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ID PMID Title PublicationDate abstract
26308347 Characteristics of Patients With Early-Onset Arthritis in Latin America: Description of th 2015 Sep BACKGROUND: While many studies have tried to show that early intervention improves the clinical outcomes of early-onset arthritis, only a few were carried out in Latin America. OBJECTIVES: The aim of this study was to describe the Pan-American Registry of Early-Onset Arthritis (REPANARC) project and report the preliminary outcomes of a cohort of patients. METHODS: The REPANARC cohort consisted of a sample of patients from 6 Latin American countries. Patients with arthritis of 1 or more joints of 1-year duration or less were assessed by a rheumatologist during 6 consecutive clinical visits for a follow-up period of 2 years. The registry included clinical characteristics, medical history, physical examination, disease activity, analytical chemistries, imaging, current treatment, and a set of patient-reported outcome measures evaluating disability, psychological distress, and quality of life. RESULTS: A total of 173 patients were included with mean age of 41.9 ± 13.2 years; 83.8% were women. The predominant presentations at onset were insidious, polyarticular, additive, bilateral, and symmetrical. The initial diagnoses were rheumatoid arthritis (50.6%), undifferentiated arthritis (40.5%), and other arthritis (8.9%). With Disease Activity Score in 28 Joints, 76.9% had moderate to high disease activity, and 61.9% had moderate to severe disability (Health Assessment Questionnaire). Considering undifferentiated arthritis, 60.3% persisted undifferentiated, 29.4% evolved as rheumatoid arthritis, 4.4% remained self-limited, and 5.9% to other forms. The frequencies of depression and anxiety were high as measured with the Hospital Anxiety and Depression Scale, and approximately 20% had significant decrements in quality of life measured with the Medical Outcomes Study Short-Form 36 Health Survey Version 2. Mean time from the first symptoms to the first visit to a rheumatologist was 126 days. Shorter delays were confirmed to be associated with better outcomes. CONCLUSIONS: The REPANARC project is a useful tool to provide valuable information regarding patients with early-onset arthritis attending rheumatology centers in Latin-America.
27538899 Ganglionic acetylcholine receptor autoantibodies in patients with autoimmune diseases incl 2017 Jul OBJECTIVES: Autonomic dysfunction is closely associated with autoimmune diseases (AID) including primary biliary cirrhosis (PBC). The objective of this study was to determine the prevalence of anti-ganglionic (nicotinic) acetylcholine receptor (gAChR) antibodies in patients with AID. METHODS: We determined the presence of gAChR antibodies in serum samples from 146 patients (systemic lupus erythematosus [SLE] = 32; rheumatoid arthritis [RA] = 43; systemic sclerosis [SSc] = 38; PBC= 33) without information regarding autonomic symptoms, as well as 34 patients with other neurological diseases [OND], and 73 healthy controls [HC]. We specifically analyzed sera for anti-gAChRα3 and -β4 antibodies using the luciferase immunoprecipitation system (LIPS) assay. RESULTS: LIPS assay detected anti-gAChRα3 and -β4 antibodies in the sera from patients with SLE (12.5%, 4/32), RA (18.6%, 8/43), SSc (13.2%, 5/38), PBC (9.1%, 3/33), OND (2.9%, 1/34), and HC (0.0%, 1/73). There were no significant correlations between the levels of anti-gAChRα3 and -β4 antibodies, and the total titers of autoantibodies in AID. CONCLUSIONS: The results demonstrated a significant prevalence of anti-gAChR antibodies in patients with AID, which is independent of the production of other autoantibodies in patients with autoimmune diseases. These anti-gAChR antibodies could mediate the autonomic dysfunction involved in the autoimmune mechanisms of AID.
24832356 Chemopreventive effects of a curcumin-like diarylpentanoid [2,6-bis(2,5-dimethoxybenzylide 2015 Jul AIM: Synovial fibroblast has emerged as a potential cellular target in progressive joint destruction in rheumatoid arthritis development. In this study, BDMC33 (2,6-bis[2,5-dimethoxybenzylidene]cyclohexanone), a curcumin analogue with enhanced anti-inflammatory activity has been synthesized and the potency of BDMC33 on molecular and cellular basis of synovial fibroblasts (SF) were evaluated in vitro. METHODS: Synovial fibroblast cells (HIG-82) were cultured in vitro and induced by phorbol-12-myristate acetate (PMA) to stimulate the expression of matrix metalloproteinase (MMPs) and pro-inflammatory cytokines. The protective effects of BDMC33 were evaluated toward MMP activities, pro-inflammatory cytokine expression and nuclear factor kappa-B (NF-κB) activation by using various bioassay methods, including zymography, Western blotting, reverse transcription polymerase chain reaction, immunofluorescense microscopy and electrophoretic mobility shift assay. RESULTS: The results showed that BDMC33 significantly inhibited the pro-gelatinase B (pro-MMP-9) and collagenase activities via suppression of MMP-1 in activated SF. In addition, BDMC33 strongly suppressed MMP-3 gene expression as well as inhibited COX-2 and IL-6 pro-inflammatory gene expression. We also demonstrated that BDMC33 abolished the p65 NF-κB nuclear translocation and NF-κB DNA binding activity in PMA-stimulated SF. CONCLUSIONS: BDMC33 represents an effective chemopreventive agent and could be used as a promising lead compound for further development of rheumatoid arthritis therapeutic intervention.
27138022 Safety, bioavailability, and pharmacokinetics of VGX-1027-A novel oral anti-inflammatory d 2016 Mar VGX-1027, a novel oral immune modulator, is under development for the treatment of rheumatoid arthritis. The safety, tolerability, and pharmacokinetics of single (1-800 mg) and multiple (40-400 mg) oral doses were evaluated in 2 clinical studies. The doses were well tolerated up to 800 mg in a single dose and 200 mg twice daily in multiple doses. Adverse events were mild to moderate in severity with no identifiable dose-related pattern. There were no clinically significant physical or laboratory findings. The pharmacokinetic data indicated that increases in Cmax and AUC0-inf were dose-proportional, and AUC0- τ was approximately dose-proportional. For the single-dose study, median Tmax ranged from 0.5 to 2 hours and mean t1/2 ranged from 4.9 to 8.7 hours. For the multiple-dose study, median Tmax ranged from 0.5 to 2.0 hours and mean t1/2 ranged from 7.05 to 10.05 hours. No accumulation of the drug was observed after day 1, indicating that steady-state concentrations were attained with single and multiple dosing for 5 days. Approximately 90% of the administered dose was excreted in urine as unchanged drug.
30766061 Insights and Implications of the VA Rheumatoid Arthritis Registry. 2015 May The VA Rheumatoid Arthritis Registry addresses the underrepresentation of veterans in rheumatoid arthritis research, serving as a repository that links banked serum, plasma, and DNA samples with an array of patient-level information.
27022509 LIMPING IN CHILDREN. 2009 Jan Limping in children is a common complaint at pediatric, pediatric orthopaedic offices and in emergency rooms. There are several causes for this condition, and identifying them is a challenge. The older the patient, the better the anamnesis and more detailed the physical examination will be, enabling an easier medical assessment for searching the source of the disorder. In order to make the approach easier, three age groups can and should be considered. Among infants (1 to 3 years old), diagnosis will most likely be: transitory synovitis, septic arthritis, neurological disorders (mild brain palsy (BP) and muscular dystrophy), congenital hip dislocation (CHD), varus thigh, juvenile rheumatoid arthritis (JRA) and neoplasias (osteoid osteoma, leukemia); in the scholar age group, between 4 and 10 years old, in addition to the diagnoses above, Legg-Calvé-Perthes disease, discoid meniscus, inferior limbs discrepancy and unspecific muscular pain; in adolescents (11 to 15 years old): slipped capital femoral epiphysis, congenital hip dislocation, chondrolysis, overuse syndromes, dissecans osteochondritis, and tarsal coalition. The purpose of this study is to provide an update on how to approach pediatric patients presenting with limping, and to discuss its potential causes.
30640995 [Effects of Longzuan Tongbi Recipe on Fas/FasL Systems in Serum and Synovium of Collagen-i 2016 Aug Objective To observe the effect of Longzuan Tongbi Recipe (LTR) on Fas/FasL sys- tems in serum and synovium of collagen-induced arthritis (CIA) rats. Methods Ten rats were randomly selected from 60 male Wistar rats as a normal control group. CIA model was prepared by injecting type II bovine collagen and incomplete Freund's adjuvant mixture in the rest 50 rats. After modeling rats were di- vided into the model group, the methotrexate (MTX) group, high, middle, and low dose LTR groups, 10 in each group. Normal saline was administered to rats in the model group by gastrogavage. MTX solution (0.27 mg/100 g) was administered to rats in the MTX group by gastrogavage, once per week for 4 succes- sive weeks. LTR (4.32, 2.16, 1.08 g/mL) was administered to rats in the 3 LTR groups by gastrogavage, twice per day for 30 successive days. Morphological changes of synovium were observed by HE staining. Expression levels of Fas/FasL in rat serum and synovium were quantitatively detected by ELISA. Results Normal synovium cells could be seen in the normal group. But they were obviously proliferated, fat cells in the lower synovium were reduced or deformed, fibroblasts were increased in the model group, accompa- nied with infiltration of lymphocytes and monocytes. All these changes were more obviously alleviated in the MTX group, and the 3 LTR groups. Compared withI the normal control group, Fas expression level in- creased in rat serum and synovium, serum FasL expression level decreased in the model group (P <0. 05, P <0. 01). Compared with the model group, Fas expression level decreased in rat serum and synovium in the MTX group, high and middle dose LTR groups; Fas expression level in rat serum increased in the MTX group and 3 LTR groups; Fas expression level in synovium increased in the MTX group, high and middle dose LTR groups (P <0. 05, P <0. 01). Compared with the MTX group, Fas expression level in serum of the low dose LTR group, and Fas expression level in synovium of low and middle dose LTR groups was elevat- ed; Fas expression level in serum and synovium of the high dose LTR group was reduced; FasL expres- sion level in serum and synovium of low and middle dose LTR groups was reduced; FasL expression level in serum and synovium increased of the high dose LTR group (P <0. 05, P <0. 01). Conclusion LTR could control and treat rheumatoid arthritis, and its mechanism might lie in regulating. Fas/FasL systems media- ted cell apoptosis, and relieving pathological reaction of rheumatoid arthritis.
26767974 Possible alendronate-induced polyarticular synovitis. 2016 Apr We present a case of polyarticular synovitis following alendronate treatment for osteoporosis. The patient had no evidence of rheumatoid arthritis, pyrophosphate arthropathy, or seronegative/seropositive arthritis. Our main aim in this study is to highlight the potential adverse effects of alendronate and to warn orthopedic surgeons about the possibility of such a side effect that might lead orthopedic surgeons to administer wrong and unnecessary treatments like arthrocentesis. The withdrawal of alendronate is found to be the treatment of choice. Alendronate should be considered as a possible cause of synovitis or polyarthritis in patients treated with this agent in the absence of any other pathology. An association between alendronate and synovitis has rarely been described in the literature. We present a patient who developed polyarticular synovitis after treatment with alendronate and responded to its withdrawal.
25557144 Risk of tuberculosis in patients treated with anti-tumor necrosis factor therapy: a nation 2015 Mar AIM: The aim of this study was to investigate the incidence of tuberculosis (TB) following anti-tumor necrosis factor (TNF) therapy in an intermediate TB burden area and to compare the risk between drugs and diseases. METHODS: The data were obtained from a nationwide database maintained by the Health Insurance Review and Assessment Service. The study population comprised of patients who were prescribed with TNF inhibitors from 2005 to 2009. TB cases were selected based on prescription of anti-TB medications. RESULTS: Of 8421 patients in the study population, 1729 patients with latent TB prophylaxis were identified and 102 patients developed TB. The incidence of TB was 1017 per 100 000 person-years. When divided into four groups according to the main diagnosis and using an ankylosing spondylitis group as a reference, the incidence of TB was highest in patients with inflammatory bowel disease (IBD) (incidence rate ratio [IRR] 5.97, 95% confidence interval [CI] 3.34-10.66), followed by patients with rheumatoid arthritis (IRR 1.02, 95% CI 0.57-1.83) and those with psoriatic arthritis (IRR 1.00, 95% CI 0.14-7.30). Comparison between drugs showed a significantly lower incidence of TB in patients treated with etanercept (reference), highest incidence in those treated with infliximab (IRR 6.8, 95% CI 3.74-12.37) and an intermediate incidence in patients treated with adalimumab (IRR 3.45, 95% CI 1.82-6.55). CONCLUSIONS: The difference in TB risk between TNF inhibitors was similar with countries of low TB burden. This study suggests that particular attention is required for patients treated with TNF monoclonal antibodies.
25687918 The role and utility of measuring red blood cell methotrexate polyglutamate concentrations 2015 Apr PURPOSE: Evidence regarding the relationship between red blood cell methotrexate polyglutamate concentration and response to treatment and adverse drug reactions in patients using methotrexate for inflammatory arthropathies is complex and in some respects appears conflicting. Accordingly, we undertook a systematic analysis of available evidence to determine the clinical utility of dosing methotrexate to a target red blood cell methotrexate polyglutamate concentration. METHODS: A systematic literature review was conducted to identify all studies that had reported an association between red blood cell methotrexate polyglutamate concentration and disease activity or adverse drug reactions in users of methotrexate for the treatment of rheumatoid arthritis, juvenile idiopathic arthritis or psoriatic arthritis. RESULTS: No randomised controlled trials were identified. Thirteen studies (ten in patients with rheumatoid arthritis and three in patients with juvenile idiopathic arthritis) were identified. All studies evaluated an association between red blood cell methotrexate polyglutamate concentration and response to treatment, and eight evaluated an association with toxicity. Eight studies identified lower disease activity with at least one higher red blood cell methotrexate polyglutamate concentration, although there was at least moderate potential for bias in all of these studies. Relatively large increases in concentration appeared to be required to produce a meaningful reduction in disease activity. Only one study identified an association between red blood cell methotrexate polyglutamate concentration and methotrexate-induced side effects, although studies were likely underpowered to detect this type of association. CONCLUSIONS: The manner in which data were presented in the included studies had many limitations that hampered its conclusive assessment, but red blood cell methotrexate polyglutamate concentrations appear to be a potentially useful guide to treatment in patients with inflammatory arthropathies, but the specific polyglutamate that should be monitored and how monitoring could be integrated into treat-to-target approaches should be clarified before it can be routinely implemented.
25601383 Surface replacement arthroplasty for glenohumeral arthropathy in patients aged younger tha 2015 Jul BACKGROUND: The role of cementless surface replacement arthroplasty (CSRA) in young individuals is currently unclear. The aim of this study was to evaluate CSRA long-term results for glenohumeral arthritis in young patients. METHODS: Between 1990 and 2003, 54 CSRAs were performed on 49 patients (25 men, 24 women) aged younger than 50 years. Mean age was 38.9 years (range, 22-50 years). Three patients (4 shoulders) died over time and 8 were lost to follow-up, leaving 38 patients (42 shoulders) with a mean follow-up of 14.5 years (range, 10-25 years). There were 17 total shoulder replacements with metal back glenoid, and 37 underwent humeral head resurfacing with microfracture of the glenoid. RESULTS: The indications were avascular necrosis, 16; rheumatoid arthritis, 20; instability arthropathy, 7; primary osteoarthritis, 5; fracture sequelae, 3; postinfection arthritis, 2; and psoriatic arthritis, 1. The mean relative Constant score increased from 11.5% to 71.8% (P < .0001), and the mean patient satisfaction at final follow-up was 8.7 of 10. The mean relative Constant score for the humeral head resurfacing with microfracture of the glenoid improved to 77.7% compared with 58.1% for total resurfacing arthroplasty. Two required early arthrodesis due to instability and deep infection. Seven were revised to stemmed prosthesis: 1 for traumatic fracture and 1 for glenoid erosion 16 years after the index procedure. Five shoulders in 4 patients (4 rheumatoid arthritis, 1 avascular necrosis) were revised at 8 to 14 years after surgery for cuff failure and loosening. Three were revised to stemless reverse total shoulder arthroplasty due to rotator cuff failure at 23, 16, and 13 years after surgery. CONCLUSIONS: CSRA provides good long-term symptomatic and functional results in the treatment of glenohumeral arthropathy in patients aged younger than 50 years in 81.6% of the patients. This improvement is maintained over more than 10 years after surgery, with high patient satisfaction (8.7 of 10). However, 10 shoulders (of 54) (18.5%) underwent revision arthroplasty. Resurfacing offers a valuable tool in treating young patients with glenohumeral arthritis, providing reasonably good long-term results in 81.6% of the patients, while allowing preservation of bone stock if the need for revision arises. All the revision arthroplasty options are preserved, including less invasive procedures.
26375613 Low Risk of Birth Defects for Infants Whose Mothers Are Treated With Anti-Tumor Necrosis F 2016 Feb BACKGROUND & AIMS: Safety data on anti-tumor necrosis factor (anti-TNF) treatment during pregnancy are limited. We studied the risk of birth defects after anti-TNF treatment in early pregnancy. METHODS: We collected data on 1,272,424 live-born infants identified from the Danish (2004-2012) and Swedish (2006-2012) population-based health registers. We determined the prevalence of birth defects among infants born to women with chronic inflammatory disease (inflammatory bowel disease, rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis, or psoriasis), with (n = 683) and without (n = 21,549) anti-TNF treatment during early pregnancy, and in the general population. We compared the risk of any major birth defect and birth defect by organ system for infants born to women with chronic inflammatory disease, with and without anti-TNF treatment. Risks were presented as odds ratios (ORs) with 95% confidence intervals (CIs). We adjusted for maternal age, parity, smoking, body mass index, multiple gestation, country, and chronic inflammatory diagnosis. RESULTS: Birth defects were more prevalent among infants born to women with chronic inflammatory disease, regardless of anti-TNF treatment status, than in the general population (4.8% vs 4.2%). Birth defects occurred in 43 of the infants born to the 683 women who received anti-TNF treatment (6.3%), and 1019 of the infants born to women with chronic inflammatory disease (4.7%). The OR for any defect in women receiving anti-TNF therapy was 1.32 (95% CI, 0.93-1.82); the OR for a cardiovascular defect was 1.60 (95% CI, 0.93-2.58), and the OR for a urinary defect was 2.22 (95% CI, 0.86-4.71). CONCLUSIONS: Based on an analysis of data from the health registries in Denmark and Sweden, women who received anti-TNF agents during pregnancy had a slightly (but not significantly) higher risk of having children with birth defects. Although larger studies are needed, the heterogeneity of the observed birth defects did not indicate a common etiology.
27089203 Sclerostin: More than a bone formation brake. 2016 Mar 16 Anti-sclerostin treatment spurs an unexpected acceleration of bone erosion in a mouse model of rheumatoid arthritis (Wehmeyeret al, this issue).
26917952 Is drug discontinuation risk of adalimumab compared with etanercept affected by concomitan 2016 OBJECTIVE: To compare drug discontinuation risk between adalimumab (ADA) and etanercept (ETN) treatment among anti-tumor necrosis factor (anti-TNF)-naïve rheumatoid arthritis (RA) patients, in particular the influence of concomitant dose of methotrexate (MTX). METHODS: This retrospective nationwide population-based cohort study identified 4,592 anti-TNF-naïve RA patients in whom ETN (n=2,609) or ADA (n=1,983) was initiated using National Health Insurance claims data. After adjustment for prior medication, concomitant medication, and baseline demographic data, the relative risk of drug discontinuation in ADA users compared with ETN users was quantified by calculating adjusted hazard ratios (aHRs) with 95% confidence intervals (CIs) using Cox proportional hazard regression analyses, stratified by the follow-up time (≤1 year, >1 year) and/or concomitant MTX dose (≤10 mg/wk, >10 mg/wk). RESULTS: ADA users had a higher risk of drug discontinuation compared with ETN users during the first year of follow-up (aHR, 1.13; 95% CI, 1.01-1.27), but not during all treatment periods (aHR, 1.06; 95% CI, 0.98-1.16) or after 1 year (aHR, 0.99; 95% CI, 0.87-1.13). However, ADA users had a significantly higher risk of drug discontinuation compared with ETN users among patients on concomitant MTX >10 mg/wk during all treatment periods (aHR, 1.27; 95% CI, 1.10-1.47), during the first year of follow-up (aHR, 1.48; 95% CI, 1.22-1.78), or after 1 year (aHR, 1.42; 95% CI, 1.06-1.90), but not among patients on concomitant MTX 0-10 mg/wk. CONCLUSION: This population-based cohort study demonstrated a modification effect of concomitant MTX dose on the relative risk of anti-TNF discontinuation for ADA compared with ETN among anti-TNF-naïve RA patients. However, the lack of exact cause of anti-TNF discontinuation limited causal inference of such a concomitant MTX dose-related modification effect.
26509060 Late-onset neutropenia after treatment with rituximab for rheumatoid arthritis and other a 2015 OBJECTIVES: To evaluate the prevalence of late-onset neutropenia and its complications in patients treated with rituximab (RTX) for rheumatoid arthritis (RA) and other autoimmune diseases (AIDs) in a prospective registry. METHODS: The AutoImmunity and Rituximab registry is an independent 7-year prospective registry promoted by the French Society of Rheumatology. For each episode of neutropenia, data were validated by the clinician in charge of the patient. RESULTS: Among 2624 patients treated with RTX for refractory AIDs, and at least 1 follow-up visit (a total follow-up of 4179 patient-years in RA and 987 patient-years in AIDs), late-onset neutropenia was observed in 40 patients (25 RA (1.3% of patients with RA, 0.6/100 patient-years), and AIDs in 15 (2.3% of patients with AIDs, 1.5/100 patient-years)). 6 patients (15%) had neutrophils <500/mm(3), 8 (20%) had neutrophils between 500 and 1000/mm(3), and 26 (65%) had neutrophils between 1000 and 1500/mm(3). Neutropenia occurred after a median period of 4.5 (3-6.5) months after the last RTX infusion in patients with RA, and 5 (3-6.5) months in patients with AIDs. 5 patients (12.5%), 4 of them with neutrophils lower than 500/mm(3), developed a non-opportunistic serious infection and required antibiotics and granulocyte colony-stimulating factor injections, with a favourable outcome. After resolution of their RTX-related neutropenia, 19 patients (47.5%) were re-treated, and neutropenia reoccurred in 3 of them. CONCLUSIONS: Late-onset neutropenia might occur after RTX and may result in serious infections. Thus, monitoring of white cell count should be performed after RTX. However, in this large registry of patients with AIDs, the frequency of RTX-induced neutropenia was much lower than that previously reported in patients treated for blood malignancies or AIDs.
25535773 Diagnostic utility of major salivary gland ultrasonography in primary Sjögren's syndrome. 2015 Jan OBJECTIVES: To investigate major salivary gland ultrasonography (US) in relation to symptoms and findings of oral and ocular dryness, and autoimmune disease, for potential use in diagnosis and follow-up of patients with primary Sjögren's syndrome (pSS). METHODS: Patients with pSS were recruited from the Department of Rheumatology, Haukeland University Hospital. The parotid and submandibular salivary glands were examined by US using a simplified scoring system for glandular homogeneity and hypoechogenic areas. Scans were graded on a scale 0-3, grades 0-1 considered corresponding to normal/non-specific changes and grades 2-3 to pathological changes. Sicca symptoms of the mouth and eyes, salivary gland capacity, tear secretion, minor salivary gland inflammation, serum autoantibodies, and fatigue were also investigated. RESULTS: US was performed in 97 patients. Oral and ocular sicca symptoms correlated with US score and decreased saliva levels. Fatigue VAS correlated with oral sicca symptoms but was inversely correlated with age. Patients with normal/non-specific US findings tended to be older than patients with pathological US findings. US score correlated with unstimulated and stimulated salivary secretion and tear secretion. Minor salivary gland inflammation correlated with major salivary gland US findings, and lymphoid organisation, germinal centre (GC)-like structures, in the minor salivary gland tissue biopsies was seemingly related to US pathology. Serum autoantibodies against Ro/SSA and/or La/SSB were associated with US pathology. CONCLUSIONS: US findings in major salivary glands correlate with subjective and objective oral and ocular items as well as systemic autoimmune features of pSS. US represents a useful imaging tool for diagnostics and follow-up of pSS.
26537778 Reevaluation for clinical manifestations of HTLV-I-seropositive patients with Sjögren's s 2015 Nov 4 BACKGROUND: The aim of the study was to reassess the prevalence and characteristics of human T lymphotropic virus type I (HTLV-I)-associated Sjögren's syndrome (SS) and SS in HTLV-I-associated myelopathy (HAM) based on the American European Consensus Group (AECG) criteria in HTLV-I endemic area, Nagasaki prefecture. METHODS: The 349 patients who underwent a minor salivary gland biopsy (MSGB) for suspected SS were retrospectively classified by AECG classification criteria and divided with or without anti-HTLV-I antibody. RESULTS: The HTLV-I data-available 294 patients were investigated. One hundred-seventy patients were classified as SS and 26.5 % were HTLV-I-seropositive. We have included 26 patients with HTLV-I-associated myelopathy (HAM) and 38.5 % were classified as having SS. The prevalences of ANA and anti-SS-A/Ro antibody of HAM + SS were significantly low compared to the HTLV-I asymptomatic carriers (AC) with SS and the HTLV-I-seronegative SS patients, although lacrimal dysfunction tended to be high in HAM + SS and significantly high in AC + SS patients compared with the patients with HTLV-I-seronegative SS. The focus scores of MSGB in the HAM + SS patients were similar to those of the AC + SS patients and the HTLV-I-seronegative patients with SS. Among the MSGB-positive patients, there was a low prevalence of ANA in the HAM + SS patients. Similar results were obtained in case of anti-SS-A/Ro or SS-B/La antibody. CONCLUSION: In HTLV-I endemic area, high prevalence of anti-HTLV-I antibody among SS as well as the characteristics of HAM + SS and AC + SS was still determined by AECG classification criteria.
26411598 [Pseudo-adult Still's disease, anasarca, thrombotic thrombocytopenic purpura and dysautono 2016 Jan INTRODUCTION: Multicentric Castleman's disease can mimic adult-onset Still disease. It is exceptionally associated with anasarca, thrombotic microangiopathy and dysautonomia. CASE REPORT: We report a 32-year-old woman with an association of oligoanuria, anasarca, thrombotic microangiopathy with features compatible with adult-onset Still disease. The outcome was initially favorable with corticosteroids, immunoglobulins and plasmapheresis but with the persistence of relapses marked by severe autonomic syndrome and necessity of high dose corticosteroids. The diagnosis of mixed type Castleman's disease, HHV8 and HIV negative, was obtained four years after the onset of symptoms by a lymph node biopsy. The outcome was favorable after tocilizumab and corticosteroids but tocilizumab had to be switched to anakinra to ensure a proper and long-lasting control of the disease. CONCLUSION: Our patient partially fits the description of TAFRO syndrome (Thrombocytopenia, Anasarca, myeloFibrosis, Renal dysfunction, Organomegaly), a MCM rare variant, recently described in Japanese patients.
27087620 Sleep quality in patients with primary Sjögren's syndrome. 2016 May OBJECTIVES: To assess the sleep quality in primary Sjögren's syndrome (pSS) patients and evaluate its relationship with the disease, quality of life and mood disorders. METHODS: The sleep quality of 29 pSS women and 29 matched controls was assessed by the Pittsburgh Sleep Quality Index (PSQI). Seven domains are grouped according to three factors: F1 perceived sleep quality (subjective sleep quality, sleep latency, use of sleeping medication), F2 sleep efficiency (sleep duration, habitual sleep efficiency) and F3 daily disturbances (sleep disturbances, daytime dysfunction). These domains are scored as a single factor of global sleep quality. The Short Form Health Survey (SF-36), Functional Assessment of Chronic Illness Therapy (FACIT) fatigue scale and Hospital Anxiety and Depression Scale (HADS) were also administered. Disease activity and damage were evaluated with the EULAR Sjögren's syndrome disease activity index (ESSDAI), the Sjögren's Syndrome Disease Activity and Damage Indexes (SSDAI, SSDDI). RESULTS: The mean PSQI global score had higher pathological values (8.6±4.6) compared with controls (5.6±2.2) (p=0.002). F1 and F3 were significantly worse in cases (p=0.01, p=0.009). A negative correlation was found between SF-36 subscales and the global PSQI, F2 and F3. The anxiety HADS correlated with F2 and F3, while depression only with F3. No correlation with FACIT and disease indexes emerged. CONCLUSIONS: Using PSQI, an impaired sleep quality was demonstrated in pSS patients, especially with perceived quality and the daily disturbances. It is associated with a reduced quality of life but not with disease-related variables.
26147835 Comparative Evaluation of Silicone Hydrogel Contact Lenses and Autologous Serum for Manage 2015 Sep PURPOSE: To comparatively evaluate the efficacy of a bandage contact lens (BCL) and autologous serum (AS) eye drops in the management of severe dry eye caused by Sjögren syndrome (SS). METHODS: In this prospective randomized study, 40 patients with SS were enrolled. Patients were divided into 2 treatment groups: BCL and AS. RESULTS: A total of 37 patients were included, 18 patients (35 eyes) in the AS group and 19 patients (36 eyes) in the BCL group. At the end of 6 weeks, the best-corrected visual acuity improved significantly in the BCL group (0.5 ± 0.3 vs. 0.3 ± 0.2, P = 0.003) but not in the AS group (0.4 ± 0.3 vs. 0.3 ± 0.3, P = 0.11). The best-corrected visual acuity remained stable up to 6 weeks after discontinuation of the BCL (0.5 ± 0.3 vs. 0.4 ± 0.2, P = 0.03). Although the Ocular Surface Disease Index scores decreased significantly after treatment in both groups, patients in the BCL group had lower Ocular Surface Disease Index scores than those in the AS group (53.4 vs. 41.8 at week 3, 47.1 vs. 31.0 at week 6, 52.7 vs. 32.0 at week 12; P = 0.014, <0.001, <0.004, respectively). The "faces" scores showed improved quality of life in both groups. Tear break-up time improved significantly in both groups except at 6 weeks after discontinuation of the AS. Patients in the BCL group had lower corneal staining scores than those of the AS group after 6 weeks of treatment and 6 weeks after discontinuation of treatment (P < 0.01). There was no significant change in Schirmer I test scores between or within groups. CONCLUSIONS: Balafilcon A silicone hydrogel contact lenses as a BCL were effective in the management of SS-associated dry eye. Clinical Trial Registration—URL: http://www.clinicaltrials.gov. Unique identifier: NCT02147509.