Comparative analysis of optical coherence tomography angiography (OCTA) results between Behçet's disease patients and a healthy control group.

PURPOSE: This study aimed to compare optical coherence tomography angiography (OCTA) findings between patients with Behçet's disease (BD) and individuals with healthy eyes. DESIGN: A cross-sectional study. METHODS: This cross-sectional study was conducted on patients (67 eyes) with BD who were referred to Feiz Hospital and healthy eyes (43 eyes). All subjects underwent Snellen visual acuity, a slit-lamp examination, measuring intraocular pressure, conducting a dilated fundus examination, OCTA imaging, and spectral-domain (SD)-OCT imaging. OCTA retinal vascular measurements including optic nerve VD, macular-associated VD( superficial and deep), foveal avascular zone (FAZ) area, FAZ perimeter (PERIM), and vessel density within a 300-µm-wide region of the FAZ (FD) were compared between the groups. RESULTS: A significant difference was evident between the two groups (healthy one group and BD group) in terms of parafoveal and perifoveal total retinal thickness, total pRNFL VD in all quadrants except the inferior sector (P < 0.05), and macular superficial, and deep VD in all regions except temporal and superior perifoveal VD (P < 0.05) following adjustments for age, gender, and signal strength index. When comparing the two groups, ocular Behçet's disease (BD) and non-ocular BD, it was evident that peripapillary vessel density (VD) exhibited a significant decrease in ocular BD eyes in all sectors except for the superior and inferior ones, as compared to non-ocular BD eyes. In addition, the comparison of ocular BD and non-ocular BD showed superficial and deep VDs were lower in ocular BD than non-ocular BD in all regions. CONCLUSION: According to these findings, peripapillary and macular vessel density is affected in BD. Key Points • The study utilized OCTA to compare retinal features in Behçet's disease (BD) patients and healthy individuals, revealing significant differences in retinal thickness and vessel density. • Ocular BD demonstrated reduced peripapillary vessel density compared to non-ocular BD. • The demonstrated association between ADMA and cIMT in patients with early SSc may suggest a role of NO/ADMA pathway in the initiation of macrovascular injury in SSc.

Predictors of severe lupus flare: a prospective follow-up study.

BACKGROUND: Flare-up of systemic lupus erythematosus (SLE) is a common characteristic that could have deleterious effects on patients' outcome and survival. The aim of this study was to identify the predictors of severe lupus flare. METHODS: 120 patients with SLE were enrolled and followed-up for 23 months. Demographic, clinical manifestations, laboratory parameters and disease activity were recorded at each visit. In addition, presence of severe lupus flare at each visit was evaluated by using the Safety of Estrogens in Lupus Erythematosus National Assessment (SELENA)-SLE disease activity index (SLEDAI) flare composite index. Predictors of severe lupus flare were obtained by backward logistic regression analyses. Predictors of SLEDAI were obtained by backward linear regression analyses. RESULTS: During the follow-up period, 47 patients had at least one episode of severe lupus flare. Mean (SD) age of patients with severe flare versus no flare was 31.7 (7.89) and 38.3 (8.24) years, respectively (P = 0.001). Ten (62.5%) out of 16 males and 37 (35.5%) out of 104 females had severe flare (P = 0.04). History of lupus nephritis (LN) was recorded in 76.5% and 44% of patients with severe flare and no severe flare, respectively (P = 0.001). Thirty-five (29.2%) patients with high anti-double-stranded DNA (anti-ds-DNA antibody) and 12 (10%) with negative anti-ds-DNA antibody had severe lupus flare (P = 0.02). By multivariable logistic regression analysis, younger age (OR = 0.87, 95% CI 0.80-0.94, P = 0.0001), history of LN (OR = 4.66, 95% CI 1.55-14.002, P = 0.006) and high SLEDAI at the first visit (OR = 1.19, 95% CI 1.026-1.38) were the main predictors of flare. When severe lupus flare after the first visit was used as the outcome variable, similar findings were observed but, SLEDAI, although left among the final predictors in the model, was not significant. SLEDAIs in future visits were mainly predicted by Anti-ds-DNA antibody, 24-h urine protein and arthritis at the first visit. CONCLUSION: SLE patients with younger age, history of previous LN or high baseline SLEDAI, may need closer monitoring and follow up.

Long-term follow-up of Iranian male patients with systemic lupus erythematosus.

This study was conducted to investigate the long-term survival in male patients with systemic lupus erythematosus (SLE) and its predictors. The main demographic and clinical manifestations at the time of disease diagnosis were recorded retrospectively. Kaplan-Meier curves were used to calculate survival rates. Predictors of mortality were determined by backward Cox regression analysis. Eighty-four male patients with SLE were enrolled. During the 23-year study period, 11 patients died. Lupus nephritis (5 cases), infections (5 cases) and alveolar hemorrhage (1 case) were the most common causes of deaths. Overall survival rates at the end of 5, 10, 15, and 20 years after SLE disease diagnosis were 86%, 84%, 84% and 84%, respectively. In multivariate backward-regression analysis, the main determinants of death at the time of SLE diagnosis were oral ulcer (p = 0.004, HR = 7.69, 95% CI 1.92-33.33), thrombocytopenia (p = 0.012, HR = 5, 95% CI 1.41-16.66) and SLE disease activity index (SLEDAI, p = 0.05, HR = 1.08, 95% CI = 0.999-1.1). Observing oral ulcer, thrombocytopenia and high SLEDAI at the time of disease diagnosis were the main prognostic factors in male lupus patients.

Role of Cystatin C in Predicting Disease Activity and Flare-Up in Systemic Lupus Erythematosus: A Longitudinal Follow-Up Study.

BACKGROUND: We aimed to determine the sensitivity of serum cystatin C (Cys-C) in predicting lupus flare-up. METHODS: In a longitudinal study, 77 patients were followed-up for up to 15 months. Cys-C, physician global assessment (PGA), and lupus activity index (SLEDAI) were recorded during each visit. Flare-up was defined as an increase ≥4 scores in SLEDAI compared to the last visit. The predictability of flare-up by Cys-C was evaluated by generalized linear-mixed effect model (GLMM) and generalized estimating equation (GEE). Predictive power of Cys-C, SLEDAI, and PGA was compared by the area under the curves (AUC) and application of receiver operating characteristic (ROC) curves. RESULTS: Lupus flare-up was observed in 14 out of 77 patients on the 1st visit, 3 out of 41 patients on the 2nd visit, 2 out of 26 patients on the 3rd visit, 1 out of 14 patients on the 4th visit, and 1 out of 3 patients on the 5th visit. Mean Cys-C levels in patients with flare-up vs. those with no flare-up in the 1st, 2nd, and 3rd visits were 1769 vs. 1603 (P = 0.6), 5701 vs. 2117 (p = 0.2) and 1409 vs. 1731 (p = 0.9), respectively. Cys-C had lower predictive power than PGA and SLEDAI for either flare-up, active nephritis or SLEDAI in GLMM/GEE models. Cys-C also showed lower sensitivity (AUC = 0.701, 95%CI = 0.579-0.823, P = 0.003) than PGA and SLEDAI, to distinguish patients prone to flare-ups. CONCLUSIONS: Although Cys-C had some sensitivity for predicting flare-up, active nephritis or SLEDAI, its sensitivity was lower than that in PGA and SLEDAI.

The impact of neutrophil extracellular trap from patients with systemic lupus erythematosus on the viability, CD11b expression and oxidative burst of healthy neutrophils.

BACKGROUND: NET (neutrophil extracellular trap) has been shown to directly influence inflammation; in SLE (systemic lupus erythematosus), it is reportedly a plausible cause for the broken self-tolerance that contributes to this pathology. Meanwhile, the role of NET is not easily explicable, and there is a serious discrepancy in the role of NET in SLE pathology and generally inflammation; in particular, the interactions of neutrophils with NET have been rarely inspected. This study evaluates the effect of NET on neutrophils in the context of SLE. The neutrophils were incubated by the collected NET (from SLE patients and healthy controls) and their expression of an activation marker, viability and oxidative burst ability were measured. RESULTS: The level of cell mortality, CD11b expression and the oxidative burst capacity were elevated in NET-treated neutrophils. Also, the elevation caused by the SLE NET was higher than that produced by the healthy NET. CONCLUSION: The decreased neutrophil viability was not due to the increase in apoptosis; rather, it was because of the augmentation of other inflammatory cell-death modes. The upregulation of CD11b implies that NET causes neutrophils to more actively contribute to inflammation. The increased oxidative burst capacity of neutrophils can play a double role in inflammation. Overall, the effects induced by NET on neutrophils help prolong inflammation; accordingly, the NET collected from SLE patients is stronger than the NET from healthy individuals.

Autologous plasma versus fetal calf serum as a supplement for the culture of neutrophils.

OBJECTIVE: Currently, the replacement of fetal calf serum (FCS) by a more suitable alternative is a sought aim in the field of tissue and cell culture research. Autologous plasma (AP) and especially autologous serum (AS) have been shown to be effective substitutes of FCS in culture media for some of the cell types. Nevertheless, there is no comparative data on the most appropriate supplement for cell media in neutrophil studies, it is now unclear whether AP have a relatively equal, superior or inferior performance to FCS in neutrophil cell culture. In the present study, human blood neutrophils were isolated and cultured in FCS- or AP-supplemented medium. After 12, 36 and 60 h of incubation, cell viability, oxidative burst and CD11b expression were determined by flow cytometry. RESULTS: Compared to the culture of neutrophils in FCS 10% medium, the culture of neutrophils in a medium with AP 10% could prolong their life span without affecting their function. The findings introduce AP as a better supplement for human neutrophil cell culture than FCS and propose a simple and economical procedure for neutrophil isolation and culture.

Nailfold microvascular changes in patients with systemic lupus erythematosus and their associative factors.

OBJECTIVES: The aim of this study was to determine capillaroscopic changes in patients with systemic lupus erythematosus (SLE) and their predictors. METHODS: Fifty-nine SLE patients and 31 controls were enrolled in a cross-sectional study. Nailfold capillaroscopy examinations were performed, and qualitative semi-quantitative and quantitative parameters were evaluated in all fingers. Demographic features and lupus characteristics, such as renal involvement, medications, laboratory data, disease activity (SLEDAI) and damage, were recorded. The predictors of capillaroscopic abnormalities were obtained by backward stepwise regression analysis. RESULTS: Capillary numbers of right hands were significantly lower in patients than in controls [8.74 (1.66) vs. 9.63 (1.80), P = 0.0001]. Capillaries were wider in patients than in controls in right [56.32 µm (16.76) vs. 50.43 µm (10.16), P = 0.002] and left hands [54.40 (15.02) vs. 49.71 (9.77), P = 0.005]. Capillaries were shorter in SLE patients than in controls. Multivariate analysis revealed that the main associative factors of microvascular abnormalities were gender, drinking tea and hydroxychloroquine use for giant capillaries, SLEDAI and low C3 for avascularity and age, lupus nephritis and corticosteroid use for ramification. CONCLUSION: Most nailfold capillaroscopic abnormalities were more common in SLE patients than in controls. Hydroxychloroquine, corticosteroids, SLEDAI, low complement and lupus nephritis may be the major prognostic factors for microvascular changes in SLE patients.

Practical issues in assessing nailfold capillaroscopic images: a summary.

Nailfold capillaroscopy (NC) is a highly sensitive, safe, and non-invasive technique to assess involvement rate of microvascularity in dermatomyositis and systemic sclerosis. A large number of studies have focused on NC pattern description, classification, and scoring system validation, but minimal information has been published on the accuracy and precision of the measurement. The objective of this review article is to identify different factors affecting the reliability and validity of the assessment in NC. Several factors can affect the reliability of the examination, e.g., physiological artifacts, the nailfold imaging instrument, human factors, and the assessment rules and standards. It is impossible to avoid all artifacts, e.g., skin transparency, physically injured fingers, and skin pigmentation. However, minimization of the impact of some of these artifacts by considering some protocols before the examination and by using specialized tools, training, guidelines, and software can help to reduce errors in the measurement and assessment of NC images. Establishing guidelines and instructions for automatic characterization and measurement based on machine learning techniques also may reduce ambiguities and the assessment time.

Image enhancement effect on inter and intra-observer reliability of nailfold capillary assessment.

BACKGROUND: Nailfold capillaroscopy (NC) is a diagnostic imaging technique that is used to assess the blood capillary network in the nailfold area. NC is routinely used for patients with microcirculation problems, such as systemic sclerosis and other connective tissue diseases. Experts commonly use subjective evaluation as a reference point in images of nailfold video capillaroscopy, so it is important to reduce the inherent ambiguities in human judgment and diagnosis. Image quality is an important factor that affects measurement error and assessment time of NC images. OBJECTIVE: In this study, a new image enhancement technique was introduced and evaluated subjectively. METHODS: In total, 475 nailfold video capillaroscopy images from 18 healthy subjects and 41 systemic lupus erythematosus patients were used. The images were randomly divided into two sets, one each with 275 and 200. Eight independent observers who were familiar with the capillaroscopy technique participated in this study. The set of 275 images was evaluated by three observers with the forced-choice pairwise comparison method. Elliptic broken line (EBL) was used to count the number of capillaries. The intra- and inter-observer reliability of the original and enhanced images was evaluated on 200 images by five observers. RESULT: Except for eight images, all observers preferred the enhanced images in the visual quality comparison method. The intra-class correlation coefficient (ICC) of intra- and inter-observer reliability increased from 0.76-0.84 to 0.82-0.89, respectively, when using the enhancement method. CONCLUSION: By improving the image quality, more capillary details will be visible, and an observer can document more details that may not be visible in the original image and can do so more efficiently.

Metabolic syndrome in Iranian patients with systemic lupus erythematosus and its determinants.

The aim of this study was to determine the prevalence of metabolic syndrome (MetS) in Iranian patients with systemic lupus erythematosus (SLE) and its determinants. In a cross-sectional study, 98 patients with SLE and 95 controls were enrolled. Prevalence of MetS was determined based on American Heart Association and National Heart, Lung, and Blood Institute (AHA/NHLBI) and 2009 harmonizing criteria. In addition, demographic features and lupus characteristics such as disease duration, pharmacological treatment, laboratory data, SLE disease activity index (SLEDAI), and Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage index (SDI) were recorded. The predictors of MetS were obtained by backward stepwise regression analysis. Using AHA/NHLBI, MetS was observed in 35 (35.7%) patients and 28 (29.8%) controls (P = 0.4). Using harmonizing criteria, MetS was observed in 37 (37.7%) patients and 33 (35.1%) controls (P = 0.7). There was no difference in frequency distribution of MetS components between the patients and the controls. In multivariate regression analysis, low C3, blood urea nitrogen (BUN), and body mass index were independent determinants of MetS in lupus patients. BUN, low C3, and body mass index were the major determinants of MetS in lupus patients.

Sensitivity analyses of four systemic lupus erythematosus disease activity indices in predicting the treatment changes in consecutive visits: a longitudinal study.

This study was conducted to assess the ability of the British Isles Lupus Assessment Group-2004 (BILAG-2004), the SLE Disease Activity Index-2K (SLEDAI-2K), the European Consensus Lupus Activity Measurement (ECLAM), and the Revised Systemic Lupus Activity Measure (SLAM-R) to detect the need to treatment change in daily clinical practice. One hundred and two patients with SLE were enrolled and followed up for 2 to 8 months and visited at least 3 times. Physician Global Assessment, BILAG-2004, SLEDAI-2K, SLAM-R, and ECLAM, were calculated in every visit. Treatment change, dependent variable, was categorized as decrease/no change vs. increase. The aforementioned indices, independent variables, were compared to learn their ability in predicting the treatment change. The probability of treatment change was measured by generalized linear-mixed effect model (GLMM) and generalized estimating equations (GEE). Adjusted odds ratios were calculated. Predictive power of indices was compared by area under the curve (AUC) in plots of sensitivity vs. 1-specificity and application of receiver operating characteristic curves (ROC). BILAG-2004 and SLEDAI-2K had substantial correlation with treatment change. Among different GLMM models, BILAG-2004 followed by SLEDAI-2K showed the highest associations with treatment change. Among various GEE models, similar findings were observed. Also, these 2 indices had the highest sensitivity (the largest AUC) towards treatment change; BILAG-2004 (AUC = 0.779, 95% CI = 0.710-0.848, p = 0.001) and SLEDAI-2K (AUC = 0.771, 95% CI = 0.698-0.843, p = 0.001). BILAG-2004 followed by SLEDAI-2K had the highest predictability of treatment change.

Prognostic factors of mortality in Iranian patients with systemic lupus erythematosus admitted to intensive care unit.

The aim was to determine the course, outcome, and determinants of mortality in patients with systemic lupus erythematosus (SLE) in intensive care unit (ICU). SLE patients admitted to ICU from 2004 to 2015 were recruited retrospectively. Demographic data, disease characteristics, causes of admission, baseline SLE disease activity index-2K (SLEDAI-2K) and Acute Physiologic and Chronic Health Evaluation II (APACHE) score, the outcome, and the causes of death were recorded. Predictors of mortality were compared between alive and dead patients by Cox regression analysis. Ninety-four patients with SLE were enrolled. Mean age at the time of ICU admission was 29.6 years. Average scores of SLEDAI and APACHE II were 11.3 and 19.8, respectively. The most common causes of ICU admission were pneumonia, diffuse alveolar hemorrhage (DAH), and seizure. Forty-seven patients (50%) died in ICU. The principal causes of death were septic shock (25.5%), multi-organ failure (12.5%), DAH (10.6%), and pneumonia (10.6%). After multivariate analysis, high APACHE II, septic shock, and duration of mechanical ventilation were indicators of survival outcome. Mean (95% CI) survival days in ICU in patients with and without respiratory failure were 14.6 (10.4-18.9) and 28.7 (17.9-39.5) days, respectively (P = 0.001). This figure for those with and without septic shock was 13.5 (4.9-11.1) and 22.3 (9.3-24.7) days, respectively (P = 0.016). High APACHE II, septic shock, and duration of mechanical ventilation were the main predictors of death in patients with SLE in ICU. Multicenter studies are needed to draw a fine picture of SLE behavior in ICU.

Elliptical broken line method for calculating capillary density in nailfold capillaroscopy: Proposal and evaluation.

Nailfold capillaroscopy is a practical method for identifying and obtaining morphological changes in capillaries which might reveal relevant information about diseases and health. Capillaroscopy is harmless, and seems simple and repeatable. However, there is lack of established guidelines and instructions for acquisition as well as the interpretation of the obtained images; which might lead to various ambiguities. In addition, assessment and interpretation of the acquired images are very subjective. In an attempt to overcome some of these problems, in this study a new modified technique for assessment of nailfold capillary density is introduced. The new method is named elliptic broken line (EBL) which is an extension of the two previously known methods by defining clear criteria for finding the apex of capillaries in different scenarios by using a fitted elliptic. A graphical user interface (GUI) is developed for pre-processing, manual assessment of capillary apexes and automatic correction of selected apexes based on 90° rule. Intra- and inter-observer reliability of EBL and corrected EBL is evaluated in this study. Four independent observers familiar with capillaroscopy performed the assessment for 200 nailfold videocapillaroscopy images, form healthy subject and systemic lupus erythematosus patients, in two different sessions. The results show elevation from moderate (ICC=0.691) and good (ICC=0.753) agreements to good (ICC=0.750) and good (ICC=0.801) for intra- and inter-observer reliability after automatic correction of EBL. This clearly shows the potential of this method to improve the reliability and repeatability of assessment which motivates us for further development of automatic tool for EBL method.

Prospective study of articular manifestations in Behçet's disease: five-year report.

AIM: To evaluate different aspects of articular involvements (prevalence, types, relation to extra-articular manifestations, etc.) in Iranian patients with Behcet's disease (BD). METHODS: In a prospective study, all patients with BD attending an outpatient BD clinic were enrolled. The type of articular involvements (peripheral or axial), involved joints, duration of attacks and their relation to extra-articular manifestations, human leukocyte antigen (HLA)-B5 and HLA-B27 were evaluated. Data analysis was done by using descriptive statistical indices such as mean and confidence interval. The comparisons were done by chi-square test. RESULTS: In a 5-year period, 2312 patients were recruited. Musculoskeletal manifestations were recorded in 430 patients (190 without previous history of these involvements). The remaining 1882 patients showed no musculoskeletal involvements, of which 753 had past histories of musculoskeletal manifestations. The 5-year incidence rate was 18.6%, and the prevalence rate was 51.2%. History of previous musculoskeletal involvements has no effect in its new development (P = 0.2). Arthritis was the most common type of involvement (289/430, 67%). The most frequent pattern was monoarthritis (191/289, 66%), and the most frequent involved joints were knees (147/289, 50.9%). The arthritic attacks were unilateral in 82.4% of cases. Ankylosing spondylitis was seen in 44/430 (10.2%). There was no association between HLA-B5 or HLA-B27 and any type of musculoskeletal involvements (P > 0.5). Pseudofolliculitis was the only extra-articular manifestation related to arthritic attacks (P = 0.046). CONCLUSION: Musculoskeletal involvement is a common manifestation of BD seen in more than half of patients. Acute knee monoarthritis was the most common pattern of articular involvement in BD.

Childhood versus adult-onset systemic lupus erythematosus: long-term outcome and predictors of mortality.

The aim of this study was to compare survival of childhood-onset systemic lupus erythematosus (c-SLE) and adult-onset SLE (a-SLE) according to initial manifestations. This was a retrospective cohort study. All patients were categorized into c-SLE (≤18 years) and a-SLE (>18 years). The clinical and serological data at the time of diagnosis were recorded and compared. Kaplan-Meier curves were used to compare survival rates between the two groups. Predictors of mortality were obtained by a backward Cox regression. One hundred eighty patients with c-SLE and 394 patients with a-SLE were enrolled. The female/male ratio was higher in c-SLE (P = 0.0001). Lupus nephritis (P = 0.002) and valvular heart disease (P = 0.025) were more common in c-SLE and a-SLE, respectively. In a 23-year follow-up, 20 patients (11.1%) with c-SLE and 35 patients (8.9%) with a-SLE died. Mortality was not significantly different between them (P = 0.4). The main causes of death were nephritis (50% in c-SLE vs. 29% in a-SLE), infections (40% in c-SLE vs. 29% in a-SLE), and circulatory disease (10% in c-SLE vs. 37% in a-SLE). The difference was not significant (P = 0.08). Cumulative survival rates after 5, 10, 15, and 20 years were 91, 87, 85, and 78% in c-SLE and 93, 90, 90, and 83% in a-SLE, respectively. By multivariate analysis, seizure, proteinuria, and nephritis in c-SLE and seizure, hematuria, and pericarditis in a-SLE had negative prognostic effect on survival. Both c-SLE and a-SLE patients with seizure or renal involvement should be monitored more carefully to prevent ominous outcomes.

Capillary density: An important parameter in nailfold capillaroscopy.

Nailfold capillaroscopy is one of the various noninvasive bioengineering methods used to investigate skin microcirculation. It is an effective examination for assessing microvascular changes in the peripheral circulation; hence it has a significant role for the diagnosis of Systemic sclerosis with the classic changes of giant capillaries as well as the decline in capillary density with capillary dropout. The decline in capillary density is one of microangiopathic features existing in connective tissue disease. It is detectable with nailfold capillaroscopy. This parameter is assessed by applying quantitative measurement. In this article, we reviewed a common method for calculating the capillary density and the relation between the number of capillaries as well as the existence of digital ulcers, pulmonary arterial hypertension, autoantibodies, scleroderma patterns and different scoring system.

Relationship between articular and nonarticular manifestations in inflammatory bowel diseases.

BACKGROUND: Musculoskeletal manifestations (MSM) of inflammatory bowel diseases (IBDs) are usually the most frequent extraintestinal manifestations. However, they are not paid enough attention during regular office visits. This cross-sectional study aimed to draw a clinical picture of MSM and their relationships with other findings in patients with IBD. MATERIALS AND METHODS: Patients of our IBD cohort between March 2012 and September 2013 were consecutively evaluated. Those with current or past history of any MSM were examined by a rheumatologist. The outcome of interest was different MSMs. Distribution of IBD manifestations between the two groups of patients with (n = 20) and without (n = 253) MSM was compared. Logistic regression analysis was employed to find the relationships of demographic, clinical, and laboratory findings with MSM. RESULTS: Two hundred and seventy-three patients were enrolled. Forty-two patients (15.4%) had extraintestinal manifestations of which twenty patients (7.5%) had at least one MSM. 7/20 patients (35%) versus 22/253 (8.7%) had other extraintestinal manifestations (P = 0.0001). 12/20 patients (57%) had arthritis (polyarthritis, 33% and oligoarthritis, 67%). The most frequent involved joints were knee and ankle observed in 8 (40%) and 7 (35%) patients, respectively. The inflammatory back pain was recorded in 5/20 patients (25%) whereas two patients (10%) had ankylosing spondylitis. In regression analysis, oral aphthous (odds ratio [OR] =8.8 [95% confidence intervals (CI), 1.7-45], P = 0.009) and other extraintestinal manifestations (OR = 5.2 [95% CI, 1.3-20], P = 0.02) were significantly related with arthritis. CONCLUSION: The most frequent extraintestinal manifestations in patients with IBD were MSM. Knee and ankle were the most frequent involved joints. Extraintestinal manifestations were determinant variables of arthritis.

The autonomic dysfunction in patients with lupus disease: An electrophysiological study.

BACKGROUND: The aim of this study was to investigate autonomic nervous system (ANS) function by using electrophysiological tests in patients with systemic lupus erythematosus (SLE). MATERIALS AND METHODS: This descriptive analytical study was done on 28 individuals with a history of lupus and ten age- and sex-matched healthy objects were being selected randomly. The autonomy questionnaire has been used to determine clinical symptom of ANS involvement. The electrophysiological assessments of ANS function were performed by sympathetic skin response (SSR). The mean values of sympathetic (SSR latency and amplitude) parameters were compared to determine any correlations between SSR parameters and clinical characteristics of ANS. RESULTS: 28 SLE patients (3 males, 25 females) with a mean age of 34.6 ± 9.74 years and 10 control subjects (4 males, 6 females) with a mean age of 36.8 ± 6.43 years were included in the study. Among patients 17 (60.7%) exhibited autonomic symptoms. Headache was the most common issue with the highest percentage rate (41.17%). The mean latency and amplitude of SSR were increased (1.52 ± 0.16 vs. 1.39 ± 0.16 and 107 ± 15.6 vs. 110 ± 15.6, respectively), compared to control. A significant difference was observed between the SSR test results of patients with lupus compared to normal healthy objects (P < 0.05). R = 0.43 correlation was found between autonomy questionnaire scores and SSR (P < 0.05). CONCLUSION: It could be concluded that latency measures in SSR test can be used as a valuable and accurate evaluation guideline for autonomic system assessment.

Anti-C1q antibody in patients with lupus nephritic flare: 18-month follow-up and a nested case-control study.

OBJECTIVES: A prospective cohort was conducted to investigate the association of anti-C1q antibody and lupus/lupus nephritis (LN) flare. METHODS: Sixty-nine consecutive patients with systemic lupus erythematosus were enrolled and followed up for 18 months. Anti-C1q was recorded at the first visit and at the time of flare. For patients with flare, age and sex matched SLE patients were considered as the control group (nested case-control study). The predictability of anti-C1q and other laboratory indices for LN flare during the 18-month follow-up was calculated. RESULTS: Fourteen out of sixty-nine (20%) had lupus flare. Fourteen patients were chosen as controls. Nine cases and three controls had positive anti-C1q at the first visit (p = 0.0001). Twenty-six (38%) and 43 (62%) patients had positive and negative anti-C1q antibody at the first visit, respectively, of whom 9 (34.5%) and 3 (7%) patients developed LN flare in the next 18 months (p = 0.003). Anti-C1q and 24-hour urine protein were found as the main predictors of LN flare. The positive and negative predictive values of anti-C1q for LN flare were 35% and 93%, respectively. Predictive values of positive anti-C1q/low C3 together were 60% and 96%, respectively. CONCLUSION: The combination of positive anti-C1q/low C3 had the highest reasonable predictive values for LN flare.

The association between initial manifestations of childhood-onset systemic lupus erythematosus and the survival.

INTRODUCTION: Childhood-onset systemic lupus erythematosus (cSLE) comprises 15-20% of patients with SLE. Although several studies have reported the outcomes of adult-onset SLE, few investigations have been conducted on cSLE in the Middle East. METHODS: In a retrospective study, all children with SLE admitted to our tertiary referral center between 1992 and 2011 were recruited. The clinical and laboratory data at the time of onset were recorded and analyzed. Kaplan-Meier analysis was used to calculate the survival rates. Cox regression analysis was applied to assess the predictors of mortality. RESULTS: One hundred and eighty-eight children diagnosed with SLE were enrolled during the study period. Nine patients were censored due to loss to follow-up (6) and incomplete data (3 cases). Mean age of patients at the time of onset was 14.4 (3.05) years. Only 22 (11.8%) children were younger than 10 years at the time of disease onset. In total, 20 patients (11%) died, all after the first decade of life. The most common cause of death was lupus nephritis (10 patients, 50% of deaths) followed by infections (35%), cerebrovascular accidents (10%) and alveolar hemorrhage (5%). Cumulative survival rate after 5, 10, 15 and 20 years was 91, 87, 85, and 79%, respectively. Having hematuria or pleurisy at the time of SLE onset had a negative effect on survival in multivariate analysis. CONCLUSION: cSLE survival in Iran was comparable to that in other developing countries. Baseline presentation with hematuria predominantly increased the mortality rate in cSLE. Prospective and larger studies in future may unfold other aspects of cSLE.