Hydroxychloroquine as an important immunomodulator: a novel insight into an old drug.

Hydroxychloroquine (HCQ) is an increasingly popular drug owing to its efficacy, long­term safety, and a wide range of therapeutic effects. Currently, due to the numerous benefits it provides, the use of the drug goes beyond the treatment of rheumatic and dermatologic diseases. As HCQ shows anti­inflammatory, immunomodulatory, antiproliferative, and photoprotective action, it has a great potential to be applied also in the treatment of oncologic diseases, multiple sclerosis, diabetes, cardiovascular diseases, or recurrent miscarriages. Nevertheless, antimalarial drugs are still most widely used in the long­term treatment of systemic rheumatic disorders, such as systemic lupus erythematosus (SLE), rheumatoid arthritis, and primary Sjögren syndrome, as they continue to offer satisfactory outcomes. They reduce the need for glucocorticoids and immunosuppressants and increase their effectiveness. In addition, they reduce the risk of possible side effects and complications. This paper presents the latest data on HCQ, its mechanisms of action, its therapeutic potential in current clinical practice as well as future perspectives. It also discusses the correct dosing regimen and long­term monitoring, with consideration of possible rare complications. Finally, it focuses on the enormous benefits for patients with rheumatic diseases in terms of reducing the disease activity and organ damage, preventing flares and pregnancy­related complications, and, most importantly, lowering mortality rates in SLE patients.

Identification of Clinical Response Predictors of Tocilizumab Treatment in Patients with Severe COVID-19 Based on Single-Center Experience.

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 manifestations and their attribution to systemic lupus erythematosus: a retrospective single-center study in a Polish population.

INTRODUCTION: Neuropsychiatric (NP) manifestations occur in patients with systemic lupus erythematosus (SLE), and it is challenging to distinguish these manifestations from other neuropsychiatric conditions. OBJECTIVES: We aimed to assess the prevalence of primary neuropsychiatric SLE (NPSLE) in a Polish cohort of SLE patients. PATIENTS AND METHODS: This retrospective, cross­sectional 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 [SLEDAI­2K] 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. RESULTS: 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 (SLEDAI­2K ≥6) was significantly higher in the NPSLE patients than in those without NP symptoms attributed to SLE (P <0.005; 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. CONCLUSIONS: 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.

Tocilizumab as a first-line biologic treatment in rheumatoid arthritis patients - the impact of concomitant methotrexate treatment and Rheumatic Disease Comorbidity Index on the clinical response - results from the multicenter observational ACT-POL study.

Objectives: According 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 methods: This 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). Results: Total 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. Conclusions: The 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.

2022 Systemic lupus erythematosus remission in clinical practice. Message for Polish rheumatologists.

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.

On-admission laboratory predictors for developing critical COVID-19 during hospitalization - a multivariable logistic regression model.

INTRODUCTION AND OBJECTIVE: 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. MATERIAL AND METHODS: 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. RESULTS: 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 glycaemia >6.8 mmol/L (OR 4.74; 95%CI 1.65-13.66; P = 0.002), immature neutrophils count >0.06/µL (OR 4.06; 95%CI 1.35-12.2; P = 0.012) and urine protein concentration >500 mg/L (OR 2.94; 95%CI 1.04-8.31; P = 0.043). CONCLUSIONS: 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.

Different blood-brain-barrier disruption profiles in multiple sclerosis, neuromyelitis optica spectrum disorders, and neuropsychiatric systemic lupus erythematosus.

AIM OF THE STUDY: 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. CLINICAL RATIONALE FOR THE STUDY: 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. MATERIAL AND METHODS: 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). RESULTS: 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. CONCLUSIONS AND CLINICAL IMPLICATIONS: 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.

Lack of Association between Serum Interleukin-23 and Interleukin-27 Levels and Disease Activity in Patients with Active Systemic Lupus Erythematosus.

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.

TYK2 as a therapeutic target in the treatment of autoimmune and inflammatory diseases.

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.

Work instability and associated factors among patients with rheumatoid arthritis in Greater Poland.

OBJECTIVES: Rheumatoid 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 METHODS: The 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, χ2 test, and Spearman's correlation. RESULTS: 140 (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]). CONCLUSIONS: Pain and disability are the main factors strongly associated with work instability among patients with RA.

Kikuchi-Fujimoto disease associated with primary Sjögren's syndrome - literature review based on a case report.

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.

Factors associated with quality of life in systemic sclerosis: a cross-sectional study.

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 (ß = 2.854, p < 0.001) and anxiety (ß = 0.404, p < 0.001). This model with two factors (functional disability and anxiety) explained 56.7% of the variance in patients with diffuse SSc and 73.2% in those with localized SSc. CONCLUSIONS: Functional disability and anxiety are significantly associated with quality of life in SSc. Interventions aimed at improving either of these factors may contribute towards improving the quality of life of people with SSc.

Autologous hematopoietic stem cell transplant for progressive diffuse systemic sclerosis: procedural success and clinical outcome in 5-year follow-up.

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.

Satisfaction and discontent of Polish patients with biological therapy of rheumatic diseases: results of a multi-center questionnaire study.

OBJECTIVES: Biologics 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 METHODS: An 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. RESULTS: A 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. CONCLUSIONS: Summing 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).

Results from Polish Spondyloarthritis Initiative registry (PolSPI) - methodology and data from - the first year of observation.

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.

Immune Cell Neurotrophin Production Is Associated with Subcortical Brain Atrophy in Neuropsychiatric Systemic Lupus Erythematosus Patients.

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.

Thrombocytopenia resistent to standard therapy in lupus patients - analysis of 3 cases and clinical statement.

Hematologic disorders, including thrombocytopenia, are a common symptom of systemic lupus - Systemic Lupus Erythematosus (SLE). An important diagnostic and therapeutic issue is the severe and recurrent thrombocytopenia resistant to standard treatment. It requires extensive diagnostics and a multi-directional view on its causes beyond the autoimmune process. Currently, there is no single treatment regimen for these disorders. Corticosteroids (CS) are the first-line drugs, but their chronic use is a big problem, they are not always successful and often generate a number of complications, especially in moderate to high doses. In the case of thrombocytopenia associated with the activity of SLE, immunosuppressive therapy is the gold standard and may result in long-term remission of symptoms and clinical stabilization. The major problem is thrombocytopenia resulting from other causes, such as infection or medications. This study discusses severe recurrent thrombocytopenia on the basis of three clinical cases, analyzing both the various causes of disorders, and providing ways of management and treatment. Special attention is paid to the correlation of thrombocytopenia with the clinical and immunological activity of SLE. It seems that severe and refractory thrombocytopenia may be a critical point and largely determine the management and treatment possibilities of SLE.

Assessment of education requirements for patients with rheumatoid arthritis, based on the Polish version of the Educational Needs Assessment Tool (Pol-ENAT), in the light of some health problems - A cross-sectional study.

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. MATERIALS AND METHOD: 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<0.001). There was a positive correlation between the demand for knowledge about the movement (r=0.194; p=0.001), self-care (r=0.134; p=0.026), assistance/support(r =0.163; p=0.006) and morning stiffness experienced. There was a negative correlation between the need for knowledge concerning managing pain, feelings and the arthritis process and daily ability assessed with HAQ DI. CONCLUSIONS: The study shows that health education should be targeted at young patients with early RA. In the case of the severity of morning joints stiffness, there is a need to increase knowledge about the methods of mobility aids, self-care and the possibility of obtaining support.

[Current view on chloroquine derivative treatment from rheumatologist perspective and possible ocular side effects]. / Wspólczesne spojrzenie na leczenie pochodnymi chlorochiny z perspektywy reumatologa a mozliwe powiklania okulistyczne.

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.

Adult-onset Still's disease as a mask of Hodgkin lymphoma.

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.