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Hyperinflammation in COVID-19 plays a crucial role in pathogenesis and severity; thus, many immunomodulatory agents are applied in its treatment. We aimed to identify good clinical response predictors of tocilizumab (TCZ) treatment in severe COVID-19, among clinical, laboratory, and radiological variables. We conducted a prospective, observational study with 120 patients with severe COVID-19 not improving despite dexamethasone (DEX) treatment. We used parametric and non-parametric statistics, univariate logistic regression, receiver operating characteristic (ROC) curves, and nonlinear factors tertile analysis. In total, 86 (71.7%) patients achieved the primary outcome of a good clinical response to TCZ. We identified forty-nine predictive factors with potential utility in patient selection and treatment monitoring. The strongest included time from symptom onset between 9 and 12 days, less than 70% of estimated radiological lung involvement, and lower activity of lactate dehydrogenase. Additional predictors were associated with respiratory function, vitamin D concentration, comorbidities, and inflammatory/organ damage biomarkers. Adverse events analysis proved the safety of such a regimen. Our study confirmed that using TCZ early in the hyperinflammatory phase, before severe respiratory failure development, is most beneficial. Considering the described predictive factors, employing simple and widely available laboratory, radiological, and clinical tools can optimize patient selection for immunomodulatory treatment with TCZ.

Neuropsychiatric (NP) manifestations occur in patients with systemic lupus erythematosus (SLE), and it is challenging to distinguish these manifestations from other neuropsychiatric conditions.We aimed to assess the prevalence of primary neuropsychiatric SLE (NPSLE) in a Polish cohort of SLE patients.This retrospective, cross‑sectional study evaluated 164 patients with SLE. NP manifestations were attributed to SLE using the Italian model. Demographic and clinical data, including disease activity (measured by the Systemic Lupus Erythematosus Disease Activity Index version 2000 [SLEDAI‑2K] and the Physician Global Assessment) and organ damage (measured by the Systemic Lupus International Collaborating Clinics / American College of Rheumatology Damage Index), were obtained in patients with and without NP manifestations attributed to SLE.The final analysis set included 143 patients, 34 of whom (23.8%) had NP manifestations attributed to SLE. The age of the patients with NPSLE and the age of disease onset were significantly lower in comparison with those without NP symptoms attributed to SLE (median [interquartile range], 38 [29-45] vs 45 [32-55] years; P = 0.009, and 35 [24-38] vs 40 [25-48] years; P = 0.03, respectively). The disease activity and proportion of patients with active disease (SLEDAI‑2K ≥6) was significantly higher in the NPSLE patients than in those without NP symptoms attributed to SLE (P0.005; 100% vs 85.3%; P = 0.01, respectively). NP manifestations in the central nervous system were the most frequent (91.5%). In the patients with NPSLE, cerebrovascular disease, seizures, cognitive dysfunction, psychosis, and cranial neuropathy occurred most often.NP manifestations occurred mainly in young patients with high disease activity. Cerebrovascular disease, seizures, psychosis, cognitive dysfunction, and cranial neuropathy were the most frequent manifestations of NPSLE.

Toczen rumieniowaty ukladowy (SLE, systemic lupus erythematosus ) ze wzgledu na liczne manifestacje kliniczne i laboratoryjne jest jedną z najbardziej zlozonych jednostek chorobowych, z ktorymi mają do czynienia reumatolodzy w swojej praktyce klinicznej. Rownie skomplikowana co sama choroba jest jej historia, ktorej początki siegają średniowiecza, ale wlaściwy opis choroby i identyfikacja poszczegolnych manifestacji miala miejsce dopiero w drugiej polowie XX wieku. Historia SLE jest rozdzialem nadal otwartym — w ostatnich latach opracowano nowe kryteria klasyfikacyjne choroby, a takze nowe, skuteczne metody leczenia. Celem pracy bylo przyblizenie rysu historycznego tocznia rumieniowatego ukladowego z uwzglednieniem postepow w zakresie jego definicji, diagnostyki i leczenia.

Recognition of patients with COVID-19 who will progress clinically and need respiratory support remains challenging. The aim of the study was to identify abnormalities in on-admission laboratory results that can precede progression from moderate or severe to critical COVID-19.Laboratory data analyzed of 190 patients admitted with moderate or severe COVID-19 to our ward. Laboratory results taken into analysis were obtained during the first 48 hours of hospitalization. Multivariate logistic regression was performed using risk factors obtained in the univariate analysis as dependent variables.42 patients were identified who developed critical COVID-19. In univariate analysis, 22 laboratory risk factors were detected that were used in logistic regression and in building model with following predictors: high-sensitive troponin I concentration (hs-TnI)26 ng/mL (OR 13.45; 95%CI 3.28-55.11; P 15 (OR 5.67; 95%CI 1.97-16.36, P 50 pg/mL (OR 5.52; 95%CI 1.86-16.37; P = 0.001), fasting glycaemia6.8 mmol/L (OR 4.74; 95%CI 1.65-13.66; P = 0.002), immature neutrophils count0.06/µL (OR 4.06; 95%CI 1.35-12.2; P = 0.012) and urine protein concentration500 mg/L (OR 2.94; 95%CI 1.04-8.31; P = 0.043).The most significant risk factors of developing critical COVID-19 during hospitalization are: elevated hs-TnI, IL-6, and glucose serum concentrations, increased immature neutrophil count, neutrophils to monocytes ratio, and proteinuria during the first 48 hours after admission. The model built with these predictors achieved better predictive performance than any other univariately analysed laboratory markers in predicting the critical development COVID-19.

ObjectivesAccording to the EULAR recommendations, remission or low disease activity (LDA) in rheumatoid arthritis should be achieved by a maximum of 6 months (M6) of treatment. Data on the use of tocilizumab (TCZ) as first-line biologic treatment in rheumatoid arthritis (RA) in routine clinical practice in Poland are lacking.Material and methodsThis multicenter, non-interventional, prospective, observational study recruited adults, presenting with moderate-to-severe RA, showing an inadequate response or intolerance to disease-modifying antirheumatic drugs, where TCZ was the first-line biologic treatment. The effectiveness of TCZ was assessed by the proportion of patients achieving remission and low disease activity following 6 months of treatment with intravenous TCZ. The impact of comorbidities on treatment outcomes was measured using the Rheumatic Disease Comorbidity Index (RDCI).ResultsTotal remission rates at months 3 and 6 were 6% and 31%, respectively. Low disease activity was reported in 10% and 92% of the patients at 3 and 6 months, respectively. The response was comparable between TCZ as monotherapy and in combination with methotrexate. Mean DAS28 decreased from 6.61 at baseline to 4.27 at the scheduled time of the assessment (3 and 6 months). The Rheumatic Disease Comorbidity Index was not correlated with the number of patients achieving LDA at M3 and M6 or remission rates at M6. Remission rates correlated with RDCI at M3. A total of 114 adverse events were reported in 61 patients, among which five were considered as serious.ConclusionsThe study confirms the effectiveness and safety of TCZ in real-world settings as a first-line biologic treatment in patients with moderate-to-severe RA. Importantly, comorbidities do not affect the results of 6-month treatment with TCZ, that is, the optimal time to achieve at least LDA. Our results may improve the effects of RA therapy in Poland, especially in patients with comorbidities and those who, for various reasons, cannot receive optimal treatment with methotrexate.

Systemic lupus erythematosus (SLE) is a complicated multiorgan disease and can lead to organ damage and increased risk of morbidity and mortality. The strategy of management while avoiding complications, especially caused by chronic glucocorticoid therapy, improves outcomes. Different definitions of the treatment goal in different configurations of lupus activity indexes have appeared over the years. In 2021 the definition of remission and recommendations for its achievement were published and it become a way to implement a treat-to-target strategy. The main goal of treatment has become DORIS (definition of remission in SLE) remission and the alternative LLDAS (low lupus disease activity state). Prolonging remission with clinical and immunological lupus activity restrictions and minimizing or stopping steroid doses reduced flares and damage accrual. The analysis and neutralization of poor prognosis predictive factors in lupus could be the most beneficial for less morbidity and mortality and better quality of life.

Primary Sjögren’s syndrome (pSS) is a chronic, autoimmune disease predominantly involving exocrine glands. Lymphadenopathy is one of the possible symptoms of pSS. It may also suggest development of non-Hodgkin lymphoma (NHL), the most severe complication of pSS, or be a symptom of less common diseases, such as Kikuchi-Fujimoto disease (KFD), presented in this paper. Kikuchi-Fujimoto disease is an extremely rare, benign and self-limiting disorder, characterized by regional lymphadenopathy. This paper presents a case of previously unreported association of pSS, KFD and renal cancer in a patient with recurrent cervical lymphadenopathy, as well as a discussion on the coexistence of these diseases based on available literature searching for PubMed, Scopus and Google Scholar databases, particularly in this subject. These three clinical entities may manifest lymphadenopathy each, causing a diagnostic dilemma. The treatment is also challenging under such circumstances. In this particular situation, it was a combination of immunosuppressive therapy and surgery.

To assess differences in BBB damage profiles by measuring serum levels of soluble vascular cell adhesion molecule-1 (sVCAM-1), soluble platelet endothelial cell adhesion molecule-1 (sPECAM-1), soluble intercellular adhesion molecule-1 (sICAM-1), and S100 calcium-binding protein B (S100B) in relapsing-remitting multiple sclerosis (RRMS), neuromyelitis optica spectrum disorders (NMOsd), and neuropsychiatric systemic lupus erythematosus (NPSLE) patients.Blood-brain-barrier (BBB) disruption is one of the key pathological processes involved in various demyelinating diseases of the central nervous system (CNS) and is associated with shedding of cell adhesion molecules and S100B into the serum compartment. Therefore, making an assessment of serum levels of the above-mentioned molecules could provide information about disease pathogenesis, severity of BBB disruption, and disease activity.We recruited 42 RRMS, 19 NMOsd and 35 NPSLE patients. Subjects were treated with beta-interferons or glatiramer acetate (RRMS), oral steroids and/or azathioprine (NMOsd, NPSLE), other immunosuppressants (NPSLE), or antimalarials (NPSLE). The clinical condition of the patients was assessed using the Kurtzke Expanded Disability Status Scale for MS and NMOsd, and the Systemic Lupus Erythematosus Disease Activity Index for NPSLE. Serum levels of sVCAM-1, sPECAM-1, sICAM-1 and S100B were determined using enzyme-linked immunosorbent assay (ELISA).We found the lowest levels of sPECAM-1, sICAM-1 and S100B in sera from NMOsd patients. The highest levels of sPECAM-1 and sICAM-1 were observed in NPSLE, and in NPSLE and MS, respectively. There were no statistically significant differences in sVCAM-1 levels between the examined groups. In MS and NMOsd, there was a negative correlation between the EDSS score and the following molecules: sPECAM-1, sICAM-1 and S100B.We conclude that there is a different profile of blood-brain-barrier disruption reflected by cell adhesion molecules shedding in the spectrum of autoimmune CNS disorders with disseminated white matter lesions. These molecules could become new biomarkers to be used in CNS demyelinating diseases differential diagnoses and monitoring disease activity, but further studies on larger groups of patients are necessary.

Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune disease characterized by the production of multiple autoantibodies, resulting in tissue and organ damage. Recent studies have revealed that interleukin-23 (IL-23) and interleukin-27 (IL-27) may be therapeutically relevant in selected SLE manifestations. This study aimed to identify associations between serum IL-27 and IL-23 levels and disease activity in Polish patients with different manifestations of SLE: neuropsychiatric lupus (NPSLE), and lupus nephritis (LN). Associations between interleukin levels and oligo-specific antibodies against double-stranded DNA (dsDNA), dose of glucocorticoids, and type of treatment were also analyzed. An enzyme-linked immunosorbent assay was used to assess anti-dsDNA antibodies and analyze the serum concentration of IL-27 and IL-23 from 72 patients aged 19–74 years with confirmed active SLE. Disease activity was measured using the Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI 2-K). No significant correlations between interleukin levels and SLEDAI score, anti-dsDNA, corticosteroid dose, or type of treatment were noted. Patients with NPSLE and LN presented the highest median scores of SLEDAI.

JAKs are intracellular protein tyrosine kinases that, through activation of STATs, are responsible for signal transduction pathways that regulate cellular responses to numerous cytokines, growth factors and hormones in many different cells. JAK-STAT signaling plays a key role in regulating immune function, and cytokines – such as IL-23, IL-12 and type I interferons – are central to the pathogenesis of autoimmune diseases, including psoriasis, inflammatory bowel disease and systemic lupus erythematosus. Here the authors review the evidence for targeting TYK2 as a more specific approach to treating these conditions. TYK2 inhibitors are clinically effective in autoimmune and inflammatory diseases and may avoid some of the complications reported with nonselective JAK inhibitors.

Introduction Systemic sclerosis (SSc) is a connective tissue disease characterized by progressive fibrosis of the skin and internal organs, leading to their failure and disturbances in the morphology and function of blood vessels. The disease affects people in different ways, and identifying how the difficulties and limitations are related to quality of life may contribute to designing helpful interventions. The aim of this study was to identify factors associated with quality of life in people with SSc. Methods This was a cross-sectional study conducted in 11 rheumatic centres in Poland. Patients diagnosed with SSc were included. Quality of life was measured using the SSc Quality of Life Questionnaire (SScQoL). The following candidate factors were entered in preliminary multivariable analysis: age, place of residence, marital status, occupational status, disease type, disease duration, pain, fatigue, intestinal problems, breathing problems, Raynaud’s symptoms, finger ulcerations, disease severity, functional disability, anxiety and depression. Factors that achieved statistical significance at the 10% level were then entered into a final multivariable model. Factors achieving statistical significance at the 5% level in the final model were considered to be associated with quality of life in SSc. Results In total, 231 participants were included. Mean age (SD) was 55.82 (12.55) years, disease duration 8.39 (8.18) years and 198 (85.7%) were women. Factors associated with quality of life in SSc were functional disability (β = 2.854, p

Systemic sclerosis is an autoimmune connective tissue disease affecting both skin and internal organs. Progressive disease with multiple organ involvement is considered to have a poor prognosis. Treatment possibilities are limited, but certain patients may benefit from autologous hematopoietic stem cell transplantation (auto-HSCT). We report a case of a 30-year-old woman with progressive diffuse systemic sclerosis treated with parenteral cyclophosphamide with unsatisfactory results. Due to progression of the disease and lack of alternative therapies auto-HSCT was performed. After instituting treatment with autologous hematopoietic stem cell transplantation no immunosuppressive therapy has been required during 5-year follow-up. Improvement in exertion tolerance, partial regression of skin lesions and stabilization of pulmonary and cardiovascular changes were observed. Currently therapeutic options in patients with progressive systemic sclerosis are limited. Hematopoietic stem cell transplantation might become an alternative therapeutic solution not only in the early phase of the disease but also among selected patients with progressive systemic sclerosis resistant to standard therapy.

ObjectivesRheumatoid arthritis (RA) affects patients’ capacity to work. The Rheumatoid Arthritis Work Instability Scale (RA-WIS) is a reliable method to measure work instability (WI) (1–3). We lack data on the relationship between RA and work instability among Polish patients. Our study aimed to assess WI and associated factors among patients with RA.Material and methodsThe authors conducted a multi-centre cross-sectional observational study. 315 patients from three rheumatology centres were enrolled and filled in questionnaires, including demographic and self-reported clinical data, RA-WIS, and the Health Assessment Questionnaire (HAQ). Swollen and tender joint counts (SJC, TJC) were assessed by the attending physician, and current erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were collected. We excluded 41 patients due to an incorrectly filled in form and analysed questionnaires of 274 patients. DAS28 (Disease Activity Score in 28 joints) and DAS28-CRP were calculated. We performed statistical analysis with Statistica v. 13.3 using the Mann-Whitney U test, 2 test, and Spearman’s correlation.Results140 (51%) patients were currently employed and their characteristics were analysed. In univariable analysis we identified the following risk factors for high risk WI: moderate-to-high disease activity (DAS28 ≥ 3.2 – OR 2.29, 95% CI 1.06–4.96, p = 0.033; DAS28-CRP ≥ 3.2 – OR 2.34, 95% CI 1.04–5.27, p = 0.038), ESR ≥ 30 mm/h in women and ≥ 20 mm/h in men (OR 2.65, 95% CI 1.20–5.89, p = 0.010), CRP ≥ 1 mg/dl (OR 4.02, 95% CI 1.78–9.10, p < 0.001), HAQ-DI > 1.0 (OR 2.23, 95% CI 1.04–4.81, p = 0.037) and at least moderate pain on the visual analogue scale (VAS p ≥ 4.5 cm – OR 5.31, 95% CI 2.36–11.96, p < 0.001). Correlations were moderate between RA-WIS and VASp (RS = 0.59, p < 0.001) and HAQ-DI (RS = 0.52, p < 0.001) but weak with disease activity indices (DAS28 [RS = 0.31, p < 0.001]; DAS28-CRP [RS = 0.28, p < 0.001]).ConclusionsPain and disability are the main factors strongly associated with work instability among patients with RA.

Objectives: Report on one-year results from the Polish Spondyloarthritis Initiative registry (PolSPI), containing the cross-sectional analysis of clinical and imaging data as well as database methodology. Material and methods: The PolSPI registry includes patients with axial (axSpA) and peripheral (perSpA) spondyloarthritis according to ASAS classification criteria, and/or patients with ankylosing spondylitis according to modified New York criteria, psoriatic arthritis according to CASPAR criteria, arthropathy in inflammatory bowel disease, reactive arthritis, juvenile spondyloarthritis or undifferentiated spondyloarthritis. Epidemiologic data and history of signs, symptoms and treatment of spondyloarthritis are collected and assessment of disease activity is performed. Radiographic images of sacroiliac joint, cervical and lumbar spine, and results of bone densitometry are collected. Every 6 months blood samples for inflammatory markers, and for long-term storage are taken. Results: During a one-year period from September 2015 to August 2016, 63 patients were registered on an electronic database; 44 (69.8%) of patients were classified as axial spondyloarthritis (axSpA) and 19 (30.2%) as peripheral spondyloarthritis (perSpA) according to ASAS criteria. Statistically significant differences between axSpA and perSpA were discovered in the percentage of HLA-B27 antigen occurrence (92.6% and 50%, respectively), BASDAI (2.8% and 4.1%, respectively), DAS 28 (2.66% and 4.03%, respectively), percentage of peripheral arthritis (20% and 88.8%, respectively), enthesitis (26.7% and 70.6%, respectively), dactylitis (6.7% and 88.9%, respectively), as well as extra-articular symptoms: acute anterior uveitis (26.7% and 5.6% , respectively) and psoriasis (6.9% and 55.6%, respectively). Patients with axSpA had significantly higher mean grade of sacroiliac involvement according to New York criteria, higher mSASSS score, and lower T-score in femoral neck in bone densitometry. Conclusions: At the early stage of the disease patients with axSpA compared to those with perSpA, have more advanced structural damage of sacroiliac joints and spine, and lower bone mineral density in the femoral neck. In the upcoming years the PolSPI registry will prospectively follow-up patients with SpA, recording response to treatment and carrying out research on interaction of inflammation and bone remodelling.

ObjectivesBiologics are medications widely applied in the management of inflammatory rheumatic diseases. The drugs were found to be effective but their application is associated with some disadvantages. Medication with biologics is relatively expensive, and in Poland, it is carried out in specialized centers. The study was designed to evaluate various aspects of satisfaction and dissatisfaction of Polish patients treated with biologics.Material and methodsAn anonymous questionnaire was distributed in 23 Polish rheumatological centers involved in the treatment; 1212 returned questionnaires were used for analysis. Responses were received from 606 patients with rheumatoid arthritis, 427 with ankylosing spondylitis, 117 psoriatic arthritis, and 62 adult patients with juvenile idiopathic arthritis (in whom administration of the drugs had been introduced before they were 18 years old). The investigated group constituted about one-fifth of all rheumatic patients on biologics in Poland.ResultsA beneficial or very beneficial influence of the medication on the state of physical health was found mostly in patients with rheumatoid arthritis (51.3 and 30.5%) and ankylosing spondylitis (51.0 and 36.8%). Family life was improved by the treatment especially in patients with ankylosing spondylitis (40.7 and 35.6% beneficial and very beneficial, respectively), sleep quality and sexual life mostly in those with ankylosing spondylitis (beneficial/very beneficial influence 41.5/38.4, and 38.7/23.9, respectively). There was a rather small influence of biological treatment on the financial situation of the patients. In general, satisfaction with the treatment was evaluated as positive or very positive in 88% of all investigated patients. In a significant part of the patients, transportation to the medical center was considered as a disadvantage of the treatment. About one-third of the patients considered laboratory and imaging tests to be done before initiation of the medication as a difficulty, and for about 40% waiting time for qualification for the medication was a significant disadvantage. The route of drug administration was without importance for 4/5 of the patients.ConclusionsSumming up, the results were similar in the patients suffering from various diseases although those with psoriatic arthritis felt the highest satisfaction (possibly due to the positive aesthetic effect), and those with ankylosing spondylitis had significant improvement in sexual life (probably due to younger age). Relatively low satisfaction was found in patients with juvenile idiopathic arthritis. There was a small influence of medication on financial status of the patients. Application of biologics has few disadvantages and most of them are associated with the organization of health services (waiting time for the tests, transportation to the medical centers).

Adult-onset Still’s disease is a rare disorder, which creates difficulties in making a proper diagnosis. Ambiguous symptoms and results of auxiliary tests, lack of unequivocal diagnostic tests and the need to exclude other causes of the disease are major problems in clinical practice. A case of a 22-year-old woman with dominated recurrent fever, significantly elevated inflammation markers and arthritis is presented. Based on clinical signs after exclusion of infection, hematological and other reasons, the patient was diagnosed with adult-onset Still’s disease. Standard treatment, with high doses of glucocorticoids and a disease-modifying drug, was applied, without the anticipated effects. The diagnostic tests were conducted again due to the lack of clinical improvement, increase of inflammatory markers and unusual response to treatment. A new symptom of significance, i.e. mediastinal lymphadenopathy, was found. After the histopathological examination of lymph nodes, Hodgkin’s disease was diagnosed and targeted therapy for hematological malignancy was applied.

Cognitive impairment is a significant clinical problem both in multiple sclerosis (MS) and systemic lupus erythematosus (SLE) patients. In MS cognitive dysfunction has been associated with brain atrophy and total demyelinating lesion volume. In SLE cognitive impairment is much less understood, and its link to structural brain damage remains to be established. The aim of this study was to identify the relationship between subcortical gray matter volume and cognitive impairment in MS and SLE. We recruited 37 MS and 38 SLE patients matched by age, disease duration and educational level. Patients underwent magnetic resonance imaging (MRI) and a battery of psychometric tests. Severity of cognitive impairment was similar in both cohorts despite larger white matter lesion load in MS patients. Psychometric scores were associated with global and subcortical gray matter atrophy measures and lesion load in MS, but not in SLE. In SLE, the lack of a relationship between cognitive impairment and structural damage, defined either as atrophy or white matter lesions, indicates a different causal mechanism of cognitive deficit.

Objective: Central nervous system (CNS) involvement in systemic lupus erythematosus (SLE) remains poorly understood. Damage within the CNS is driven by the autoimmune response; however, immunopathophysiology of neuropsychiatric (NP) SLE is multifactorial. Immune cell neurotrophin production could be neuroprotective against autoimmunity-driven CNS damage, as has been shown in multiple sclerosis. The aim of this study was to establish whether immune cell neurotrophin production is associated with damage severity in NPSLE. Methods: Selected neurotrophins (BDNF, NGF, NT-3, and NT-4/5) were measured with ELISA within peripheral blood mononuclear cells (PBMCs) isolated from 38 NPSLE patients matched with 39 healthy controls. Subcortical and cortical structure volumes were segmented with the Freesurfer 5.3 pipeline on T1-weighted isotropic images acquired on a 1.5-T MRI scanner. Results: BDNF and NGF levels in PBMCs were reduced in NPSLE compared to the healthy population. The PBMC BDNF level was associated with reduced thalamus, caudate, and putamen volumes. The NGF level correlated with lateral ventricles enlargement and thalamic volume loss. Conclusions: In NPSLE, immune cell BDNF and NGF levels are linked with subcortical atrophy. Higher BDNF levels are associated with higher midsagittal atrophy, which may reflect compensatory mechanisms, upregulating BDNF when neuroprotection is needed. These data require further confirmation.

Introduction Patients with chronic rheumatoid arthritis (RA) need advice in order to face the problems of everyday life, as well as suffering associated with the disease. Health professionals should attempt to raise the level of resourcefulness and independence of the patient. Objective To assess the relationship between the deficit of knowledge about RA and the degree of pain, fatigue, morning stiffness, assessment of disease activity as well as functional efficiency. Material and Methods The study was conducted on 277 patients with RA in 7 rheumatologic centres in Poland. The method applied was the questionnaire Pol-ENAT (0–156); HAQ DI (0–3); analog scales (0–100). Results Mean (SD) age was 53.28 (13.01) and disease duration 13.70 (10.63) years. The mean (SD) value was 54.93 (23.17) for pain, 52.97 (21.98) for fatigue, 48.28 (24.76) for morning stiffness (0–100 mm). HAQ DI was 1.40 (0.66), with an upward trend with duration of disease (p

Anti-malarial drugs specifically hydroxychloroquine (HCQ) or chloroquine (CQ) are very effective in treating and preventing the symptoms of systemic lupus erythematosus and other connective tissue diseases. These medications have shown to improve joint and muscle pain and arthritis, skin rashes, fatique, fever and also to control systemic signs of lupus as pericarditis or pleuritis. Shortterm and long-term treatment reduce cholesterol and have anti-platelet effect with decreasing risk of cardiovascular disease. The lupus patients on anti-malarials have also lower risk of cumulative organ damage due to reduce the amount of steroids. They may help to decrease lupus flares, mortality and are the key to controlling lupus long term outcome. Some lupus patients should be on anti-malarials for the rest of their life. For this reason, the key question is weather these drugs are absolutely safe and can be long term used in all lupus patients as a background therapy? Potential non-specific side effects occur very rare and are usually minor and last for short period. The major concerns are retinal deposits damage which could be potential reversible especially during hydroxychloroquine treatment. Nevertheless, ophthalmologist examination is still needed before starting to take HCQ or CQ and at to follow-up visits every 6-12 months. In conclusion it seems that anti-malarials are safe and have more clinical benefits than risks and from rheumatologist point of view should be more widely use in all lupus patients.

Choroba Dercuma jest bardzo rzadko spotykanym i rozpoznawanymw praktyce klinicznej przewlekłym, postępującym schorzeniemo nieznanej etiologii. Cechą najbardziej charakterystycznąjest obecność mnogich i bolesnych tłuszczaków w tkance podskórnejróżnych okolic ciała. Zmiany guzowate wywołują uciski powodują znaczące dolegliwości bólowe. W patogenezie tej chorobyistotną rolę odgrywają cytokiny zapalne, szczególnie czynnikmartwicy nowotworów α (tumour necrosis factor – TNF-α). Dolegliwościbólowe są związane głównie z zaburzeniami autonomicznegoukładu nerwowego. Najczęściej choroba dotyczy dorosłychotyłych kobiet w wieku pomenopauzalnym. U mężczyzn występujerzadziej – u ok. 16% w wieku dorosłym.Opisano przypadek 49-letniej pacjentki z chorobą zwyrodnieniowąstawów po endoprotezoplastyce prawego stawu biodrowego, którazgłosiła się do lekarza z powodu uporczywych dolegliwości bólowychlędźwiowo-krzyżowego odcinka kręgosłupa oraz licznych bo -les nych guzów tkanki podskórnej. Zmiany guzowate pojawiały sięsukcesywnie od ok. 2 lat, początkowo małe i niebolesne zwiększałyswoje rozmiary, zajmowały nowe obszary ciała i powodowały znaczącedolegliwości bólowe z przeczulicą skóry. Chora była leczonaprzeciwbólowo i przeciwzapalnie bez pożądanej poprawy. Wcześ niejna oddziale dermatologicznym, z uwagi na niewspółmiernie dużedolegliwości bólowe w stosunku do stwierdzanych klinicznie zmianskórnych, wysunięto podejrzenie choroby Dercuma i wykonanobiopsję potwierdzającą zmiany o typie adipositas dolorosa. ChorobaDercuma po raz pierwszy została opisana w 1892 r. przez amerykańskiego neurologa Francisa Xaviera Dercuma z Filadelfii i do dziśpozostaje rzadką, trudną do diagnostyki, różnicowania i leczenia jednostkąkliniczną, która może mieć znaczenie w praktyce reumatologicznej.W artykule omówiono obraz kliniczny choroby Dercuma, jejpatogenezę, podział zmian skórnych (tab. I) oraz określono jej ścisłyzwiązek z codzienną praktyką lekarza reumatologa. Przedstawionodokumentację fotograficzną zmian, które powodowały największedolegliwości bólowe (ryc. 1–3).

Sklerostyna jest białkiem wydzielanym przez osteocyty, które mawłaściwości hamujące proces kościotworzenia. Zidentyfikowanonowe locus w pobliżu genu SOST w miejscu 17q21, które kodujewytwarzanie tego białka. Sklerostyna łączy się z koreceptoramiLRP5 i LRP6 dla lipoprotein o niskiej gęstości i wpływa na aktywnośćbiałek morfogenetycznych kości (BMP) oraz moduluje ścieżkęsygnałową Wnt/β-katenina. Prawdopodobnie ta ścieżka sygnałowaodgrywa najważniejszą rolę w procesie osteogenezy i jest najwszechstronniejw ostatnich latach studiowaną drogą regulatorowąmetabolizmu kości. Mimo olbrzymiego postępu osiągniętegow ostatnich latach w zrozumieniu molekularnych aspektów metabolizmukości, obecnie nie jest dostępna jakakolwiek całkowiciesatysfakcjonująca terapia niskiej masy kostnej i zapobiegająca złamaniom.Prowadzonych jest wiele badań naukowych mających nacelu identyfikację potencjalnych celów terapeutycznych związanychze ścieżką Wnt/β-katenina.

Chora, lat 22, zgłosiła się do szpitala z powodu narastających dolegliwościbólowych w części piersiowo-lędźwiowej kręgosłupa.Pacjentka miała już rozpoznaną homocystynurię, która jest dziedziczonymautosomalnie recesywnie zaburzeniem przemiany aminokwasów,charakteryzującym się uszkodzeniem narządu wzroku,ośrodkowego układu nerwowego, układu sercowo-naczyniowego,układu kostno-szkieletowego oraz tkanki łącznej.W badaniu morfometrycznym (vertebral fracture assessment –VFA) metodą densytometryczną (DXA) stwierdzono wielopoziomowe(T4–L4) złamania trzonów kręgów w części piersioweji lędźwiowej kręgosłupa. Zarówno w badaniu densytometrycznymkości w części L1–L4 kręgosłupa, jak i całego kośćca (total body)wykazano znacznie zmniejszoną gęstość mineralną kości w stosunkudo oczekiwanej dla płci i wieku. Rozpoczęto leczenie kwasemzoledronowym (Aclasta), preparatem z grupy bisfosfonianów,podawanym raz na rok w formie dożylnej. Chora bardzo dobrzetolerowała podanie leku i pozostaje nadal pod ścisłą obserwacją.Osteoporoza w przebiegu homocystynurii stanowi część obrazuklinicznego choroby. Monitorowanie metabolizmu tkanki kostnej,gęstości mineralnej kości oraz zmian strukturalnych w obrębiekręgosłupa za pomocą VFA metodą DXA podczas stosowania bisfosfonianówmoże stanowić istotną wartość dla oceny skutecznościzastosowanego leczenia.

Aim. Assessment of infections prevalence rate, type and severity in patients diagnosed with progressive systemic sclerosis (PSS), treated with cyclophosphamide (CTX), during 12 months of observations. Material and methods. A retrospective analysis of mild, moderate and severe infections in 17 women with a mean age of 58.8 ± 10.0, based on an interview, physical examination, additional tests, and available medical records. Results. 46 various infections were diagnosed in the analysed group of patients. 32 (69.6%) infections involved the respiratory system, and 14 (30.4%) infections concerned the urinary tract. The average frequency per one patient was 2.7 ± 3.5 (median: 2) events during 12 months of observations. The majority of infections, 60.9 % (n = 28), were mild ones of slight intensity, and 37.0% (n = 17) were moderate ones. Only one person (2.2% of all infections) had a severe infection requiring hospitalisation.Conclusions. In the studied group the infection prevalence rate was comparable to that in a healthy population. The majority of infections were mild and involved the respiratory system. Basis of conducted analysis 12 months intravenous administration of CTX is not a factor significantly increasing a risk of severe infections in the studied group of patients. In PSS patients CTX pulse therapy is relatively safe, as it does not cause severe infections requiring hospitalisation

To examine changes in serum levels of the bone remodelling molecules dickkopf-1 (Dkk-1), sclerostin, wingless-type protein-3a (Wnt-3a), and bone morphogenetic protein-7 (BMP-7) during 6 months of anti-tumour necrosis factor (anti-TNF) treatment in ankylosing spondylitis (AS) patients with high disease activity.We included 40 patients with axial AS: 20 patients with high disease activity were assigned to treatment with TNF inhibitor and 20 with low disease activity were assigned to non-steroidal anti-inflammatory drug (NSAID) treatment. Markers of bone remodelling and inflammation were assessed at baseline and after 6 months.In the TNF inhibitor-treated group Dkk-1 decreased significantly from 196.8 pg/mL [95% confidence interval (CI) 94.1-399.0] to 116.3 pg/mL (95% CI 38.0-301.6) and BMP-7 increased significantly from 1.4 pg/mL (95% CI 0-23.0) to 20.3 pg/mL (95% CI 4.9-41.3). There was a significant negative correlation between Dkk-1 and BMP-7 at 6 months (r = -0.64, p = 0.004) in this group. Non-parametric regression analysis adjusted for disease duration, age, sex, baseline modified Stoke's Ankylosing Spondylitis Spine Score (mSASSS), and baseline C-reactive protein (CRP) confirmed a statistically significant effect of treatment on time-related changes of Dkk-1 and BMP-7. Erythrocyte sedimentation rate (ESR), CRP, and also the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) score decreased significantly in the anti-TNF-treated group.Among the potential biomarkers of bone remodelling in AS, Dkk-1 and BMP-7 displayed significant time alterations and correlative interactions during anti-TNF treatment.

Systemic lupus erythematosus (SLE) is a heterogeneous disease, in which B lymphocyte activation and chronic inflammation play the key role. Both the disease itself and its treatment cause damage to multiple organs and systems. So far, despite intensive treatment, disease remission has been achieved in few patients, and the ratio of organ complications has increased significantly. This is caused by a long‑term glucocorticoid therapy with a relatively rare use of immunosuppressive drugs. With a new treatment strategy and modern immunotherapy, it is possible to reduce the mortality rate, limit multiple‑organ damage, thereby significantly improving the quality of life and prognosis of patients with SLE. The "treat‑to‑target" strategy enables targeted treatment resulting in a long‑term symptom remission. It is based on an intensive immunosuppressive treatment with simultaneous reduction of glucocorticoid doses, and limiting their use solely to exacerbations in disease activity. The current idea for treatment is also the conscious use of the beneficial potential of background SLE treatment including antimalarial agents and standard immunosuppressive therapy. With the first biological agent approved for SLE treatment, the new age of therapy has dawned. Biologics offer new prospects and possibilities to induce clinical and immunological remission of SLE.

Asymmetric dimethylarginine (ADMA) is associated with endothelial injury. Increased ADMA levels are found in rheumatoid arthritis (RA) and ankylosing spondylitis (AS). We set out to assess the ADMA and symmetric dimethylarginine (SDMA) levels in AS, RA, and healthy controls, and in the anti-TNF treated patients with active AS.In 78AS patients and 29 RA patients who were anti-TNF treatment naive at baseline, along with 23 healthy control subjects, we assessed erythrocyte sedimentation rate (ESR), high-sensitivity C-reactive protein (hsCRP), ADMA, and SDMA. For AS patients, the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), back pain VAS and patient's global activity of disease were calculated. After 6 months, we repeated the assessment in 30 out of the 78 AS patients in whom the anti-TNF treatment was initiated.The baseline mean (SD) plasma ADMA concentration of AS patients was 0.64 (0.19) μmol/l and did not differ from controls (0.65 [0.19] μmol/l, p0.05). In the RA group, ADMA concentration was higher than in controls (0.77 vs. 0.65 μmol/l, p0.05). Both at baseline and at follow-up, ADMA levels correlated positively with BASDAI (R = 0.52, p = 0.02, and R = 0.47, p = 0.04, baseline and follow-up, respectively). Six months of anti-TNF treatment did not influence ADMA concentration (0.51 [0.12] vs. 0.51 [0.11] μmol/l, p = 0.70).An absence of changes in plasma ADMA levels in the anti-TNF treated AS group despite the improvement in disease activity (BASDAI) and inflammation (ESR, CRP) may suggest either a lack of effect, or, even if such an effect were to take place, it needs not imply measurable changes in blood ADMA.

Selective anticytokine therapy improves clinical outcome in patients with rheumatoid arthritis but does not produce complete remission. The inflammatory network with multiple cell types and cytokines is a multi-factorial phenomenon involving the synovial membrane. Specific inhibition of one factor may result in activation of an alternative (by-pass) route. Thus, the search continues for novel, more aggressive combination therapies. It seems possible to administer a combination of anticytokine drugs or to combine one anticytokine with one B cell-depleting agent (different mechanisms of action). Initial reports on this combination therapy show that it is relatively effective and safe, especially as regards the second option. Results of ongoing clinical trials as well as information on the cost-effectiveness of these therapies are awaited.

Coronary angioplasty (PTCA) is a common treatment method in patients with coronary heart disease, but its effects on heart rate variability (HRV) have not been well established.To verify whether the localisation of coronary lesion undergoing PTCA affects HRV parameters.Ninety six consecutive individuals underwent elective coronary angiography with subsequent ad hoc successful PTCA. Two five-minute ECG were recorded, one before PTCA and the second 24-hour after PTCA. The HRV indices were determined by means of classical and 'new' mathematical models.The PTCA-induced changes in HRV variables depended on the localisation of dilated lesion. PTCA of the circumflex artery revealed the most significant HRV changes--a decrease in value of domain indices: Yeh DI (0.033+/-0.031 vs. 0.011+/-0.006 un/unitless, p=0.005), Yeh II (0.053+/-0.039 vs. 0.032+/-0.013 un, p=0.017), Organ BAND (9.101+/-9.245 vs. 4.62+/-2.205 bpm/beat per minute, p=0.031), Huey STV (208.821+/-262.248 vs. 76.444+/-35.281 bpm, p=0.013), Dalton MABB (15.733+/-16.575 vs. 7.57+/-4.89 ms, p=0.015), Dalton SD (48.741+/-37.468 vs. 27.759+/-10.533 ms, p=0.015), Zugaib STV (0.0129+/-0.0132 vs. 0.005+/-0.003 un, p=0.005), SDNN (27.204+/-18.592 vs. 21.329+/-32.784 ms, p=0.044), rMSSD (56.239+/-19.751 vs. 51.496+/-43.889 ms, p=0.025) and increased LF/HF (2.384+/-2.072 vs. 5.632+/-5.379 un, p=0.044). Angioplasty of the right coronary artery resulted in decreased AR TP (18.273+/-2.296 vs. 17.085+/-2.256 ms(2), p=0.017) and alteration of the sympathovagal balance of the autonomic nervous system towards predominance of sympathetic activity: AR LF (0.264+/-0.029 vs. 0.284+/-0.040 un, p=0.007), LF/HF (4.310+/-4.457 vs. 6.958+/-7.013 un, p=0.018), HF (0.199+/-0.165 vs. 0.141+/-0.157 un, p=0.031), AR HF (0.647+/-0.043 vs. 0.621+/-0.054 un, p=0.014). PTCA of the left anterior descending artery caused no change.Changes in heart rate variability caused by coronary angioplasty depend on the localisation of coronary lesions.

Background Systemic sclerosis (SSc) affects patients9 functioning, quality of life and diminishes their life expectancy. More importantly the chronic progressive nature of the disease threatens the patients9 life. In planning patient education in people with SSc and their families, it is extremely important to identify their health problems as these may determine their educational needs. Objectives (1) To identify health problems and the educational needs in people with SSc. (2) To determine the relationship between the health problems and their educational needs. Methods This was a multicentre cross-sectional study conducted at the Department of Rheumatology and Internal Diseases, in Medical University of Bialystok, and in six other rheumatology centres in Poland. The inclusion criterion was diagnosis of SSc (based on the ACR/EULAR 2013 criteria) and a minimum age of 18 years. Health problems were defined as disability (HAQ-DI), pain (Pain-VAS), fatigue (Fatigue-VAS), morning stiffness (Min), intestinal problems, breathing problems, Raynaud9s phenomenon, fingers ulcers and overall disease severity from the patients perspective (0-100mm VAS). The educational needs were measured using the Polish version of educational needs assessment tool (PolENAT). We used Pearson correlation coefficient (r) to report the effect sizes, 0.10, 0.20 and 0.50 representing small, medium and large effects respectively. Results The sample comprised 140 patients, women being the majority (n=125; 89.3%). They had a mean (±SD) age of 54 years ±14.23 and disease duration of 11 years ±10.27. Reported health problems were particularly severe in patients over 60 years. The biggest influence on daily activity in the whole study group was pain (r=0.294, p There was a positive linear relationship between educational needs and disability (r=0.290, p Conclusions All patients declared the need for education, with greater intensity in the elderly, and longer duration of SSc. Educational needs relate mainly to issues related to the principles of arthritis process, managing pain and self-help measures. Interest in education is dependent on the subjective assessment of the disease and the reported sense of discomfort. References Sierakowska M., et al. Developing the Polish Educational Needs Assessment Tool (Pol-ENAT) in rheumatoid arthritis and systemic sclerosis: a cross-cultural validation study using Rasch analysis. Qual Life Res. 2014. Disclosure of Interest None declared

The current analysis of heart rate variability (HRV) is one of the noninvasive methods of cardiovascular system assessment. The quantitative characteristics of the RR interval sequence and its dynamics are still under development as regards measurement techniques and development of new HRV interpretation models. The practical clinical application of the standard measurements is still insufficient and improvement of sensitivity and specificity of HRV parameters is needed.To assess a novel mathematical model of HRV interpretation and compare it with standard HRV measurements for patients with stable coronary artery disease (CAD), based on the virtual instrumentation technique.The study group consisted of 24 patients with CAD confirmed by coronary angiography and a control group of 15 volunteers. Short-term electrocardiographic signals were recorded by a computer system and analysed for estimation of several HRV descriptors in time, frequency and combined time-frequency domains. Calculations included standard HRV measures and the Allan factor, a parameter based on the Haar wavelet transform.None of the investigated measurements derived from power spectral analysis has shown a statistically significant difference between healthy controls and patients with CAD, with the exception of rMSSD (Wilcoxon test: supine position *p=0.0018, erect position p=0.0708; discriminant function analysis: supine position *p=0.0069, erect position p=0.7851). Compared with standard HRV variables, the Allan factor better discriminated patients with CAD from healthy subjects (Wilcoxon test: supine position *p=0.0172, erect position *p=0.0001; discriminant function analysis: supine position p=0.8962, erect position *p=0.0200).The observations related to the novel parameter based on combined time and frequency domains may provide better quantitative measurements of heart rate variability in patients with coronary artery disease and require further investigations to assess their sensitivity and specificity.

Background Belimumab is a monoclonal antibody, inhibitor of soluble B-cell activating factor, that is used in the adjuvant treatment of adult patients with active seropositive systemic lupus erythematosus (SLE) [1]. Belimumab was approved in Poland in 2011. Objectives To analyze the influence of belimumab therapy on the severity of SLE (expressed as SLEDAI score) and glucocorticoid (GC) use in Polish patients qualified to Early Access Program (EAP). Methods Recruitment to EAP was started in April 2012 in 8 Polish tertiary rheumatology centers. A total of 23 subjects were enrolled. The data of 22 patients (mean age 38.5±8.7 years, 18 women) with at least 3-month follow-up were analyzed. Median time elapsed between the diagnosis of SLE and the enrollment was 7 years (range: 1-39 years). Median SLEDAI score at baseline equaled 13.5 points (range: 6-32 points), and median baseline prednisone equivalent amounted to 11 mg (range: 5-35 mg). Results Median SLEDAI score at 3 months (7 points, range: 3-14 points) was significantly lower than the respective baseline value (p Conclusions The obtained results point to the efficacy of belimumab in the management of SLE. Our findings are similar to those documented in the BLISS trial [2]. References Ginzler EM, Wallace DJ, Merrill JT, et al. Disease control and safety of belimumab plus standard therapy over 7 years in patients with systemic lupus erythematosus. J Rheumatol. 2013 [Epub ahead of print]. van Vollenhoven RF, Petri MA, Cervera R, et al. Belimumab in the treatment of systemic lupus erythematosus: high disease activity predictors of response. Ann Rheum Dis. 2012 Aug;71(8):1343-9. Acknowledgements GlaxoSmithKline provided support for data analysis. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.2880

Background Clinical activity of ankylosing spondylitis (AS) predicts the natural course of the disease and the response to treatment. Several molecules are involved in new bone formation resulting in structural damage in patients with AS. However, the link between the clinical and molecular phenomena has not yet been fully established. The aim of the study was to investigate the relation between markers of bone remodeling and inflammation with clinical activity and structural damage in AS. Methods We assessed the serum levels of sclerostin, Dickkopf-1 protein, Wingless protein-3a, bone morphogenic protein-7, matrix metalloproteinase-3, osteoprotegerin, bone alkaline phosphatase and inflammatory markers in 50 AS patients with high disease activity (BASDAI ≥ 4), 28 with low disease activity (BASDAI