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ID PMID Title PublicationDate abstract
23053721 Diagnosis and treatment of primary Sjögren syndrome-associated peripheral neuropathy: a s 2013 Sep OBJECTIVES: The clinical and therapeutic aspects of primary Sjögren syndrome (PSS) in patients with peripheral neuropathy were analyzed and the specifics of individual case studies are discussed. METHODS: We retrospectively studied six patients (four women, two men; mean age 64.5 years) presenting with PSS with peripheral neurological involvement over a five-year period (2008-2012). All patients had neurological examinations, including nerve conduction studies, somatosensory evoked potentials, and sural nerve biopsies. Treatment regimens included corticosteroids, intravenous gammaglobulin, or immunosuppressive treatment. RESULTS: Peripheral neuropathy was observed in six (7.9 %) of 76 patients with SS as the underlying disease; three were cases of multiple mononeuropathy, two cases had sensory ataxic neuropathy, one of which was autonomic neuropathy, and one case was diagnosed as painful sensory neuropathy without sensory ataxia. Four of the six patients were diagnosed with SS after the onset of neurological symptoms. Individual peripheral neuropathies had distinct neurological, electrophysiological, and pathological characteristics. The effect of steroids and intravenous gammaglobulin differed depending on the case. CONCLUSIONS: In PSS patients, a precise diagnosis is important, because the therapeutic strategy and response varies depending on the type of neuropathy. In clinical practice, it is important to consider a diagnosis of SS when patients present with peripheral neuropathy.
24786926 Interleukin 18: a biomarker for differential diagnosis between adult-onset Still's disease 2014 Jun OBJECTIVE: The differential diagnosis between rheumatic diseases and infectious conditions is a great challenge in clinical practice. Adult-onset Still's disease (AOSD) is a rare systemic inflammatory syndrome that shares several clinical and laboratory variables with sepsis. Interleukin (IL)-18 is overexpressed in AOSD, suggesting a possible role as a disease biomarker. The aim of our study was to detect IL-18 serum levels in a cohort of patients with AOSD and sepsis and to address its possible role as a biomarker for differential diagnosis. METHODS: A group of unselected patients with AOSD diagnosed according to the Yamaguchi criteria and consecutive patients with sepsis diagnosed according to the American College of Chest Physicians/Society of Critical Care Medicine Consensus Conference criteria were enrolled. The clinical and laboratory data were collected. In the AOSD group, disease activity was assessed by Pouchot's and Rau's criteria. IL-18 serum levels were detected by ELISA. RESULTS: Thirty-nine patients with AOSD and 18 patients with sepsis were enrolled. Two out of 18 patients with sepsis (11.1%) also fulfilled the Yamaguchi criteria. A significant difference was found in IL-18 serum levels between patients with active and inactive disease (p < 0.001), and it positively correlated with disease activity (p = 0.0003), ferritin serum level (p = 0.016), and erythrocyte sedimentation rate (p = 0.041). IL-18 was significantly increased in patients with AOSD when compared with sepsis (p = 0.014). For a cutoff of 148.9 pg/ml, this test had a specificity of 78.3% and a sensitivity of 88.6%. CONCLUSION: We have demonstrated that IL-18 can be a biomarker for differential diagnosis between AOSD and sepsis.
24637981 A short neuropsychological evaluation of patients with primary Sjögren's syndrome. 2014 Jan OBJECTIVE: To investigate cognitive deficits in patients with primary Sjögren's syndrome (PSS). METHOD: Eighteen patients with PSS, aged between 25 and 61 years, were subjected to a short neuropsychological battery and compared with 18 patients with multiple sclerosis and 18 healthy controls. RESULTS: The analysis of variance (ANOVA) revealed that the clinical groups had significantly worse performance than the control group on the Rey Auditory Verbal Learning Test 3; (F(2,53) =3.500, p=0.038) and 7 (F(2,53) =5.068, p=0.010). The clinical groups had elevated levels of depression on the Beck Depression Inventory (BDI); (p=0.003). The analysis of the data from the Trail Making Test B-A revealed a significant difference between the clinical and control groups (p=0.023). The analysis of covariance with BDI score as a covariate, did not change the outcome. CONCLUSION: Our study revealed cognitive deficits in patients with PSS detectable by a short neuropsychological battery.
24490899 The role of innate and lymphoid IL-22-producing cells in the immunopathology of primary Sj 2014 Apr In primary Sjögren's syndrome (pSS) a complex of interconnections between epithelial barrier, innate and adaptive immunity occurs. IL-22 is a pleiotropic cytokine that in pSS may be placed at the intersection of the adaptive and innate branches of immunity. Some evidence suggests that, in pSS, IL-22 may play a prominent pro-inflammatory role driving the early phase of tissue and systemic inflammation and participating in the self-perpetuation of disease. Despite contradictory data in literature about the role of NK cells in pSS, recent data also suggest an important contribution of this subset of cells of the innate immune system in the development and perpetuation of inflammation. Here, we discuss the role of IL-22 in the pathogenesis of pSS and in epithelial barrier function.
24159086 A study of the short-term effect of artificial tears on contrast sensitivity in patients w 2013 Dec 5 PURPOSE: Primary Sjögren's syndrome often includes dry eye manifestations, including a reduction in optical quality from the compromised ocular surface. This study was designed to measure the effect of different artificial tears on the contrast sensitivity of Sjögren's syndrome patients from 5 minutes to 4 hours after instillation. METHODS: Ten normal subjects and 10 subjects with ocular manifestations of Sjögren's syndrome were compared at baseline, including staining scores, a dry eye questionnaire, and contrast sensitivity testing. Changes in contrast sensitivity in the Sjögren's syndrome group were measured over a 4-hour period after instillation of a variety of artificial tears. RESULTS: Statistically significant differences in staining, subjective questionnaire scores, and contrast sensitivity were measured between the normal and Sjögren's syndrome groups. Statistically significant changes in contrast sensitivity were measured over time after artificial tear instillation, with the greatest effect at 3 to 6 cycles/deg. The more mucoadhesive artificial tears demonstrated a significantly greater effect. CONCLUSIONS: The effects of artificial tears on measured contrast sensitivity in dry eye patients in the postinstillation period of 5 minutes to 4 hours appear limited, but an artificial tear with more mucoadhesive properties showed more benefit than those that do not. Modest effects on contrast sensitivity, primarily at medium spatial frequencies, were observed with the more mucoadhesive formulations.
23893093 Diagnosis of pathological minor salivary glands in primary Sjogren's syndrome by using Ram 2014 Mar The lip biopsy is essential for the diagnosis of primary Sjogren's syndrome (SS) but an invasive method can cause some disadvantages. The purpose of this study is to apply Raman spectroscopy to detect the pathological minor salivary glands in primary SS and establish the diagnostic model of Raman spectra of the primary SS samples. Raman spectra from the primary samples and control samples were obtained by Raman microscope and were compared to find the differences. The principal component analysis (PCA) and discrimination function analysis (DFA) were employed to analyze the spectra and establish the diagnostic model. The differences of Raman spectra demonstrated the biochemical molecular alterations between the different samples. Compared with the control samples, the content of proteins, nucleic acids, and keratin increased in the primary SS samples but the content of lipids decreased. PCA and DFA displayed a powerful role in the classification of the Raman spectra. The sensitivity and specificity of the diagnostic model reached above 91 and 92%, respectively. The total accuracy is 92.4%. Raman spectroscopy combined with PCA-DFA algorithm will provide an effective and accurate technology for the diagnosis of the pathological minor salivary glands in primary SS, which may replace the lip biopsy in the future.
23884464 When killers become helpers. 2013 Jul 24 Interplay between a natural killer (NK)-cell receptor, NKp30, and other cells in the salivary glands profoundly affects pathogenesis of the autoimmune disease Sjögren's syndrome (Rusakiewicz et al., this issue).
25261962 High-resolution HLA analysis of primary and secondary Sjögren's syndrome: a common immuno 2015 Apr To compare the distribution of HLA-A, B, DRB1 and DQB1 alleles among Mexican patients with primary Sjögren Syndrome (pSS), secondary SS (sSS), connective tissue disease (CTD) without (w/o) SS and historical ethnically healthy controls. We included 28 pSS, 30 sSS, 96 CTD w/o SS patients and 234 controls. HLA-A, B, DRB1 and DQB1 were amplified and sequenced using the Allele SEQR Sequenced Based Typing Kits and analyzed on the ABI Prism*3130 DNA Analyzer using the Assign software. Gene frequencies were obtained by direct counting. Contingence tables of 2 × 2 were generated and analyzed by the Mantel-Haenzel χ (2) or Fisher's test (EPIINFO program). We reported odds ratios (OR) and corrected p values. SS patients showed increased frequencies of A*68:01 and DRB1*14:06 alleles when compared to CTD w/o SS (OR 4.43, 95 % CI 1.35-14.14, p = 0.007 and OR 14, 95 % CI 1.68-116, p = 0.001, respectively) and a higher prevalence of DRB1*01:01 (OR 5.9, 95 % CI 2.13-16.56, p = 0.003) and HLA-B*35:01 (OR 3.70, 95 % CI 1.92-7.12, p = 0.004) when compared with controls. pSS patients had a higher frequency of DRB1*14:06 allele than sSS (OR 16, 95 % CI 1.59-390, p = 0.001). Anti-Ro/SSA positivity was associated with B*51:01 (OR 10.11, 95 % CI 1.09-245, p = 0.02) and DRB1*03:01 alleles (OR 4.26, 95 % CI 1.01-18.89, p = 0.029), whereas the A*01:01 allele was associated with anti-La/SSB positivity (OR 4.75, 95 % CI 1.32-16.92, p = 0.003). In our population, the DRB1*14:06 allele was associated with primary and secondary SS implying that both varieties bear a similar immunogenetic background.
24162151 Systemic involvement in primary Sjogren's syndrome evaluated by the EULAR-SS disease activ 2014 Feb OBJECTIVE: To evaluate systemic involvement in primary SS in a large cohort of Spanish patients using the EULAR-SS disease activity index (ESSDAI) definitions. METHODS: Systemic involvement was characterized using ESSDAI definitions for the 10 clinical domains (constitutional, lymphadenopathy, glandular, articular, cutaneous, pulmonary, renal, peripheral nervous system, central nervous system and muscular). ESSDAI scores at diagnosis, during follow-up and cumulated at the last visit were calculated. RESULTS: The cohort consisted of 921 patients. After a mean follow-up of 75 months, 77 (8%) patients still had an ESSDAI score of zero at the last visit. Organ by organ, the percentage of patients who developed activity during the follow-up (ESSDAI score ≥ 1 at any time) ranged between 1.4% and 56%, with articular, pulmonary and peripheral neurological involvement being the most common. Logistic multivariate regression analysis showed the following features at diagnosis and had the closest association with systemic activity (statistically significant independent variables in at least two domains): cryoglobulinaemia in five domains; anaemia, lymphopenia and low C3 levels in three domains each and age <35 years in two domains. Sicca features, ANA and RF at diagnosis were not associated with a higher cumulated activity score in any clinical domain. CONCLUSION: Primary SS is undeniably a systemic disease, with the joints, lungs, skin and peripheral nerves being the most frequently involved organs. Cytopenias, hypocomplementaemia and cryoglobulinaemia at diagnosis strongly correlated with higher cumulated ESSDAI scores in the clinical domains. Clinically the ESSDAI provides a reliable picture of systemic involvement in primary SS.
23751410 Reactive macrophage activation syndrome possibly triggered by canakinumab in a patient wit 2013 Dec Macrophage activation syndrome (MAS) is a rare and serious complication of adult-onset Still's disease. We describe a case in a 49-year-old woman with Still's disease refractory to glucocorticoids, methotrexate, and infliximab. Anakinra provided satisfactory disease control for 1 year, after which escape phenomenon occurred. After four tocilizumab injections, cutaneous melanoma developed. The persistent systemic manifestations prompted treatment with two canakinumab injections. Ten days later, she had a spiking fever, dyspnea, low back pain, abdominal pain, odynophagia, and hepatomegaly. Laboratory tests showed liver cytolysis (180 IU/L; N: 10-35), acute renal failure (creatinine, 407 μmol/L; N:50-100), thrombocytopenia (60 G/L; N: 150-400), leukocytosis (12,200/mm(3); N: 4000-10,000), hypertriglyceridemia (5070 mmol/L; N: 0.4-1.6), lactate dehydrogenase elevation (4824 IU/L; N: 135-250), and hyperferritinemia (97 761 μg/L; N:15-150). Examination of a bone marrow biopsy showed phagocytosis. Tests were negative for viruses and other infectious agents. Glucocorticoid therapy (1.5 mg/Kg/d) and intravenous polyvalent immunoglobulins (0.5 g/Kg/d) were given. Her condition improved despite the many factors of adverse prognostic significance (thrombocytopenia, absence of lymphadenopathy, and glucocorticoid therapy at diagnosis). This is the first reported case of MAS after canakinumab therapy in a patient with adult-onset Still's disease.
23797265 Pulmonary manifestations of Sjögren's syndrome. 2013 Aug Sjögren's syndrome (SS) is primarily defined by its impact on the oral and ocular system resulting in xerostomia and xerophthalmia. However, SS can also manifest throughout the respiratory system. Subclinical pulmonary involvement is common. Clinically significant involvement can result in a 4-fold increased risk of death. Thus, recognizing the many potential presentations of SS in the lung is critical in caring for patients with SS. Additionally, SS should be included in the differential diagnosis of a number of forms of interstitial lung disease.
24498865 Spondylodiscitis and Achilles tendonitis due to gout. 2014 Nov The patient, a 62-year-old man with a 3-year history of hyperuricemia, presented with severe neck pain, Achilles enthesopathy and polyarthralgia. He consumed alcohol heavily. The biochemical profile was normal except for elevated levels of CRP (3.6 mg/dl; normal < 0.3), uric acid (UA) (10.9 mg/dl; normal 2.5-7.5) and creatinine (1.7 mg/dl; normal 0.5-1.0). Bone scintigraphy showed polyarthritis at the right elbow, wrist and bilateral first MTP joints. Notably, bone scintigraphy with computed tomography also revealed spondylodiscitis of C5-C6, which was confirmed by MRI, and left Achilles tendonitis. Moreover, left Achilles tendonitis was also confirmed by ultrasonography, indicating enthesitis with low-echoic lesion and calcification. Needle aspiration yielded a white viscous liquid, with numerous urate crystals identified on polarized light microscopy. He was diagnosed with gouty arthritis associated with spondylodiscitis and Achilles tendonitis. After the treatment with allopurinol, colchicine and predonisolone, his symptoms were improved, and serum CRP and UA levels were normalized. The cervical spine and Achilles tendon are rare and notable sites of involvements in gout, and differential diagnosis of gouty arthritis from spondyloarthritis, rheumatoid arthritis, tumor, pseudogout, and infection is necessary. When the patient was noted to have neck pain and Achilles enthesopathy, we should always recognize gouty arthritis.
23749581 Early remission is associated with improved survival in patients with inflammatory polyart 2014 Sep OBJECTIVES: This study aimed to evaluate whether the early achievement of clinical remission influences overall survival in an inception cohort of patients with inflammatory polyarthritis (IP). METHODS: Consecutive early IP patients, recruited to a primary care based inception cohort from 1990 to 1994 and from 2000 to 2004 were eligible for this study. Remission was defined as absence of clinically detectable joint inflammation on a 51-joint count. In sensitivity analyses, less stringent definitions of remission were used, based on 28-joint counts. Remission was assessed at 1, 2 and 3 years after baseline. All patients were flagged with the national death register. Censoring was set at 1 May 2011. The effect of remission on mortality was analysed using the Cox proportional hazard regression model, and presented as HRs and 95% CIs. RESULTS: A total of 1251 patients were included in the analyses. Having been in remission at least once within the first 3 years of follow-up was associated with a significantly lower risk of death: HR 0.72 (95% CI 0.55 to 0.94). Patients who were in remission 1 year after the baseline assessments and had persistent remission over time had the greatest reduction in mortality risk compared with patients who never achieved remission within the first 3 years of follow-up: HR 0.58 (95% CI 0.37 to 0.91). Remission according to less stringent definitions was associated with progressively lower protective effect. CONCLUSIONS: Early and sustained remission is associated with decreased all-cause mortality in patients with IP. This result supports clinical remission as the target in the management of IP.
23911410 Comparative antigen-induced gene expression profiles unveil novel aspects of susceptibilit 2013 Dec Lewis (LEW) and Wistar Kyoto (WKY) rats of the same major histocompatibility complex (MHC) haplotype (RT.1(l)) display differential susceptibility to adjuvant-induced arthritis (AIA). LEW are susceptible while WKY are resistant to AIA. To gain insights into the mechanistic basis of these disparate outcomes, we compared the gene expression profiles of the draining lymph node cells (LNC) of these two rat strains early (day 7) following a potentially arthritogenic challenge. LNC were tested both ex vivo and after restimulation with the disease-related antigen, mycobacterial heat-shock protein 65. Biotin-labeled fragment cRNA was generated from RNA of LNC and then hybridized with an oligonucleotide-based DNA microarray chip. The differentially expressed genes (DEG) were compared by limiting the false discovery rate to <5% and fold change ≥2.0, and their association with quantitative trait loci (QTL) was analyzed. This analysis revealed overall a more active immune response in WKY than LEW rats. Important differences were observed in the association of DEG with QTL in LEW vs. WKY rats. Both the number of upregulated DEG associated with rat arthritis-QTL and their level of expression were relatively higher in LEW when compared to WKY rat; however, the number of downregulated DEG-associated with rat arthritis-QTL as well as AIA-QTL were found to be higher in WKY than in LEW rats. In conclusion, distinct gene expression profiles define arthritis-susceptible versus resistant phenotype of MHC-compatible inbred rats. These results would advance our understanding of the pathogenesis of autoimmune arthritis and might also offer potential novel targets for therapeutic purposes.
25409665 Universal nature of spondyloarthropathy as a reactive disease, reflecting differential sen 2013 Tendency to afflict one part of the skeleton, rather than another, could be referred to as the osseotropism of the disease. That term would also include which part of the particular bone was affected. That, in addition to characteristics of erosions, facilitates distinguishing spondyloarthropathy from rheumatoid arthritis, calcium pyrophosphate deposition disease and gout. Spondyloarthropathy, however, is not limited to humans. Initially recognized in 20% of gorillas and rhesus macaques, it was subsequently identified in 25% of bears and 35% of rhinoceros. It is truly a pan-mammalian phenomenon, extending from marsupials and rodents to whales and as ancient as dinosaurs.
23510070 Perspectives on epigenetic-based immune intervention for rheumatic diseases. 2013 Mar 14 Rheumatic disease can loosely be described as any painful condition affecting the loco-motor system, including joints, muscles, connective tissues, and soft tissues around the joints and bones. There is a wide spectrum of rheumatic diseases, many of which involve autoimmunity, including systemic lupus erythematosus and rheumatoid arthritis. A significant body of evidence now links aberrant epigenetic regulation of gene expression with rheumatic disease and points toward the use of epigenetic targeting agents as potential new treatment options, particularly for those conditions associated with an autoimmune element. In this perspective, I will briefly cover the current knowledge surrounding this area in the field of rheumatology.
24274266 Chemical profiling of Wu-tou decoction by UPLC-Q-TOF-MS. 2014 Jan Wu-tou decoction (WTD), a traditional Chinese medicine (TCM) formula, is composed of Aconiti Radix Cocta, Ephedrae Herba, Paeoniae Radix Alba, Astragali Radix and Glycyrrhiza Radix Preparata, and it has been used for more than a thousand years to treat rheumatic arthritis, rheumatoid arthritis and pain of joints, while the active constitutions of WTD are unclear. In this research, an ultra performance liquid chromatography coupled with quadrupole time-of-flight mass spectrometry (UPLC-Q-TOF-MS) method in both positive and negative ion mode was established to investigate the major constitutions in WTD. A Waters ACQUITY UPLC BEH C18 column was used to separate the aqueous extract of WTD. Acetonitrile and 0.1% aqueous formic acid (v/v) were used as the mobile phase. 74 components including alkaloids, monoterpene glycosides, triterpene saponins, flavones and flavone glycosides were identified or tentatively characterized in WTD based on the accurate mass within 15 ppm error and tandem MS behavior. All the constitutions were also detected in the corresponding individual herbs. These results will provide a basis for further study in vivo of WTD and the information of potential new drug structure for treating rheumatic arthritis and rheumatoid arthritis.
23533423 Validation of a prediction rule for the diagnosis of rheumatoid arthritis in patients with 2013 Objectives. To validate van der Helm-van Mil score (vHvM) and new ACR/EULAR criteria for the diagnosis of rheumatoid arthritis (RA) in patients with undifferentiated arthritis (UA). Patients and Methods. Adult patients with UA (swelling ≥2 joints of less than 6 months duration, without diagnosis, and never treated with disease modifying drugs). Results. Ninety-one patients were included. Mean age: 55.6 years (SD: 17.4), 74% females. Median symptoms duration was 2 months (IR: 1-4 months). Mean van der Helm-van Mil score was 6.9 (SD: 2). After a mean followup of 6.2 months (SD: 6), 40.7% patients fulfilled ACR 1987 RA classification criteria, 28.6% fulfilled other diagnostic criteria, and 31% remained as UA. Receiver operator characteristic curve's (ROC's) area under the curve (AUC) for the vHvM score for diagnosis of RA was 0.83. A cutoff value of 6.94 showed sensitivity of 81% and 79.7% specificity. For the new ACR/EULAR criteria, the ROC AUC was 0.93, and a value equal to or greater than 6 showed 86.5% sensitivity and 87% specificity. Conclusion. van der Helm-van Mil prediction score and the new ACR/EULAR criteria proved to be valuable for the diagnosis of RA in patients with early UA.
23728960 Lower extremity arthroplasty in patients with inflammatory arthritis: preoperative and per 2013 Jun Spondylarthritis, which includes conditions such as ankylosing spondylitis and psoriatic arthritis, and rheumatoid arthritis are the most common forms of inflammatory arthritis. Joint inflammation and damage may result in the need for arthroplasty, and the surgeon must be aware of the perioperative challenges associated with these systemic diseases. In patients with inflammatory arthritis who have polyarticular disease and spinal involvement at the time of presentation for lower extremity arthroplasty, preoperative evaluation must include careful evaluation of all joints, including the cervical spine. Preoperative assessment and perioperative management must be appropriate to minimize cardiac and pulmonary complications. Finally, the perioperative management of medications used to manage inflammatory arthritis is critical because these medications may increase the risk of infection and compromise wound healing.
25434627 WITHDRAWN: Polymyositis of bilateral upper legs seen on FDG-PET/CT in a patient with rheum 2014 Nov 27 This article has been withdrawn at the request of the author and editor. The Publisher apologizes for any inconvenience this may cause. The full Elsevier Policy on Article Withdrawal can be found at http://www.elsevier.com/locate/withdrawalpolicy.