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Sicca Syndrome

Laura C Cappelli, Ami A Shah, Clifton O Bingham
Immune checkpoint inhibitors (ICIs) are newly approved treatments for advanced malignancies that are increasing survival. The mechanism of these drugs, non-specifically activating T cells, also leads to immune-mediated damage of tissue or immune-related adverse events (IRAE). IRAEs with rheumatic phenotypes are increasingly being recognised. Inflammatory arthritis, sicca syndrome, inflammatory myopathy, vasculitis and lupus nephritis have been described as a result of ICIs. Use of ICIs will be expanding in the coming years for several reasons...
2016: RMD Open
Jianjun Gu, Jiajie Zhai, Guiqing Liao, Jiaqi Chen
PURPOSE: To describe the results of Boston keratoprosthesis implantation in a patient with Stevens-Johnson syndrome following autologous submandibular gland transplantation. DESIGN: Observational case report. METHODS: We report the case of a man diagnosed with Stevens-Johnson syndrome. Autologous submandibular gland transplantation was performed in the right eye in 2008 to ameliorate tear film deficiency. Due to the improvement in the microenvironment of the ocular surface, Boston keratoprosthesis implantation was performed in the right eye in 2011...
October 11, 2016: Ocular Immunology and Inflammation
Petr Husa
Extrahepatic manifestations of hepatitis C virus infection (HCV) are very common. The most common of these is mixed cryoglobulinaemia. Anti-HCV antibodies and viral ribonucleic acid, HCV RNA, can be found in the cryoprecipitates, together with the rheumatoid factor. Cryoglobulins consist of a complex of immunoglobulins that in vitro precipitate upon the cooling bellow the human body temperature. Vasculitis is caused by the deposition of such immune complexes in the small blood vessels. A link with the HCV infection is considered to be established with membranoproliferative glomerulonephritis, leukocytoclastic vasculitis, lymphoproliferative disorders (in particular B cell lymphoma), Sjögren and sicca syndrome, lichen planus, porfyria cutanea tarda and diabetes mellitus...
2016: Vnitr̆ní Lékar̆ství
Silvia Bosello, Giusy Peluso, Federica Iavarone, Barbara Tolusso, Irene Messana, Gavino Faa, Massimo Castagnola, Gianfranco Ferraccioli
BACKGROUND: In the present study, we investigated whether thymosin β (Tβ) in saliva and in minor salivary glands is differentially expressed in patients with primary Sjögren's syndrome (pSS) and patients with autoimmune diseases (systemic sclerosis [SSc], systemic lupus erythematosus [SLE], and rheumatoid arthritis [RA], with and without sicca syndrome [ss]). METHODS: Saliva specimens of nine patients with pSS, seven with ss/SSc, seven with ss/SLE, seven with ss/RA, seven with SSc, seven with SLE, and seven with RA, as well as ten healthy subjects, were analyzed using a high-performance liquid chromatograph coupled with a mass spectrometer equipped with an electrospray ionization source to investigate the presence and levels of Tβ4, Tβ4 sulfoxide, and Tβ10...
October 6, 2016: Arthritis Research & Therapy
T G Coursey, F Bian, M Zaheer, S C Pflugfelder, E A Volpe, C S de Paiva
In both humans and animal models, the development of Sjögren syndrome (SS) and non-SS keratoconjunctivitis sicca (KCS) increases with age. Here, we investigated the ocular surface and lacrimal gland (LG) phenotype of NOD.B10.H2(b) mice at 7-14, 45-50, and 96-100 weeks. Aged mice develop increased corneal permeability, CD4(+) T-cell infiltration, and conjunctival goblet cell loss. Aged mice have LG atrophy with increased lymphocyte infiltration and inflammatory cytokine levels. An increase in the frequency of CD4(+)Foxp3(+) T regulatory cells (Tregs) was observed with age in the cervical lymph node (CLN), spleen, and LG...
October 5, 2016: Mucosal Immunology
Divi Cornec, Sandrine Jousse-Joulin, Sebastian Costa, Thierry Marhadour, Pascale Marcorelles, Jean-Marie Berthelot, Eric Hachulla, Pierre-Yves Hatron, Vincent Goeb, Olivier Vittecoq, Emmanuel Nowak, Jacques-Olivier Pers, Valérie Devauchelle-Pensec, Alain Saraux
PURPOSE: To determine whether the severity of salivary-gland involvement, assessed using salivary gland ultrasonography [SGUS], histological focus score, or the unstimulated whole salivary flow [UWSF], was associated with the response to rituximab in patients with primary Sjögren's syndrome [pSS]. MATERIALS AND METHODS: Among the 120 patients with pSS enrolled in the randomised TEARS trial of rituximab versus placebo, 35 underwent either centralised minor salivary-gland biopsy or SGUS at inclusion...
2016: PloS One
Vatinee Y Bunya, Satasuk Joy Bhosai, Ana Maria Heidenreich, Kazuko Kitagawa, Genevieve B Larkin, Thomas M Lietman, Bruce D Gaynor, Esen K Akpek, Mina Massaro-Giordano, M Srinivasan, Travis C Porco, John P Whitcher, Stephen C Shiboski, Lindsey A Criswell, Caroline H Shiboski
PURPOSE: To identify a screening strategy for dry eye patients with a high likelihood of having Sjogren's syndrome (SS) through the evaluation of the association of ocular surface tests with the extraocular signs used for the diagnosis of SS. DESIGN: Multi-center cross-sectional study. METHODS: The Sjogren's International Clinical Collaborative Alliance (SICCA) registry enrolled 3,514 participants with SS or possible SS from 9 international academic sites...
September 16, 2016: American Journal of Ophthalmology
Clodoveo Ferri, Manuel Ramos-Casals, Anna Linda Zignego, Luca Arcaini, Dario Roccatello, Alessandro Antonelli, David Saadoun, Anne Claire Desbois, Marco Sebastiani, Milvia Casato, Peter Lamprecht, Alessandra Mangia, Athanasios G Tzioufas, Zobair M Younossi, Patrice Cacoub
Hepatitis C virus (HCV) infection is responsible for both hepatic and extra-hepatic disorders (HCV-EHDs); these latter are correlated on one hand clearly with HCV lymphotropism causing immune-system dysregulation as well as with viral oncogenic potential, and on the other hand probably with chronic inflammatory status causing cardio-metabolic complications as well as neurocognitive disturbances. The spectrum of HCV-EHDs ranges from mild or moderate manifestations, such as arthralgia, sicca syndrome, peripheral neuropathy, to severe, life-threatening complications, mainly vasculitis and neoplastic complications...
September 15, 2016: Autoimmunity Reviews
Monisha Billings, Bruce A Dye, Timothy Iafolla, Alan N Baer, Margaret Grisius, Ilias Alevizos
BACKGROUND: Xerostomia is a chief complaint of patients with Sjögren's syndrome (SS). However, newer proposals for SS classification remove xerostomia and hyposalivation from the criteria list. Given these developments and the importance of patient-centered research outcomes, we sought to evaluate the utility of patient-reported xerostomia with implications for classification criteria, and clinical trials targeting SS treatment modalities. METHODS: A nested case-control study was designed within The National Institute of Dental and Craniofacial Research/National Institutes of Health (NIDCR/NIH) SS Cohort - one of the largest SS cohorts in the US...
September 9, 2016: EBioMedicine
E Apostolou, E K Kapsogeorgou, O D Konsta, I Giotakis, M I Saridaki, E Andreakos, A G Tzioufas
Type III interferons (IFNs) or IFN-λs (IFN-λ1/IL29, IFN-λ2/interleukin (IL)-28A and IFN-λ3/IL-28B) consist of a recently identified group of IFNs, implicated initially in several human diseases, including cancer and autoimmunity. In this study, we sought to investigate the expression of type III IFNs and their common receptor IFN-λR1/IL-28Ra in Sjögren's syndrome (SS). Type III IFN expression was examined in minor salivary gland tissues (MSG), peripheral blood mononuclear cells (PBMCs), sera and resting or Toll-like receptor (TLR)-stimulated salivary gland epithelial cells (SGEC) from SS patients and sicca-complaining controls...
September 10, 2016: Clinical and Experimental Immunology
Amel Rezgui, Imene Ben Hassine, Monia Karmani, Fatma Ben Fredj, Chadia Laouani
The occurrence of renal and multiple organ Amyloïdosis is currently considered exceptional in the course of systemic lupus erythematosus. We report a case of a concomitant SLE and Amyloïdosis in a 57 year old female patient with hypothyroidism history, who presented with erythema nodosum, fever, arthralgia and sicca syndrome. Biological findings showed an inflammatory syndrome, renal failure, proteinuria (1g / 24h), positive auto antibodies and anti DNA. Lung radiology revealed medistinal lymphadenopathy, pleural nodules, ground glass infiltrates and pleuritis...
2016: Pan African Medical Journal
Susanna Maddali Bongi, Martina Orlandi, Angelina De Magnis, Daniela Moncini, Angela Del Rosso, Felice Galluccio, Alessandro Franchi
The goal of this study was to evaluate the characteristics and the prevalence of histopathologic and immunohistochemical changes in vulvar biopsies, in women with Primary Sjögren Syndrome (pSS) and non-Sjögren Sicca Syndrome (nS-SS). Twenty-one women with pSS and 11 with nS-SS (investigated by xerophtalmia and xerostomia tests, biopsy of minor salivary glands, gynecological history, and gynecologic symptoms score) underwent vulvar biopsies, evaluated for histopathologic and immunohistochemicalchanges, and compared with those obtained from 26 patients with lichen sclerosus...
November 2016: International Journal of Gynecological Pathology
Claudia Kedor, Jan Zernicke, Anja Hagemann, Lorena Martinez Gamboa, Johanna Callhoff, Gerd-Rüdiger Burmester, Eugen Feist
UNLABELLED: This study aims to investigate the efficacy and safety of low-dose cyclosporine A (CyA) in patients with primary Sjögren's syndrome (pSS) and articular involvement. This phase II open-label clinical study included 30 patients meeting the American-European Consensus group criteria for pSS with active joint involvement under stable symptomatic therapy. Treatment consisted of approximately 2 mg kg(-1) body weight of CyA day(-1) over a period of 16 weeks. The primary endpoint was defined as a reduction in the number of painful and/or swollen joints at end of treatment (EOT)...
September 2016: Clinical Rheumatology
Frédéric Chiambaretta, Serge Doan, Marc Labetoulle, Nicolas Rocher, Lamia El Fekih, Riadh Messaoud, Moncef Khairallah, Christophe Baudouin
PURPOSE: This study compared the efficacy and safety of hyaluronic acid (HA)-trehalose, a new eyedrop containing trehalose (a natural bioprotectant) and HA, to an established formulation containing only HA. METHODS: This was a phase III, randomized, active-controlled, investigator-masked, multicenter study in France and Tunisia. In all, 105 adult patients (≥18 years) with moderate to severe dry eye disease (DED) received either HA-trehalose (n = 52) or HA (n = 53) 3-6 times per day for 84 days...
July 20, 2016: European Journal of Ophthalmology
Joana Campos, Maarten R Hillen, Francesca Barone
Primary Sjögren syndrome (pSS) can be considered a systemic autoimmune disease with a strong organ bias. The involvement of the exocrine glands is prevalent and drives the pathognomonic manifestations of dryness that define the sicca syndrome. The salivary glands also represent the hub of pSS pathology. Elements belonging to both innate and acquired immune responses have been described at this site that contribute to disease establishment and progression. The interaction between those elements and their relative contributions to the clinical manifestations and lymphoma progression largely remain to be addressed...
August 2016: Rheumatic Diseases Clinics of North America
Asghar Hajiabbasi, Irandokht Shenavar Masooleh, Yousef Alizadeh, Amir Sadredin Banikarimi, Pooneh Ghavidel Parsa
UNLABELLED: Sjogren syndrome (SS) can occur alone, primary Sjogren syndrome, or in association with other rheumatic diseases, secondary Sjogren syndrome (sSS), such as Rheumatoid arthritis (RA). The occurrence of Sjogren syndrome with RA makes it course worse and increases high morbidity and mortality of RA. In this exploratory study we aim to determine the prevalence of sSS (diagnosed based on the revised version of American-European consensus Group Classification Criteria: AUCG-criteria), sicca symptoms (dry eye, dry mouth), positive autoantibody tests (Anti RO or Anti-LA antibodies), UWSFR (Unstimulated Whole Salivary Flow Rate), Schirmer and Lissamine test...
July 2016: Acta Medica Iranica
Maureen Rischmueller, Joanna Tieu, Susan Lester
Primary Sjögren's syndrome (pSS) is a relatively common autoimmune systemic rheumatic disease. In addition to sicca syndrome and swollen salivary glands, systemic features manifest in the majority of patients, and are severe in 15%, particularly affecting the joints, skin, lungs, and peripheral nervous system. A recent meta-analysis estimated a pooled relative risk of 13.76 for the development of non-Hodgkin lymphoma, particularly in pSS patients who have parotid enlargement, vasculitis, cryoglobulinemia, and antibodies to Ro and La...
February 2016: Best Practice & Research. Clinical Rheumatology
Pulukool Sandhya, Biji Theyilamannil Kurien, Debashish Danda, Robert Hal Scofield
Sjögren's syndrome is a common autoimmune disease that presents with sicca symptoms and extraglandular features. Sjögren's syndrome is presumably as common as RA; yet it is poorly understood, underdiagnosed and undertreated. From the usual identity as an autoimmune exocrinopathy to its most recent designate as an autoimmune epithelitis - the journey of SS is complex. We herein review some of the most important milestones that have shed light on different aspects of pathogenesis of this enigmatic disease. This includes role of salivary gland epithelial cells, and their interaction with cells of innate and adaptive immune system...
July 14, 2016: Current Rheumatology Reviews
Alain Saraux, Jacques-Olivier Pers, Valérie Devauchelle-Pensec
Primary Sjögren syndrome (pSS) is a progressive autoimmune disease characterized by sicca and systemic manifestations. In this Review, we summarize the available data on topical and systemic medications, according to clinical signs and disease activity, and we describe the ongoing studies using biologic drugs in the treatment of pSS. Expanding knowledge about the epidemiology, classification criteria, systemic activity scoring (ESSDAI) and patient-reported outcomes (ESSPRI) is driving active research. Treatment decisions are based on the evaluation of symptoms and extraglandular manifestations...
August 2016: Nature Reviews. Rheumatology
Maartje J L Colaris, Mintsje de Boer, Rene R van der Hulst, Jan Willem Cohen Tervaert
In this study, we compared one hundred patients with autoimmune/inflammatory syndrome induced by adjuvants (ASIA) due to silicone implant incompatibility syndrome diagnosed in 2014 in Maastricht, the Netherlands, with one hundred historical patients with adjuvant breast disease diagnosed in the Baylor College of Medicine, Houston, USA, between 1985 and 1992. Similarities and differences between these two cohorts were identified to determine whether the spectrum of silicone-related disease changed during the last 30 years...
July 13, 2016: Immunologic Research
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