the SGUS score. SGUS should be included in future classification criteria for SS.

Objective. The aim of this study was to investigate the current use of musculoskeletal US (MSUS) and the most relevant areas of interest for this imaging modality in paediatric rheumatology. Methods. A questionnaire was developed by the paediatric subgroup of the OMERACT US task force and e-mailed to the members of the main international paediatric rheumatology networks and societies. Responses were entered in an electronic database. Results were analysed quantitatively or summarized qualitatively in the case of open questions. Results. The overall response rate was 36% (262/719). The use of MSUS varied among members of the various networks/societies. MSUS was considered of high relevance for improvement of diagnostic skills, for the guidance of joint injections and for the assessment of specific joints, namely the hip, ankle, midfoot and wrist. It was considered useful for early detection of synovitis and in determining disease activity and disease remission. Conclusion. Although at present MSUS is not widely used by paediatric rheumatologists, there is considerable interest in this imaging technology among members of the international networks. The results of this survey suggest that the next objective in the research agenda should be the standardization of the assessment of joints in healthy children. This will then help differentiate pathological (i.e. synovitic) joints from normal joints. The initial target joints should be the hip, ankle, midfoot and wrist. MSUS training focused on the assessment of paediatric patients might be very important in implementing the use of this technique in clinical practice and research.

US has demonstrated higher sensitivity in detecting synovitis, tenosynovitis and enthesitis than clinical examination in JIA. US has also allowed earlier assessment of cartilage and bone abnormalities than conventional radiology. Although it seems that US detects a high frequency of subclinical synovitis in JIA patients with clinically inactive disease, further studies are needed to clarify those findings. In addition, assessment of structural damage changes in growing children should be taken with caution until more information about normal US articular anatomy in healthy children is available.