Incorporation of Salivary Gland Ultrasonography Into the American College of Rheumatology/European League Against Rheumatism Criteria for Primary Sjögren's Syndrome

Objective: To assess whether the addition of salivary gland ultrasonography (SGUS) or replacement of current criteria items by SGUS influences the performance of the American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) criteria for primary Sjögren's syndrome. Methods: Included were consecutive patients with complete data on all ACR/EULAR items (n = 243) who underwent SGUS in our primary Sjögren's syndrome expertise center. Clinical diagnosis by the treating physician was used as the gold standard. Separate analyses were performed for patients who underwent labial or parotid gland biopsies. The average score for hypoechogenic areas in 1 parotid and 1 submandibular gland was determined (range 0–3). Next, performance of the ACR/EULAR criteria was evaluated after addition of SGUS or replacement of current items by SGUS. Results: Receiver operating characteristic analysis showed an optimal cutoff value of ≥1.5 for SGUS. The optimal weight for SGUS positivity was 1. Cutoff for ACR/EULAR fulfilment remained ≥4. In patients who underwent a labial gland biopsy (n = 124), the original criteria showed an area under the curve (AUC) of 0.965, sensitivity of 95.9%, and specificity of 92.2%. After the addition of SGUS, the AUC was 0.966, with a sensitivity of 97.3% and specificity of 90.2%. In patients who underwent a parotid gland biopsy (n = 198), similar results were found. Sensitivity of the criteria decreased substantially when SGUS replaced salivary gland biopsy or anti-SSA antibodies, while performance remained equal when SGUS replaced the ocular staining score, Schirmer's test, or unstimulated whole saliva flow. Conclusion: Validity of the ACR/EULAR criteria remains high after incorporation of SGUS. With SGUS, clinicians are offered a larger array of tests to evaluate fulfillment of the ACR/EULAR criteria

Histopathology, salivary flow and ultrasonography of the parotid gland:three complementary measurements in primary Sjögren's syndrome

OBJECTIVE: The involvement of salivary glands in primary Sjögren's syndrome (pSS) can be assessed in different ways: histopathology, salivary flow and ultrasonography. To understand the relative value of these different approaches, it is crucial to understand the relationship between them. As we routinely perform these three modalities in the parotid gland for disease evaluation, our aim was to investigate the construct validity between these modalities in one and the same gland. METHODS: Consecutive sicca patients underwent a multidisciplinary diagnostic work-up including parotid gland biopsy, collection of parotid gland-specific saliva and parotid gland ultrasonography. Patients who were classified as pSS according to the ACR-EULAR criteria were included. Construct validity was assessed using Spearman's correlation coefficients. RESULTS: The 41 included pSS patients completed a full work-up within mean time interval of 2.6 months. Correlations between histopathological features and stimulated parotid salivary flow were fair (ρ=-0.123 for focus score, and ρ=-0.259 for percentage of CD45+ infiltrate). Likewise, poor correlations were observed between stimulated parotid salivary flow and parotid ultrasonography (ρ=-0.196). Moderate to good associations were found between the histopathological items focus score and percentage of CD45+ infiltrate, with parotid ultrasound scores (total ultrasound score: ρ = 0.510 and ρ = 0.560; highest for homogeneity: ρ = 0.574 and ρ = 0.633). CONCLUSION: Although pSS associated ultrasonographic findings did correlate with histopathological features, the three modalities that evaluate salivary gland involvement assess different (or at best partly related) constructs. Therefore, histopathology, salivary flow and ultrasonography are complementary measurements and cannot directly replace each other in the work-up of pSS

Health-related quality of life in young adults with symptoms of constipation continuing from childhood into adulthood

<p>Abstract</p> <p>Background</p> <p>Children with functional constipation report impaired Health-related Quality of Life (HRQoL) in relation to physical complaints and long duration of symptoms. In about one third of children with constipation, symptoms continue into adulthood. Knowledge on HRQoL in adults with constipation persisting from childhood is lacking.</p> <p>Objectives</p> <p>To assess HRQoL in adults with constipation from early childhood in comparison to that of their peers. Furthermore to gain insight into the specific social consequences related to continuing symptoms of constipation and/or fecal incontinence at adult age.</p> <p>Methods</p> <p>One HRQoL questionnaire and one self-developed questionnaire focusing on specific consequences of symptoms of constipation continuing into adulthood were administrated to 182 adults with a history of childhood constipation. Successful clinical outcome was defined as a defecation frequency three or more times per week with less than two episodes of fecal incontinence per month, irrespective of laxative use. HRQoL of both adults with unsuccessful and successful clinical outcome were compared to a control group of 361 peers from the general Dutch population.</p> <p>Results</p> <p>No differences in HRQoL were found between the whole study population and healthy peers, nor between adults with successful clinical outcome (n = 139) and the control group. Adults with an unsuccessful clinical outcome (n = 43) reported significantly lower HRQoL compared to the control group with respect to scores on bodily pain (mean ± SD 77.4 ± 19.6 versus 85.7 ± 19.5, p = 0.01) and general health (67.6 ± 18.8 versus 74.0 ± 18.1, p = 0.04). Adults with an unsuccessful clinical outcome reported difficulties with social contact and intimacy (20% and 12.5%, respectively), related to their current symptoms. Current therapy in these adults was more often self-administered treatment (e.g. diet modifications) (60.4%) than laxatives (20.9%).</p> <p>Conclusion</p> <p>Overall, young adults with constipation in childhood report a good quality of life, as HRQoL of adults with successful clinical outcome was comparable to that of their peers. However, when childhood constipation continues into adulthood, it influences HRQoL negatively with social consequences in 20% of these adults.</p

Imaging in Primary Sjögren’s Syndrome

Primary Sj&ouml;gren&rsquo;s syndrome (pSS) is a systemic autoimmune disease characterized by dysfunction and lymphocytic infiltration of the salivary and lacrimal glands. Besides the characteristic sicca complaints, pSS patients can present a spectrum of signs and symptoms, which challenges the diagnostic process. Various imaging techniques can be used to assist in the diagnostic work-up and follow-up of pSS patients. Developments in imaging techniques provide new opportunities and perspectives. In this descriptive review, we discuss imaging techniques that are used in pSS with a focus on the salivary glands. The emphasis is on the contribution of these techniques to the diagnosis of pSS, their potential in assessing disease activity and disease progression in pSS, and their contribution to diagnosing and staging of pSS-associated lymphomas. Imaging findings of the salivary glands will be linked to histopathological changes in the salivary glands of pSS patients