How are we treating our systemic patients with primary Sjögren syndrome? Analysis of 1120 patients

Gheitasi, H.; Kostov, Belchin; Solans, Roser; Fraile, G.; Suárez-Cuervo, Carlos; Casanovas, A.; Rascόn, F.-J.; Qanneta, Rami; Pérez-Álvarez, R.; Ripoll, M.; Akasbi, M.; Pinilla, Blanca; Bosch, J.; Nava-Mateos, J J; Díaz-Lόpez, B.; Morera-Morales, Maria Lluisa; Retamozo, Soledad; Ramos‐Casals, Manuel; Brito-Zerón, Pilar

doi:10.1016/j.intimp.2015.03.027

Cited by 27 publications

(21 citation statements)

References 16 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Common immunotherapies include hydroxychloroquine low-dose glucocorticoids (<20 mg/day), and high-dose glucocorticoids (>20 mg/day). Second-tier options include rituximab (RTX) and intravenous immunoglobulins (IVIg) [19]. Their relative benefit for neuropathy is unknown, as most randomized clinical trials (RCT) for SS did not include neurological end points.…”

Section: Immune-mediated Painful Sensory Ganglionitis/neuronitismentioning

confidence: 99%

Immunotherapy Prospects for Painful Small-fiber Sensory Neuropathies and Ganglionopathies

Oaklander

2016

Neurotherapeutics

View full text Add to dashboard Cite

The best-known peripheral neuropathies are those affecting the large, myelinated motor and sensory fibers. These have well-established immunological causes and therapies. Far less is known about the somatic and autonomic "small fibers"; the unmyelinated C-fibers, thinly myelinated A-deltas, and postganglionic sympathetics. The small fibers sense pain and itch, innervate internal organs and tissues, and modulate the inflammatory and immune responses. Symptoms of small-fiber neuropathy include chronic pain and itch, sensory impairment, edema, and skin color, temperature, and sweating changes. Small-fiber polyneuropathy (SFPN) also causes cardiovascular, gastrointestinal, and urological symptoms, the neurologic origin of which often remains unrecognized. Routine electrodiagnostic study does not detect SFPN, so skin biopsies immunolabeled to reveal axons are recommended for diagnostic confirmation. Preliminary evidence suggests that dysimmunity causes some cases of small-fiber neuropathy. Several autoimmune diseases, including Sjögren and celiac, are associated with painful small-fiber ganglionopathy and distal axonopathy, and some patients with "idiopathic" SFPN have evidence of organ-specific dysimmunity, including serological markers. Dysimmune SFPN first came into focus in children and teenagers as they lack other risk factors, for example diabetes or toxic exposures. In them, the rudimentary evidence suggests humoral rather than cellular mechanisms and complement consumption. Preliminary evidence supports efficacy of corticosteroids and immunoglobulins in carefully selected children and adult patients. This paper reviews the evidence of immune causality and the limited data regarding immunotherapy for small-fiberpredominant ganglionitis, regional neuropathy (complex regional pain syndrome), and distal SFPN. These demonstrate the need to develop case definitions and outcome metrics to improve diagnosis, enable prospective trials, and dissect the mechanisms of small-fiber neuropathy.

show abstract

Section: Immune-mediated Painful Sensory Ganglionitis/neuronitismentioning

confidence: 99%

Immunotherapy Prospects for Painful Small-fiber Sensory Neuropathies and Ganglionopathies

Oaklander

2016

Neurotherapeutics

View full text Add to dashboard Cite

show abstract

“…Suppression of an excessive abnormal immune response is critical to attenuate the symptoms of patients with pSS, and glucocorticoids or immunosuppressive therapy is therefore frequently utilized . Furthermore, commonly used immunosuppressive agents include hydroxychloroquine (HCQ), rituximab, methotrexate and tumour necrosis factor inhibitors . When used for treatment, these drugs also have many adverse effects, such as macular degeneration, gastrointestinal adverse effects and an increased risk of tumour development.…”

mentioning

confidence: 99%

CP‐25 Alleviates Experimental Sjögren's Syndrome Features in NOD/Ltj Mice and Modulates T Lymphocyte Subsets

Zhang

et al. 2018

Basic Clin Pharma Tox

View full text Add to dashboard Cite

Primary Sjögren's syndrome (pSS) is a chronic inflammatory autoimmune illness of the moisture-producing glands such as salivary glands that is characterized by various immune abnormalities. The aetiology of pSS remains unclear and there is no curative agent. In this study, we investigated the putative therapeutic effects on a NOD/Ltj mouse model of Sjögren's syndrome-like disorders of an ester derivative of paeoniflorin, paeoniflorin-6'O-benzene (termed CP-25). Our study showed that CP-25 alleviated effectively clinical manifestations in NOD/Ltj mice resulting, for example, in increased salivary flow and reduced histopathological scores. Furthermore, CP-25 decreased lymphocyte viability in NOD/Ltj mice and attenuated the infiltration of Th1 cells and Th2 cells into the salivary glands of NOD/Ltj mice. In the spleen on NOD/Ltj mice, CP-25 skewed the ratio of Th17 and regulatory T cells towards regulatory T cells. After treatment, concentrations of anti-La/SSB and IgG antibodies were reduced and the titre of the inflammatory cytokines IFN-γ, IL-4, IL-6 and IL-17A in the serum on NOD/Ltj mice was alleviated. Thus, we define CP-25 as a novel compound that is a potent therapeutic agent for pSS by modulating T lymphocyte subsets. Future studies will validate the use of CP-25 as a therapeutic strategy for the treatment of pSS.

show abstract

“…Suppression of an excessive abnormal immune response is critical to attenuate the symptoms of patients with pSS, and glucocorticoids or immunosuppressive therapy is therefore frequently utilized [22]. The commonly used immunosuppressive drugs include hydroxychloroquine (HCQ), rituximab, methotrexate and TNF inhibitors, but these agents inevitably induce a series of side effects, such as osteoporosis, infection, gastric ulcer, myelosuppression, hepatorenal toxicity, and immunosuppression [23]. It is not only the expectation of doctors but also the demand of patients that a new, safe and effective drug that can be quickly promoted and effectively used in the clinical treatment of Sjögren's syndrome be found.…”

Section: Discussionmentioning

confidence: 99%

Melatonin alleviated the immune response and improved the salivary gland function in primary Sjögren’s syndrome

Liu¹,

Weng²,

Yu³

et al. 2020

Preprint

View full text Add to dashboard Cite

Background Excessive inflammatory reactions participate in primary Sjögren’s syndrome (pSS) progression. In addition, biological clock genes have been detected in the salivary glands, which indicates that clock genes regulate the growth and development of the salivary glands as well as the quality and quantity of saliva secretion. Melatonin is an amine hormone secreted by the pineal gland that has many physiological functions, such as regulating immunity and correcting disorder in the biological clock rhythm. The purpose of this study was to clarify the correlation between pSS and the biological clock rhythm and explore the possibility of applying melatonin to treat pSS. Methods Melatonin (10 mg/kg/d or 15 mg/kg/d) or vehicle was administered to NOD/Ltj mice by intraperitoneal injection for 4 weeks. Clock gene expression levels in labial gland biopsy specimens from pSS patients and submandibular gland specimens from mice were measured by Western blotting (WB) and RT-PCR. The salivary flow rate of mice was measured at 12, 14, and 16 weeks. The severity of lymphocyte infiltration in the salivary glands was analysed by haematoxylin and eosin (H&E) staining. Enzyme-linked immunosorbent assay (ELISA) and immunohistochemical staining were used to detect the expression levels of related inflammatory factors in mice. The percentages of Th17, Th2, and Treg cells were analysed by flow cytometry. Results There was a distinct expression profile for clock genes in pSS patients compared with controls. Continuous melatonin administration improved salivary gland function in NOD/Ltj mice, with decreased lymphocyte infiltration in the submandibular glands and reduced related inflammatory factor expression in the serum and salivary glands. Melatonin treatment skewed T cells towards the Treg and Th2 subsets while suppressing Th17 responses. Additionally, melatonin administration regulated clock gene expression in NOD/Ltj mice. Conclusion pSS pathogenesis and progression are correlated with abnormal circadian gene expression. Melatonin improves the hypofunction of the salivary glands and inhibits the inflammatory development of pSS in NOD/Ltj mice. This study provides a theoretical basis and potential approach for the clinical prevention and treatment of pSS.

show abstract

How are we treating our systemic patients with primary Sjögren syndrome? Analysis of 1120 patients

Cited by 27 publications

References 16 publications

Immunotherapy Prospects for Painful Small-fiber Sensory Neuropathies and Ganglionopathies

Immunotherapy Prospects for Painful Small-fiber Sensory Neuropathies and Ganglionopathies

CP‐25 Alleviates Experimental Sjögren's Syndrome Features in NOD/Ltj Mice and Modulates T Lymphocyte Subsets

Melatonin alleviated the immune response and improved the salivary gland function in primary Sjögren’s syndrome

Contact Info

Product

Resources

About