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Overall survival of systemic lupus erythematosus (SLE) patients significantly increased in recent decades, however, the relative risk of mortality is still high. Long-term survival outcome of pediatric SLE remains unclear. This study aims to explore the long-term survival rate and its predictors in patients with systemic lupus erythematosus (SLE).A retrospective, hospital-based cohort study was performed between 2004 and 2018 in a tertiary referral medical center in Taiwan. Data on comorbidities, medications, and causes of admission were collected for risk factor analysis using time-dependent multivariate Cox proportional hazards models.A total of 2392 adults and 115 pediatric SLE patients were enrolled (female, n = 2157 and 95, respectively). The 10-year survival rates were 93.2%, 90.2%, 98.9%, and 100% in adult women, adult men, girls, and boys with SLE, respectively. The overall mortality rate was 2.09 case/100 patient-years (PY) for male SLE and 1.39 case/100 PY for female SLE patients. Male SLE patients did not have a statistically significantly higher mortality rate than female SLE patients in each age stratification. Infectious disease (n = 119), heart failure (n = 21), and cerebrovascular accident (n = 14) were the leading causes of death in adult SLE patients. Advanced age (hazard ratio [HR]: 1.04, 95% confidence interval [CI]: 1.03-1.05), treatment with mean dosage of systemic glucocorticoid equivalent to10 mg/d of prednisolone (HR: 1.71, 95% CI: 1.14-2.57), comorbidities with malignancy (HR: 1.94, 95% CI: 1.22-3.09), chronic kidney disease (HR: 1.86, 95% CI: 1.25-2.77), hypertension (HR: 1.42, 95% CI: 1.01-1.98), and admission due to bacterial pneumonia (HR: 1.92, 95% CI: 1.12-3.31) and sepsis (HR: 2.78, 95% CI: 1.51-5.13) were independent risk factors for mortality in SLE patients.SLE patients with advanced age, malignancy, chronic kidney disease, hypertension, treated with a higher average dosage of glucocorticoids, and admission due to bacterial pneumonia and sepsis have an increased risk of mortality.

To investigate the association of blood pressure and hypertension with disease severity among the patients with ankyloing spondylitis (AS). There were 167 AS patients enrolled in the cross-sectional study. Blood pressure was measured and the presence of hypertension was recorded. Patient's disease severity, including disease activity, functional ability, patient's global assessments, physical mobility and radiographic damage were evaluated. ESR and CRP levels were tested. We recorded patient's medication use of NSAIDs, DMARDs and TNF-α blockers. Smoking, exercise habit, diabetes mellitus, hypercholesterolemia and obesity indices were assessed. Multivariate linear regression showed that systolic blood pressure was associated with TNF-α blocker [standard coefficient (β) = 0.194, P = .007], DMARDs (β = 0.142, P = .046), age (β = 0.211, P = .003), male gender (β = 0.242, P = .001) and body mass index (BMI) (β = 0.245, P = .001). Diastolic blood pressure was associated with cervical rotation (β = -0.174, P = .037), lateral lumbar flexion (β = -0.178, P = .019), m-SASSS (β = 0.198, P = .038) and BMI (β = 0.248, P = .003). Notably, multivariate logistic regression showed that hypertension was associated with m-SASSS (OR = 1.033, P = .033), age (OR = 1.098, P = .0010) and BMI (OR = 1.210, P = .003). Using ROC cure analyses, age, BASMI, BASRI-Total, m-SASSS, waist circumference, BMI and waist-to-height ratio were useful in predicting hypertension, and m-SASSS is the best (AUC = 0.784, P .001). Advanced radiographic damage is an independent risk factor of hypertension in AS, and m-SASSS is the most useful disease severity parameter in predicting the presence of hypertension. Advanced radiographic damage, poor cervical rotation, lateral lumbar flexion, older age, male gender, TNF-α blocker, DMARDs use and obesity are associated with increased blood pressure.

In Taiwan, the incidence and prevalence of psoriatic arthritis (PsA) have risen significantly in recent years. Moreover, data from the Taiwan National Health Insurance Research Database (NHIRD) show that more than 85% of PsA patients are treated with just non-steroidal anti-inflammatory drugs (NSAIDs) and/or conventional synthetic disease-modifying anti-rheumatic drugs (csDMARDs). Taiwanese clinicians have also expressed concerns regarding uncertainties in the diagnosis of PsA and the delayed, interrupted, and/or tapered use of biologics, as well as differences in therapeutic preferences between and within dermatologists and rheumatologists. To address these issues, the Taiwan Rheumatology Association and the Taiwanese Association for Psoriasis and Skin Immunology jointly convened a committee of 28 clinicians from the fields of rheumatology, dermatology, orthopedics, and rehabilitation, to develop evidence-based consensus recommendations for the practical management of PsA in Taiwan. A total of six overarching principles and 13 recommendations were developed and approved, as well as a treatment algorithm with four separate tracks for axial PsA, peripheral PsA, enthesitis, and dactylitis. Psoriasis (PsO) management was not discussed here, as the Taiwanese Dermatological Association has recently published a comprehensive consensus statement on the management of PsO. Together, these recommendations provide an up-to-date, evidence-based framework for PsA care in Taiwan.

AIM To investigate total and central obesity in ankylosing spondylitis (AS), and assess the association with inflammation, disease severity and cardiovascular risk factors. METHODS There were 105 AS patients enrolled. Anthropometry was measured to determine total (body mass index [BMI]) and central obesity (waist circumference [WC], waist-to-height ratio [WHtR]). We evaluated patients' disease activity, functional ability, global assessment, physical mobility, radiographic damage and health index. Erythrocyte sedimentation rate, C-reactive protein (CRP) and blood biochemistry profile were tested. Retrospective radiographic change was assessed in 39 patients. Presence of diabetes and hypertension were examined. RESULTS The obese AS patients had higher inflammation (CRP), disease activity (Ankylosing Spondylitis Disease Activity Score [ASDAS] - CRP), physical mobility (Bath Ankylosing Spondylitis Metrology Index [BASMI]), radiographic damage (modified Stoke Ankylosing Spondylitis Spinal Score [m-SASSS]), liver function and blood pressure (all P

Objective Axial spondyloarthritis (axSpA) is a chronic inflammatory disease bearing challenges in early diagnosis. To improve clinical diagnosis and management of axSpA, recommendations were developed with current axSpA classification criteria and recent advances in medical imaging applications. Methods A systematic literature review was conducted by 10 rheumatologists and radiologists in Taiwan to retrieve research evidence on the utilization of imaging modalities, including conventional radiography (CR), magnetic resonance imaging (MRI), computed tomography (CT), ultrasound (US), quantitative sacroiliac scintigraphy (QSS), and dual-energy X-ray absorptiometry (DXA). The panel of experts proposed six key issues on the role of imaging in early diagnosis of axSpA, monitoring of disease activity and structural changes, predicting treatment effects, and assessing complications such as osteoporosis and spinal fracture. The consensus was established on the basis of research evidence, clinical experiences and expert opinions. For each recommendation statement, the level of evidence was evaluated, the strength of recommendation was graded and the final level of agreement was determined through voting. Results In total, four overarching principles and 13 recommendations were formulated. These recommendations outlined different imaging approaches in the diagnosis and management of axSpA disease progression. Considering CT is easy to perform when MRI is less available in Taiwan, the expert panel proposed a concise and practical diagnostic scheme to strengthen the valuable role of MRI and CT in the diagnostic evaluation of axSpA without evident radiographic features. Conclusion These modified recommendations provide guidance for rheumatologists, radiologists and healthcare professionals on timely diagnosis of axSpA and disease management with appropriate imaging modalities.

ObjectiveWe sought to test the hypothesis that Polygenic Risk Scores (PRSs) have strong capacity to discriminate cases of ankylosing spondylitis (AS) from healthy controls and individuals in the community with chronic back pain.MethodsPRSs were developed and validated in individuals of European and East Asian ethnicity, using data from genome-wide association studies in 15 585 AS cases and 20 452 controls. The discriminatory values of PRSs in these populations were compared with other widely used diagnostic tests, including C-reactive protein (CRP), HLA-B27 and sacroiliac MRI.ResultsIn people of European descent, PRS had high discriminatory capacity with area under the curve (AUC) in receiver operator characteristic analysis of 0.924. This was significantly better than for HLA-B27 testing alone (AUC=0.869), MRI (AUC=0.885) or C-reactive protein (AUC=0.700). PRS developed and validated in individuals of East Asian descent performed similarly (AUC=0.948). Assuming a prior probability of AS of 10% such as in patients with chronic back pain under 45 years of age, compared with HLA-B27 testing alone, PRS provides higher positive values for 35% of patients and negative predictive values for 67.5% of patients. For PRS, in people of European descent, the maximum positive predictive value was 78.2% and negative predictive value was 100%, whereas for HLA-B27, these values were 51.9% and 97.9%, respectively.ConclusionsPRS have higher discriminatory capacity for AS than CRP, sacroiliac MRI or HLA-B27 status alone. For optimal performance, PRS should be developed for use in the specific ethnic groups to which they are to be applied.

Aim To assess the real-world effectiveness and safety of rituximab (RTX) at 24 months in patients with established rheumatoid arthritis (RA) and to identify predictors of low disease activity/remission and a good European League Against Rheumatism (EULAR) response. Methods Seventy RTX-treated RA patients were enrolled. Predictors for low disease activity/remission and a good EULAR response at 24 months were identified by multivariate analyses. Results At 24 months, the mean Disease Activity Score of 28 joints-erythrocyte sedimentation rate (DAS28-ESR) decreased from 6.88 ± 0.85 at baseline to 3.47 ± 0.85. Twenty-nine patients (41.4%) reached low disease activity/remission, while all patients had a moderate/good EULAR response. After adjustment by multivariate analyses, we found concomitant methotrexate at a dosage >10 mg/week (odds ratio [OR] 5.17; 95% CI 1.34-19.93; P = 0.017) predicted low disease activity/remission, and baseline DAS28 ≤6.5 (OR 4.97; 95% CI 1.22-20.30; P = 0.026) predicted good EULAR response at 24 months. The most common adverse events were infusion-related (5.7%), and there was no incidence of malignancy or mortality during the treatment. Conclusions RTX was effective and safe in real-life management of RA patients with high disease activity. Patients taking concomitant methotrexate and with lower baseline DAS28-ESR were more likely to benefit from RTX.

Background/Purpose: To determine serum semaphorin 3A (Sema 3A) levels in ankylosing spondylitis (AS). Methods: Serum Sema 3A was measured in 46 AS patients and 30 healthy controls (HCs). For the patients, we recorded demographic data, disease activity, functional index & global assessment, detected human leukocyte antigen-B27 (HLA-B27), and measured erythrocyte sedimentation rate (ESR) & C-reactive protein (CRP). Results: Sema 3A was higher in AS patients than in HCs (3.98 ± 2.57 vs. 1.34 ± 0.48 ng/ml, p = 0.013). Area under the curve (AUC) of standard receiver operating characteristic (ROC) has suggested that Sema 3A > 2 ng/ml is better to predict the higher Bath Ankylosing Spondylitis Disease Activity Index (BASDAI, > 4) than ESR or CRP. There were good correlations between higher Sema 3A and uveitis, Schöber's test, as well as interstitial lung disease. AS patients undergoing anti-tumor necrosis factor therapies for 3 months exhibited a positive correlation of change in Sema 3A (ΔSema 3A) with disease activity fluctuation [ΔBASDAI, ΔBath Ankylosing Spondylitis Functional Index (BASFI) and ΔBath Ankylosing Spondylitis - Global score (BAS-G)]. Conclusion: Serum Sema 3A level was increased in AS patients and was inversely correlated to Schöber's test. Serum Sema 3A is better as a bio-marker than ESR or CRP to correlate with high disease activity in AS patients, and it is also a good indicator for monitoring disease activity and functional status during anti-TNF treatment. Also, Sema 3A may be taken as a predictor for extra-articular presentations in AS, but this needs further study to elucidate. Keywords: Ankylosing spondylitis, Semaphorin 3A, Osteoimmunology, Bone remodeling, Biomarker

Background/purpose: To investigate the Janus kinase-1 and 3 (JAK-1 and 3) expression in peripheral blood mononuclear cells (PBMCs) in ankylosing spondylitis (AS). Methods: The levels of JAK-1 and JAK-3 mRNA in PBMCs, CD3+ T cells and CD14+ monocytes were measured by RT-PCR in 52 AS patients and 31 healthy controls (HCs). The demographic features, BASDAI, BASFI, HLA-B27, ESR, CRP and serum immunoglobulin A (IgA) level were recorded and correlated with the JAK-1 & JAK-3 transcripts in patients and HCs as appropriate. Results: JAK-1 and JAK-3 expression in PB CD3+ T cells plus CD14+ monocytes was significantly higher in AS patients than in HCs (p 4) and BASFI (>4) than ESR or CRP in AS patients. Conclusion: In AS, JAK-1 expression in PB cells rather than ESR or CRP might be regarded as a bio-marker for monitoring disease activity and functional index in AS. These findings have also suggested that JAK-1 and JAK-3 expression may play a role in the inflammatory processes in patients with AS. Keywords: Ankylosing spondylitis, Janus kinase (JAK), Acute-phase reactant

Additional file 1: Figure S1. Dose-dependent binding of CTLA-4-Ig on activated human B cells. Purified blood CD19+ B cells were stimulated with or without anti-IgM (5 μg/ml) and anti-CD40 (1 μg/ml) antibodies for 3 days. (A) After washing, levels of CD80 and CD86 were examined using immunofluorescent staining and flow cytometry analysis. Medium, untreated B cells; IgM+CD40, activated B cells. Gray peaks, isotype control; black line, anti-CD80/86 antibody. (B) The binding of various concentrations (1, 10, and 100 μg/ml) of CTLA4-Ig (black lines) or L6 control protein (Grey peaks) on untreated and activated human B cells. Data are representative of 2-4 independent experiments. Figure S2. The effect of CTLA-4-Ig on the levels of CD80/CD86 on the surface of activated human B cells. (A) CTL4-Ig treatment prevented anti-CD80 antibody binding to TD stimulation-activated B cells. Purified blood CD19+ B cells were stimulated with anti-IgM (5 μg/ml) and anti-CD40 (1 μg/ml) antibodies in the presence of 100 μg/ml CTLA-4-Ig or L6-Ig control protein (Ctrl-Ig) for 2 days. The activated cells were split in half. One half of the cells were incubated with acid elution buffer for 4 mins at room temperature (Acid wash) and the other half were left untreated (w/o acid wash). After PBS washing, both parts of the cells were stained with anti-CD80, anti-CD86, and anti-IgG-Fc antibodies. Anti-IgG-Fc antibody was used to detect CTLA-4-Ig bound on the cell surface. Black lines, cells activated in the presence of CTLA-4-Ig; gray peaks, cells activated in the presence of Ctrl-Ig. The numbers in the upper right corner is the percentage of marker positive cells in the Ctrl-Ig treated (gray) or CTLA-4-Ig treated (bold) cells. The peak in the right of the anti-IgG-Fc staining histogram is surface IgG+ (class switched memory) B cells. (B) CTLA-4-Ig treatment reduced SAC-induced CD80 and CD86 levels on the surface of the B cells. CD19+ B cells were stimulated SAC in the presence of various concentrations (10, 30, or 100 μg/ml) of CTLA-4-Ig or L6-Ig control protein (Ctrl-Ig) for 2 days. After acid wash, the levels of CD80 and CD86 on the CTLA-4-Ig- (black lines) or Ctrl-Ig- (grey peaks) treated cells were examined using immunofluorescent staining. One representative experiment out of 4 was shown. Figure S3. The effect of abatacept on the levels of CD80/CD86 on the surface of the memory B cells from 3 patients with RA. The PBMCs isolated from 3 patients with RA were split in half. One half of the cells were incubated with acid elution buffer for 4 mins at room temperature (acid wash) and the other half were left untreated. After PBS washing, both parts of the cells were stained with anti-CD80, anti-CD86, anti-CD27, anti-IgD, anti-CD20, and anti-IgG-Fc antibodies. The label on top of the histogram indicates the time after abatacept injection. (A) The levels of CD80 and CD86 in the memory B cells of one of the 3 RA patients were shown. The analysis of CD80 or CD86 level was gated on memory (CD20+CD27+) cells. Black lines, the cells treated with acid wash; gray peaks, the cells without acidic elution. (B) The levels of CD80 and CD86 on the surface of the memory B cells in the PBMCs of the 3 RA patients. Gray dots, samples without acidic elution; open circles, samples with acidic elution. (C) The trend of CD80 and CD86 expression on the memory B cells of the same 3 RA patients in B before (top) and after acid wash (bottom).

Background & Aims In patients who have resolved hepatitis B virus (HBV) infection, treatment of rheumatoid arthritis (RA) can result in reappearance of hepatitis B surface antigen (HBsAg), called reverse seroconversion. We investigated clinical features and outcomes of reverse seroconversion in patients who received immunosuppressant or biologic therapy for RA. Methods We identified 1494 patients with RA (925 who resolved HBV infection) and available data on levels of antibody to HB core antigen and HBsAg who had attended Taipei Veterans General Hospital from January 2007 through December 2017. We identified 17 cases (median age, 66 years) who were negative for HBsAg before treatment of RA and reverse seroconversion (HBsAg reappearance) after glucocorticoid treatment (n = 13) and/or biologic therapy (adalimumab, n = 2; etanercept, n = 1; rituximab, n = 9; or abatacept, n = 4). Four patients were positive for antibodies against HBsAg (seroconverted) before the immunosuppressive treatment. Results The median time from immunosuppressive treatment to reverse seroconversion was 120 months (range, 20–264 months), whereas the time from biologic therapy treatment to reverse seroconversion was 66 months (range, 10–105 months). After reverse seroconversion, 8 individuals (47.1%) were positive for HB e antigen; 9 cases (52.9%) did not have a flare of alanine transaminase. However, 3 patients (17.6%) developed liver decompensation. Conclusions In patients who resolved HBV infection and received immunosuppressant treatment of RA, risk of reversal of seroconversion is low but persists for up to 10 years. Patients with RA who previously resolved HBV infections should be monitored for levels of HBsAg and HBV DNA once immunosuppressive treatment of RA begins.

Although human leucocyte antigen (HLA)‐B27 is strongly associated with ankylosing spondylitis (AS), the association of unfolded protein response (UPR) induced by HLA‐B27 misfolding in AS remains controversial. Since dendritic cells (DCs) are crucial in induction of AS in HLA‐B27‐transgenic rats, and plasmacytoid DCs (pDCs) belong to one type of DCs, we here aim to study the relevance of pDCs and UPR in AS. Peripheral pDCs were isolated from 27 HLA‐B27(+) AS patients and 37 controls. The bone marrow (BM) and synovium of inflamed hips from AS patients and controls were obtained. We found a significantly higher frequency of pDCs in the peripheral blood, BM, or inflamed synovium of hips, which is associated with the enhanced expression of pDC trafficking molecules, CCR6 and CCL20 in the synovium of AS patients. Functional analysis further revealed that several inflammatory cytokines, including TNFα, IL‐6, and IL‐23, secreted by pDCs were significantly increased in AS patients as compared with those in controls. Remarkably, protein kinase RNA‐like endoplasmic reticulum kinase (PERK) pathway in UPR was up‐regulated in pDCs of AS patients. Notably, PERK inhibitor treatment significantly inhibited the enhanced cytokine production by pDCs of AS patients. Further, the extent of PERK activation was significantly associated with the increased disease severity of AS patients. Our data uncover the aberrant distribution and function of pDCs in AS patients. The up‐regulated PERK pathway in UPR of pDCs not only contributes to enhanced cytokine production of pDCs, but also is associated with increased disease activity of AS patients.

Ankylosing spondylitis (AS) is a type of axial inflammation. Over time, some patients develop spinal ankylosis and permanent disability; however, current treatment strategies cannot arrest syndesmophyte formation completely. Here, we used mesenchymal stem cells (MSCs) from AS patients (AS MSCs) within the enthesis involved in spinal ankylosis to delineate that the HLA-B27–mediated spliced X-box–binding protein 1 (sXBP1)/retinoic acid receptor-β (RARB)/tissue-nonspecific alkaline phosphatase (TNAP) axis accelerated the mineralization of AS MSCs, which was independent of Runt-related transcription factor 2 (Runx2). An animal model mimicking AS pathological bony appositions was established by implantation of AS MSCs into the lumbar spine of NOD-SCID mice. We found that TNAP inhibitors, including levamisole and pamidronate, inhibited AS MSC mineralization in vitro and blocked bony appositions in vivo. Furthermore, we demonstrated that the serum bone-specific TNAP (BAP) level was a potential prognostic biomarker to predict AS patients with a high risk for radiographic progression. Our study highlights the importance of the HLA-B27–mediated activation of the sXBP1/RARB/TNAP axis in AS syndesmophyte pathogenesis and provides a new strategy for the diagnosis and prevention of radiographic progression of AS.

Objectives The aim of this study was to investigate the role of regulatory T cells (Tregs) in ankylosing spondylitis (AS). Methods We included 69 AS patients (15 of them received anti-tumor necrosis factor-apha agent-adalimumab) in the study and used a questionnaire to record the demographic data, disease activity index, functional index, human leukocyte antigen-B27, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Thirty healthy subjects were used as controls. The peripheral blood mononuclear cells (PMBCs) were stained with anti-CD4, anti-CD25 and anti-Forkhead/winged helix transcription factor P3 (anti-FoxP3) antibodies and flow-cytometry was used to determine cell populations. Results The percentages of Tregs in PMBCs were significantly higher in AS patients than in healthy controls. In AS patients who had poor disease functional index with higher levels of ESR and CRP were positively and significantly correlated with Tregs percentages in PMBCs. After adalimumab treatment in 15 patients, the percentages of Tregs, the ESR/CRP levels and the Bath Ankylosing Spondylitis Disease Activity Index/Bath Ankylosing Spondylitis Functional Index were significantly and gradually decreased over time. Conclusions The high expression of FoxP3 and CD25 on CD4 + T cells in PBMCs in AS patients was noted, and could be reversed by adalimumab therapy. These findings suggest that Tregs may play a role in modulating the inflammatory process in AS. Whether Tregs can be taken as a predictor for disease activity or treatment outcome is unclear and requires further study.

Aim To establish guidelines for the clinical management of axial spondyloarthritis that take into account local issues and clinical practice concerns for Taiwan. Method Overarching principles and recommendations were established by consensus among a panel of rheumatology and rehabilitation experts, based on analysis of the most up‐to‐date clinical evidence and the clinical experience of panelists. All Overarching Principles and Recommendations were graded according to the standards developed by the Oxford Centre for Evidence Based Medicine, and further evaluated and modified using the Delphi method. Results The guidelines specifically address issues such as local medical considerations, National Health Insurance reimbursement, and management of extra‐articular manifestations. Conclusion It is hoped that this will help to optimize clinical management outcomes for axial spondyloarthritis in Taiwan.

To investigate the effect of inflammation on expressions of bone morphogenetic proteins (BMPs) in peripheral blood mononuclear cells (PBMCs) and its association with individual radiographic changes in patients with ankylosing spondylitis (AS).The changes in BMP-2, -4, and -7 gene expressions in PBMCs were measured after stimulation by tumor necrosis factor-α (TNF-α) and interleukin-1β (IL-1β). The correlation of increase in gene expression with clinical and radiographic findings in patients with AS were analyzed.Both TNF-α and IL-1β could enhance BMP-2 expression in PBMCs from AS patients. Increases in BMP-2, -4, and -7 expressions in PBMCs positively correlated with total modified Stoke Ankylosing Spondylitis Spinal Score (all p0.05). Moreover, increases in BMP-2, -4, and -7 gene expressions after TNF-α and IL-1β stimulation were greater among AS patients with versus without severe sacroiliitis (all p0.05). Increases in BMP-2 and -7 expressions were greater in PBMCs from 4 patients with total (cervical, thoracic, and lumbar) spinal ankylosis than in the 8 patients who did not have total spinal ankylosis (all p0.05).In AS, inflammation upregulates the expression of BMPs in PBMCs which may lead to the radiographic progression with new bone formation.

In patients with systemic lupus erythematosus (SLE), low bone mineral density (BMD) is associated with increased age, prolonged disease, low body mass index (BMI), and overlap with rheumatoid arthritis (RA). Elevated fibroblast growth factor (FGF)-23 in cyclosporine A (CsA) users with SLE are associated with decreased active vitamin D and osteocalcin.The objective of this study was to investigate the steroid and CsA effect on bone metabolism and serum FGF-23 in SLE patients.Seventy-two SLE patients and 10 age- and sex-matched healthy individuals underwent blood tests for bone metabolic biomarkers and FGF-23, and lumbar spine dual-energy X-ray absorptiometry for BMD.Comparisons between patients and controls were made in premenopausal women/men younger than 50 years and postmenopausal women/men older than 50 years separately. SLE patients had more frequent low Z-score (≤-2.0, 8.5 vs. 0%), osteopenia (-2.5T-score-1.0, 52 vs. 50%), and osteoporosis (T-score≤-2.5, 12 vs. 0%), than the healthy age-compatible counterparts. BMD was significantly lower in patients with advanced age, longer disease duration, lower BMI, and overlap with RA (all p0.05 by multiple linear regression analyses). Serum FGF-23 was significantly higher and 1,25-dihydroxyvitamin D (1,25(OH)2D3) lower in SLE patients treated with glucocorticoid and CsA than in those not taking both of them (p=0.027 and 0.002, respectively). The cumulative dose of glucocorticoid was inversely correlated with serum intact parathyroid hormone (r=-0.299, p=0.011), C-terminal telopeptide of type I collagen (r=-0.581, p0.001), and osteocalcin (r=-0.648, p0.001). FGF-23 and the cumulative dose of CsA were positively correlated (r=0.38, p=0.001) and both were negatively correlated with 1,25(OH)2D3 (r=-0.266, p=0.016 and r=-0.55, p0.001) and osteocalcin (r=-0.234, p=0.034 and r=-0.274, p=0.02).SLE patients treated with glucocorticoid and CsA exhibited markedly decreased bone turnover. Those taking CsA had higher serum FGF-23 associated with suppression of 1,25(OH)2D3 and bone formation. Such high-risk patients necessitate regular screening of osteoporosis.

Primary Sjogren's syndrome (pSS) is a systemic autoimmune disease and may complicate with interstitial lung disease. The risk of Mycobacterium tuberculosis (TB) infection in patients with pSS has not been determined. This nationwide population-based study aimed to explore the incidence and risk factors of TB infection in patients with pSS. We identified 4,822 pSS patients from the Taiwan National Health Insurance database and compared the incidence rates of TB infection in these patients with 48,220 randomly selected age-, sex-, and comorbidity-matched subjects without pSS. The Cox proportional hazard model was used to identify risk factors for TB in patients with pSS. The risk of TB was higher in the pSS cohort than in the control cohort with an incidence rate ratio (IRR) of 1.58 (95% confidence interval [95% CI] 1.13-2.18, p = 0.006). The risk factors for TB in the pSS cohort were age ≥60 years (hazard ratio [HR] 3.22, 95% CI 1.78-5.84; p < 0.001), and corticosteroid usage, which had a dose-dependent effect in the pSS patients compared to the nonusers (daily prednisolone dose or equivalent less than 5 mg/day: HR 2.34; p = 0.020, 95% CI 1.14-4.78; 5 mg/day to less than 10 mg/day: HR 4.79, 95% CI 2.15-10.68; p < 0.001; 10 mg/day or more: HR 12.19, 95% CI 4.42-33.63; p < 0.001). Patients with pSS had a higher risk of pulmonary TB in Taiwan, which was related to age ≥60 years and corticosteroid usage.

Objectives This study aimed to identify the risk factors for mortality and the impact of dialysis modalities on the survival in SLE patients with end-stage renal disease (ESRD). Methods This retrospective nationwide population-based study using the National Health Insurance Research Database in Taiwan collected data from 1073 SLE ESRD patients starting maintenance dialysis between March 1997 and December 2006. A multivariate Cox regression hazard model was applied to identify factors predicting mortality in cohorts using different dialysis modalities and the impact of dialysis modalities on the survival outcome of these patients of both genders. Results The major threat to SLE patients on maintenance dialysis was infections. For SLE patients undergoing regular haemodialysis (HD), age, male sex, and high or absence of daily steroid dosing were predictive of higher mortality. For those undergoing regular peritoneal dialysis (PD), age and high daily steroid dosing were the predictive factors. After adjusting confounding factors, male patients with HD had a significantly poorer outcome than the counterpart with PD or female patients with HD. There was no survival difference among female SLE patients with different dialysis modalities. Conclusion No underlying comorbidities were identified to increase the mortality of patients receiving particular dialysis modalities after correcting for age and steroid factors. There was no impact of different dialysis modalities on survival of female SLE patients. However, male SLE patients seemed susceptible to fatal events complicated by HD, which led to an inferior survival rate.

Primary Sjogren's syndrome (PSS) is an autoimmune disease targeting exocrine glands. It ten times more dominantly affects women than men with an onset peak at menopause. The genetic factor predisposing women to PSS remains unclear. Therefore, we aimed to identify susceptibility loci for PSS in women. We performed genome-wide association study (GWAS) using 242 female PSS patients and 1444 female control in Han Chinese population residing in Taiwan. Replication was conducted in an independent cohort of 178 female PSS and 14,432 control subjects. We identified rs117026326 on GTF2I with GWAS significance (P = 1.10 × 10-15) and rs13079920 on RBMS3 with suggestive significance (P = 2.90 × 10-5) associating with PSS in women. The association of RBMS3 was further evidenced by imputation in which rs13072846 (P = 4.89 × 10-5) was identified and confirmed as female PSS associating SNP within the same LD with rs13079920. PSS pathogenesis involves both immune (effector) and exocrine (target) system. We suggested that while GTF2I is a previously reported associating gene which may function in immune system, RBMS3 is a novel susceptibility gene that predisposes women to PSS potentially through modulating acinar apoptosis and TGF-β signaling in target exocrine system.

Spondyloarthritis (SpA) is often diagnosed late in the course of the disease and improved methods for early diagnosis are required. We have tested the ability of genetic profiling to diagnose axial SpA (axSpA) as a whole group, or ankylosing spondylitis (AS) alone, in a cohort of chronic back pain patients.282 patients were recruited from centres in the United Kingdom, Germany, Taiwan, Canada, Columbia and Turkey as part of the ASAS classification criteria for axSpA study (ASAS cohort). Subjects were classified according to the ASAS axSpA criteria, and the modified New York Criteria for AS. Patients were genotyped for ~200,000 immune-mediated disease SNPs using the Illumina Immunochip.We first established the predictive accuracy of genetic data comparing 9,638 healthy controls and 4,428 AS cases from the homogenous International Genetics of AS (IGAS) Consortium Immunochip study which showed excellent predictive power (AUC=0.91). Genetic risk scores had lower predictive power (AUC=0.83) comparing ASAS cohort axSpA cases meeting the ASAS imaging criteria with IGAS controls. Comparing genetic risk scores showed moderate discriminatory capacity between IGAS AS and ASAS imaging positive cases (AUC 0.67±0.05), indicating that significant differences in genetic makeup exist between the cohorts.In a clinical setting of referred back pain patients suspected to have axial SpA we were unable to use genetic data to construct a predictive model better than that based on existing clinical data. Potential confounding factors include significant heterogeneity in the ASAS cohort, possibly reflecting the disease heterogeneity of axSpA, or differences between centres in ascertainment or classification performance.

Background Ankylosing spondylitis (AS) is a chronic arthritis with a pathogenesis which is not fully understood. A third subset of IL-17-producing T helper cells, called Th17 cells, has been discovered and characterized. We investigated whether IL-17 and IL-23, two Th17-related cytokines, play any roles in the pathogenesis of, and have any correlations with, disease activity and clinical manifestations in AS. Methods This cross-sectional study included 49 AS patients and 25 healthy control subjects. The serum IL-17 and IL-23 levels were measured using enzyme-linked immunosorbent assay kits. At the same time, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Bath Ankylosing Spondylitis Functional Index (BASFI) and Bath Ankylosing Spondylitis Patient Global Score (BAS-G) levels were measured, and physical examinations were performed on study participants to determine their extent of physical mobility. Results The serum IL-17 and IL-23 levels of the AS patients were significantly higher than those of the healthy controls. In the AS patients, the BASDAI scores had a better correlation with the serum IL-17 or IL-23 levels (IL-17, r = 0.351, p = 0.014; IL-23, r = 0.398, p = 0.005) than with ESR (r = 0.078, p = 0.600) and CRP (r = 0.012, p = 0.993). IL-17 or IL-23 correlate to the BASFI, BAS-G and parameters related to physical mobility. In the receiver operating characteristic (ROC) analysis, the serum IL-17 and IL-23 levels act better in discriminating patients with BASDAI≥4 (AUC value 0.88, p = 0.001) than ESR and CRP (AUC value 0.727, p = 0.008). Conclusion Serum IL-17 and IL-23 levels were significantly higher in AS patients than in healthy controls and the levels correlate to disease activity measured by BASDAI scores, but not parameters of functional ability and spinal mobility. These results suggest the existence of a role of IL-17 and IL-23 in the pathogenesis of inflammation in AS.

Objective.The aim of our study was to compare the clinical, functional, and radiographic outcomes at different ages of onset in patients with ankylosing spondylitis (AS).Methods.A total of 546 patients were enrolled consecutively and classified into 3 groups based on their age at symptom onset: (1) juvenile-onset AS (age ≤ 16 years; JoAS); (2) adult-onset AS (> 16 but < 40 years; AoAS); and (3) late-onset AS (≥ 40 years; LoAS). We compared the differences among the 3 groups. OR for disease outcomes were calculated and adjusted for sex, HLA-B27, and disease duration.Results.There were 67 patients (12.3%) with JoAS, 460 (84.2%) with AoAS, and 19 (3.5%) with LoAS. Male sex and HLA-B27 were associated with a younger age at onset (p < 0.001). Compared to patients with AoAS, patients with JoAS were more likely to present with peripheral arthritis, while patients with JoAS and LoAS were less likely to have back pain at the onset of AS (p < 0.05). After controlling for multiple covariates, JoAS was found to be associated with a worse functional outcome and global assessment, and a high serum immunoglobulin A level (p < 0.05). Patients with JoAS had less lumbar spinal radiographic severity (p < 0.05). There were no statistical differences in clinical or functional outcome between the LoAS and AoAS groups. None of the LoAS patients had radiographic hip involvement.Conclusion.Sex and HLA-B27 are significantly associated with age at onset of AS. Both JoAS and LoAS have their distinctive symptoms/signs at onset and different disease outcomes.

Introduction Systemic lupus erythematosus (SLE) is prevalent among young female adults, particularly in Asia. In Taiwan, features of end-of-life SLE patients remain unclear. Material and methods Data regarding SLE patients whose hospitalization ended up with death were collected and analyzed from the repository of the National Health Insurance Research Database, Taiwan, from 2005 to 2007. Results A total of 302 subjects were enrolled and the majority of these were young to middle-aged women (45.8 ±18.5 years); only one third of them were treated by rheumatologists. Eight patients (2.6%) with comorbid cancers received hospice care. Sepsis/bacteremia (42.1%) was the major acute comorbidity. Nephropathy/nephritis (35.1%) represented the major chronic comorbidity. Among 27 subjects with comorbid cancers, gynecological cancers were the most common (18%). Among the inpatient costs, the cost of prescriptions accounted for the majority (21.7 ±11.5%). Under a multivariate logistic regression, advanced age (≥ 65 years) correlated positively with acute lower respiratory conditions (ALRC) and diabetes mellitus (DM), and male gender correlated negatively with nephropathy/nephritis. The nephropathy/nephritis correlated positively with hospital stays > 14 days. The ALRC was closely associated with acute respiratory failure, but not with shock. However, shock was closely associated with hospital stays ≥ 14 days and sepsis/bacteremia. Cancer development was inversely correlated to nephropathy/nephritis, acute respiratory failure, and shock (all p < 0.05). Conclusions The end-of-life SLE patients revealed aforementioned characteristics and relationships. Sepsis/bacteremia, acute respiratory failure, and ALRC contributed most frequently to the ultimate death of acutely ill SLE patients.

The role of hepatitis B virus (HBV) infection in patients with primary Sjogren's syndrome (pSS) remains unclear. Therefore, we investigated the prevalence and clinical significance of HBV infection in Taiwanese patients with pSS. One hundred seventy-five patients with pSS who fulfilled the 2002 American-European Revised Classification Criteria were enrolled. Eighteen (10.3%) patients were positive for hepatitis B surface antigen (HBsAg). There were 4 males and 14 females, with the mean age of 54.4 years. The main immunological feature was rheumatoid factor (13 of 18, 72.2%), which was significantly higher than those negative for HBsAg (28.1%, p

Sjogren’s syndrome (SS) is a common immune disease that mainly affects the exocrine glands, which clinically present xerostomia, keratoconjuctivitis sicca and serological autoantibodies including rheumatoid factor, antinuclear antibody, anti-SS-A and anti-SS-B. The prevalence of SS is high but underestimated in the general population. SS may involve many other organs and tissues including lung (interstitial lung disease), kidney (renal tubular acidosis and hypokalemia), nerve (peripheral neuropathy), vessel (vasculitis, Raynauld’s phenomenon), bladder (interstitial cystitis), lymph node (lymphaenopathy), liver (autoimmune hepatitis), pancreas (pancreatitis) and GI (reflux esophagitis, peptic ulcer). In 2010, a new disease activity, the European League Against Rheumatism (EULAR) Sjogren Syndrome Disease Activity Index (ESSDAI) was proposed. Diagnosis of SS is based upon the 2002 classification criteria of SS, which originated from the 1996 European classification criteria. Methods for diagnosis of SS inclu...

To determine the factors associated with radiographic spinal involvement and hip involvement in ankylosing spondylitis (AS) and assess the influence of the damage seen in the radiographs on functional outcome in patients with AS.We included 531 consecutive patients and recorded the clinical, laboratory, and radiographic data. Based on the spinal radiographs, patients were classified into 3 categories: (1) no spinal involvement; (2) spinal involvement without fusion; and (3) spinal involvement with fusion. Hip involvement was assessed by the Bath Ankylosing Spondylitis Radiology Hip Index and defined by a score of at least 2. Logistic regression analyses were used to investigate the factors associated with the radiographic spine and hip involvements.Ninety-eight (18.5%) patients had radiographic evidence of spinal fusion and 48 (9.0%) had radiographic evidence of hip involvement. Patients who had longer disease duration, elevated C-reactive protein levels, advanced sacroiliitis, and radiographic hip involvement were significantly more likely to have spinal fusion (P0.05). Elevated C-reactive protein levels and advanced sacroiliitis were also significantly associated with the presence of spinal involvement without fusion (P0.05). Early disease onset and more radiographic severity in the spine and sacroiliac joints were the predictors of radiographic hip involvement (P0.05). Patients with either spine or hip involvement had significantly higher Bath Ankylosing Spondylitis Functional Index scores (P0.001).There is a relationship between radiographic sacroiliitis, spinal fusion, and hip involvement in patients with AS. Damage to the spine and hip seen radiographically can contribute to functional impairment.

The objective of the study was to investigate the role of receptor activator of nuclear factor-kappaB ligand (RANKL) and osteoprotegerin (OPG) in ankylosing spondylitis (AS). Serum levels of soluble RANKL (sRANKL) and OPG were measured in 42 AS patients and 26 healthy controls. We evaluated the AS patient's disease activity, functional ability, global assessment, and physical mobility and tested markers of systemic inflammation, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) levels. Serum levels of sRANKL [mean (SD), 4.75 (1.88) vs. 3.70 (1.14) pmol/l, p = 0.015] and OPG [mean (SD), 5.18 (1.19) vs. 4.52 (0.85) pmol/l, p = 0.026] were significantly higher in the 42 AS patients than the 26 healthy controls. Interestingly, serum OPG levels correlated significantly with ESR (r = 0.417, p = 0.007), CRP (r = 0.524, p0.001), tragus-to-wall distance (r = 0.556, p0.001), fingertip-to-floor distance (r = 0.423, p = 0.007), and occiput-to-wall distance (r = 0.465, p = 0.002) and correlated inversely with modified Schober index (r = -0.525, p = 0.001), cervical rotation (r = -0.403, p = 0.022), lateral lumbar flexion (r = -0.587, p0.001), and chest expansion (r = -0.553, p0.001). Moreover, in the AS patients with higher (or =4.925 pmol/l, n = 21) serum OPG levels, there were significant increases in the tragus-to-wall distance (p = 0.007), fingertip-to-floor distance (p = 0.023), and CRP levels (p = 0.014) and decreased in the modified Schober index (p = 0.012), lateral lumbar flexion (p = 0.019), and chest expansion (p = 0.005). Serum levels of sRANKL and OPG are increased in the AS patients and may participate in the disease process of AS. Production of OPG has association with poor physical mobility and may reflect systemic inflammation in AS.

Aims: To assess the predictive and prognostic value of antinuclear antibodies (ANA) and rheumatoid factor (RF) in primary Sjogren’s syndrome (pSS). Methods: This retrospective study includes 201 patients that fulfilled the 1993 European preliminary classification criteria for pSS. The patients were further categorized by the 2002 revised criteria, with or without the inclusion of ANA and RF as classification criteria, and were further subgrouped by the presence of ANA, RF, anti-SS-A, and anti-SS-B, and different ANA titers. The clinical manifestations, serological markers, and results of lip biopsies among these subgroups were compared. Results: Our results showed pSS patients who are seropositive for one of the following markers: ANA, RF, anti-SS-A, or anti-SS-B are younger, predominantly female, and had more serological abnormalities than those with seronegativity of ANA, RF, anti-SS-A, or anti-SS-B. Higher ANA titers (≥ 1 : 640) correlated with higher frequency of serum anti-SS-A+ and anti-SS-B+, and elevations of serum immunoglobulin G and A in all three different classification criteria groups. The clinical manifestations and laboratory results in the 2002 revised criteria groups with or without the inclusion of ANA and RF as classification criteria items were highly concordant. Conclusion: Regardless of the classification criteria for pSS, patients who are seropositive for one of the ANA, RF, anti-SS-A and anti-SS-B biomarkers are more likely to have autoimmune-related Sjogren’s syndrome. ANA and RF have shown to possess the predictive and prognostic values for those who do not fulfill the higher stringent 2002 revised criteria but are indicated for immunomodulatory therapy. Thus we suggest that ANA and RF should be reconsidered as items of classification criteria for pSS.

Objective. To investigate the role of HLA-G in AS. Methods. Serum levels of soluble HLA-G (sHLA-G) were measured in 80 AS patients and 30 healthy controls. The expression of HLA-G on the peripheral blood mononuclear cell (PBMC) surface was investigated in the same 80 AS patients and 40 healthy controls by flow cytometry. The response of HLA-G after 3 months of TNF-a blocker therapy (adalimumab) was evaluated in 14 AS patients. We evaluated Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Bath Ankylosing Spondylitis Functional Index (BASFI), Bath Ankylosing Spondylitis Patient Global Score (BAS-G), physical mobility, ESR and CRP levels. Results. Serum levels of sHLA-G were significantly lower in 80 AS patients than 30 healthy controls [mean (S.D.) 22.47 (26.8) vs 34.78 (32.01) U/ml, P ¼ 0.028], and correlated significantly with modified Schober index (r ¼ 0.326; P ¼ 0.009), chest expansion (r ¼ 0.319; P ¼ 0.011), lateral lumbar flexion (r ¼ 0.377; P ¼ 0.002), cervical rotation (r ¼ 0.396; P ¼ 0.004), whereas inversely correlated with fingertip-to-floor distance (r ¼� 0.282; P ¼ 0.026) and tragus-to-wall distance (r ¼� 0.270; P ¼ 0.031). The expression of HLA-G on PBMCs was significantly higher in 80 AS patients than 40 healthy controls [mean (S.D.) 18.5 (6.10)% vs 15.41 (4.84)%; P ¼ 0.012], and correlated significantly with ESR (r ¼ 0.421; P < 0.001) and CRP (r ¼ 0.419; P < 0.001). The expression of HLA-G on PMBCs decreased significantly after 3 months of adalimumab therapy [third month vs baseline, 13.46 (5.38)% vs 19.87 (7.31)%; P ¼ 0.016]. Conclusions. Lower serum levels of sHLA-G contribute to susceptibility to AS, and predispose to poor spinal mobility. The expression of HLA-G on PMBCs is up-regulated in AS, correlates with acute phase reactants and decreases after TNF-a blocker therapy, suggesting an index of disease activity.

Background: To study the clinical effectiveness and adverse reactions of etanercept in patients with active rheumatoid arthritis (RA), in whom combination therapies with disease-modifying antirheumatic drugs (DMARDs) had failed. Methods: One hundred and thirty-three patients with active RA who had been treated without satisfactory effect with DMARDs were entitled, by the Taiwan Bureau of National Health Insurance, to undergo etanercept injection (25 mg subcutaneously, twice weekly) along with oral methotrexate (15 mg weekly) in Taipei Veterans General Hospital. The disease activity score in 28 swollen and 28 tender joints (DAS28), erythrocyte sedimentation rate (ESR), serum C-reactive protein (CRP), rheumatoid factors (RFs), tender joint count (TJC), and swollen joint count (SJC) were recorded at the beginning, 3, 6, 9, and 12 months after treatment. Any adverse event, relevant or irrelevant to the therapy, was recorded throughout the whole course of treatment. Results: Ninety-four patients completed the 1-year therapeutic program. There were significant improvements in all parameters (DAS28, ESR, CRP, TJC and SJC), which approached satisfactory values at the end of the first 3 months and which were sustained thereafter in most patients. Patients also tolerated the treatment protocol well, with adverse events occurring sporadically. Significant clinical response occurred as early as 3 months after the start and might last beyond 1 year in some patients. Adverse effects such as injection site reaction or infections rarely occurred. Conclusion: Combination therapy with etanercept and DMARDs seemed to be effective at improving the aching symptoms associated with rheumatoid activity and was well tolerated in this cohort study. It was generally safe, though a small number of non-fatal infections were observed. [J Chin Med Assoc 2009;72(11):581–587]

An extensive histopathogical analysis of synovial tissues is a good method to understand how specific cellular or molecular events developed in spondyloarthropathy (SpA). Although it is difficult to take biopsy sampling (the axial skeleton, sacroiliac joint, the enthesis, etc.), some histologic studies of peripheral syovial specimen (knee, hip etc.) have demonstrated the specific cellular or molecular features in SpA. The immunohistologic findings in SpA synovial tissues showed increased vascularity, synovial hyperplasia, inflammatory cells infiltration (especially macrophage subset, CD163+) and matrix metalloproteinase (MMP) expression (especially MMP3). Numbers of CD163+ macrophage and polymorphonuclear cells was correlated to global disease activity in SpA. TNF-α blockade have been shown not only to significantly improve the signs and symptoms of SpA but also to improve functional status and quality of life. CD163+ macrophages and MMP3 are rapidly downregulated by effective treatment with TNF-α blockade, and they are valuable synovial biomarkers for response to treatment in SpA. In the future, more molecular or cellular studies still need to be explored to understand detailed pathogenesis of SpA.

Objective.To measure serum concentration and analyze the expression of interleukin 18 (IL-18) mRNA in mononuclear cells of patients with systemic lupus erythematosus (SLE).Methods.IL-18 concentrations in sera and culture supernatants of peripheral blood mononuclear cells (PBMC) from healthy controls and patients with active SLE were measured by ELISA. PBMC and polymorphonuclear leukocytes (PMN) purified from patients with active SLE were stimulated with phytohemagglutinin (PHA), pokeweed mitogen (PWM), and lipopolysaccharide (LPS). Expression of IL-18 mRNA in cells was analyzed by RT-PCR.Results.Serum IL-18 levels were significantly higher in SLE patients than in controls, and correlated with disease activity in SLE patients (r2= 0.602). Two patients receiving intravenous methylprednisolone therapy (1.0 g/day for 3 days) showed profound decreases in serum IL-18 levels after therapy. The quiescent PBMC from SLE patients (30/30) expressed IL-18 transcript more frequently than control PBMC (20/30). PBMC from SLE patients produced more IL-18 than control PBMC after 72 hours of incubation, by RT-PCR. PHA and PWM inhibited the production of IL-18 in PBMC from both SLE patients and controls. Inhibition by PWM was more pronounced than that by PHA, especially in SLE-PBMC. Control and SLE-PMN with or without LPS stimulation produced negligible IL-18.Conclusion.IL-18 is involved in the autoimmune derangement of leukocyte function in patients with active SLE.

A 71-year-old man presented with quadriplegia, seizures, dysarthria, motor aphasia and urinary incontinence lasting for several years. The development of proteinuria and increased susceptibility to infections brought the physician's attention to possible underlying autoimmune diseases. Laboratory investigations revealed evidence for systemic lupus erythematosus (SLE) and antiphospholipid syndrome. Imaging studies showed obstructive hydrocephalus. Several courses of methylprednisolone therapies followed by maintenance therapy with low-dose steroid, ventriculoperitoneal shunt, and antihypertensives improved the proteinuria and dysarthria but not the urinary incontinence or dementia. A thromboembolic event in the central nervous system secondary to phospholipid antibodies or lupus activity may represent a pathogenetic basis for hydrocephalus. When encountering a patient with hydrocephalus but without apparent predisposing factors, it is always important to include SLE as a differential diagnosis.

To estimate the prevalence of spondyloarthritis (SpA) and the clinical features of human leucocyte antigen (HLA)-B27-associated acute anterior uveitis (HLA-B27 uveitis) in Chinese patients.We conducted a retrospective cohort study using a structured chart review to record the complete ocular history, including the onset of uveitis, month of uveitis attack, specific eye involvement, the time of first attack, and rheumatic manifestations from 1987 to 2004. A total of 504 patients with HLA-B27 uveitis were consequently enrolled consecutively from the uveitis clinic of Taipei Veterans General Hospital.In total, 1719 attacks of uveitis in 504 patients were recorded. Females tended to have a higher frequency of attack than males, and those with a disease course of less than 5 years showed more uveitis recurrence. The same eye attacks were observed in 156 of 332 patients (47%), more than the expected percentage compared with attacks with random-eye occurrence (p0.001). A significantly higher number of uveitis attacks occurred in winter. SpA-related acute anterior uveitis (AAU) was found in 387 patients (76.8%). Ankylosing spondylitis (AS) occurred in 214 patients (42.5%), with a significantly higher prevalence in males than in females (p0.001). Undifferentiated SpA (USpA)-related AAU occurred in 150 patients (29.8%), with a significantly higher prevalence in females than in males (p0.001). Patients with SpA had an earlier onset of uveitis (p = 0.01) and a greater number (or = 6) of attacks (p = 0.03).The prevalence of SpA was high in the Chinese population with HLA-B27-associated uveitis. The association with SpA indicated an earlier age of uveitis onset and a greater likelihood of having a higher number of uveitis attacks.