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      Imaging in Primary Sjögren’s Syndrome

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          Abstract

          Primary Sjögren’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.

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          Most cited references103

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          Pathogenetic mechanisms in the initiation and perpetuation of Sjögren's syndrome.

          Sjögren's syndrome (SS), a chronic autoimmune disorder, particularly compromises the function of exocrine glands. The involvement of these glands is characterized by focal, mononuclear cell infiltrates that surround the ducts and replace the secretory units. The pathogenetic mechanisms of this autoimmune exocrinopathy have not been fully elucidated. Immunologically-activated or apoptotic glandular epithelial cells that expose autoantigens in genetically predisposed individuals might drive autoimmune-mediated tissue injury. Alterations in several immune mediators, such as upregulation of type I interferon-regulated genes, abnormal expression of B-cell-activating factor and activation of the interleukin-23-type 17 T-helper cell pathway, have been reported. Extension of the pathological process that affects the exocrine glands into periepithelial and extraepithelial tissue can cause a considerable percentage of patients to exhibit systemic findings that involve the lungs, liver or kidneys. These manifestations develop as a result of lymphocytic invasion or an immune-complex-mediated process, or both, and present as skin vasculitis coupled with peripheral neuropathy or glomerulonephritis (or both). Patients with systemic extraepithelial manifestations display low serum levels of the complement component C4 and mixed type II cryoglobulins, and show an increased risk of developing non-Hodgkin lymphoma, thereby reflecting an overall worse prognosis with higher mortality rates than those without extraepithelial manifestations.
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            Systemic involvement in primary Sjogren's syndrome evaluated by the EULAR-SS disease activity index: analysis of 921 Spanish patients (GEAS-SS Registry).

            To evaluate systemic involvement in primary SS in a large cohort of Spanish patients using the EULAR-SS disease activity index (ESSDAI) definitions.
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              Characterizing IgG4-related disease with 18F-FDG PET/CT: a prospective cohort study

              Purpose IgG4-related disease (IgG4-RD) is an increasingly recognized clinicopathological disorder with immune-mediated inflammatory lesions mimicking malignancies. A cohort study was prospectively designed to investigate the value of 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) in characterizing IgG4-RD. Methods Thirty-five patients diagnosed with IgG4-RD according to the consensus criteria were enrolled with informed consent. All patients underwent baseline 18F-FDG PET/CT evaluation. Among them, 29 patients underwent a second 18F-FDG PET/CT scan after 2 to 4 weeks of steroid-based therapy. Results All 35 patients were found with 18F-FDG-avid hypermetabolic lesion(s); 97.1 % (34/35) of these patients showed multi-organ involvement. Among the 35 patients, 71.4 % (25/35) patients were found with more organ involvement on 18F-FDG PET/CT than conventional evaluations including physical examination, ultrasonography, and computed tomography (CT). 18F-FDG PET/CT demonstrated specific image characteristics and pattern of IgG4-RD, including diffusely elevated 18F-FDG uptake in the pancreas and salivary glands, patchy lesions in the retroperitoneal region and vascular wall, and multi-organ involvement that cannot be interpreted as metastasis. Comprehensive understanding of all involvement aided the biopsy-site selection in seven patients and the recanalization of ureteral obstruction in five patients. After 2 to 4 weeks of steroid-based therapy at 40 mg to 50 mg prednisone per day, 72.4 % (21/29) of the patients showed complete remission, whereas the others exhibited > 81.8 % decrease in 18F-FDG uptake. Conclusion F-FDG PET/CT is a useful tool for assessing organ involvement, monitoring therapeutic response, and guiding interventional treatment of IgG4-RD. The image pattern is suggested to be updated into the consensus diagnostic criteria for IgG4-RD.
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                Author and article information

                Journal
                J Clin Med
                J Clin Med
                jcm
                Journal of Clinical Medicine
                MDPI
                2077-0383
                03 August 2020
                August 2020
                : 9
                : 8
                : 2492
                Affiliations
                [1 ]Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, 9713 GZ Groningen, The Netherlands; e.mossel@ 123456umcg.nl (E.M.); f.g.m.kroese@ 123456umcg.nl (F.G.K.); h.bootsma@ 123456umcg.nl (H.B.)
                [2 ]Department of Nuclear Medicine and Molecular Imaging, University of Groningen, University Medical Center Groningen, 9713 GZ Groningen, The Netherlands; a.w.j.m.glaudemans@ 123456umcg.nl
                [3 ]Department of Pathology and Medical Biology, University of Groningen, University Medical Center Groningen, 9713 GZ Groningen, The Netherlands; b.van.der.vegt@ 123456umcg.nl
                [4 ]Department of Oral and Maxillofacial Surgery, University of Groningen, University Medical Center Groningen, 9713 GZ Groningen, The Netherlands
                Author notes
                [* ]Correspondence: m.s.van.ginkel@ 123456umcg.nl (M.S.v.G.); a.vissink@ 123456umcg.nl (A.V.); Tel.: +316-50-36-16-604 (M.S.v.G.); +316-50-36-13-841 (A.V.)
                Author information
                https://orcid.org/0000-0002-0867-0282
                https://orcid.org/0000-0001-8081-0641
                https://orcid.org/0000-0002-2613-1506
                https://orcid.org/0000-0003-2581-4361
                Article
                jcm-09-02492
                10.3390/jcm9082492
                7463854
                32756395
                480dffc4-be87-484a-9ee8-a95e74bd522a
                © 2020 by the authors.

                Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license ( http://creativecommons.org/licenses/by/4.0/).

                History
                : 10 July 2020
                : 30 July 2020
                Categories
                Review

                primary sjögren’s syndrome,imaging,salivary gland,sialography,salivary gland ultrasonography,magnetic resonance imaging,sialendoscopy,salivary gland scintigraphy,positron emission tomography

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