Adjuvant rituximab improves sensory ataxia in CIDP-related Sjögren syndrome

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune neuropathy characterised by insidious onset, progressive course, proximal and distal symmetrical weakness, and sensory impairment. It may affect patients of any age with varying degrees of clinical involvement and response rates to existing treatments. Sjögren syndrome (SS) is a systemic autoimmune disorder that primarily affects the exocrine glands causing a sicca syndrome. It may affect the peripheral nervous system, usually causing painful small fibre or pure sensory axonal neuropathy, ganglioneuronopathy or a predominantly sensory CIDP. We report the case of a 71-year-old man diagnosed with a debilitating and difficult-to-treat CIDP who, 5 years later, developed SS with pulmonary involvement. Due to lack of response to treatments other than periodic intravenous immunoglobulin (IVIg) every 12 days, we started adjuvant treatment with rituximab which increased the time interval between IVIg therapies by 50%, providing better quality of life for the patient.

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Activation of Stimulator of Interferon Genes (STING) and Sjögren Syndrome

Journal of Dental Research ◽

10.1177/0022034518760855 ◽

2018 ◽

Vol 97 (8) ◽

pp. 893-900 ◽

Cited By ~ 11

Author(s):

J. Papinska ◽

H. Bagavant ◽

G.B. Gmyrek ◽

M. Sroka ◽

S. Tummala ◽

...

Keyword(s):

Gene Expression ◽

Viral Infections ◽

Critical Role ◽

Autoimmune Disorder ◽

Sjögren Syndrome ◽

Lymphoid Cells ◽

Type I Interferons ◽

Sjogren Syndrome ◽

Type I ◽

Type I Ifns

Sjögren syndrome (SS), a chronic autoimmune disorder causing dry mouth, adversely affects the overall oral health in patients. Activation of innate immune responses and excessive production of type I interferons (IFNs) play a critical role in the pathogenesis of this disorder. Recognition of nucleic acids by cytosolic nucleic acid sensors is a major trigger for the induction of type I IFNs. Upon activation, cytosolic DNA sensors can interact with the stimulator of interferon genes (STING) protein, and activation of STING causes increased expression of type I IFNs. The role of STING activation in SS is not known. In this study, to investigate whether the cytosolic DNA sensing pathway influences SS development, female C57BL/6 mice were injected with a STING agonist, dimethylxanthenone-4-acetic acid (DMXAA). Salivary glands (SGs) were studied for gene expression and inflammatory cell infiltration. SG function was evaluated by measuring pilocarpine-induced salivation. Sera were analyzed for cytokines and autoantibodies. Primary SG cells were used to study the expression and activation of STING. Our data show that systemic DMXAA treatment rapidly induced the expression of Ifnb1, Il6, and Tnfa in the SGs, and these cytokines were also elevated in circulation. In contrast, increased Ifng gene expression was dominantly detected in the SGs. The type I innate lymphoid cells present within the SGs were the major source of IFN-γ, and their numbers increased significantly within 3 d of treatment. STING expression in SGs was mainly observed in ductal and interstitial cells. In primary SG cells, DMXAA activated STING and induced IFN-β production. The DMXAA-treated mice developed autoantibodies, sialoadenitis, and glandular hypofunction. Our study demonstrates that activation of the STING pathway holds the potential to initiate SS. Thus, apart from viral infections, conditions that cause cellular perturbations and accumulation of host DNA within the cytosol should also be considered as possible triggers for SS.

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Pulmonary Involvement in Sjögren Syndrome

Seminars in Respiratory and Critical Care Medicine ◽

10.1055/s-0034-1371529 ◽

2014 ◽

Vol 35 (02) ◽

pp. 255-264 ◽

Cited By ~ 28

Author(s):

Kristin Highland ◽

Maryl Kreider

Keyword(s):

Pulmonary Involvement ◽

Sjögren Syndrome ◽

Sjogren Syndrome

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Pulmonary involvement in systemic lupus erythematosus, Sjögren syndrome and mixed connective tissue disease

Pulmonary Manifestations of Systemic Diseases ◽

10.1183/2312508x.10014319 ◽

2019 ◽

pp. 106-123

Author(s):

Katerina M. Antoniou ◽

Eirini Vasarmidi ◽

Athina Trachalaki ◽

Eleni Bibaki ◽

Bruno Crestani

Keyword(s):

Systemic Lupus Erythematosus ◽

Connective Tissue ◽

Connective Tissue Disease ◽

Lupus Erythematosus ◽

Mixed Connective Tissue Disease ◽

Pulmonary Involvement ◽

Sjögren Syndrome ◽

Sjogren Syndrome ◽

Systemic Lupus

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Sjögren’ Syndrome - Quality of Life as a Focus in a Complex Therapeutic Strategy

Aktuelle Rheumatologie ◽

10.1055/s-2005-857944 ◽

2005 ◽

Vol 30 (1) ◽

pp. 13-14

Author(s):

Ch Tomiak

Keyword(s):

Quality Of Life ◽

Therapeutic Strategy ◽

Sjögren Syndrome ◽

Sjogren Syndrome

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Pulmonary Involvement in Sjögren Syndrome

Clinics in Chest Medicine ◽

10.1016/j.ccm.2019.05.002 ◽

2019 ◽

Vol 40 (3) ◽

pp. 531-544 ◽

Cited By ~ 5

Author(s):

Jake G. Natalini ◽

Chadwick Johr ◽

Maryl Kreider

Keyword(s):

Pulmonary Involvement ◽

Sjögren Syndrome ◽

Sjogren Syndrome

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Quality of Sexual Life in Women with Primary Sjögren Syndrome

The Journal of Rheumatology ◽

10.3899/jrheum.141475 ◽

2015 ◽

Vol 42 (8) ◽

pp. 1427-1431 ◽

Cited By ~ 23

Author(s):

Roberta Priori ◽

Antonina Minniti ◽

Martina Derme ◽

Barbara Antonazzo ◽

Filippo Brancatisano ◽

...

Keyword(s):

Quality Of Life ◽

Disease Activity ◽

Mood Disorders ◽

Short Form ◽

Sjögren Syndrome ◽

Sexual Life ◽

Sjogren Syndrome ◽

Primary Sjögren Syndrome ◽

Vaginal Ph

Objective.To assess the quality of sexual life of women with primary Sjögren syndrome (pSS) and to identify its correlations with disease activity and damage, quality of life, and mood disorders.Methods.The quality of sexual life of 24 women with pSS was assessed with the Female Sexual Function Index (FSFI). Twenty-four healthy women, matched by age and hormonal status, were enrolled as controls. Mood disorders and quality of life were investigated using the Hospital Anxiety and Depression Scale (HADS) and the Medical Outcomes Study Short Form-36. Patients underwent a gynecological visit with vaginal pH measurement, cervicovaginal swabs, and Pap smears. Disease activity and damage were assessed by the European League Against Rheumatism Sjögren syndrome disease activity and damage indexes.Results.Patients with pSS showed a pathological mean FSFI score (19.1 ± 7.33) significantly different from controls (p = 0.004), both in menstruating women (p = 0.006) and in menopausal women (p = 0.03). Major differences between the 2 groups were detected in dyspareunia (p < 0.005), lubrication (p = 0.006), desire (p = 0.004), and arousal (p = 0.018). The FSFI score was inversely correlated with age (p = 0.008) and anxiety HADS (p = 0.031). No early anatomical changes, swabs, and Pap smear alterations were revealed in patients with pSS; however, vaginal pH was higher than normal in premenopausal patients (6.0 ± 0.77).Conclusion.Both premenopausal and postmenopausal women with pSS have a worse sexual quality of life. We reported a greater prevalence of dyspareunia that is statistically significant when compared with controls. The FSFI could be a useful tool to assess this topic, but has been neglected in the care of patients with pSS heretofore.

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Minor Salivary Gland Inflammatory Lesions in Sjögren Syndrome: Do They Evolve?

The Journal of Rheumatology ◽

10.3899/jrheum.130256 ◽

2013 ◽

Vol 40 (9) ◽

pp. 1566-1571 ◽

Cited By ~ 43

Author(s):

Efstathia K. Kapsogeorgou ◽

Maria I. Christodoulou ◽

Demosthenes B. Panagiotakos ◽

Spyros Paikos ◽

Anna Tassidou ◽

...

Keyword(s):

Salivary Gland ◽

Minor Salivary Gland ◽

Cell Types ◽

Sjögren Syndrome ◽

Time Interval ◽

Sjogren Syndrome ◽

T And B Cells ◽

Biopsy Specimens ◽

Lymphocytic Infiltrates ◽

Significant Disease

Objective.The lymphocytic infiltrates of minor salivary gland (MSG) lesions of Sjögren syndrome (SS) vary in grade and composition and are generally thought to develop in stepwise manner. Their progression over time is not well defined.Methods.We studied repetitive MSG biopsy specimens from 28 patients with primary SS.Results.The infiltration grade and prevalence of the major infiltrating cell types (T and B cells, macrophages, dendritic cells, natural killer cells) remained largely unchanged during a median 55 month biopsy time interval followup (quartiles 42–81).Conclusion.We found significant disease progression involving the development of mucosa-associated lymphoid tissue lymphoma in patients expressing adverse serologic prognostic factors, such as low serum C4 complement levels and cryoglobulinemia.

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