Disease Activity Features Research Articles

Generalized lymphadenopathy as an initial presentation of systemic lupus erythematosus: case report and review of the literature

BackgroundSystemic lupus erythematosus (SLE) is a chronic multisystemic autoimmune disorder with a wide array of clinical manifestations. Lymphadenopathy is occasionally observed in patients with SLE, and although it is sometimes a feature of active disease, it is rarely the initial presenting feature at the time of diagnosis.Case presentationWe report the case of a 35-year-old woman with clinical features of generalized lymphadenopathy, fatigue, fever, and malaise of approximately 3 months duration with no other constitutional disorders or systemic involvement suggestive of SLE.Despite extensive laboratory, radiological, and histological evaluations for malignancies and infectious causes of generalized lymphadenopathy, all results returned normal. About a month later, she developed inflammatory polyarthritis, with a markedly elevated erythrocyte sedimentation rate (ESR) of 120 mm/h, and a subsequent serologic workup confirmed the diagnosis of SLE.Autoimmune serology testing revealed positive results for various antibodies, including antinuclear antibody (ANA) at a titre of 1:5120, homogenous and speckled pattern, anti-Sm, anti-dsDNA, U1-snRNP, Ku, SSA-AntiRo, SSB-AntiLa, anti-SM/RNP, anti-Histone, ribosomal-p protein, and anti-nucleosome antibodies. The diagnosis of SLE was established using the 2019 European League Against Rheumatism/American College of Rheumatology (EULAR/ ACR) criteria.She was on oral prednisolone, hydroxychloroquine, and calcium/vitamin D3 tablets. The patient improved remarkably, all swollen lymph nodes regressed spontaneously and she had no further need for a hematology review.ConclusionWhile similar cases have been reported in Europe and some parts of Asia, in Africa, a careful search of the literature revealed that such cases have been reported only from Egypt in North Africa and not from other parts of the continent; hence, this case report highlights the importance of recognizing atypical presentations of SLE to improve awareness and diagnosis in different regions.

Tuberculosis remains a major burden in systemic lupus erythematosus patients in Durban, South Africa.

Infections are common in systemic lupus erythematosus (SLE), with tuberculosis (TB) being important in an endemic environment. We studied the prevalence and spectrum of TB in SLE in Durban, South Africa. A medical records review of SLE patients seen over 13-year period, and the demographic data, clinical manifestations, laboratory findings, treatment and outcome were noted. There were 512 SLE patients and 72 (14.1%) had TB. Thirty (41.7%) had pulmonary TB (PTB) and 42 (58.3%) had extra-pulmonary TB (EPTB). The prevalence of TB among the different ethnic groups was 36/282 (12.8%) for Indian people, 29/184 (15.8%) Black African people, 7/26 (26.9%) admixed African people and none among the 18 White people. Comparison of the 72 SLE-TB patients with 72 SLE controls showed no difference in gender, age at SLE diagnosis and disease duration. The SLE-TB patients had a significant increase in the clinical and laboratory features of disease activity (arthritis, mucocutaneous lesions, renal involvement, vasculitis, low complement, raised ds-DNA antibodies), and cumulative prednisone use over the preceding 3 months.Compared to PTB, the EPTB patients were significantly younger, developed TB earlier after SLE diagnosis, and had higher disease activity. The EPTB patients also had increase in features of disease activity (renal, thrombocytopenia, ds-DNA antibodies), and increase in ever use of intravenous methylprednisolone (IV-MP) and mycophenolate mofetil (MMF). On multivariate analysis, the independent risk factors for EPTB were ever use of MMF (p = 0.003) and IV-MP (p = 0.027). Analysis of the cumulative SLE criteria showed renal involvement was an independent risk factor for EPTB. The outcome was similar in both groups. We show an increased prevalence of TB (14.1%) and EPTB (58.3%) in SLE in an endemic area and confirm that features of disease activity and use of immunosuppressive therapy are the major risk factors. Renal involvement (as a cumulative criterion) is an independent risk factor for EPTB.

Can Patient-Reported Symptoms Be Used to Measure Disease Activity in Systemic Sclerosis?

To evaluate the association between patient-reported symptoms and changes in disease activity over time in systemic sclerosis (SSc). Using data from 1,636 patients enrolled in the Australian Scleroderma Cohort Study, we used generalized estimating equations to determine the relationship between patient-reported worsening of Raynaud's phenomenon (RP), skin involvement, and breathlessness in the month preceding each study visit and features of disease activity in the corresponding organ systems. The associations between the following parameters were analyzed: patient-reported worsening RP and the presence of new-onset digital pitting and digital ulcers; patient-reported worsening skin involvement and increasing modified Rodnan skin thickness score (MRSS); new areas of skin involvement and new-onset joint contractures; patient-reported worsening breathlessness and deteriorating respiratory functions test (RFT) results, indicated by a 10% decrease in forced vital capacity (FVC) and a 15% decrease in diffusing capacity for carbon monoxide (DLco), new-onset interstitial lung disease (ILD), and new-onset pulmonary arterial hypertension (PAH). We found a significant association between patient-reported worsening RP and the presence of digital ulcers (odds ratio [OR] 1.53 [95% confidence interval (95% CI) 0.60-0.93]), patient-reported worsening skin involvement and increasing MRSS (OR 2.10 [95% CI 1.54-2.86]), and worsening patient breathlessness and deteriorating RFTs (FVC OR 2.12 [95% CI 1.70-2.65]; DLco OR 1.97 [95% CI 1.34-2.02]), new-onset ILD (OR 1.91 [95% CI 1.40-2.61]), and new-onset PAH (OR 5.08 [95% CI 3.59-7.19]). These results demonstrate that patient-reported symptoms are associated with clinically meaningful changes in disease activity in patients with SSc. This suggests that when objective measures of change in disease status are unavailable, patient-reported symptoms could be used to indicate a change in SSc disease activity.

Associations between Disease Activity/Severity and Damage and Health-Related Quality of Life in Adult Patients with Localized Scleroderma-A Comparison of LoSCAT and Visual Analogue Scales.

Localized scleroderma (LoS) is a chronic fibrosing disorder of the skin and, less commonly, subcutaneous tissues. As the disease causes subjective symptoms, cosmetic defects, and, at times, functional disability, subjects with LoS experience deterioration of their health-related quality of life (HRQoL). The influence of disease activity/severity and damage status on HRQoL measures in patients with LoS is scarcely known. Physician-reported measures (modified LoS skin severity index, LoS skin damage index, physician global assessments of the disease activity/severity and damage) and patient-derived measures (patient global assessments of the disease activity/severity and damage) were obtained in adult LoS patients. Their HRQoL was measured with Skindex-29 and Short Form-36. The patients’ assessments of disease activity/severity and damage in LoS differed from the assessments by the physicians. The patients’ predominant concerns centered on LoS-related damage, whereas the physicians’ concerns focused on features of disease activity. Visual analogue scales bore some relation to the HRQoL, and they seem to be important in a holistic approach to the patient and should not be omitted in LoS evaluation.

Long-term outcomes in Juvenile Myositis patients

ObjectiveJuvenile idiopathic inflammatory myopathies (JIIM) are rare, chronic autoimmune muscle diseases of childhood, with the potential for significant morbidity. Data on long-term outcomes is limited. In this study we investigate correlations between clinical and demographic features with long-term outcomes in a referral population of adult patients with JIIM. MethodsForty-nine adults with JIIM were assessed at two referral centers between 1994 and 2016. Features of active disease and damage at a cross-sectional assessment were obtained. Regression modeling was used to examine factors associated with long-term outcomes, defined by the presence of calcinosis or a higher adjusted Myositis Damage Index (MDI) score. A multivariable model of MDI was constructed using factors that were statistically significant in bivariate models. ResultsAt a median of 11.5 [IQR 4.5–18.9] years following diagnosis, median American College of Rheumatology (ACR) functional class was 2 [1.5–3.0], Health Assessment Questionnaire (HAQ) score was 0.4 out of 3.0 [0.0–1.0], and manual muscle testing (MMT) score was 229 out of 260 [212.6–256.8]. Median MDI score was 6.0 [3.5–8.9], with the most commonly damaged organ systems being cutaneous and musculoskeletal. Factors associated with an elevated MDI score were the presence of erythroderma and other cutaneous manifestations, disease duration, and ACR functional class. Calcinosis was present in 55% of patients. The strongest predictors of calcinosis were disease duration, periungual capillary changes, and younger age at diagnosis. ConclusionIn a tertiary referral population, long-term functional outcomes of JIIM are generally favorable, with HAQ scores indicative of mild disability. Although most patients had mild disease activity and virtually all had significant disease damage, severe or systemic damage was rare. Certain clinical features are associated with long-term damage and calcinosis.

Pro-inflammatory S100A11 is elevated in inflammatory myopathies and reflects disease activity and extramuscular manifestations in myositis

BackgroundS100A11 (calgizzarin), a member of the S100 family, is associated with oncogenesis, inflammation and myocardial damage. Our aim was to analyse S100A11 in idiopathic inflammatory myopathies (IIMs) and its association with disease activity features and cancer development. MethodsS100A11 in muscle was determined by immunohistochemistry in polymyositis (PM), dermatomyositis (DM), myasthenia gravis (MG) and in subjects without autoimmune inflammatory disease (HC). S100A11 in plasma was measured in 110 patients with IIMs (PM, DM, and cancer associated myositis (CAM) patients) and in 42 HC. Disease activity was assessed by myositis disease activity assessment (MYOACT), muscle enzymes and C-reactive protein (CRP) were measured by routine laboratory techniques; autoantibodies by immunoprecipitation or by immunoblot. ResultsWe observed an accumulation of S100A11 in the cytoplasm of regenerating and necrotizing muscle fibres of PM and DM patients. S100A11 was increased in plasma of all myositis patients compared to HC (3.8 (1.5–16.8) vs 2.8 (1.7–11.2) ng/ml, p = 0.011) and in DM and CAM patients compared to HC (4.0 (2.2–14.9) and 4.5 (1.5–9.1) vs 2.8 (1.7–11.2) ng/ml, p < 0.001 and p = 0.022, respectively). In all myositis patients, S100A11 correlated with the levels of lactate dehydrogenase (r = 0.256, p = 0.011), aspartate aminotransferase (AST) (r = 0.312, p = 0.002), CRP (r = 0.254, p = 0.022) and MYOACT (r = 0.245, p = 0.022). S100A11 was associated with MYOACT (r = 0.377, p = 0.030) and pulmonary and cutaneous disease activity in DM patients (r = 0.408, p = 0.017 and r = 0.417, p = 0.01, respectively). S100A11 was related to the levels of AST (r = 0.412, p = 0.027) in PM and to the levels of creatine phosphokinase (r = 0.432, p = 0.028) in CAM patients. ConclusionsWe show for a first time a potential implication of S100A11 in the local inflammatory and tissue remodelling processes in myositis and an association of circulating S100A11 with disease activity and extra muscular manifestations in DM.

The association of low complement with disease activity in systemic sclerosis: a prospective cohort study.

BackgroundIn some rheumatic diseases such as systemic lupus erythematosus (SLE), low serum complement (‘hypocomplementaemia’) is a feature of active disease. However, the role of hypocomplementaemia in systemic sclerosis (SSc) is unknown. We sought to determine the frequency, clinical associations and relationship to disease activity of hypocomplementaemia in SSc.MethodsThe study included 1140 patients fulfilling the 2013 American College of Rheumatology criteria for SSc. Demographic, serological and clinical data, obtained prospectively through annual review, were analysed using univariable methods. Linear and logistic regression, together with generalised estimating equations, were used to determine the independent correlates of hypocomplementaemia ever, and at each visit, respectively.ResultsAt least one episode of hypocomplementaemia (low C3 and/or low C4) occurred in 24.1 % of patients over 1893 visits; these patients were more likely to be seropositive for anti-ribonucleoprotein (OR = 3.8, p = 0.002), anti-Ro (OR = 2.2, p = 0.002), anti-Smith (OR = 6.3, p = 0.035) and anti-phospholipid antibodies (OR = 1.4, p = 0.021) and were more likely to display features of overlap connective tissue disease, in particular polymyositis (OR = 16.0, p = 0.012). However, no association was found between hypocomplementaemia and either the European Scleroderma Study Group disease activity score or any of its component variables (including erythrocyte sedimentation rate) in univariate analysis. Among patients with SSc overlap disease features, those who were hypocomplementaemic were more likely to have digital ulcers (OR = 1.6, p = 0.034), tendon friction rubs (OR = 2.4, p = 0.037), forced vital capacity <80 % predicted (OR = 2.9, p = 0.008) and lower body mass index (BMI) (OR for BMI = 0.9, p < 0.0005) at that visit, all of which are features associated with SSc disease activity and/or severity.ConclusionsWhile hypocomplementaemia is not associated with disease activity in patients with non-overlap SSc, it is associated with some features of increased SSc disease activity in patients with overlap disease features.

Incidence and predictors for cardiovascular events in patients with psoriatic arthritis

ObjectiveTo assess the incidence and risk factors of cardiovascular events in patients with psoriatic arthritis (PsA).MethodsA cohort analysis was conducted involving patients recruited and followed over the period from 1978...

Depression is a stronger predictor of the risk to consider work disability in early arthritis than disease activity or response to therapy

ObjectivesTo evaluate the factors that influence patients with early inflammatory arthritis to consider a disability pension.MethodsA total of 528 patients aged 63 or younger from an early arthritis cohort with...

Spectrum of infections and outcome among hospitalized South Africans with systemic lupus erythematosus.

Our aim was to determine reasons for admission, the prevalence and spectrum of infections, and the outcomes in a multiethnic cohort of hospitalized systemic lupus erythematosus (SLE) patients in Durban, South Africa. We reviewed the records of hospitalized SLE patients seen over a 79-month period; the demographic data, clinical manifestations, laboratory findings, reasons for admission, nature of infection, and outcome were recorded. Our 167 patients, comprising 59.3% Indians, 33.5% African Blacks, 5.4% Coloreds, and 1.8% Whites, had 327 admissions. Active disease and infections accounted for 218 (66.7%) and 115 (35.2%) admissions respectively, with 58 (17.7%) due to both active disease and infection. Features of active disease were mucocutaneous 33.0%, hematological 30.3%, renal 28.9%, and vasculitis 27.1%. Overall, 83 patients (49.7%) had 155 infections; pneumonia (36.8%), cutaneous sepsis (18.1%), tuberculosis (13.5%), urinary tract infections (12.9%), and septicemia (7.1%) were the most common. The organisms commonly isolated were Staphylococcus aureus 25.4%, Escherichia coli 20.3%, and Klebsiella species and Mycobacterium tuberculosis in 13.6% each. Serositis (odds ratio (OR) = 2.7, p = 0.005) and seizures (OR = 4.8, p = 0.007) were associated with increased risk of infection. Twenty-four (14.4%) patients died from infection and active disease; the patients who died had higher SLEDAI scores (p = 0.02) and longer duration of hospitalization (p = 0.03) but no significant associations on multiple logistic regression analysis. Bacterial infections, including tuberculosis, are common in SLE, and they are a major cause of mortality.

Tu1190 Clinical Outcome and Prediction of Final Diagnosis in Pediatric Inflammatory Bowel Disease Unclassified (IBD-U) Patients

Background: Inflammatory bowel disease (IBD) is a chronic inflammatory condition associated with significant morbidity, including psychiatric disease such as depression. The prevalence of depression, and the effect of anti-tumor necrosis factor alpha (TNFα) therapy on cognitive and somatic symptoms is not yet fully understood. Objectives: To examine (1) the prevalence of depression, using the validated Beck Depression Inventory-II (BDI-II) in patients with IBD, and (2) the association between depression with anti-TNFα therapy, disease type, disease activity, CRP, anemia, fatigue, and health related quality of life (HRQoL). Methods: Using a cross-sectional design in adult outpatients with a confirmed diagnosis of IBD, participants completed questionnaires assessing depression (BDI-II), fatigue (Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-F)), andHRQoL (Short IBDQuestionnaire (SIBDQ)). The BDI-II is categorized into minimal (0-13), mild (14-19), moderate (2028) and severe (29-63) depression, and can be grouped into cognitive and somatic domains. Demographic characteristics were also obtained. Results: Two hundred and ninety-five patients (50.8% female) with IBD (69.8% CD), including 55.2% anti-TNFα were enrolled. The prevalence of mild (14.2%), moderate (11.5%), and severe (7.5%) depressive symptoms in IBD patients was higher than reported population prevalences (13.7%; 7.1%; 2.1%, respectively). The proportion of depressed female patients (37.3%) was not statistically different to that of depressed males (28.9%, p=0.13). There was a trend seen in mean BDIII scores between CD (12.4±10.0) and UC (10.2±8.4, p=0.07) patients. Anti-depressant use (17.1±11.8, p=0.002), clinical assessment of active disease (15.0±10.0, p<0.001), and steroid use (14.1±11.0, p=0.043) were associated with a higher mean depression inventory, but anti-TNFα use (12.4±9.8, p=0.17), high CRP (13.5±9.3, p=0.29), and anemia (13.4±10.6, p=0.25) were not significantly different. In depressed patients, mean HRQoL scores (SIBDQ 3.7±1.4) were significantly lower than non-depressed patients (5.2±1.5, p<0.001). Somatic scores were significantly higher in IBD patients with features of active disease (7.7±4.2 vs 5.1±3.7, p=0.005) or high CRP (7.4±4.0 vs 5.8±4.3, p<0.001). Conversely, those with antiTNFα therapy had significantly more cognitive depressive symptoms (6.3±6.1 vs 4.9±5.7, p=0.047) than those not on biologics, but not somatic symptoms (6.2±4.1 vs 6.0±4.2, p= 0.69). Conclusions: Prevalence of depression in adult outpatient IBD practice is significantly higher compared with the general population, especially in those patients with active disease. AntiTNFα therapy was associated with increased cognitive, but not somatic depressive symptoms.

MxA as a clinically applicable biomarker for identifying systemic interferon type I in primary Sjögren's syndrome

ObjectiveTo establish an easy and practical assay for identifying systemic interferon (IFN) type I bioactivity in patients with primary Sjögren's syndrome (pSS). The IFN type I signature is present in...

THU0209 Efficacy of anti-TNF therapy in 15 patients with refractory takayasu’s arteritis: Long term unicentric follow-up

BackgroundTakayasu arteritis (TA) is a rare chronic-relapsing granulomatous vasculitis involving primarily the aorta and its major branches1. TA is associated with considerable morbidity and mortality2. Therapy is based on corticosteroids...

Sa1261 Association of Family Planning Issues and Quality of Life and in Inflammatory Bowel Disease

In the last decades an ever-growing body of evidence has suggested the involvement of intestinal microvascular endothelial cells in development and maintenance of inflammatory bowel diseases (IBD). Changes in structure and function of endothelium, mediated by a multifaceted network of chemokines, cytokines and inflammatory growth factors, are a distinctive feature of active disease. In addition, human intestinal microvessels taken from inflamed mucosa exhibit consistent degrees of endothelial dysfunction, with significant impairment of acetylcholine-induced vasodilation based on decreased endothelial nitric oxide (NO) synthase (eNOS) activity. By “endothelial dysfunction” is comprehensively meant a condition in which the varied homeostatic functions of endothelium are impaired. Endothelial function can be measured through the flow-mediated dilation (FMD) technique, a wellestablished non invasive procedure in which changes in the calibre of the brachial artery induced by an increased vessel wall shear stress are measured ultrasonographically. AIM: The present study was designed to evaluate whether FMD at the brachial artery was impaired in a sample of patients with Crohn's disease (CD) and ulcerative colitis (UC) in comparison to healthy control subjects. As a secondary measure of interest, intima-media thickness at the common carotid artery (ccIMT) was also calculated. MATERIALS AND METHOD: 49 patients (25 males; mean age, 41 yrs ± 16) with CD (26) and UC (23) in moderate-severe activity phase were included. Endothelial function was assessed through FMD and shear stress reactive hyperemia determination; moreover, ccIMT was measured ultrasonographically. Statistical analyses were made by using Statistica 6.1 software (StatSoft Inc.. Tulsa. OK. USA). RESULTS: In IBD patients FMD% values were significantly lower than in controls (6.3±3.3 vs 8.1±3.4, p=0.013). Such difference was not related to age, sex, disease duration, or comorbidity (such as, hypertension or diabetes). On the contrary, ccIMT measurements did not differ consistently between cases and controls. CONCLUSIONS: IBD group showed significant levels of endothelium dysfunction compared with controls. In our view, this result was more likely related to systemic inflammation than to traditional risk factors. By contrast, the lack of significant differences in ccIMT was possibly related to the low mean age and disease duration in our study population. Future studies of endothelial function are warranted comparing case series before and after remission obtained under elective treatments.

Sa1260 Non Invasive Evaluation of Endothelial Function in Inflammatory Bowel Diseases

In the last decades an ever-growing body of evidence has suggested the involvement of intestinal microvascular endothelial cells in development and maintenance of inflammatory bowel diseases (IBD). Changes in structure and function of endothelium, mediated by a multifaceted network of chemokines, cytokines and inflammatory growth factors, are a distinctive feature of active disease. In addition, human intestinal microvessels taken from inflamed mucosa exhibit consistent degrees of endothelial dysfunction, with significant impairment of acetylcholine-induced vasodilation based on decreased endothelial nitric oxide (NO) synthase (eNOS) activity. By “endothelial dysfunction” is comprehensively meant a condition in which the varied homeostatic functions of endothelium are impaired. Endothelial function can be measured through the flow-mediated dilation (FMD) technique, a wellestablished non invasive procedure in which changes in the calibre of the brachial artery induced by an increased vessel wall shear stress are measured ultrasonographically. AIM: The present study was designed to evaluate whether FMD at the brachial artery was impaired in a sample of patients with Crohn's disease (CD) and ulcerative colitis (UC) in comparison to healthy control subjects. As a secondary measure of interest, intima-media thickness at the common carotid artery (ccIMT) was also calculated. MATERIALS AND METHOD: 49 patients (25 males; mean age, 41 yrs ± 16) with CD (26) and UC (23) in moderate-severe activity phase were included. Endothelial function was assessed through FMD and shear stress reactive hyperemia determination; moreover, ccIMT was measured ultrasonographically. Statistical analyses were made by using Statistica 6.1 software (StatSoft Inc.. Tulsa. OK. USA). RESULTS: In IBD patients FMD% values were significantly lower than in controls (6.3±3.3 vs 8.1±3.4, p=0.013). Such difference was not related to age, sex, disease duration, or comorbidity (such as, hypertension or diabetes). On the contrary, ccIMT measurements did not differ consistently between cases and controls. CONCLUSIONS: IBD group showed significant levels of endothelium dysfunction compared with controls. In our view, this result was more likely related to systemic inflammation than to traditional risk factors. By contrast, the lack of significant differences in ccIMT was possibly related to the low mean age and disease duration in our study population. Future studies of endothelial function are warranted comparing case series before and after remission obtained under elective treatments.

Optimising diagnostic and prognostic investigations in myeloma

In recent years an increasing number of investigations has been devised to improve diagnosis and prognostication in myeloma. Standard investigations range from the characterisation of paraprotein by serum and urine protein electrophoresis, immunofixation and bone marrow biopsy, with the definition of the ‘CRAB’ features of disease activity (hypercalcaemia, renal impairment, anaemia and bone lesions), to the International Staging System based on beta-2 microglobulin and albumin. Plasma cell labelling index measures proliferation by slide-based or flow cytometrical quantitation of replicating S-phase cells, with clear prognostic implications. More recently the quanitita-tion of serum free light chains was developed, based on antibody detection of light chain epitopes that are hidden when bound to heavy chains. The assay is useful, particularly in light chain myeloma, and in the assessment of responses including stringent complete response (sCR). Unique junctional epitopes between heavy and light chain constant regions can also be identified in the ‘heavy-light chain assay’. Response criteria based on para-protein levels correlate with survival and are crucial to the assessment of new therapies; in the detection of minimal residual disease, sCR was added to denote the absence of clonal cells by immunohistochemistry or flow cytometry, in conjunction with a normal free light chain ratio. Conventional and molecular cyto-genetics are some of the most important developments, ranging from the detection of IgH translocations, chromosomal deletions and ploidy changes. Myeloma can be divided into hyperdiploidy and non-hyperdiploidy groups, denoting standard and poor prognosis, respectively, the latter enriched for changes such as del13 and IgH translocations e.g., t(4;14). Basic panels comprise the detection of ploidy changes and del13 by conventional cytoge-netics, t(4;14), t(14;16) and del17p by FISH as high risk markers, and t(11;14) and t(6;14) as standard/good risk markers. Gene expression profiling has provided information on new pathogenic molecules and risk stratification, with specific patterns correlating with proliferation and responsiveness to novel therapies.

A cross-sectional study of the Birmingham Vasculitis Activity Score version 3 in systemic vasculitis

Assessment of disease activity in vasculitis can be achieved using the BVAS, a clinical checklist of relevant symptoms, signs and features of active disease. The aim of this study was to revalidate the BVAS version 3 (BVAS v. 3) in a cohort of patients with systemic vasculitis. A total of 238 patients with vasculitis from seven countries in Europe were evaluated at a single time point. Spearman's correlation coefficients were calculated between BVAS v. 3 scores, vasculitis activity index (VAI), physician's global assessment (PGA), the physician's treatment decision, CRP and the vasculitis damage index (VDI) to demonstrate that the BVAS v. 3 measures disease activity. WG (63%), Churg-Strauss syndrome (9%) and microscopic polyangiitis (9%) were the most common diagnoses. The BVAS v. 3 showed convergent validity with the VAI [ρ = 0.82 (95% CI 0.77, 0.85)], PGA [ρ = 0.85 (95% CI 0.81, 0.88)] and the physician's treatment decision [ρ = 0.54 (95% CI 0.44, 0.62)]. There was little or no correlation between BVAS v. 3 and the CRP level [ρ = 0.18 (95% CI 0.05, 0.30)] or with the VDI [ρ = -0.10 (95% CI 0.22, 0.03)]. The inter-observer reliability was very high with an intra-class correlation coefficient (ICC) of 0.996 (95% CI 0.990, 0.998) for the total BVAS v. 3 score. The BVAS v. 3 has been evaluated in a large cohort of patients with vasculitis and the important properties of the tool revalidated. This study increases the utility of the BVAS v. 3 in different populations of patients with systemic vasculitis.

Clinical characteristics and treatment of subglottic stenosis in patients with Wegener’s granulomatosis

Subglottic stenosis is a complication of Wegener’s granulomatosis caused by tracheal tissue damage and scarring. It may occur as a presenting feature of the disease leading to diagnosis, or instead as a late-stage manifestation. Frequently, it occurs or progresses independently of other features of active disease, and sometimes appears while the general disease is in remission under therapy. The diagnosis of isolated subglottic stenosis may be difficult histologically. Thus, a combination of clinical, histopathological and immunological tests is needed to establish the diagnosis. The management of subglottic stenosis is challenging. A subset of patients may not be responsive to immunosuppressant medication and fixed lesions may require surgical repair. An individualized approach that may include medical and interventional therapies is recommended.

THE CLINICAL DISPLAYS AND THE FUNCTIONAL ACTIVITYOF RIBOSOMAL GENES IN ASTHMATIC CHILDREN

Background. To analyze the interrelations between the parameters of the functional activity of ribosomal genes and some clinical features in asthmatic children. Material and methods. 78 asthmatic patients were included in the study. Standard methods of production and colouring of cytogenetic preparations were used. The functional activity of ribosomal genes was defined on five-point system in arbitrary units. Statistical data processing was spent with the help of applied programs Statistica 6.0. Results. The data about the level of the functional activity of ribosomal genes of asthmatic children (18,57 arbitrary units) were obtained. In all patients the general functional activity of ribosomal genes parameters were correlated with the number of leukocytes and the functional activity of ribosomal genes localized in GHchromosomes parameter -with the level of immunoglobulin E. We constructed the discrimination model according to the disease severity index which included 4 significant parameters: bands number, disease manifestation age, blood sedimentation rate and active ribosomal genes copies number. Conclusion. Our data give evidence about the interrelations between the functional activity of ribosomal genes parameters and some clinical features in asthmatic children. The number of active ribosomal genes copies is considered to be one of the significant features of disease activity.

EULAR Sjögren's syndrome disease activity index: development of a consensus systemic disease activity index for primary Sjögren's syndrome

ObjectiveTo develop a disease activity index for patients with primary Sjögren's syndrome (SS): the European League Against Rheumatism (EULAR) Sjögren's syndrome disease activity index (ESSDAI).MethodsThirty-nine SS experts participated in an...