The ethiopathogenesis of increased apoptosis of lymphocytes in systemic lupus erythematosus (SLE) is still incompletely understood but anti-C1q autoantibodies have been shown to induce apoptosis in lymphocytes from healthy donors and certain cell lines.

This study was undertaken to investigate the relationship between peripheral lymphocyte apoptosis and serum levels of anti-C1q autoantibodies in SLE patients.

The sera of 124 patients with SLE involving 62 active SLE and 62 inactive SLE, fulfilling America College of Rheumatology (ACR) classification criteria for SLE (1997) were incubated with peripheral blood lymphocytes of healthy donors. The results were compared with 124 sex- and age-matched normal controls. Apoptotic lymphocytes (AL) were detected by flow cytometry using annexin V and propidium iodide binding. Anti-C1q autoantibodies were detected by an enzyme-linked immunoassay kit for all SLE patients.

Results demonstrated that the percentage of AL in the peripheral blood of active SLE patients was significantly higher (n = 62, 34.95 ± 12.78%) than that of the inactive SLE patients (n = 62, 30.69 ± 10.13%, P = 0.042, 95%CI = 0.16–8.36) and normal controls (n = 124, 27.92 ± 10.22%, P = 0.001, 95%CI = 3.33–10.73). The percentage of AL significantly correlated with serum levels of anti-C1q autoantibodies in the active SLE patients (r = 0.263, P = 0.039) but not in the inactive SLE patients (r = 0.170, P = 0.185).

The results of this study suggest that increased serum levels of anti-C1q autoantibodies are responsible for apoptosis and may play a pathogenic role in SLE patients, especially in active disease.

Apoptosis is induced by binding of death receptor ligands, members of the tumor necrosis factor (TNF) superfamily, to their cognate receptors. It is suggested that TNF-related apoptosis inducing ligand (TRAIL) is involved in pathogenesis of juvenile-onset systemic lupus erythematosus (JSLE). This study aimed to assess TRAIL concentrations in sera of JSLE children and to determine their potential relationship with disease activity, anti-double-stranded DNA (anti-dsDNA) levels, neutropenia and renal involvement.

Circulating levels of TRAIL were measured by enzyme-linked immunosorbent assay (ELISA) in serum samples obtained from 40 JSLE patients (20 with active and 20 with inactive disease) and 20 controls.

The mean (SEM) serum TRAIL concentration in JSLE was 1750.7 (440.2) pg/mL. Serum TRAIL concentrations in patients were higher than those in controls (P < 0.01). Serum TRAIL concentrations for children with inactive disease (1854.8 [485.4] pg/mL) and those with activity (1646.6 [390.6] pg/mL) were statistically comparable. JSLE children with positive anti-dsDNA antibodies had significantly higher TRAIL levels (mean = 1846 [456] vs. 1455 [325] pg/mL; P < 0.05). Serum TRAIL concentrations were significantly higher in classes III and IV nephritis compared to classes I and II nephritis (1970 [512] vs. 1330 [331] pg/mL; P < 0.01). Serum TRAIL concentrations in patients with neutropenia were higher than those without neutropenia (1805 [505] vs. 1516 [400] pg/mL; P = 0.042) and in controls (P = 0.024).

Our data indicate that an increased level of TRAIL is a feature of JSLE that correlates with disease activity, anti-dsDNA titers neutropenia and lupus nephritis.

Chronic low back pain is an important health problem Sri Lankan males.

To determine the association between low back pain and posture, exercise, family history, level of education, level of income, smoking, consumption of alcohol and animal proteins.

A case control study was carried out among 166 cases (patients with low back pain) and 196 controls (without low back pain). Data was collected using a pretested structured interviewer-administered questionnaire, enquiring about demographic data and details of risk factors. Heights and weights were measured to calculate body mass index (BMI).

Age range was 18–85 years. Mean age (SD) for cases was 47.8 (16) years. Mean age (SD) for controls was 42.6 (17) years. Bad posture (odds ration [OR] = 107.4), lack of exercise (OR = 16.4), positive family history (OR = 42.3), moderate education (OR = 1.8), daily alcohol consumption (OR = 2.4) and not consuming animal proteins (OR = 4.6) were significant risk factors for low back pain. BMI, level of income and smoking did not have a significant association with low back pain.

Posture, exercise, family history, level of education, consumption of animal proteins and consumption of alcohol had significant associations with low back pain. Most risk factors were similar to other countries. However, being marginally over weight and smoking regularly were not significantly associated with low back pain.

Fibromyalgia (FM) is a chronic disorder characterized by widespread musculoskeletal pain and fatigue. It is a less frequently diagnosed disease in China, thus Chinese rheumatologists may have lower awareness of FM compared with colleagues in Western countries. The aim of this study is to investigate the perceptions of FM in Chinese rheumatologists and analyze their therapeutic approach in clinical practice.

An anonymous questionnaire survey was conducted among a nationwide sample of Chinese rheumatologists at the 15th National Rheumatology Conference in 2010. The 20-question survey included questions regarding background, work experience, perceptions of diagnosis and behaviors of treatment related to FM. Continuing medical education (CME) information was also collected in the survey.

Seven hundred and seven rheumatologists responded to the questionnaire, a response rate of 60%. Less than one-fifth of the respondents were experienced in dealing with FM. Although most of the respondents regarded FM as a distinct pathological entity, nearly 30% of Chinese rheumatologists believed that FM was only a psychological disorder. The respondents recognized some of the FM-related symptoms, but had limited knowledge on the diagnostic criteria. Eighty percent of the respondents declared they had difficulties in treating FM patients. However, nearly all (90.8%) respondents believed that the prognosis of FM patients was usually benign. Our data also showed that most Chinese rheumatologists were eager for CME on FM.

The awareness and perception of FM are still low among Chinese rheumatologists. CME on FM is needed for improving the quality of health care in China.

Besides joint destruction, extra-articular complications (outside the locomotor system) are frequent in rheumatoid arthritis (RA) patients, especially cardiovascular, hematological and metabolic disorders. Here, we evaluated and compared the protective activity of two different doses of mixture of ginger and turmeric rhizomes powder (1 : 1) suspended in distilled water (GTaq) in alleviating both articular and extra-articular manifestations in rat adjuvant-induced arthritis (AIA).

Arthritis was induced by a single intra-dermal injection of 0.1 mL of Complete Freund's adjuvant (containing heat-killed Mycobacterium tuberculosis) into the palmar surface of the left hind paw after the rats were subjected to light diethyl ether anesthesia. Arthritic rats received orally and daily (for 28 consecutive days) distilled water as vehicle, indomethacin (1.0 mg/kg body weight), or GTaq (200 or 400 mg/kg body weight) from the day of arthritis induction.

The present study showed that GTaq (especially the high dose) was more effective (4.2–38.4% higher, P < 0.05–0.001) than indomethacin (a non-steroidal/anti-inflammatory drug) in alleviating the loss in body weight gain, the histopathological changes observed in ankle joints, blood leukocytosis and thrombocytosis, iron deficiency anemia, serum hypoalbuminemia and globulinemia, the impairment of kidney functions, and the risks for cardiovascular disease in arthritic rats. These protective effects of GTaq were mediated through increasing the food intake and decreasing the systemic inflammation that occur at the appearance of polyarthritis, oxidative stress and dyslipidemia.

Ginger-turmeric rhizomes mixture may be effective against RA severity and complications as shown in an AIA rat model.

In our study, we aimed to evaluate left ventricular function in patients with Sjögren syndrome (SS) using tissue Doppler echocardiography (TDE) and myocardial performance index (MPI) in addition to conventional echocardiographic methods.

We evaluated 50 patients with SS and 48 healthy volunteers with similar demographic characteristics. Systolic and diastolic functions of the left ventricle were analyzed with standard two-dimensional (2D) echocardiography, M-mode echocardiography, pulsed-wave (PW) Doppler and tissue Doppler imaging.

Septal part of the mitral annulus PW TDE showed that systolic myocardial wave (Sm), early diastolic myocardial wave (Em), late diastolic myocardial wave (Am) and Em/Am ratios are significantly lower, and myocardial isovolumetric relaxation time (IVRTm) and MPI values are significantly higher in patients with SS. Lateral site of the mitral annulus PW TDE showed that Em, Sm and Em/Am ratios are significantly lower, and IVRTm and MPI values are significantly higher in patients with SS compared with healthy controls.

In this study, it was shown that both left ventricle systolic and diastolic functions of patients with SS were disturbed.

To evaluate the diagnostic value of anti-mutated citrullinated vimentin antibodies (anti-MCV), anti-cyclic citrullinated peptides antibodies (anti-CCP), anti-glucose-6-phosphate isomerase antibodies (anti-GPI) and anti-keratin antibodies (AKA) and rheumatoid factor (RF) in rheumatoid arthritis (RA).

The five auto-antibodies were detected in serum samples of 56 patients with RA, 21 patients with systemic lupus erythematosus (SLE), 11 with ankylosing spondylitis (AS), six with Sjögren's syndrome (SS), four with connective tissue disease (CTD) and 20 healthy controls. Anti-MCV, anti-CCP and anti-GPI were detected by enzyme-linked immunosorbent assays (ELISA), AKA was determined by indirect immunofluorescence and RF was determined by rate nephelometry.

In RA, anti-MCV and anti-GPI had the highest sensitivity (78.6% and 75.0%, respectively), anti-CCP and AKA had the highest specificity (97.6%). Anti-GPI had the lowest specificity (64.3%), and AKA had the lowest sensitivity (48.2%). When two antibodies were detected together, the sensitivity of anti-MCV/anti-CCP/RF were highest (92.9%) with a lower specificity (73.8%). The combination of anti-MCV/anti-CCP had a slightly decreased sensitivity (89.3%) and the same specificity (73.8%).

The combination RF/anti-MCV/anti-CCP or anti-MCV/anti-CCP are usefully serologic tests for the diagnosis of RA in Chinese patients.

To identify risk factors for symptomatic knee osteoarthritis (OA) and explain the geographical disparities in its occurrence.

A population-based case control study used data from a national Community Oriented Program for the Control of Rheumatic Diseases (COPCORD) study conducted in Lebanon in 2009. The sample included 59 incident cases of symptomatic knee OA with no past knee injury, knee pain for a period of < 12 months, and were examined by rheumatologists. One hundred and eighteen randomly sampled population-based controls were frequency matched with cases by age and gender.

Obesity, overweight and area of residence were significant risk factors for knee OA, after adjusting for type of job, monthly income and family history of joint problems.

Determinants of symptomatic knee OA in Lebanon may differ by geographical location, potentially reflecting differences in social conditions, biological elements and environmental factors. The geographical differences remained significant even after accounting for investigated factors. Thus, further research is needed to explore other potential determinants, such as living conditions, biomechanical and hormonal factors.

Knee osteoarthritis (OA), is the most common degenerative joint disease. Several non-pharmacological interventions have been used for this purpose such as insoles. There are contradictory data about the superiority and effectiveness of laterally wedged compared with neutrally wedged insoles. This study was designed to compare the effectiveness of laterally and neutrally wedged insoles in management of knee OA.

In this double-blind, parallel treatment trial, 118 patients with knee OA according to American College of Rheumatology (ACR) criteria were enrolled and were followed for 2 months. Patients were randomly divided into two groups. Fifty-seven of them were treated with 5° laterally elevated wedged insoles (group A) and 61 patients were treated with neutrally wedged insoles (group B). Edinburg Knee Functional Scale (EKFS) was used to evaluate knee function before and after interventions. At the end of 2 months, severity of knee pain during the previous 2 days, numbers of non-steroid anti inflammatory drugs (NSAIDs) used for pain relief within the last 2 weeks and EKFS were assessed.

Severity of knee pain decreased in both groups after intervention. The mean difference in groups A (laterally wedged insole) and B (neutrally wedged insole) were 29.3 (95% confidence interval [95% CI]: 25.12, 33.55) and 6.25 (95% CI: 3.09, 9.4), respectively (P < 0.001 for both). In addition, at the end of the study, EKFS improved significantly in group A (mean: 7.54, 95% CI: 6.3, 8.8; P < 0.001), while in group B we could not find significant improvement (mean: 0.54, 95% CI: −0.41, 1.5; P = 0.166). Numbers of NSAIDs used during the two final weeks of the study significantly decreased compared with baseline in group A (P = 0.001; mean: 2.6, 95% CI: 1.3, 3.9); while in group B this was not shown (P = 0.9; mean: 0.05, 95% CI:−0.87, 0.97).

This study suggests that laterally elevated wedged insoles are more effective than neutrally wedged insoles, in pain relief of knee OA.

Despite several attempts made during the last decade, the exact pathogenesis of exceedingly high thrombotic events and bad obstetric outcome in antiphospholipid syndrome (APS) remains elusive. Anti-annexin A5 (aANX IgG) is thought to have a role in pathophysiology of APS. We studied role of aANX IgG in the pathogenesis of hypercoagulable state in APS patients.

We estimated levels of aANX IgG in 112 patients with APS (86 primary and 26 secondary). We also estimated aANX IgG levels in 40 age- and sex-matched healthy controls, 10 patients with systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) each, without any history of thrombosis or pregnancy morbidity, 10 patients of non-APS thrombosis and 10 patients of pregnancy loss without APS.

Only three healthy controls, two SLE (P = 0.239), one RA patient (P = 0.794), three non-APS thrombosis patients (P = 0.086) and two patients with pregnancy loss without APS (P = 0.258) had marginally elevated values, whereas 53 primary APS (P < 0.001) and 16 secondary APS (P < 0.001) were positive. We also compared aANX IgG levels in different groups. Mean ± standard errors of the mean of healthy controls was 3.77 ± 0.49, in SLE patients it was 4.88 ± 1.17 (P = 1.000), in RA patients it was 4.67 ± 0.97 (P = 1.000), in non-APS thrombosis it was 7.93 ± 0.88 (P = 0.488) and in pregnancy loss without APS it was 6.80 ± 0.93 (P = 0.789). However, it was significantly elevated in primary APS (12.87 ± 1.07, P < 0.001), secondary APS (11.98 ± 1.41, P = 0.001) and total APS patients (12.68 ± 0.88, P < 0.001).

From the above observations it appears that aANX IgG plays a significant role in producing a hypercoagulable state in primary and secondary APS.

This study was designed to evaluate iron deficiency as a predisposing factor for resistant oral aphthosis in patients with Behcet's disease (BD).

In a case control study 220 consecutive BD patients with oral aphthosis were enrolled. All patients had been treated for at least 3 months. They were divided into two groups according to their treatment response (75 patients in the Case and 145 in the Control group). Demographic and clinical characteristics of the disease, serum iron, total iron binding capacity and serum ferritin were determined in each patient. We used independent t-test and Mann–Whitney U-test to compare the quantitative variables and chi-square test for qualitative variables. Odds ratio (OR) and confidence interval at 95% (95% CI) were calculated for each item.

There was no significant difference between the two groups in demographics or clinical characteristics of the disease. We found iron deficiency in 72 patients (32.7%, 95% CI: 6.2), higher in the Case group than Control (39.2% vs. 30.1%; P = 0.17). Despite the higher frequency of iron deficiency in men (26.8% vs. 14.5%), the difference was not statistically significant (P = 0.09). Multivariate logistic regression analysis showed that none of the iron deficiency or sex variables could predict the development of resistant oral aphthosis. The OR for iron deficiency was 1.52 (95% CI: 0.81–2.86) and for male sex was 1.04 (95% CI: 0.56–1.91).

Despite the higher frequency of iron deficiency in BD patients with resistant oral aphthosis, we were not able to attribute this resistance to this deficiency.

Rheumatoid arthritis (RA) is associated with increased cardiovascular morbidity and mortality. In the general population, an increased heart rate is associated with increased mortality. Only a few studies have investigated heart rate in RA patients and compared the results with patients that do not have RA (n-RA). Therefore, little is known as to whether an increased heart rate, at least in part, could explain the increased mortality found in RA patients. The aim of the present study was to investigate whether heart rate is increased in RA patients.

In this cross-sectional study, heart rate was determined in a total of 282 patients (131 RA, 151 n-RA). In addition, non-invasive pulse wave analysis of the radial artery was performed to determine cardiac ejection duration using the Sphygmocor apparatus. Furthermore, the subendocardial viability ratio (SEVR), a marker of cardiac workload, was investigated, whereby higher values indicate a more favorable supply/demand relationship for the myocardium. Patients using chronotropic drugs were not included in the study.

Heart rate was virtually the same in RA patients (71.9 ± 11.2 beats/min [bpm]) as compared with controls (72.3 ± 11.7 bpm; P > 0.05). Also SEVR (RA 144 ± 25% vs. n-RA 147 ± 27%; P > 0.05) and ejection duration (RA 321 ± 24 ms vs. n-RA 318 ± 24 ms; P > 0.05) were comparable between the groups.

It could not be shown that heart rate in RA patients differs significantly from heart rate in controls. Therefore, heart rate does not appear to explain or contribute to the increased cardiovascular risk found in RA patients.

The issue whether patients with rheumatoid arthritis (RA) have certain personality characteristics has been discussed. The temperament and personality characteristics of the patient may influence success in competing with stress. The aims of the study were to determine the most common dominant affective temperaments in patients with RA and whether temperament affects the disability.

A total of 88 patients with RA participated in this cross-sectional study. The Turkish version of the Temperament Evaluation of Memphis, Pisa, Paris and San Diego Auto Questionnaire scale was used to determine the dominant affective temperament, and the Health Assessment Questionnaire (HAQ) was used to determine functional ability.

Depressive temperament was found in 52 patients (59.1%) as the most common dominant affective temperament followed by irritable temperament in 17 patients (19.3%). There was no significant difference in HAQ scores of the patients according to the subscales of the Temperament Evaluation of Memphis, Pisa, Paris and San Diego Auto Questionnaire. Disability levels of patients were found as low-level disability in 65 patients and high-level disability in 23 patients. Multiple linear regression analysis indicated that HAQ score was not associated with gender, body mass index, duration of the disease, depressive temperament, anxious temperament, cyclothymic temperament or irritable temperament.

The current study showed that depressive and irritable temperaments are the most common affective temperaments in patients with RA. The dominant affective temperament does not affect the functional ability of patients with RA.

The first objective was to investigate the behavioural activity in the systems of Gray's theory; these are the Behavioural Inhibition System (BIS) and Behavioural Approach System (BAS), in fibromyalgia (FM) patients. The second aim was to assess in FM patients whether there is an association between BIS or BAS with self-reported somatic symptoms. Twenty FM patients and 20 healthy controls completed questionnaire measures of BIS and BAS activity (Sensitivity to Punishment and Sensitivity to Reward Questionnaire), self-reported somatic symptoms (Somatic Symptoms Scale Revised), positive and negative affect (Positive and Negative Affect Schedule) and health status (EuroQoL Visual Analogue Scale). The results showed that FM patients had lower Sensitivity to Reward (SR) scores than controls. The SR score correlated with different somatic symptoms groups. The partial correlation (controlling for other variables measured) showed that the SR score correlated specifically with musculoskeletal symptoms. Furthermore, in regression analysis, SR score significantly predicted musculoskeletal symptoms, after controlling for other variables measured in this study. Our findings suggest that FM patients show BAS hypoactivity. This BAS activity in FM is similar to patients with depression, where a lower BAS functioning has also been found. The BAS activity predicts the musculoskeletal self-reported symptoms in FM better than other measures included in this study. Although this is a preliminary study, it suggests the importance of BAS activity in FM.

To translate and adapt the Health Assessment Questionnaire Disability Index (HAQ-DI) into Bengali (B-HAQ) for use in Bangladeshi populations and to test its reliability and validity in patients with rheumatoid arthritis (RA).

The HAQ-DI was translated using rigorous forward-backward protocols and the translated version was subsequently cognitively pretested in a sample of 30 outpatients with RA. The pre-final version of the questionnaire was psychometrically tested for internal consistency and construct validity in a new sample of 100 consecutive RA outpatients.

Ten questions were changed to suit the Bengali culture. Pretests showed that the items included in the B-HAQ were well understood by Bengali patients, while some of the original items were difficult to understand for a majority of patients. The resulting B-HAQ showed good internal consistency and construct validity in the psychometric validation study.

This study suggests that the B-HAQ is a reliable and valid instrument for measuring functional disability in a Bengali-speaking population with RA. Future studies should examine the test–retest reliability and responsiveness of the B-HAQ before it can be confidently recommended as an outcome measure in intervention studies.

The aim of our study was to: (i) map out the presence of peripheral vascular disease in a sample of systemic lupus erythematosus (SLE) patients; and (ii) correlate our findings with disease characteristics, activity indices, traditional risk factors of atherosclerosis and thrombotic variables.

The study population comprised 120 SLE patients and 100 controls. Clinical data were collected for patients and controls with stress on clinical issues of SLE patients, including British Isles Lupus Assessment Group index score, anti-double stranded DNA titer C3 and C4 levels, and treatment taken, mainly steroids. Measurements of thrombotic variables were performed. Non-invasive arterial assessment was performed, including carotid duplex scanning and measurement of carotid artery intima-media thickness (IMT) and peripheral arterial assessment and measurement of ankle–brachial pressure index (ABPI).

The mean age of SLE patients was 32 years and mean disease duration was 8 years. There were no statistically significant differences in the traditional vascular risk factors measured between SLE patients and controls. There were significantly higher plasma levels of thrombotic variables in SLE patients. The average IMT was statistically significantly greater in SLE patients compared to controls. Thirty SLE patients (25%) had an ABPI < 1.0 compared with six controls (6%), which was statistically significant.

This study showed an increased prevalence of peripheral arterial disease in SLE patients as shown by the higher carotid artery IMT and lower ABPI in such patients compared with controls. Multiple risk factors are likely to be involved in such findings.

Recent studies report that cardiovascular mortality is more common in patients with spondyloarthropathy (SpA) compared with the normal population. In this study, we aimed to determine left ventricular systolic and diastolic functions using tissue Doppler echocardiography (TDE) in addition to conventional methods in undifferentiated SpA (uSpA) patients.

A total of 45 patients and 44 age and sex matched healthy controls participated in the present study. Left ventricular systolic and diastolic functions were assessed with two dimensional (2D) echocardiography, M-mode echocardiography, pulsed-wave (PW) echocardiography and tissue Doppler echocardiography. The peak systolic velocity (Sm), early diastolic myocardial peak velocity (Em), and late diastolic myocardial peak velocity (Am), myocardial isovolumetric contraction time (IVCTm), myocardial ejection time (ETm), myocardial isovolumetric relaxation time (IVRTm) and myocardial performance index (MPI) were measured at septal and lateral mitral annulus.

Left ventricular diastolic inflow velocities showed that isovolumetric relaxation time (IVRT) and deceleration time (DT) were significantly longer in the uSpA group. Left ventricular lateral wall PW tissue Doppler echocardiography showed that Em was significantly lower in uSpA group. Septal PW tissue Doppler echocardiography showed that Em was lower and IVRT was longer in the uSpA group compared with healthy controls.

In this study we determined that left ventricular systolic function is preserved in patients with uSpA. Although frequency of diastolic dysfunction was similar in both groups, deterioration of some diastolic parameters in the uSpA group might be considered for possible cardiac involvement in patients with uSpA.

Joint hypermobility when associated with symptoms in the absence of systemic rheumatologic disease is termed as benign joint hypermobility syndrome (BJHS). BJHS is often an under-recognised and a poorly managed entity. Indian studies on BJHS are very few and none have been carried out in any of the service rheumatology centres. Hence this retrospective study was carried out at a tertiary medical institute of the Indian Army to assess the varied clinical profile of BJHS.

All patients consecutively diagnosed as BJHS at the rheumatology clinic of the Army Hospital (Research and Referral) Delhi from May 2010 to May 2011 were included in the study. Their age, sex, presenting features, clinical profile, laboratory and radiological parameters were studied.

The mean age of these patients was 30 ± 5.71 years with a median duration of symptoms of 42 (06–120) months. There were 45 males and 39 females (male : female = 1.15 : 1.00). The median Beighton's score in these patients was 6/9 (range 4–9). Most of our patients were military personnel (43/84), and all had knee joint pain with evidence of degenerative changes in 19 and synovitis in two patients. Eleven patients including nine military personnel had evidence of soft tissue rheumatism with associated fibromyalgia in four and anxiety disorder in one. Out of 18 patients with a Beighton's score of ≥ 7, nine had incidental findings of lateral head tilt on frontal observation. There was evidence of carpal tunnel syndrome in a patient with wrist synovitis and one patient had associated skin laxity without features of Ehlers–Danlos syndrome.

BJHS is often under-recognized in clinical practice and is usually missed because of a lack of awareness. A high index of clinical suspicion to diagnose this entity is essential due to its associated morbidities, especially among those exposed to strenuous physical activities.

To investigate the short- to medium-term effectiveness of ultrasound (US)-guided steroid injections for shoulder pain in patients who previously failed to respond to unguided steroid injections.

We examined 60 consecutive patients who had undergone US examination and US-guided steroid injection. Patients were categorised into having had a good response (i.e., good pain relief at time of follow-up), some response (improvement for 2–4 weeks) or no response, as documented by their usual rheumatologist in their subsequent routine out-patient review appointments.

Average age was 64.4 ± 11.5 years and 42 were female. Median interval between US-guided injection and follow-up was four (interquartile range 2–5) months. Thirty-four (56.6%) patients reported a good response, 13 (21.7%) some response and another 13 (21.7%) no response.

US guidance of steroid injections may achieve good short- to medium-term benefit in the majority of patients with chronic shoulder pain due to a variety of clinical syndromes.

This study aimed to clarify whether different anti-tumor necrosis factor (TNF) drugs can improve endothelial function better than conventional disease-modifying anti-rheumatic drugs (DMARDs) in a series of Japanese patients with rheumatoid arthritis (RA).

Twenty-five patients who met the American College of Rheumatology 1987 revised diagnostic criteria for RA were randomly selected for this study. The percentage of brachial flow-mediated dilation (%FMD) and maximum carotid intima-media thickness were examined by ultrasonography.

The %FMD in the group treated with anti-TNF therapy was significantly higher than that in the group treated with DMARDs (P < 0.001). The %FMD was significantly correlated with anti-TNF therapy (r = 0.684, P < 0.001) and Disease Activity Score C-reactive protein (r = –0.404, P < 0.05). Multiple regression analysis revealed that anti-TNF therapy was significantly associated with %FMD (β = 0.684, P < 0.001).

Anti-TNF therapy may influence endothelial function more than conventional DMARD therapy. Prospective longitudinal studies examining whether anti-TNF therapy was able to improve endothelial function are required.

We decided to determine the effectiveness of oral bromocriptine in patients with active rheumatoid arthritis (RA) who are in methotrexate (MTX) therapy.

Patients receiving stable doses of MTX were randomized to one of two groups and received 3 months of double-blind bromocriptine (5 mg/day) or matching placebo. The moderate and major outcome measures were the proportion of patients with > 0.6 and > 1.2 improvement in RA based on the Disease Activity Score 28 (DAS28) at 3 months. Safety measures included adverse events and laboratory assessments.

On a background treatment of MTX, the percentage of patients with moderate and major DAS28 responses at 3 months in the bromocriptine group (73.8%/59.5%) was not significantly different from placebo (63.1%/31.6%). Side effects were typically mild and included mild nausea and sleep disturbance; we did not have any adverse events resulting in discontinuation of the study drug.

In patients with active RA receiving stable doses of MTX, bromocriptine showed non-significant improvement in efficiency outcomes compared to placebo.

Systemic sclerosis (SSc) is a rare systemic connective tissue disease characterized by abnormal fibroblast proliferation and micro-vascular inflammatory changes.

To assess serum B-cell activating factor (BAFF) levels in patients with systemic sclerosis and to correlate this with disease features and disease severity.

This is a case-control study in which patients with the established diagnosis of SSc were recruited. The diagnosis of SSc was established according to the American Rheumatology Association 1980 criteria for the classification of scleroderma. Patients' assessment included evaluation of skin involvement using the Modified Rodnan score and disease severity using the Medsger score. Twenty-five healthy matching controls were included. The sandwich enzyme-linked immunosorbent assay technique was used for direct assessment of serum BAFF in patients and controls.

The study included 60 patients (54 female and 6 male), with a mean age of 38.18 ± 12.06 years, with mean disease duration of 7.85 ± 4.075 years. Serum BAFF in patients ranged 98.2–5015 pg/mL with mean BAFF 1100 ± 835.4 pg/mL. In controls serum BAFF levels ranged 188.5–2314 pg/mL with mean BAFF 546.1 ± 471.1 pg/mL, showing a statistically significant elevation of serum BAFF levels in SSc patients (P = 0.0001) with insignificant correlation to skin disease or total Medsgar Score of the study population (P > 0.05). Serum BAFF levels showed significant correlation with episodes of pseudo-obstruction and methotrexate (MTX) use in the patients studied (P < 0.05).

Serum BAFF levels were significantly elevated in patients with SSc irrespective of disease subtype, disease duration or age of patients. This elevation in serum BAFF significantly related to gastrointestinal track involvement and MTX therapy.

To describe the spectrum of diseases seen in an outpatient setting in the Singapore General Hospital, the largest tertiary referral centre in Singapore.

In this cross-sectional study, medical records of patients scheduled for an appointment at the rheumatology specialist outpatient clinics over a 4-month period (10 August 2010–31 December 2010) were reviewed. Primary diagnoses documented by the attending physician at the latest visit were recorded.

Among 4180 patients (29.5% male; mean [SD] age: 53.5 [15.1] years; 77.0% Chinese, 8.0% Malay, 8.8% Indian and 6.2% others), the spectrum of diseases seen was as follows [disease – definite n (%), probable n (%)]: Arthritis: rheumatoid arthritis – 958 (22.9%), 68 (1.6%); osteoarthritis – 452 (10.8%), 39 (0.9%); crystal arthritis – 417 (10.0%), 18 (0.4%); spondyloarthritis – 227 (5.4%), 61 (1.5%); psoriatic arthritis – 158 (3.8%), 9 (0.2%); other inflammatory arthritis – 153 (3.7%), 94 (2.2%); Connective tissues diseases: systemic lupus erythematosus – 412 (9.9%), 26 (0.6%); vasculitis – 105 (2.5%), 22 (0.5%); Sjögren's syndrome – 81 (1.9%), 32 (0.8%); overlap syndromes – 73 (1.8%); scleroderma – 50 (1.2%), 4 (0.1%); undifferentiated connective tissue diseases – 45 (1.1%), 106 (2.5%); myositis – 41 (1.0%), 12 (0.3%); antiphospholipid syndrome – 22 (0.5%), 7 (0.2%); polymyalgia rheumatica – 16 (0.4%); Others: soft tissue rheumatism – 155 (3.7%); osteoporosis – 61 (1.5%); other non-rheumatologic conditions – 189 (4.5%); other rheumatologic conditions – 67 (1.6%).

Rheumatoid arthritis, osteoarthritis and crystal arthritis were the three most common rheumatological diseases seen in a tertiary referral centre serving a multi-ethnic urban Asian population in Singapore.

To assess effects of ankylosing spondylitis (AS) on working life and physical activity in Australia; to quantify changes in working life and physical activity that occur after anti-tumor necrosis factor-alpha (TNF-α) treatment; and to assess efficacy of anti-TNF-α therapy for AS in an Australian context.

This is a multi-centre observational study of people with AS on anti-TNF-α therapy. All participants satisfied the New York Modified Criteria and had active and refractory disease at anti-TNF-α therapy commencement. Participation involved a standardized interview, a metrology assessment, assessment of disease remission and medical record review. Interviews and patients' records were used to compare working life (employment, sick leave and productivity) and physical activity (participation rate, hours/week, and physical intensity) between the pre-AS, post-AS and post-anti-TNF-α therapy periods.

Fifty-two patients took part. Participants were on average 44.8 years old, predominately male (86.5%) and had been on anti-TNF-α therapy for 29 months; 39% were in partial remission and 75% had 50% reduction in the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI). Responders to anti-TNF-α therapy were 10.5 years younger than non-responders (P = 0.004). Post-anti-TNF-α therapy participants gained 6.6 h/week of work (P = 0.02), and productivity improved 31% (P < 0.001) compared to immediately prior to commencing treatment. Physical activity participation increased from 71% to 85% (P = 0.039) and activity intensity increased by 33% (P = 0.002) post-treatment. Participants gained 1.8 h/week of sport (P = 0.001) and 2.2 h/week of recreational physical activity (P < 0.001).

Australians with AS have their working life and physical activity severely affected by this disease. Treatment with anti-TNF-α therapy results in significant improvement in these parameters.

To establish an improved substrate for an indirect immunofluorescence test (IIF) to detect anti-Sm antibody.

Full-length Smith protein D1(Sm-D1) complementary DNA was obtained from human larynx carcinoma cell line HEp-2 by reverse transcription – polymerase chain reaction (RT-PCR) and cloned into the mammalian expression vector pEGFP-C1. The recombinant plasmid pEGFP-Sm-D1 was transfected into HEp-2 cells. The expression, localization and antigenicity of fusion proteins of green fluorescent protein (GFP) in transfected cells were confirmed by means of immunoblotting (IBT), confocal fluorescence microscopy and IIF analysis. Transfected HEp-2 cells were analyzed with reference serum and compared with untransfected HEp-2 cells by IIF.

Stable expression of the Sm-D1-GFP was maintained for more than ten generations. This Sm-D1-GFP showed the antigenicity of Sm-D1 with a characteristic phenotype in IIF.Six of 12 serum specimens from systemic lupus erythematosus contained both 29/28 and 13.5 kDa proteins and showed characteristic immunofluorescent patterns. The same phenomenon appeared in 3/6 serum samples which contained 29/28 kDa proteins only. Sera from 10 healthy donors did not react with HEp-Sm-D1 or HEp-2 at 1:80 attenuant degrees. No alteration in expression, localization and morphology was observed when HEp-Sm-D1 or HEp-2 interacted with the reference sera which could react with Ro/SSA, La/SSB, β2GP1, centromere, histone, and Scl-70 antibodies in routine IIF tests.

As a new kind of substrate of IIF, HEp-Sm-D1 can be used to detect anti-Sm antibodies. Transfected HEp-2 cells keep the immunofluorescent property of HEp-2 cells in immunofluorescence anti-nuclear antibody (IFANA) test and could potentially be used as substrate for routine IFANA detection.

Measurements of salivary calcium level may be a useful screening tool for osteoporosis in postmenopausal women. The purpose of this study was to clarify whether this measure is valid compared with dual-energy X-ray (Bone Mineral Density) screening tools in osteoporosis.

A case-control study was carried out in 40 postmenopausal women with osteoporosis (T-score ≤ −2.5) and 40 women without osteoporosis (T-score > −1 bone mineral density). Salivary samples were collected and calcium concentrations were measured and expressed as mg/dL. Receiver operating characteristic curve analyses was used to determine the optimal cut-off thresholds for salivary calcium in healthy postmenopausal women.

The cut-off point for salivary calcium was 6.1 mg/dL. The sensitivity and specificity, respectively, for identifying women with osteoporosis, were 67.5 (95%CI 52.33–82.67) and 60% (95%CI 44.62–75.38). The area under curve (AUC) was 0.678 (95%CI 0.56–0.79), the positive predictive value (PPV) was 62.79 (95%CI 47.74–77.84) and negative predictive value (NPV) was 64.86% (95%CI 49.27–80.46). The positive likelihood ratio was 1.688 and the negative likelihood ratio was 0.542.

Salivary calcium concentration discriminates between women with and without osteoporosis and constitutes a useful tool for screening for osteoporosis.

The aim of this study was to investigate depression and sexual dysfunction in female patients with mucocutaneous Behçet's disease (BD).

Twenty-five consecutive, sexually active premenopausal female patients with mucocutaneous BD (mean age: 34.76 ± 4.61 SD years) followed at a rheumatology outpatient clinic were enrolled into the study. The control group consisted of 27 age-matched (mean age: 37.0 ± 4.6 SD years), sexually active, healthy volunteers. The Female Sexual Function Index (FSFI) and Beck Depression Inventory (BDI) were used for sexual and psychiatric assessment.

Depression was found in four of 27 (14.8%) in the control group and eight of 25 (32%) in the BD group (P = 0.01). The median total FSFI score for patients with BD was 21.85 (interquartile range [IQR]: 18.25–27.9) and for healthy controls, 27 (IQR 21.5–29.3; P = 0.03). Female sexual dysfunction was diagnosed in 14 of 25 (56%) in the BD group and in 11 of 27 (41%) in the control group (P = 0.27). The pain domain was significantly higher in the BD group at 5.6 (4.4–6.0) than in the control group at 4.4 (3.2–5.5; P = 0.03). None of the other domains (desire, arousal, lubrication, orgasm and satisfaction) of the BD and control groups were different. There were no statistically significant differences between the FSFI, BDI scores and presence of genital ulceration in the BD patients.

Depression and FSD were more common in the patients with BD than in the healthy subjects. This might have been a result of the depressive effect of chronic disease as well as BD and low androgen levels.

To investigate the rheumatic complications of inflammatory bowel disease (IBD) Arab patients in relation to the clinical manifestations of IBD using the Montréal classification system in a hospital-based population in Kuwait.

A cohort of 130 consecutive patients with IBD, either ulcerative colitis (UC) or Crohn's disease (CD) attending gastroenterology and rheumatology clinics of Kuwait University hospital from January to December 2010 were recruited. IBD diagnosis, classification, and the rheumatologic characteristics of patients were assessed and noted on a pro forma.

In the 130 IBD patients (mean age 32.6 ± 12.3 years), 45 (34.6%) had UC and 85 (65.4%) had CD. Forty-five (34.6%) IBD patients developed rheumatic manifestations; the difference in proportion was not significant among UC and CD patients (18 [40.0%] vs. 27 [31.7%], P = 0.215). Peripheral arthritis was seen in 41 (31.5%) IBD patients. Axial skeletal involvement presenting as a combination of spondyloarthritis with sacroiliitis was seen in 11 (8.5%) out of 130 IBD patients. Isolated sacroiliitis was seen in four (3.1%) IBD patients. Enthesopathy was seen in seven (5.4%) and dactylitis in two (1.5%) IBD patients. No statistically significant difference (P > 0.05) was detected between the frequency of the rheumatic manifestations and the IBD clinical subtypes.

This study delineates the rheumatic complications in relation to clinical manifestations (phenotypes) of IBD using the Montréal classification, in a hospital-based cohort of an Arab population. The rheumatic manifestations of IBD in our study were comparable to previously published data from other parts of the world.

This case control study was designed to determine the patterns of angiotensin converting enzyme insertion/deletion (ACE I/D) gene polymorphism in rheumatoid arthritis (RA) patients and healthy controls.

The study population was divided into two groups: the study group included 66 RA patients diagnosed according to the American College of Rheumatology (ACR) classification criteria for RA, and the control group included 66 healthy adults who were age-and sex-matched to the RA group. All RA patients were assessed by Disease Activity Score (DAS28), ACR Classification of Global Functional Disability Status and Sharp's score as outcome measures. Gene investigations for ACE I/D polymorphism were performed by polymerase chain reaction (PCR) in both groups.

The ACE I/D polymorphism was the (D/D) genotype in 60.6% (n = 40) of RA patients, the (I/D) genotype in 31.8% (n = 21) and the (I/I) genotype in 7.6% (n = 5). The frequency of (D) carriage was significantly higher in the RA cases than in the control group (76.5% vs. 53.8%, respectively, P = 0.0002). ACE D allele carriers were at higher risk of RA, 2.8 times higher than (I) carriers and those who had the homozygote (DD) genotype had 5.6 times the possibility of having RA. No correlations were observed between the homozygote (DD) genotype and disease activity or severity in RA patients.

Our study suggests that high frequency of the ACE D allele contributes to the heritability of RA susceptibility compared to other ACE alleles. On the other hand, no association was detected between ACE I/D polymorphism and the severity of RA.

Leprosy classically presents with cutaneous and neural involvement. Rheumatological manifestations are frequent, although often under-recognized. At times, these may present to a rheumatology clinic prior to the diagnosis of leprosy. Herein, we present our experience with patients referred with various rheumatological disorders who were subsequently diagnosed as having leprosy.

This retrospective study (January 2001–September 2010) was carried out at the Department of Clinical Immunology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, in northern India. Patients who were confirmed as having leprosy were included. Details regarding demographic and clinical presentations were collected.

Forty-four cases (30 male, mean age 40 ± 13.6 years and mean disease duration 18.7 ± 24.3 months) were identified. Musculoskeletal manifestations included arthritis (n = 22), swollen hands and feet syndrome (SHFS) (n = 11), tenosynovitis (n = 9), painful swollen feet (n = 9), arthralgias (n = 7) and vasculitis (n = 1). Distribution of joints mimicked rheumatoid arthritis (n = 14) and spondyloarthropathy (n = 7). Arthritis and/or tenosynovitis were part of spontaneous onset lepra reaction in 28 cases. Other clinical manifestations were: paresthesias (n = 28), erythematous nodules (n = 25) and anesthetic patches (n = 7). Thirty-one patients had thickened nerves (ulnar n = 28, common peroneal n = 21). Eight patients did not have any cutaneous manifestations and had presented with SHFS and arthritis or tenosynovitis. These were labeled as pure neuritic leprosy. Most of the patients responded to multidrug anti-leprosy therapy and glucocorticoids.

Rheumatological presentations of leprosy may mimic RA, spondyloarthropathy or vasculitis. Pure neuritic variety and spontaneous type 2 lepra reaction pose unique diagnostic challenges. Increased awareness may avoid delay in diagnosis.

Foot involvement is not uncommon and occurs early in the disease course of rheumatoid arthritis (RA). Inflammation and ongoing synovitis of foot joints lead to joint destruction and instability, tendon dysfunction, and eventually collapse of the medial longitudinal arch and pes planovalgus that contributes to difficulty in walking and gait abnormalities. This article reviews foot-related problems in patients with RA, focusing on the prevalence, natural history and role of imaging in both diagnosis and management of midfoot and subtalar joint disease in RA.

Cardiovascular disease is a substantial contributor to increased morbidity and mortality in rheumatoid arthritis (RA). The aim of this audit was to determine the rate of cardiovascular events in a cohort of newly diagnosed RA patients.

The inpatient clinical database from Christchurch Hospital, Christchurch, New Zealand, was searched using the International Classification of Diseases 9th Revision (ICD9) and 10 codes representing RA and cardiovascular disease between 1 January 1999 and 31 December 2008. Notes were reviewed with additional demographic and medication data sought. Outpatient data for RA patients was collated from the Rheumatology Department's letter database.

Four hundred and six patients were identified with combined ICD9 or 10 codes for RA and ischemic heart disease, of whom 194 had a confirmed myocardial event. Of these, 34 were diagnosed with RA between January 1999 and December 2008 prior to their myocardial event. Kaplan–Meier analysis showed risk of a cardiovascular event at 1 and 10 years was 0.64% and 9.4%, respectively. There were 26 confirmed deaths in the study period. The risk of death at 1 and 10 years was 0.48% and 8.16%, respectively.

We have shown a relatively low prevalence of cardiovascular events in this RA population diagnosed within a 10 year period. This is consistent with other reports and likely reflects the short follow-up period. Prospective longer-term studies will be required to further investigate the relative contribution of disease activity and other parameters to cardiovascular events in patients with early RA.

Cyclo-oxygenase (COX)-2 inhibitors have been the target of severe criticism, more so following the withdrawal of Rofecoxib. Post-marketing surveillance of Celecoxib in Asian Indians, who are predisposed to premature athero-thrombotic events, has not been studied.

To study the adverse effects of Celecoxib and compare them with those of other non-steroidal anti-inflammatory drugs (NSAIDs) in an Asian Indian cohort.

This is a retrospective chart review with convenience sampling of patients on NSAIDs (at least five tablets a week, for at least 3 months prior to the study), attending the Rheumatology clinic of a tertiary care institution in south India between June 2004 and November 2004. Those with pre-existing heart disease, hypertension, thrombo-embolic disease, peptic ulcer and patients on corticosteroids were excluded. All the recorded adverse events were noted and compared between the Celecoxib and non-selective NSAID users. Univariate analysis using Chi-square test was performed.

Of the 1387 patients included, 915 were on Celecoxib. In the NSAID group, 204 had used multiple NSAIDs in sequence. Of the Celecoxib users, 164 had switched over to an NSAID during the study period. New onset of hypertension was significantly higher in the Celecoxib users as compared to non-selective NSAID users (3.06% vs. 1.27%, P = 0.04). However, those who had switched over to NSAIDs did not show this trend. NSAID users, on the other hand, had significant gastrointestinal (GI) toxicity (2.54% vs. 0.327%, P = 0.001). A significant number of Celecoxib users who switched over to NSAIDs also developed GI toxicity (6.1% vs. 1.21%, P = 0.018) over a shorter time span, as compared to the continuous NSAID users. Multiple NSAID users had higher adverse events (6.37% vs. 2.23%, P = 0.023) as compared to single NSAID users.

Celecoxib significantly increased the incidence of new onset hypertension in this cohort of Indian patients with rheumatic diseases. No thromboembolic events were documented.

This Clinical Guidance is aimed to help practitioners assess, diagnose and manage their patients with osteoporosis (OP), using the best available evidence.

A literature search using PubMed (MEDLINE) and The Cochrane Library identified all relevant articles on OP and its assessment, diagnosis and treatment, from 2005, to update from the previous edition published in 2006. The studies were assessed and the level of evidence assigned; for each statement, studies with the highest level of evidence were used to frame the recommendation.

This article summarizes the diagnostic and treatment pathways for OP, highlighting the new data that have changed the way we assess and treat OP. Instead of starting treatment based on bone mineral density alone, there has been a move to assessing 10-year fracture risk before treatment, using tools such as the Fracture Risk Assessment Tool (FRAX). There has been a re-evaluation on calcium supplementation and more emphasis on the importance of vitamin D. There has been concern about the potential adverse effects of the long-term usage of bisphosphonates, which we have discussed fully. New drugs that have been licensed since 2006 in Malaysia have been included.

Adequate intake of calcium (1000 mg from both diet and supplements) and vitamin D (800 IU) daily remain important in the treatment of OP. However, in confirmed OP, pharmacological therapy with anti-resorptives is the mainstay of treatment. Patients need to be regularly assessed while on medication and treatment adjusted as required.

Reports of hospitalized systemic connective tissue disorders (SCNTD) are mostly disease-specific reports from institutional databases.

To clarify the admission rate, disease determination, hospital mortality rate, length of stay and hospital charges among hospitalized patients diagnosed with SCNTD.

The data were extracted from the 2010 national database of hospitalized patients provided by the Thai Health Coding Center, Bureau of Policy and Strategy, Ministry of Public Health, Thailand. Patients over 18 years having International Classification of Diseases (ICD)-10 codes for a primary diagnosis related to SCNTD were included.

There were 6861 admissions coded as disorders related to SCNTD during the fiscal year 2010. The admission rate was 141 per 100 000 admissions. Among these, systemic lupus erythematosus (SLE) was the most common, followed by systemic sclerosis (SSc) and dermatomyositis/polymyositis (DM-PM). The overall mean length of hospital stay was 6.8 days. Small vessel vasculitis and Sjögren syndrome had the longest and the shortest hospital stays respectively (14.5 vs. 5.3 days). Hospital charges were highest among systemic vasculitis and DM-PM patients.

The admission rate for SCNTD in Thailand was 141 per 100 000 admissions among which SLE was the most common. Overall hospital mortality was 4.1%. Although a lower prevalence was found among systemic vasculitis, it had a higher mortality rate, longer length of stay and greater therapeutic cost.

To determine the prevalence and to identify the risk factors of chloroquine maculopathy (CM), and to evaluate the association of plasma chloroquine (CQ) and desethylchloroquine (DCQ) levels and CM.

Rheumatoid arthritis (RA) patients who had taken CQ for at least 6 months and stable CQ dosage for at least 2 months were included. CM was diagnosed by dilated ocular examination and automated visual field. Plasma CQ and DCQ levels were determined by liquid chromatography tandem mass spectrometry method. Logistic regression was used to explore risk factors associated with CM.

One hundred and ninety-three patients were included with median CQ duration (range) of 50.2 months (6.0–269.8) and cumulative dose of 137.4 g (16.4–1226.5). The prevalence of CM was 13.5%. Factors associated with CM identified from univariate analysis were age > 60 years, and creatinine clearance with odds ratio (OR) (95%CI) of 5.79 (2.42, 13.84), and 0.98 (0.96, 1.00). In multivariate analysis, older age, usage > 5 years, and current dose from 2.5 mg/kg ideal body weight [IBW]/day were the factors significantly associated with CM with OR of 5.89 (2.38, 14.57), 2.94 (1.10, 7.83), and 3.32 (1.04, 10.60), respectively, while plasma CQ and DCQ showed no association with CM.

The prevalence of CM was 13.5% among RA patients taking CQ for at least 6 months. Age > 60 years, duration of CQ usage > 5 years and current CQ dose ≥2.5 mg/kg IBW/day were the risk factors for CM. The plasma CQ or DCQ levels demonstrated no correlation in developing CM.

To report the long-term outcome of Saudi children with systemic lupus erythematosus (SLE).

Charts of all children with SLE treated between 1990 and 2010 at King Faisal Specialist Hospital and Research Center Riyadh, were reviewed. The long-term outcome measured by pediatric adaptation of the Systemic Lupus International Collaborating Clinics American College of Rheumatology Damage Index (pSDI) and death related to SLE were determined. The data included: gender, age at disease onset, clinical features and treatment at last follow-up visit.

One hundred and fifty-two patients (129 girls and 23 boys) were included. The mean age at onset of SLE was 8.8 ± 2.6 years, while the mean age at diagnosis was 9.5 ± 2.6 years and the mean disease duration was 7.5 ± 4.6 years. All patients were treated with corticosteroid and immunosuppressive drugs. Eighty (52.6%) patients had damage with a mean SDI score of 1.3 ± 1.7. Damage accrual was mostly in the growth (26.8%), renal (17.1%) and neuropsychiatric (15.8%) domains. Due to progressive renal disease, 14 patients required dialysis; five of them underwent renal transplant. There were nine deaths related to SLE, eight of them due to infection. Based on logistic regression, patient disease damage was significantly associated with young age at disease onset and long disease duration. Similarly, death related to SLE was influenced by early-onset disease. In contrast, gender, disease duration and therapy did not affect the suggested outcome measures.

Our results are comparable to reports from other tertiary centers. Early-onset disease probably influences the long-term outcome of SLE in children. Infection remains an important cause of death in children with SLE.

To retrospectively analyze disease activity and damage-associated factors in granulomatosis with polyangiitis (GPA) in Turkey.

A retrospective analysis was carried out in 21 GPA patients. Assessments for activity were performed with the Birmingham Vasculitis Activity Score for GPA (BVAS/GPA) and for permanent organ damage by the Vasculitis Damage Index (VDI).

Lower BVAS/GPA (P = 0.002), absence of renal involvement (P = 0.003) and higher creatinine clearence (P = 0.000) at diagnosis increased the likelihood of achieving remission at 6 weeks. Relapses were associated with high creatinine clearence (P = 0.021), low BVAS/GPA (P = 0.014), absence of renal involvement (P = 0.036) and proteinuria (< 0.5/24 h) (P = 0.013) at diagnosis, whereas achieving remission at 6 weeks (P = 0.012) was associated with absence of co-trimoxazole usage (P = 0.038) and less severe clinical subgroup (P = 0.034). Lower cumulative first 6 months of cyclophosphamide and methylprednisolone were associated with earlier (≤ 12 months) relapses (P = 0.048 and P = 0.083, respectively). Baseline damage (VDI ≥ 1) was associated with a delay in diagnosis (P = 0.032), presentation with milder clinical subgroups (P = 0.052) and low serum creatinine (P = 0.013). The increase in VDI in the first 12 months (early damage) constituted most (91%) of the total damage measured at the end of follow-up.

Despite high early remission rates, relapse represents a major problem in localized GPA in our study. Baseline damage was associated with longer diagnostic delay and lower baseline serum creatinine. The initial phase of the disease seems to be the most crucial period for mortality and accumulated damage.

Phosphatidylserine-rich microparticles derived from endothelial cells, platelets and leukocytes have been implicated as surrogate markers of cellular activation in systemic lupus erythematosus (SLE). Because microparticles have also been associated with many primary neurologic diseases, this study investigated whether cellular-derived microparticles are also implicated in neuropsychiatric SLE (NPSLE).

Plasma microparticles were measured in 51 SLE patients and 22 age- and gender-matched controls. Acute NPSLE was defined as major NPSLE (acute stroke, transient ischemic attack, psychosis, isolated seizures, major cognitive disorder, or acute confusional state) and NPSLE disease activity was measured with the neurologic components of the SLE Disease Activity Index (Neuro-SLEDAI).

Neuro-SLEDAI levels varied widely in SLE patients, consistent with variable NPSLE activity. When considering all patients with SLE, there was no difference in total microparticles relative to matched controls, 2158/μL (interquartile range [IQR] 1214–3463) versus 2782/μL (IQR 1586–2990; P = 0.57) nor differences in microparticles derived from either platelets (P = 0.40), monocytes (P = 0.15) or endothelial cells (P = 0.32). However, levels of circulating monocyte-derived microparticles significantly and independently correlated with NPSLE (r = −0.28; P = 0.045), corticosteroid dosage (r = −0.38; P = 0.006) and levels of circulating C5a (r = 0.54; P < 0.0001). Non-neurologic SLE disease activity was not associated with microparticles.

Circulating cell-derived microparticles are reduced in active NPSLE, although the relative contribution of reduced microparticle production, increased consumption or intravascular sequestration, remain uncertain.

Ankylosing spondylitis (AS) is a chronic rheumatic disorder with gender differences. The aim of study was to investigate the association between polymorphisms of the androgen receptor (AR) gene and the susceptibility to AS in Taiwanese men of Han Chinese descent.

We conducted a case-control study with 92 male AS patients and 108 healthy controls. Trinucleotide (CAG and GGC) repeats and seven single nucleotide polymorphisms (SNPs) rs962458, rs6152, rs1204038, rs5918757, rs2361634, rs6624304 and rs1337080 in the AR gene were genotyped.

We found that only one patient had polymorphic SNPs of the AR gene. None of the genotyped SNPs in the AR gene, originally found in Caucasians, was polymorphic in the Taiwanese men. Neither CAG nor GGC repeat lengths in the AR gene had a significant relationship with human leukocyte antigen (HLA)-B27 positivity or disease severity in AS.

There were no differences in CAG and GGC lengths in the AR gene between AS and the controls. None of the genotyped SNPs in AR gene are detected to be polymorphic in male Taiwanese, which indicates that the effect of AR gene on AS may be ethnic-specific and may be conserved in East Asians compared to Caucasian populations. Still, additional studies using large sets of subjects deserve further attention, since our sample size was small with limited statistical power and supporting evidence for association between the AR gene and AS risk in the Japanese population exists.

The objectives are to detect the frequency of sicca symptoms and Sjögren's syndrome (SS) in patients with systemic sclerosis (SSc) based on the diagnostic criteria of the American–European Consensus Group (AECG) and to evaluate demographic, clinical and serologic characteristics.

One hundred and eighteen SSc patients referred to our hospital were included in this study. All SSc patients were questioned with respect to sicca symptoms. Levels of rheumatoid factor (RF), anti-nuclear antibodies (ANA), anti-Ro and anti-La antibodies were measured; non-stimulated saliva amounts were recorded and Schirmer test and break-up time were applied to all patients. Minor salivary gland biopsy samples were obtained from those patients giving ≥ 3 positive answers to sicca symptom questions, patients with positive xerostomia/xerophthalmia test results, and patients with at least one antibody being positive. Patients presenting with grade 3 and/or grade 4 sialoadenitis based on Chisholm criteria were considered pathological.

Sicca symptoms were present in 84 of 118 patients with SSc (71.2%). Minor salivary gland biopsy samples were obtained from 74 patients. Grade 3 and/or grade 4 sialoadenitis was detected in 40 (33.9%) patients and they were diagnosed with SS. Compared to patients diagnosed with SSc alone, systemic sclerosis patients diagnosed with SS had lower pulmonary hypertension and less diffuse lung involvement. Statistically significant difference was detected in terms of sclerodactylia and telangiectasia between SSc–SS and SSc patient groups (P = 0.045 and P = 0.011, respectively). Serological assessments revealed that in the SSc–SS group, 13 patients were anti-Ro antibody positive, six were anti-La antibody positive and 37 were anti-topoisomerase 1 antibody positive. RF, ANA and anti-centromere antibody levels were higher in the SSc–SS group.

In the present study, highly frequent sicca symptoms and Sjögren's syndrome based on AECG criteria were noted in patients with systemic sclerosis. The SSc–SS patient group had less severe clinical course and lung involvement.

We describe a 42-year-old man who presented with painless obstructive jaundice, organomegaly and lymphadenopathy. Biopsy of the ampulla of Vater revealed the presence of increased populations of plasma cells which stained positively for immunoglobulin G4. He was treated with prednisolone and demonstrated significant clinical improvement 1 month later. A further case is described and a review of the literature is also provided.