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ID PMID Title PublicationDate abstract
31262506 Adherence to Current Vaccination Recommendations for Patients With Rheumatoid Arthritis in 2021 Mar BACKGROUND: Patients with RA have a two to four-fold increased risk of developing infections compared to the general population. For this reason, the administration of influenza, pneumococcal and shingles vaccines is recommended for all patients with RA, preferably prior to initiating treatment, Previous studies have demonstrated the low prevalence of vaccination as well as adherence to current recommendations by rheumatologists in other regions. OBJECTIVE: To determine the knowledge and adherence to the current vaccination recommendations for patients with RA by rheumatology members of the Mexican College of Rheumatology (MCR), and to identify barriers to their application in this population. METHODS: A cross-sectional study was conducted through a survey sent to 577 rheumatologists from Mexico in January 2017. RESULTS: We received completed surveys from 122 individuals, representing 21.14% of the 577 rheumatologists in our registry. Fifty percent responded that they recommended immunization against influenza to 76%-100% of their patients, 36.07% recommended immunization against pneumococcus to 76%-100% of their patients, and 69.67% of the survey responders did not recommend shingles immunization routinely to their patients. CONCLUSIONS: The data collected in this study show there is poor adherence to immunization schedules recommended for the RA population. This data suggests there is misinformation about the effectiveness, safety and optimal timing of immunization in patients with RA in Mexico.
31643549 Chloroquine. 2012 Chloroquine is an aminoquinoline used for the prevention and therapy of malaria. It is also effective in extraintestinal amebiasis and as an antiinflammatory agent for therapy of rheumatoid arthritis and lupus erythematosus. Chloroquine is not associated with serum enzyme elevations and is an extremely rare cause of clinically apparent acute liver injury.
34400910 Sclerosing Sialadenitis Is Associated With Salivary Gland Hypofunction and a Unique Gene E 2021 PURPOSE: To develop a novel method to quantify the amount of fibrosis in the salivary gland and to investigate the relationship between fibrosis and specific symptoms associated with Sjögren's syndrome (SS) using this method. MATERIALS AND METHODS: Paraffin-embedded labial salivary gland (LSG) slides from 20 female SS patients and their clinical and LSG pathology data were obtained from the Sjögren's International Collaborative Clinical Alliance. Relative interstitial fibrosis area (RIFA) in Masson's trichrome-stained LSG sections was quantified from digitally scanned slides and used for correlation analysis. Gene expression levels were assessed by microarray analysis. Core promoter accessibility for RIFA-correlated genes was determined using DNase I hypersensitive sites sequencing analysis. RESULTS: RIFA was significantly correlated with unstimulated whole saliva flow rate in SS patients. Sixteen genes were significantly and positively correlated with RIFA. In a separate analysis, a group of differentially expressed genes was identified by comparing severe and moderate fibrosis groups. This combined set of genes was distinct from differentially expressed genes identified in lung epithelium from idiopathic pulmonary fibrosis patients compared with controls. Single-cell RNA sequencing analysis of salivary glands suggested most of the RIFA-correlated genes are expressed by fibroblasts in the gland and are in a permissive chromatin state. CONCLUSION: RIFA quantification is a novel method for assessing interstitial fibrosis and the impact of fibrosis on SS symptoms. Loss of gland function may be associated with salivary gland fibrosis, which is likely to be driven by a unique set of genes that are mainly expressed by fibroblasts.
34188206 Current and future therapies for primary Sjögren syndrome. 2021 Aug Primary Sjögren syndrome (pSS) is a systemic autoimmune disease that is characterized by a triad of symptoms that affect all patients (dryness, pain and fatigue). In addition, systemic involvement can affect between one-third and one-half of patients. The management of patients with pSS has been negatively affected by a lack of effective treatments; however, knowledge of the epidemiology of pSS has increased, and advances in developing classification criteria, systemic disease activity scoring and patient-reported outcomes have been made during the past decade. Progress has also been made in understanding the mechanisms that underlie the pathogenesis of pSS, which has enabled a more targeted therapeutic approach to be taken. At present, therapeutic decisions rely on the evaluation of symptoms and systemic manifestations and are mostly formed on the basis of experience rather than evidence, and on similarities with other autoimmune diseases, although the 2019 management recommendations from EULAR are now being used to inform clinical management of pSS. This Review summarizes the available evidence for systemic treatments for pSS and includes discussions of advances in outcome assessment, the current evidence for DMARD use and an overview of promising future therapeutics.
33914202 Clinical features and risk factors of Raynaud's phenomenon in primary Sjögren's syndrome. 2021 Oct OBJECTIVE: The aim at the current study was to investigate the clinical characteristics and risk factors of Raynaud's phenomenon (RP) in patients with primary Sjögren's syndrome (pSS). METHODS: Retrospective analysis of the medical records of 333 new-onset pSS patients was performed. Demographic, clinical, and serological data were compared between individuals with and without RP. Logistic regression analysis was used to identify risk factors. RESULTS: RP was present in 11.41% of the pSS patients. pSS-RP patients were younger (49.74±14.56 years vs. 54.46±13.20 years, p=0.04) and exhibited higher disease activity (11 [5.75-15] vs. 7 [4-12], p=0.03) than those without. The prevalence of lung involvement was significantly higher in pSS patients with RP (60.53% vs. 17.29%; p<0.001). A significantly higher proportion of patients with pSS-RP tested positive about antinuclear (ANA), anti-RNP, and anti-centromere antibodies (ACA) compared to those without (p=0.003, <0.001, and 0.01, respectively). Multivariate analysis identified lung involvement (odds ratio [OR]=8.81, 95% confidence interval [CI] 2.02-38.47; p=0.04), anti-RNP positive status (OR=79.41, 95% CI 12.57-501.78; p<0.0001), as well as ACA (OR=13.17, 95% CI 2.60-66.72; p=0.002) as prognostic factors for pSS-RP. CONCLUSION: The presence of RP defined a subset of pSS with a unique phenotype, manifesting as increased lung involvement and a higher frequency of anti-RNP antibodies and ACA, as well as greater disease activity. These results suggest that RP has clinical and prognostic value of pSS patients. Further prospective studies with a larger number of subjects are warranted to confirm our findings and assess the prognostic and treatment implications of RP in pSS patients. Key Points • Raynaud's phenomenon (RP) was present in 38 (11.41%) of 333 patients with primary Sjögren's syndrome (pSS), with patients with RP exhibiting a younger age and higher disease activity. • The presence of RP indicates a subset of pSS with a unique phenotype, with manifestations including increased lung involvement and a higher frequency of anti-RNP antibodies and anti-centromere antibodies. • Patients with pSS and RP need close follow-up and long-term observation (including assessment of microangiopathy), with specific attention paid to the possible development of clinical features of systemic sclerosis.
33530719 Evaluation of the effect of topical tacrolimus 0.03% versus cyclosporine 0.05% in the trea 2022 Jan PURPOSE: To compare the effect of topical application of tacrolimus 0.03% eyedrops versus cyclosporine 0.05% in Sjogren syndrome subjects with severe dry eyes. DESIGN: A prospective single-blinded simply randomized controlled study. METHODOLOGY: 60 Sjogren patients were randomized intoGroup A: 30 patients were instructed to put tacrolimus 0.03% eyedrops in one eye for 6 months and placebo eyedrops in the other eye, (N = 30, 44.9 ± 12.58 years).Group B: 30 patients were instructed to put cyclosporine 0.05% eyedrops in one eye for 6 months and placebo eyedrops in the other eye (N = 30, 49.4 ± 12.92 years).Main outcome measures: Patients were evaluated at day 0, 90, and 180 for Ocular Surface Disease Index Questionnaire (OSDI), frequency of use of artificial tears, average fluorescein tear break up time (TBUT), ocular surface staining scores, Schirmer I test, meibum quality, and expressibility scores. RESULTS: Upon comparing both eyedrops, the mean value of OSDI decrease was 38.25 ± 18.29% versus 31.69 ± 18.57% (p-value 0.09), SICCA score decrease was 2.97 ± 1.92 versus 2.27 ± 2.02 (p-value 0.124) the decrease in artificial tear substitute use was 3.90 ± 2.22 versus 3.63 ± 1.92 (p-value 0.616), increase in Schirmer I values were 4.10 ± 4.21 and 4.26 ± 2.00 (p-value 0.590) in eyes treated with tacrolimus and cyclosporine respectively. Neither of them affected meibum quality or expressibility scores. CONCLUSION: Both tacrolimus and cyclosporine significantly improved patient symptoms, frequency of artificial tears use and ocular surface staining compared to placebo-controlled eyes. However, no significant difference regarding the efficacy of both eyedrops at the end of 6 months treatment of severe dry eyes of Sjögren syndrome patients. TRIAL REGISTRATION: ClinicalTrials.gov. Identifier: NCT03865888.
31644047 Hydroxychloroquine. 2012 Hydroxychloroquine is a derivative of chloroquine that has both antimalarial and antiinflammatory activities and is now most often used as an antirheumatologic agent in systemic lupus erythematosis and rheumatoid arthritis. Hydroxychloroquine therapy has not been associated with liver function abnormalities and is an extremely rare cause of clinically apparent acute liver injury.
34706704 Detection of xerostomia, Sicca, and Sjogren's syndromes in a national sample of adults. 2021 Oct 27 OBJECTIVES: To assess the prevalence and determinants of xerostomia among adults and identify how many of the ones experiencing xerostomia have Sicca and Sjogren's syndromes. MATERIALS AND METHODS: This cross-sectional study included 1405 35-74-year-old Lithuanians (51.7% response rate) from the five largest Lithuanian cities and 10 peri-urban and rural areas that were randomly selected from each of the 10 Lithuanian counties. Xerostomia was determined by the self-reported experience of dry mouth as "often" or "always". A dentist diagnosed Sicca syndrome by unstimulated whole sialometry and the Schirmer's test, and all cases were referred to a rheumatologist to confirm Sjogren's syndrome. Self-reported questionnaires collected data about the determinants. RESULTS: The prevalence of xerostomia was 8.0% (n = 112), Sicca syndrome was diagnosed for 8 participants (0.60%), and Sjogren's syndrome for 2 participants (0.14%), with this being the first time it was diagnosed. Experiencing xerostomia was associated with older age (OR 1.7, 95% CI 1.1-2.6), urban residence (OR 3.3, 95% CI 1.6-5.0), presence of systemic diseases (OR 2.5, 95% CI 1.4-3.3), and the use of alcohol (OR 0.6, 95% CI 0.4-0.9). The higher proportion of participants with Sicca syndrome involved females, of older age, having systemic diseases, and using medications. CONCLUSIONS: The prevalence of xerostomia was 8.0% and the determinants of xerostomia were older age, urban residence, systemic diseases, and absence of using alcohol. In total, 0.6% of participants had Sicca syndrome, which was more prevalent among females, older subjects, those with systematic diseases, and those using medications. Sjogren's syndrome was diagnosed in 0.14% of participants. Clinical relevance Dental clinicians need to be trained to identify potential Sjogren's syndrome cases.
34409540 Meibomian gland atrophy with duration of Sjogren's syndrome in adult females. 2022 Jan BACKGROUND: To investigate the correlation between the duration of Sjögren syndrome (SS) and ocular surface parameters in patients with SS-related dry eye. METHODS: We analyzed 108 eyes of 108 female patients with primary SS-related dry eye. All patients underwent rheumatoid serologic tests and ocular surface assessments. The ocular surface assessment included the Standard Patient Evaluation of Eye Dryness (SPEED) score, meibomian gland (MG) atrophy, lipid layer thickness (LLT), partial and total blinking, partial blinking rate, Schirmer's I test, non-invasive tear break-up time, and ocular surface staining score. Correlations between the duration of SS and ocular surface assessments were calculated. RESULTS: The average age and SS duration of the participants were 56.7 ± 10.2 (range 21-78) years and 54.15 ± 41.10 (range 1-134) months, respectively. There was a strong positive correlation between SS duration and MG atrophy (r = 0.766, p < 0.001). The correlation between SS duration and MG atrophy rate remained significant after controlling for age (r = 0.559, p < 0.001). Average, maximum, and minimum LLTs showed weak negative correlations with SS duration (r = - 0.310, - 0.211, and-0.304, respectively, p = 0.014, 0.028, and 0.022, respectively) and MG atrophy (r = - 0.191, - 0.326, and - 0.299, respectively, p = 0.049, 0.002, and 0.009, respectively). SPEED score showed a weak positive correlation to SS duration (r = 0.303, p = 0.042) and a moderate positive correlation to MG atrophy (r = 0.450, p = 0.029). CONCLUSIONS: Longer duration of primary SS was related to more severe MG atrophy. Therefore, it is necessary to perform meibography in SS patients to verify MG atrophy status. A comparative study with non-SS dry eye patients is required to validate this study.
33495973 Pulmonary amyloidosis and cystic lung disease in primary Sjögren's syndrome: a case repor 2021 Aug This study presents a rare case of pulmonary amyloidosis and cystic lung disease associated with primary Sjögren's syndrome. In February 2019, a 76-year-old woman with primary Sjögren's syndrome presented to our rheumatology department for lung problems. Four years before presentation, the patient underwent chest computed tomography that revealed multiple nodules with numerous thin-walled lumen in both lungs. Pulmonary nodulectomy for hemoptysis was performed 2 years before presentation. Pathological findings suggested chronic inflammation of the bronchus and amyloid deposition in the lung tissue. The nodules and vesicles were generally larger from January 2015 to October 2018 when she did not receive any special medication, but the nodules and vesicles tended to be stable from February to August 2019 when she was treated with hydroxychloroquine sulfate regularly, suggesting that hydroxychloroquine sulfate may be helpful in this patient.
32657185 Evaluation of changes in oral health-related quality of life over time in patients with Sj 2021 May OBJECTIVES: To assess oral health-related quality of life (OHRQoL) and changes in OHRQoL in 3 years of patients with Sjögren's symdrome (SS). METHODS: Thirty-five SS patients and 23 non-SS individuals were enrolled. OHRQoL were quantitatively evaluated using the shortened Oral Health Impact Profile (OHIP-14). After 3 years, 22 patients and 14 controls tool the OHIP-14 survey again. RESULTS: The SS group had a significantly higher OHIP-14 score, which indicated a lower OHRQoL, than the non-SS group. Among individual questions in the OHIP-14, scores for 'trouble pronouncing words', 'uncomfortable to eat foods', 'self-conscious', and 'diet unsatisfactory' were markedly higher in the SS group than in the non-SS group. The OHIP-14 score significantly increased in 3 years in the SS group. Furthermore, there was an inverse correlation between the change rate of salivary flow rate and change of OHIP-14 scores in 3 years in patients with SS whose OHIP-14 score increased. Scores for 'irritable with other people', 'difficulty doing usual jobs', 'felt life less satisfying', and 'unable to function' significantly increased in 3 years. CONCLUSION: In SS, OHRQoL decreased in 3 years, which was associated with a decrease in saliva secretion. Moreover, troubles related to psychosocial aspects in SS patients were found to intensify over time.
34891605 A federated AI strategy for the classification of patients with Mucosa Associated Lymphoma 2021 Nov Mucosa Associated Lymphoma Tissue (MALT) type is an extremely rare type of lymphoma which occurs in less than 3% of patients with primary Sjögren's Syndrome (pSS). No reported studies so far have been able to investigate risk factors for MALT development across multiple cohort databases with sufficient statistical power. Here, we present a generalized, federated AI (artificial intelligence) strategy which enables the training of AI algorithms across multiple harmonized databases. A case study is conducted towards the development of MALT classification models across 17 databases on pSS. Advanced AI algorithms were developed, including federated Multinomial Naïve Bayes (FMNB), federated gradient boosting trees (FGBT), FGBT with dropouts (FDART), and the federated Multilayer Perceptron (FMLP). The FDART with dropout rate 0.3 achieved the best performance with sensitivity 0.812, and specificity 0.829, yielding 8 biomarkers as prominent for MALT development.
34323636 An update of targeted therapeutic options for primary Sjögren syndrome: current status an 2021 Dec Introduction: Primary Sjögren syndrome (pSS) is a systemic autoimmune disease that may affect 3 in 1,000 people within the general population. The therapeutic scenario is complex, and no therapy has proved to be able to modify the natural course of the disease, nor to prevent the most severe systemic complications.Areas covered: Recently, the EULAR 2020 Recommendations for pSS have underlined the low level of evidence supporting efficacious therapeutic approaches, lacking a definition of specific treatment targets and being far from the 'disease modification' concept that is frequently used in other diseases. Herein, the authors review the status of current targeted therapies and provide the reader with their expert opinion.Expert opinion: The progress in discovering novel treatments for pSS seem to be focused on searching new biological therapies as highly-selective drugs that can be effective without the adverse effects related to the wide, nonspecific immunosuppression induced by the drugs currently used. Most likely, the more disruptive therapeutic approach in pSS that could be seen in a few years is the use of combination strategies targeting different etiopathogenic pathways.
35000785 Recurrent or persistent salivary gland enlargement in children: When is it Sjögren's? 2022 Feb OBJECTIVES: To describe characteristic features in children with recurrent or persistent salivary gland enlargement and to propose a diagnostic algorithm with specific consideration for Sjögren's disease (SD). METHODS: In this single-center, prospective study, 45 patients < 18 years, with recurrent or persistent salivary gland enlargement of unknown etiology were enrolled from 2006 to 2019. We collected detailed clinical information to characterize this group of patients including specific details of their major salivary gland signs and symptoms. We compared clinical, laboratory and radiological parameters between 4 groups based on the results of labial salivary gland biopsy (LSGB) and between patients who met existing SD criteria or not. RESULTS: 44 patients, with a mean age of 6.8 years and female to male ratio 21:23 were observed over a mean of 3.8 years. Characteristics of salivary gland swelling episodes varied considerably between individuals, but the majority experienced ≤5 episodes per year, lasting ≤ 1 week, with swelling affecting either or both glands. Ocular and oral dryness symptoms were observed only in 25% and 59% patients, respectively. The majority were positive for ANA, but negative for SD-specific antibodies. A total of 75% patients fulfilled at least one of the existing SD criteria. CONCLUSION: SD is a major cause of recurrent salivary gland enlargement in children. For children meeting adult criteria, the diagnosis of SD is clear. However, for the many children without dryness symptoms, objective dryness, or SD-specific antibodies, further workup including a combination of salivary gland imaging and histopathological examination can help establish the diagnosis of SD.
34328795 Aquaporin5-Targeted Treatment for Dry Eye Through Bioactive Compounds and Gut Microbiota. 2021 Oct Dry eye and dry mouth are the principal sources of morbidity for patients with Sjögren's syndrome (SS). There are few effective treatments, particularly systemic ones. Targeting aquaprin-5 (AQP5)-mediated tear secretion has been tested as a novel ancillary strategy and has proved promising. Patients have a great interest in using complementary medicine, including nutraceuticals and bioactive compounds to alleviate their symptoms. Potential mechanisms by which phytocompounds and bioactive compounds may benefit SS ocular and mouth symptoms through modulation of AQP5 activity are presented within this review. Supplementation with prebiotics (such as polyphenols with high bioavailability) in SS patients with lower Firmicutes/Bacteroides (F/B) community ratio phenotype, through administration of butyrate-producing diets, is proposed as ancillary strategy for dry eye and mouth. The potential use of natural bioactive compounds to treat dry eye could also apply to dry mouth occurring in the context of aging and SS. This novel hypothesis could have implications with respect to planning a successful dietary regimen for achieving and maintaining a normal gut microbiota in SS patients. This regimen would include augmenting butyrate-producing foodstuffs and/or polyphenol-rich syrups, and high amounts of some specific probiotic-rich foodstuffs such as yogurt, soy yogurt, or as probiotic supplements. There are applications for pharmaceutical and nutraceutical products aiming to relieve dry eye and mouth.
34270142 Nerve ultrasound findings in Sjögren's syndrome-associated neuropathy. 2021 Nov BACKGROUND AND PURPOSE: The phenotype of Sjögren's syndrome-associated neuropathy has been better characterized in recent years. However, Sjögren's syndrome-associated neuropathy remains an underdiagnosed entity with only few insights considering the pathomechanisms of nerve damage. Nerve ultrasound has proven to be a useful and efficient tool in detecting nerve damage of autoimmune origin. We, therefore, aimed to evaluate this method for Sjögren's syndrome-associated neuropathy. METHODS: Patients with Sjögren's syndrome and clinical signs of neuropathy underwent sonographic examination of both median and ulnar nerves. Nerve thickening was classified for cross-sectional areas of >12 mm² at the median nerve and for >10 mm² at the ulnar nerve. Fascicle thickening was documented for cross-sectional areas ≥5 mm² at the median and ≥3 mm² at the ulnar nerve. RESULTS: Forty-three patients were included in the analysis (median age 60 years [interquartile range 53-73 years], female rate 60%). 31/43 patients (72%) showed abnormalities on nerve ultrasound, while nerve thickening was found more frequently than fascicle thickening (90% vs. 52% of patients with sonographic abnormalities, respectively). Abnormal findings were observed more frequently at the median nerve and in proximal localization. Abnormal findings on nerve conduction studies were evident in 36/43 patients (84%). Nerve conduction studies revealed a tendency of demyelinating nerve damage patterns being associated with abnormal findings on nerve ultrasound. CONCLUSIONS: In addition to nerve conduction studies, nerve ultrasound may have a supporting role in the diagnosis of Sjögren's syndrome-associated neuropathy. Also, our data support an immune-mediated inflammatory demyelinating pathogenesis of Sjögren's syndrome-associated neuropathy.
33619703 Therapeutic Recommendations for the Management of Older Adult Patients with Sjögren's Syn 2021 Apr Primary Sjögren's syndrome (SjS) is a systemic autoimmune disease most commonly diagnosed in middle-aged women. Although the disease can occur at all ages, it is diagnosed between 30 and 60 years of age in two-thirds of patients. In more than 20% of cases, the people are older than 65 years. In this review, we focus on the therapeutic management of primary SjS in older patients, following the recently published 2020 European League Against Rheumatism (EULAR) recommendations for the management of the disease with topical and systemic therapies. These recommendations are applicable to all patients with primary SjS regardless of age at diagnosis, although the therapeutic management in older patients requires additional considerations. Older patients are more likely to have pulmonary, liver, kidney, or heart-related comorbidities (even cognitive disturbances); caution is required when most drugs are used, including muscarinic agents, systemic corticosteroids and synthetic immunosuppressants. It is also important to monitor the use of eye drops containing steroids due to the increased risk of developing cataracts, a frequent ocular complication in the older population. In contrast, the majority of drugs that can be used topically (pilocarpine rinses, eye drops containing topical non-steroidal anti-inflammatory drugs (NSAIDs) or cyclosporine A, topical dermal formulations of NSAIDs) have shown an acceptable safety profile in older patients, as well as rituximab. A rigorous evaluation of the medical history of older patients is essential when drugs included in the EULAR guidelines are prescribed, with special attention to factors frequently related to ageing, such as polypharmacy, the existence of organ-specific comorbidities, or the enhanced susceptibility to infections.
33416998 Impact of comorbid Sjögren syndrome in anti-aquaporin-4 antibody-positive neuromyelitis o 2021 May BACKGROUND: Neuromyelitis optica spectrum disorders (NMOSD) are autoimmune neurological diseases of the central nervous system, which are characterized by the presence of serum anti-aquaporin-4 autoantibodies (AQP4-IgG). An association between Sjögren syndrome (SjS) and AQP4-IgG-positive NMOSD has been proposed, but the rate of coexistence has not been determined. METHODS: In this study, 4,447 patients suspected of having NMOSD with acute neurological episodes were evaluated for the positivity of serum AQP4-IgG, serum SS-A/Ro antibody, and the presence of SjS-related symptoms (dry eye, dry mouth). RESULTS: Of the 4,447 patients, 1,651 were positive for serum AQP4-IgG, and the remaining 2,796 were negative. A significantly higher proportion of AQP4-IgG-positive patients were positive for serum anti-SSA/Ro antibody (26.3 vs. 4.5%; p < 0.0001) and anti-SSB/La antibody (7.2 vs. 1.2%; p < 0.0001) and had dry eye (9.1 vs .4.9%; p < 0.0001) and dry mouth symptoms (8.9 vs. 3.7%; p < 0.0001). More than 80% of the patients with SjS with acute neurological events such as myelitis or optic neuritis were AQP4-IgG positive. AQ4-IgG-positive patients with comorbid SjS showed a higher female rate (97.1 vs. 89.0%; p = 0.0062), a higher positivity rate for oligoclonal bands (15.4 vs. 7.5%; p = 0.029), and a higher relapse frequency (p = 0.027) than AQP4-IgG-positive patients without comorbid SjS. CONCLUSIONS: The prevalence of SjS is higher among AQP4-IgG-positive than AQP4-IgG-negative patients, with the potential prevalence of 10-20% at the diagnosis of AQP4-IgG-positive NMOSD. Comorbid SjS is more prevalent in females, and it has a higher relapse frequency among AQP4-IgG-positive patients.
33128084 Neurofilament light is a biomarker of brain involvement in lupus and primary Sjögren's sy 2021 Apr BACKGROUND: To test the hypothesis that neurofilament light (NfL) in CSF is a biomarker of CNS involvement in patients with systemic lupus erythematosus (SLE) and primary Sjögren's syndrome (pSS), we measured NfL in CSF from 52 patients with lupus and 54 with pSS and explored associations with clinical, structural, immunological and biochemical abnormalities. METHODS: In CSF, we measured NfL, anti-P antibodies, protein S100B and TWEAK by ELISA and anti-NR2 antibodies by electrochemiluminescence. Anti-phospholipid antibodies and routine immunological tests were performed in blood. IgG and albumin were measured in CSF and serum for assessment of the blood-brain barrier function (Q-albumin) and intrathecal IgG production (IgG index). Cerebral MRI and neuropsychological testing were performed. RESULTS: A multivariable regression model showed that increasing CSF anti-NR2 antibody levels were associated with increasing NfL levels in patients with SLE (B 1.27, 95% CI 0.88-1.65, p < 0.001). Age contributed significantly in the model (B 0.04, 95% CI 0.03-0.05, p < 0.001). Similar findings were observed in the pSS group. Adjusted for age and sex, no associations were found between NfL levels and any MRI data. In SLE patients, higher NfL concentrations were associated with impairments in psychomotor speed and motor function, and in pSS with motor dysfunction. These associations remained in multivariable regression models. CONCLUSIONS: Increased concentration of NfL in CSF is a marker of cerebral involvement in patients with SLE and pSS, is strongly associated with the presence of anti-NR2 antibodies, and correlates with cognitive impairment in several domains.
34588240 Prevalence of dry eye in patients with systemic lupus erythematosus: a meta-analysis. 2021 Sep 29 OBJECTIVES: To investigate dry eye disease (DED) in patients affected by systemic lupus erythematosus (SLE). METHODS: We conducted a systematic search of the literature on PubMed, EMBASE and the Cochrane Library databases from conception to 30 April 2020 for studies related to dry eye, secondary Sjögren's syndrome (sSS) and SLE. Original full-text articles with the number of patients diagnosed with SLE of over 15 were included. The risk of bias was evaluated with a validated critical appraisal tool which assessed study quality based on confounding factors, selection bias, bias related to measurement and bias related to data analysis. Data were extracted and pooled to evaluate the overall prevalence of DED with the random-effect model and sSS with the fixed effect model. RESULTS: A total of 29 studies were included and 18 273 participants were involved. The pooled data showed that the overall prevalence of DED was 16% (95% CI 10% to 21%, p<0.001) in patients of SLE. Dry eye symptoms and abnormal Schirmer's test were found in 26% (95% CI 20% to 32%, p<0.001) and 24% (95% CI 14% to 34%, p<0.001) of patients with SLE, respectively. 12% (95% CI 9% to 15%, p<0.001) of patients also met the criteria of sSS. The OR of DED in patients with SLE was 4.26 (95% CI 3.47 to 5.05, p<0.001) compared with healthy controls. The meta-regression analysis showed that the sample size (p=0.004) and study location (p=0.022) could be the source of heterogeneity. CONCLUSIONS: DED and sSS are both common in patients with SLE.