Sjögren’s syndrome: state of the art on clinical practice guidelines

Romão, Vasco C; Talarico, Rosaria; Scirè, Carlo Alberto; Vieira, Ana; Alexander, Tobias; Baldini, Chiara; Gottenberg, Jacques-Éric; Gruner, Heidi; Hachulla, É.; Mouthon, Luc; Orlandi, Martina; Pamfil, Cristina; Chambrun, Marc Pineton de; Taglietti, Marco; Toplak, Nataša; Daele, Paul Van; Laar, Jacob M van; Bombardieri, Stefano; Schneider, Matthias; Smith, Vanessa; Cutolo, Maurizio; Mosca, Marta; Mariette, Xavier

doi:10.1136/rmdopen-2018-000789

Cited by 40 publications

(24 citation statements)

References 31 publications

(41 reference statements)

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“…Thus, pSS patients with a normal-appearing SGUS may represent a clinical phenotype with milder disease and probably a lower lymphoma risk based on present knowledge (36)(37)(38)(39)(40). Improving the identification of a milder disease subset is an unmet need in pSS (41), and therefore, the SGUS could be of major value to this end. Moreover, the SGUS may be employed to detect and to guide diagnostic biopsy of salivary gland lymphoma (42,43), which is one of the main causes of increased mortality in pSS (36), and to follow-up pSS patients with parotid swelling, i.e., a major risk factor or an early manifestation of lymphoma itself (44).…”

Section: Discussionmentioning

confidence: 99%

Normal-Appearing Salivary Gland Ultrasonography Identifies a Milder Phenotype of Primary Sjögren's Syndrome

Callegher¹,

Zabotti²,

Giovannini³

et al. 2020

Front. Med.

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Objective: Salivary gland ultrasound (SGUS) is emerging as a valid tool in the management of primary Sjögren's syndrome (pSS). This study aimed to investigate whether pSS patients with normal-appearing or pathological SGUS findings showed different clinical, laboratory, and pathologic pSS-related features, and to compare the results by using two different SGUS scores.Methods: Consecutive pSS patients, according to the ACR-EULAR classification criteria, were evaluated. Salivary glands were scored using the early 1992 score by De Vita et al. and the latest 2019 OMERACT score, both being semiquantitative 0–3 scoring systems focused on ultrasonographic parenchymal inhomogeneity (grades 0 and 1, normal-appearing; grades 2 and 3, pathological). The patients were then divided into two groups: “SGUS normal-appearing” if all the salivary glands had normal-appearing parenchyma (grade 0 or 1), or “SGUS pathological” if the grade was 2 or 3 in at least one salivary gland. The associations between SGUS and pSS-related clinical, laboratory, and pathological features were then investigated in the two groups.Results: One hundred pSS patients were evaluated, the mean age (±SD) was 60.9 ± 12.0 years, and mean disease duration was 11.7 ± 7.2 years. Twenty-nine out of 100 (29%) patients were in the “SGUS normal-appearing” group and 71/100 (71%) were in the “SGUS pathological” group. A normal-appearing SGUS was significantly associated with the absence of anti-La/SSB antibodies (p < 0.001) and normal unstimulated salivary flow rate (p = 0.02) by both univariate and multivariate analyses. By univariate analysis, a normal-appearing SGUS was significantly associated also with the absence of rheumatoid factor (p = 0.002) and of serum monoclonal component (p = 0.003), ESSDAI < 5 (p = 0.03), and with a negative lip biopsy (p = 0.029). No associations were found with other items, including anti-Ro/SSA (p = 0.145), Schirmer's test (p = 0.793), ESSPRI (p = 0.47), and demographic data. No differences in these results were observed by using the two SGUS scoring systems.Conclusion: The SGUS allowed the identification of different phenotypes of pSS, and different SGUS scores focused on salivary gland inhomogeneity may be effective to this end.

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Section: Discussionmentioning

confidence: 99%

Normal-Appearing Salivary Gland Ultrasonography Identifies a Milder Phenotype of Primary Sjögren's Syndrome

Callegher¹,

Zabotti²,

Giovannini³

et al. 2020

Front. Med.

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“…In countries with a gatekeeping system, general practitioners (GPs) have a fixed practice population and they are normally the first point of contact with the health care system. Routinely recorded electronic health care data in primary care may be used to develop early detection models or estimate population prevalences for diseases such as pSS defined as "complex with rare complications" 10 and in general, to study the disease in a "real life" situation, outside the setting of a specialized clinical center. 11 In the Netherlands, and in many other countries in Europe (eg, the United Kingdom, Italy, and Spain), primary care practices use an EHR system to record the care delivered to their patients and the health problems presented.…”

Section: Primary Care Datamentioning

confidence: 99%

Fitness for purpose of routinely recorded health data to identify patients with complex diseases: The case of Sjögren's syndrome

Wiegersma

Flinterman

Seghieri

et al. 2020

Learning Health Systems

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Background: This study is part of the EU-funded project HarmonicSS, aimed at improving the treatment and diagnosis of primary Sjögren's syndrome (pSS). pSS is an underdiagnosed, long-term autoimmune disease that affects particularly salivary and lachrymal glands. Objectives: We assessed the usability of routinely recorded primary care and hospital claims data for the identification and validation of patients with complex diseases such as pSS. Methods: pSS patients were identified in primary care by translating the formal inclusion and exclusion criteria for pSS into a patient selection algorithm using data from Nivel Primary Care Database (PCD), covering 10% of the Dutch population between 2006 and 2017. As part of a validation exercise, the pSS patients found by the algorithm were compared to Diagnosis Related Groups (DRG) recorded in the national hospital insurance claims database (DIS) between 2013 and 2017. Results: International Classification of Primary Care (ICPC) coded general practitioner (GP) contacts combined with the mention of "Sjögren" in the disease episode titles, were found to best translate the formal classification criteria to a selection algorithm for pSS. A total of 1462 possible pSS patients were identified in primary care (mean prevalence 0.7‰, against 0.61‰ reported globally). The DIS contained 208 545 patients with a Sjögren related DRG or ICD10 code (prevalence 2017: 2.73‰). A total of 2 577 577 patients from Nivel PCD were linked to the DIS database. A total of 716 of the linked pSS patients (55.3%) were confirmed based on the DIS.

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“…Pain and fatigue are often treated with hydroxychloroquine. Patients and clinicians have identified unmet medical needs for SS and the necessity for novel drug development (Romao et al, 2018).…”

Section: Background Informationmentioning

confidence: 99%

Spontaneous Model of Sjögren's Syndrome in NOD Mice

et al. 2019

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The non‐obese diabetic (NOD) mouse model is the most widely described and validated method for investigating human primary Sjögren's syndrome (SS) and represents a useful model for translational studies. However, the systemic disease manifestation in NOD mice is sensitive to the housing environment, as stress modulates the immune system, so it is essential to confirm that readouts are robust, reproducible, and sensitive to known clinical treatments. This protocol describes the establishment of the spontaneous NOD model of SS and underscores the necessity of model validation to ensure that the housing environment is compatible. © 2019 by John Wiley & Sons, Inc.

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Sjögren’s syndrome: state of the art on clinical practice guidelines

Cited by 40 publications

References 31 publications

Normal-Appearing Salivary Gland Ultrasonography Identifies a Milder Phenotype of Primary Sjögren's Syndrome

Normal-Appearing Salivary Gland Ultrasonography Identifies a Milder Phenotype of Primary Sjögren's Syndrome

Fitness for purpose of routinely recorded health data to identify patients with complex diseases: The case of Sjögren's syndrome

Spontaneous Model of Sjögren's Syndrome in NOD Mice

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