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ID PMID Title PublicationDate abstract
21229358 The prevalences of some rheumatic diseases in western Turkey: Havsa study. 2012 Apr To study the prevalence major rheumatic diseases in western Turkey. This survey was conducted in Havsa which have a total population of 18,771. Physicians and interns visited every household, interviewed face to face a questionnaire about the symptoms of rheumatic disorders. The individuals replied positively to any question were examined at the nearest health center. Those have no objective findings related to any rheumatic diseases were excluded. People could not be clinically diagnosed were asked to come to the hospital for further evaluation. A total 17,835 of 18,771 residents participated. We estimated the prevalence of Behçet's Disease (BD) as 0.019%; ankylosing spondylitis: 0.120%; rheumatoid arthritis: 0.321%; knee osteoarthritis (OA): 5.351%; hand OA: 1.110%; hand and knee OA: 1.958%; total OA: 8.420%; primary Raynaud's: 1.192%; psoriasis: 0.424 %; psoriatic arthritis: 0.050%; rheumatic fever: 0.318%; rheumatic heart disease: 0.200%; inflammatory bowel disease: 0.023%; lupus: 0.059%; gout: 0.018%; systemic sclerosis: 0.022%; juvenile rheumatoid arthritis: 0.032%; temporal arteritis: 0.020%, and familial Mediterranean fever (FMF) as 0.006%. Figures were adjusted for age-sex of the general Turkish population. The prevalence's of BD and FMF are considerably lower in Havsa as compared to other regions in Turkey.
21030175 Comparison of the manual and computer-aided techniques for evaluation of wrist synovitis u 2011 Feb OBJECTIVE: Traditional methods for assessment of synovial inflammation in rheumatoid arthritis such as clinical examination, immunohistology of bioptic samples, scintigraphy, and radiography have several limitations, including lack of sensitivity, need of invasive techniques, and administration of radioactive material. MRI lacks on standardisation and the data are often analysed using laborious, relatively rigid scoring methods. MATERIALS AND METHODS: This study introduces a standardized computer-aided method for quantitative analysis of MRI of the wrist on a dedicated scanner. Assessment of the synovial inflammation was performed using a semi-automated model-based method in conjunction with patient motion reduction algorithms. Further, the new method was compared with the traditional user-dependent ROI-based technique. RESULTS: The computer-aided technique generated robust and reproducible results. Application of motion reduction algorithms allowed for significant improvements of the signal to noise ratio, which is especially important in the datasets acquired with low-field scanners. CONCLUSION: The use of the computer software can be beneficial for diagnostic decision in cross sectional as well as longitudinal MRI examinations of the wrist in rheumatoid arthritis.
21245532 Structural and biochemical analyses of the human PAD4 variant encoded by a functional hapl 2011 Feb PAD4 is a peptidylarginine deiminase that catalyzes citrullination, a type of post-translational modification. In this reaction, arginine residues in proteins are converted to citrulline. PAD4 promotes the deimination of arginine residues in histones and may regulate transcription in the context of the chromatin. Single-nucleotide polymorphisms (SNP) in the gene encoding PAD4 identified it as one of the genes associated with susceptibility to rheumatoid arthritis. The PAD4 SNP involve three amino-acid substitutions: Ser55 to Gly, Ala82 to Val and Ala112 to Gly. Autoantibodies for improperly citrullinated proteins have been found in rheumatoid arthritis patients, suggesting that the PAD4(SNP) mRNA is more stable than the conventional PAD4 mRNA and/or the PAD4(SNP) protein possesses a higher citrullination activity than the PAD4 protein. In order to study the effects of the three amino-acid substitutions found in PAD4(SNP), the crystal structure of PAD4(SNP) was determined and it was found that the amino-acid substitutions in PAD4(SNP) only induced conformational changes within the N-terminal domain, not in the active centre for citrullination located in the C-terminal domain. Biochemical analyses also suggested that the citrullination activity of PAD4(SNP) may not substantially differ from that of conventional PAD4. These structural and biochemical findings suggested that the improper protein citrullination found in rheumatoid arthritis patients is not caused by defects in the citrullination activity of PAD4(SNP) but by other reasons such as enhanced PAD4(SNP) mRNA stability.
21162100 Berberine induces dendritic cell apoptosis and has therapeutic potential for rheumatoid ar 2011 Apr OBJECTIVE: To investigate the effects of berberine on dendritic cell (DC) apoptosis and its potential as a therapeutic agent in rheumatoid arthritis (RA). METHODS: Bone marrow (BM)-derived myeloid DCs (MDCs) and plasmacytoid DCs (PDCs) were generated by culturing BM cells with granulocyte-macrophage colony-stimulating factor/interleukin-4 or flt3L, respectively. Splenic DCs, T cells, and B cells were purified using a magnetic-activated cell sorting system. In vitro apoptosis was assessed by annexin V/propidium iodide or Hoechst 33258 staining. The in vivo effects of berberine were examined in mice with collagen-induced arthritis (CIA). Immune responses against type II collagen (CII) were determined by assaying serum antibody levels, lymphocyte proliferation, and cytokine production. The proportions of DCs and apoptosis of different immune cell subsets in spleens and lymph nodes were analyzed by flow cytometry and immunohistochemistry after subset-specific surface marker labeling and TUNEL staining. RESULTS: Exposure of MDCs to berberine during BM cell differentiation reduced cell recovery by inducing apoptosis. Sensitivity to berberine-induced apoptosis was acquired starting on day 3 of DC differentiation, and mature DCs were more sensitive to berberine than immature DCs. Murine peritoneal macrophages, RAW 264.7 cells, and Jurkat cells were insensitive to berberine-induced apoptosis. Splenic DCs were more sensitive to berberine than T and B cells. Susceptibility of PDCs to berberine-induced apoptosis was similar to that of MDCs. In mice with CIA, berberine treatment ameliorated arthritis, suppressed CII-specific immune responses, and selectively increased the incidence of apoptosis in DCs within spleens and lymph nodes. CONCLUSION: These findings show that berberine selectively induces apoptosis in DCs. Berberine may thus represent a novel therapeutic agent for RA.
22121127 Physical activity and physical activity cognitions are potential factors maintaining fatig 2012 May BACKGROUND: Fatigue is a prevalent and debilitating problem in Sjögren's syndrome. It has been suggested that physical activity and cognitions about physical activity can influence fatigue. OBJECTIVE: The aim of this study was to examine fatigue and physical activity levels in patients with Sjögren's syndrome and the associations of physical activity and physical activity cognitions with fatigue. METHODS: In 300 patients with primary Sjögren's syndrome and 100 demographically matched people from the general population (mean age 57 years, 93% female), fatigue (five dimensions of the multidimensional fatigue inventory) and physical activity (three dimensions of the international physical activity questionnaire) were assessed. The physical activity cognitions 'activity avoidance' and 'somatic focus' of the Tampa scale of kinesiophobia were assessed in the Sjögren's group only. RESULTS: Sjögren's patients had higher scores on all five fatigue dimensions (p<0.001) and lower scores on moderate and vigorous intensity activity (p≤0.001) compared with control participants. In the Sjögren's group, lower physical activity and higher activity avoidance and somatic focus were associated with more severe fatigue on most fatigue dimensions. For general fatigue and physical fatigue, especially the combination of low physical activity and high activity avoidance was associated with more severe fatigue (p<0.05). CONCLUSIONS: The results suggest that fatigue in patients with Sjögren's syndrome might be reduced by targeting both physical activity and physical activity cognitions. This suggestion requires verification in clinical experimental studies.
22041431 [Simultaneous relapse of Basedow's disease in a patient with adult-onset Still's disease]. 2011 This report describes a 50-year-old woman with coexisting Basedow's disease and adult-onset Still's disease (AOSD) that relapsed simultaneously. She was diagnosed with Basedow's disease in 1999, and treatment with antithyroid agents was started. However, the treatment was soon stopped because of severe side effects. A partial thyroidectomy was performed and the thyroid function stayed well-controlled after the surgery. In August 2007, she was admitted to our hospital with fever, a sore throat, skin rashes, arthritis and leukocytosis, and was diagnosed with AOSD. At the same time, her laboratory data revealed decreased serum TSH and elevated serum free T4, suggesting a relapse of Basedow's disease. After initiation of steroid pulse therapy accompanied by oral prednisolone, both diseases improved significantly. Prednisolone was gradually reduced, and the disease activity remained in remission. Immediately after prednisolone reached 3 mg/day in November 2009, both diseases relapsed. Prednisolone was increased to 30 mg/day, and the diseases became well-controlled again. In this case, Basedow's disease was aggravated when AOSD was in the active stage. Literature searches revealed five previously reported cases with coexisting Basedow's disease and AOSD. In four of the six cases, including our case, both diseases were activated simultaneously. AOSD in the active stage is known to cause hypercytokinemia and immunological derangement. Our case indicated that the pathogenesis of AOSD might lead to relapse of coexisting Basedow's disease.
21773715 Retrospective study of 61 patients with adult-onset Still's disease admitted with fever of 2012 Jan Adult-onset Still's disease (AOSD), as a category of connective tissue diseases, has about 5∼9% of fever of unknown origin (FUO) cases. Diagnosis of AOSD was challenging because of its nonspecific characteristics. The present study analyzed clinical manifestations and laboratory findings in a series of patients with AOSD from eastern China. Medical records of 61 patients admitted with FUO and with a discharge diagnosis of AOSD were retrospectively evaluated and analyzed with special focus on clinical manifestations and laboratory findings. Compared with previous reports, most features of our patients had a similar incidence rate. Rash (79%), arthralgia (80%), and sore throat (84%) were the most frequent clinical manifestations in our series. Leukocytosis (80%), elevated ESR (98%) and CRP (100%), negative ANA (90%) and RF (93%), and high ferritin level (94%) were the most sensitive laboratory findings in our patients. AOSD was not a rare reason of FUO in eastern China. Fever, arthralgia, rash, sore throat, leukocytosis, neutrophilia, elevated ESR and CRP, negative ANA and RF, and high ferritin level were the most common clinical features in our series. The lack of highly specific characteristic makes the diagnosis of AOSD difficult compared with other diseases in FUO.
19809819 A case of adult-onset Still's disease complicated with atypical pulmonary defect. 2011 Feb Adult-onset Still's disease (AOSD) is an uncommon inflammatory condition of unknown origin and pathogenesis. Pulmonary involvement is rare and includes pleural effusion and transient pulmonary infiltrates. We describe and discuss difficulties in the diagnosis of severe respiratory symptoms with uncommon radiological and histological findings and serious prognostic implication in a 24-year-old woman with a severe form of AOSD with destructive arthritis and hematological complications.
22018200 Glucocorticoid treatment in spondyloarthritis. 2011 Sep Spondyloarthritides (SpA) are chronic inflammatory rheumatic diseases that usually affect the axial skeleton and may involve entheses and peripheral joints. The main subtypes are ankylosing spondylitis (AS) and psoriatic arthritis (PsA). Other subtypes are reactive arthritis, arthritis associated with chronic inflammatory bowel diseases and undifferentiated axial and peripheral spondyloarthritis. Although SpA were regarded as variants of rheumatoid arthritis (RA) until the 1970s, it is now well established that the pathogenesis of SpA is quite different from that of RA. There is a lack of good clinical studies on glucocorticoid therapy in the SpA. While there is no reasonable doubt that intraarticular local therapies in SpA are as effective as in RA and other forms of arthritis, the evidence for a systemic use is at best marginal. While very high doses may be effective in some patients with AS, the possible value of low-dose corticosteroid therapy in patients with PsA has never been well addressed, with respect to either clinical efficacy or inhibition of radiographic progression. Future studies are needed to clarify this important issue for usual patient care.
20495923 Aseptic meningitis in adult onset Still's disease. 2012 Dec Adult onset Still's disease (AOSD) is a systemic inflammatory disease characterized by high-fevers, articular involvement, maculopapular rash, hepatosplenomegaly, lymphadenopathy, and a neutrophilic leukocytosis. Though systemic complications of AOSD or its treatment are well described in the literature, CNS involvement in AOSD is exceedingly rare and can have protean manifestations. We present a patient with AOSD who developed chronic meningitis and sensorineural hearing loss on treatment, with a review of prior reported cases of aseptic meningitis, to highlight this rare complication of this uncommon illness.
21744183 Active immunological profile is associated with systemic Sjögren's syndrome. 2011 Oct BACKGROUND: The aim of this paper was to study the evolution of primary Sjögren's syndrome (pSS) immunological profile, its impact on pSS activity and long-term evolution in a bicentric cohort of French patients with pSS (n = 445, mean age 53.6 ± 14 years, mean follow-up 76.1 ± 51 months). METHODS: This is a retrospective cohort study. RESULTS: Two hundred twelve patients were Sjögren's syndrome A (SSA) positive, and 131 were both SSA and Sjögren's syndrome B (SSB) positive. Sixty-eight patients (15%) had cryoglobulinemia. Active systemic profile (i.e., hypergammaglobulinemia, rheumatoid factor (RF), and anti-Sjögren's syndrome A (anti-SSA), anti-Sjögren's syndrome B (anti-SSB) positivity), associated with multisystemic involvement, leads to an increased utilization of corticosteroid and hydroxychloroquine. Multivariate analysis pointed out independent statistical association between hypergammaglobulinemia, anti-SSA, anti-SSB, and RF. Cryoglobulinemia is associated with multi-systemic involvement, lymphoma, and pSS-related death. CONCLUSION: The subset of patients with active immunological profile is characterized by systemic complications leading to immunosuppressive drug utilization and polyclonal B-cell activation profile.
21689965 Treatment of dry eye: an analysis of the British Sjögren's syndrome association comparing 2011 Dec PURPOSE: This study aims to address the lack of independent subjective efficacy data on artificial tear substitutes in the treatment of dry eye due to the anecdotal association of 'thicker' products being more effective. METHODS: This is an independent study of the subjective use and efficacy of topical treatments used by members of the British Sjögren's Syndrome Association (BSSA) related to product viscosity. 2000 members of the BSSA were sent a questionnaire regarding their physical condition and the use of artificial tear substitutes. Viscosity analysis was performed on the most popular preparations. Statistical comparison is made between subjective efficacies related to substitute tear viscosity. RESULTS: 1088 patients responded giving information regarding their condition together with the subjective use and efficacy data of artificial tear substitutes. Visco-analysis was performed on the most popular preparations; these had more than 50 patients using them. In terms of subjective benefits related to viscosity for 'frequency' and 'duration' the data suggests a general trend toward viscous preparations being instilled less frequently and lasting longer; however this was not shown to be significantly correlated and some interesting comparisons are reported. CONCLUSIONS: The results confirm high levels of ocular lubricant use in the BSSA population. Our data investigates the often-anecdotal evidence that thicker preparations are more effective. However, we did not find this correlation to be statistically significant suggesting further study into factors related to subjective product efficacy. These results lay foundations for the development of future products in the treatment of severe dry eye.
23233115 Inclusion body myositis associated with Sjögren's syndrome. 2013 Dec Inclusion body myositis (IBM) belongs to the group of idiopathic inflammatory myopathies. It is a poorly understood disease, which affects skeletal muscles. IBM usually occurs as an isolated condition, but in some cases, it may be associated with another autoimmune disorder, Sjögren's syndrome. We report a case of a 47-year-old woman with headaches, symptoms of trigeminal neuralgia, progressive weakness in muscles of the upper and lower extremities and symptoms of dry eyes and mouth. On admission, creatine kinase level was increased to 6,956 IU/mL and lactate dehydrogenase (LDH) to 1,011 U/L in the serum. The increase in inflammatory factor (CRP, ESR) levels was not found. The diagnosis of inclusion body myositis associated with Sjögren's syndrome was established on the basis of clinical picture and diagnostic tests. In this therapy, methotrexate and methylprednisolone were administered. The considerable improved muscle strength in the upper and lower extremities, improved speech and swallowing, disappearance of headache and reduction in CPK and LDH levels were found 8 months after establishing the diagnosis. Treatment with methotrexate and methylprednisolone improved the clinical symptoms and quality of life of this patient and may offer a therapeutic option for some patients with IBM and concomitant Sjögren's syndrome.
22513885 The clinical value and histopathological correlation of lacrimal scintigraphy in patients 2012 Jul PURPOSE: To evaluate the lacrimal gland function in patients with primary Sjögren's syndrome (SS) using lacrimal scintigraphy (LS) and to determine the relationship between clinicopathological stage of primary SS and LS findings. METHODS: Seventy eyes from 35 patients with primary SS and 20 eyes from 10 healthy age-matched and sex-matched individuals who served as the control group were included in this study. The patients with primary SS were divided into two groups - early stage and advanced stage - according to the labial biopsy scores (23 patients in early stage and 12 in advanced stage). LS, the Schirmer-1 test, determination of break-up time (BUT), Rose bengal ocular surface vital staining and assessment of functional parameters including ocular surface disease index (OSDI) scores were performed for all cases. LS was used to evaluate tear clearance. Lacrimal scintigrams were evaluated for the presence and severity of delayed tear clearance. Delay in clearance was also subclassified according to the severity of delay (mild, moderate, severe and very severe). LS findings and ophthalmological test results of patients in early stage of disease were compared with the results of those in advanced stage and with those of normal individuals. RESULT: The Schirmer-1 test and BUT values of patients in advanced stage of primary SS decreased significantly, whereas the Rose bengal and OSDI values increased significantly when compared with the values of patients in early stage of primary SS and with those of normal individuals (P<0.05 and P<0.001, respectively). Tear clearance of patients in advanced-stage disease was delayed significantly, compared with the tear delay in patients in early stage of disease and that of normal individuals (P<0.001). An inverse correlation existed between tear clearance and both the Schirmer-1 test and BUT value in all patients (P<0.001). However, there was a positive correlation between both the Rose bengal ocular surface staining values and OSDI scores and tear clearance in all cases (P<0.001). LS findings and ophthalmologic test results were significantly different between early-stage and advanced-stage primary SS. CONCLUSION: The results indicate that LS, which is correlated closely with ophthalmological test results, may be a useful method for the assessment of the lacrimal gland function in patients with primary SS. LS findings and ophthalmological tests may be used for determining disease severity in primary SS.
22408868 [Epidemiology and physiopathogy of Sjögren's syndrome]. 2012 Feb Sjögren's syndrome is defined as an autoimmune disease characterized by a progressive sicca syndrome associated with anti-SSA or SSA positivity and/or significant infiltrate at minor salivary gland biopsy. This autoimmune disease is relatively frequent, with a clinical diagnosis among women after the 5 decade. Its physiopathology, complex, associates the intervention of both environmental and genetic factors. Salivary epithelial cell is at the center of the pathological immune response of primary Sjögren's syndrome, characterized by a chronic B cell polyclonal activation potentially leading to the development of B cell lymphoma.
22235044 Still's disease, lupus-like syndrome, and silicone breast implants. A case of 'ASIA' (Shoe 2012 Feb In recent years, four conditions, siliconosis, Gulf War syndrome (GWS), macrophagic myofasciitis syndrome (MMF) and post-vaccination phenomena, were linked to a previous exposure to an adjuvant, suggesting a common denominator, and it has been proposed to incorporate comparable conditions under a common syndrome entitled Autoimmune/inflammatory Syndrome Induced by Adjuvants (ASIA). We report a case of a female who at the age of 11 years was diagnosed with Still's disease. At the age of 22 she underwent silicone breast implants and presented with a transient lupus-like syndrome. Then, at 25 years old she had a severe activation of Still's disease in association with rupture of silicone breast implants. When the prostheses were removed, the clinical picture improved. This case fulfills the criteria for ASIA and complements seven previous reports of Still's disease in association with silicone breast implants.
22020671 Leukocytapheresis (LCAP) for treating refractory adult-onset Still's disease (AOSD). 2012 Jun This is the first report on the efficacy of leukocytapheresis (LCAP) in a patient with refractory systemic-type adult-onset Still's disease (AOSD). A 17-year-old Japanese woman with AOSD who had been treated with prednisolone and cyclosporine A presented with relapse of typical systemic AOSD, including high fever, rash, and liver dysfunction. Steroid pulse therapy (methylprednisolone 500 mg/day) was performed, which failed to stabilize the disease. Therefore, LCAP (twice/week for a total of five courses) was introduced in combination with high-dose steroids plus cyclosporin A. Elevated levels of serum ferritin and transaminases and neutrophil CD64 expression normalized after the patient's disease was successfully controlled by the induction of LCAP. In this case, elevated levels of interleukin (IL)-1β and IL-18 were normalized after LCAP induction, suggesting that LCAP treatment modulates the deregulated cytokine-mediated inflammatory responses observed in AOSD. Our clinical observations suggest that LCAP may be beneficial for flare-up of systemic manifestations of AOSD refractory to conventional treatment, including high-dose steroids and immunosuppressants.
21996338 A five-year prospective study of fatigue in primary Sjögren's syndrome. 2011 INTRODUCTION: Fatigue is prevalent in primary Sjögren's syndrome (pSS), and contributes to the considerably reduced health related quality of life in this disease. The symptom is included in proposed disease activity and outcome measures for pSS. Several studies indicate that there is an inflammatory component of fatigue in pSS and other chronic inflammatory rheumatic diseases. The purpose of this study was to investigate fatigue change in pSS in a longitudinal study, and explore whether any clinical or laboratory variables at baseline, including serum cytokines, were associated with a change in fatigue scores over time. METHODS: A clinical and laboratory investigation of 141 patients fulfilling the American-European consensus criteria of pSS was undertaken in the period May 2004 to April 2005. Median time since diagnosis was 5.5 years. Examinations included the fatigue questionnaires: fatigue severity scale (FSS), fatigue visual analogue scale (VAS), functional assessment of chronic illness therapy-fatigue (FACIT-F) and medical outcome study short form-36 (SF-36) vitality, which were repeated in a follow-up investigation in January and February 2010. RESULTS: A total of 122 patients (87%) responded at both time-points. Thirty-five percent of patients experienced a clinically significant FSS increase. On the group level, fatigue measures did not change except that there was a slight deterioration in SF-36 vitality score. High serum anti-Sjögren's syndrome A antigen (anti-SSA) showed weak associations with high baseline fatigue, and patients with increasing fatigue had lower baseline unstimulated whole salivary volume. Weak associations between increasing fatigue and serum immunoglobulin G (IgG), and the pro-inflammatory cytokine interleukin-17 (IL-17), were observed. Baseline sicca symptoms correlated with higher fatigue both at baseline and with increasing fatigue over time. Linear regression analysis did not identify any predictive ability of clinical or laboratory measures on fatigue change over time. CONCLUSIONS: Fatigue remained mainly unchanged over time. Using multivariate models did not reveal any clinical or laboratory predictors of fatigue change over time.
23253002 Successful treatment of macrophage activation syndrome complicating adult Still disease wi 2012 Dec A previously healthy 20-year-old man presented with adult Still disease (ASD). He developed life-threatening macrophage activation syndrome (MAS), which was refractory to standard immunosuppression but responded dramatically to the IL-1 receptor antagonist anakinra. Subsequent immunological investigations included assessment of the perforin expression of natural killer (NK) cells and CD8+ T cells, which confirmed MAS.
21457124 Biological therapies in primary Sjögren's syndrome. 2011 Jul INTRODUCTION: Primary Sjögren's syndrome (PSS) is a relatively common immune-mediated condition characterized by oral and ocular dryness, fatigue, musculoskeletal pain and poor health-related quality of life. Other extra-glandular organs can also be affected and PSS is associated with a markedly increased risk of lymphoma. Furthermore, the health-economic cost for PSS is substantial. There is currently no effective treatment available. With better understanding of the pathophysiology of PSS and advances in technologies, it is now possible to develop biological therapies to target specific molecules or molecular pathways that are important in PSS pathogenesis. Indeed, a limited number of biological therapies have already been tested in PSS with mixed successes. AREAS COVERED: Published data on the use of biological therapies in PSS, the possible roles for other biological therapies and the potential challenges for their use. EXPERT OPINION: The use of biological agents targeting key cellular and molecular pathways in PSS pathogenesis represents a promising therapeutic strategy. Clinical trials assessing the efficacy of biological therapies in PSS should be encouraged but patient selection and outcome measures used in these studies must be carefully considered to ensure that the true effects of biological therapies on the outcomes of PSS are being appropriately evaluated.