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Systemic sclerosis (SSc) is a rare, chronic autoimmune disease with unknown etiology. Its prominent features are fibrosis, vasculopathy and impaired immune response. Disease can also affect eyes leading to various findings in ophthalmological examination. The objective of this study was to determine the prevalence and type of ocular involvement in patients with SSc. A systematic literature review was conducted using electronic databases. A combination of following keywords was used: “systemic sclerosis” and ophthalmology-related search terms, including the keywords “eye”, “ocular” and “ophthalmic”. In conclusion, eyelid and conjunctival abnormalities and dry eye disease are among the most common ocular manifestations of SSc. Their diversity is connected to complexity of the disease.

The development of tumor necrosis factor alpha (TNF-) inhibitors is probably one of the most significant advancement in the management of a number of inflammatory rheumatic disorders to date. High efficacy of the treatment is associated with relatively rare and very heterogeneous adverse reactions. The reactions are gradually recognized with an increase in number of the patients receiving medication and prolonged period of the drug usage. Neurological adverse events in patients receiving TNF- inhibitors are not common. This finding emergence a question of coincidental occurrence of the nervous system involvements as opposite to suggestion of induction of the nervous system disease or unmasking of latent disease due to the drug administration. Potential association of anti-TNF- therapy with demyelination is one of neurological adverse reaction, and is an important concern for every day clinical practice of rheumatologist as well as is an interesting problem from pathophysiological point of view.

Purpose The aim of the study was to assess the predictors of major adverse cardiovascular events (MACE) in patients with systemic sclerosis (SSc) without pulmonary arterial hypertension. Methods The study comprised 68 patients with SSc who were followed up for the median time of 97 (74.5; 105) months. The main exclusion criteria involved tricuspid regurgitation maximal velocity >2.8 m/s and structural heart disease. At baseline the patients underwent clinical assessment of cardiovascular risk factors, 6-minute walk test, transthoracic echocardiography and biomarker testing for fetuin-A, growth differentiation factor 15 (GDF-15) and N-terminal pro-B-type natriuretic peptide. The primary composite endpoint was onset of MACE defined as death, myocardial infarction, myocardial revascularization and hospitalization for heart failure. The follow-up consisted of outpatient visits at 1 year intervals and telephone interview every 6 months. Results The baseline analysis revealed that chronic kidney disease (HR 28.13, 95% CI: 4.84–163.38), lung fibrosis on high resolution computed tomography (HR 4.36, 95% CI: 1.04–18.26) and GDF-15 concentration (unit HR 1.0006, 95% CI: 1.0002–1.0010) were independent predictors of MACE occurrence. CHLD score was formulated which assigned 1 point for the presence of arterial hypertension, hyperlipidaemia, diabetes mellitus and chronic kidney disease. After inclusion of CHLD score in Cox proportional model, it remained the only independent predictor of MACE onset (unit HR per 1 point 3.46; 95% CI: 2.06–5.82, p Conclusion Joint assessment of traditional risk factors in the form of CHLD score may serve as a reliable predictor of long-term outcome in patients with SSc without pulmonary arterial hypertension. Funding Acknowledgement Type of funding sources: Public Institution(s). Main funding source(s): School of Medicine in Katowice, Medical University of Silesia, Katowice, Poland Figure 1

Introduction: Real-world data indicate disparities in biologic access across Europe. Objectives: To describe the national structure of PsA care in Poland, with a particular focus on the population of inadequate responders (IRs) and difficulties associated with biologic therapy access. Methods: A pool of rheumatologic and dermatologic care centers was created based on National Health Fund contract lists (n = 841), from which 29 rheumatologic and 10 dermatologic centers were sampled randomly and successfully met the inclusion criterium. Additionally, 33 tertiary care centers were recruited. For successful center recruitment, one provider had to recruit at least one patient that met the criteria for one of the four pre-defined clinical subgroups, in which all patients had to have active PsA and IR status to at least 2 conventional synthetic disease-modifying drugs (csDMARDs). Self-assessment questionnaires were distributed among physicians and their patients. Results: Barriers to biologic DMARD (bDMARD) treatment are complex and include stringency of reimbursement criteria, health care system, logistic/organizational, and personal choice factors. For patients who are currently bDMARD users, the median waiting time from the visit, at which the reimbursement procedure was initiated, to the first day of bDMARD admission was 9 weeks (range 2–212, 32% &lt, 4 weeks, 29% 5–12 weeks, 26% 13–28 weeks, 13% with &gt, 28 weeks delay). Out of all inadequate responder groups, bDMARD users are the only group with “good” therapeutic situation and satisfaction with therapy. Patient satisfaction with therapy is not always concordant with physician assessment of therapeutic status. Conclusions: Despite the fact that over a decade has passed since the introduction of biologic agents, in medium welfare countries such as Poland, considerable healthcare system barriers to biologic access are present. Out of different IR populations, patient satisfaction with treatment is often discordant with physician assessment of disease status.

Dercum’s disease (adiposis dolorosa) is a rare disease of unknown etiology characterized by painful subcutaneous adipose tissue deposits with various localization over the body. The deposits occur histologically as lipomas and are associated with overweight or obesity and a variety of psychiatric disturbances (anxiety, depression, sleep disturbances). Classification of Dercum’s disease is related to size and location of adipose nodules (generalized diffuse, generalized nodular, localized nodular and juxta-articular forms). Diagnosis in based on clinical presentation and exclusion of a number of other disorders associated with lipomas. There is no generally accepted management of the patients. Liposuction or lidocaine application has been reported successful in some cases. Other therapeutic methods have been reported but their effectiveness is based on anecdotal descriptions only, and were not confirmed in clinical trials.

Erectile dysfunctions are a common feature in patients with systemic sclerosis. They are resulted from a complex pathomechanism. The main role is attributed to vascular alterations and fibrosis of the carvernous bodies. Depression and disturbed autonomy nervous system function may be additional factors for development of erectile dysfunctions. Diagnosis is based on subjective patient’s data and functional tests. Treatment of erectile dysfunctions in scleroderma patients is difficult, multifactorial and sometimes ineffective. Forum Reumatol. 2019, tom 5, nr 3: 118–123

Whipple’s disease is a rare, chronic, systemic disorder caused by Tropheryma whipplei infection. The most common symptoms are weight loss, arthralgia, diarrhea and abdominal pain. Other organ involvement can also occur in the patients. Joint manifestations may mimic rheumatoid arthritis or spondyloarthritis. Arthalgia, arthritis, spondylodiscitis, bursitis and/or tenosynovitis are seen in the majority of the patients. This explains why some of the symptoms are misdiagnosed as those of rheumatic diseases. Understanding of Whipple’s disease is important for differential diagnostics of several rheumatic symptoms.

Systemic sclerosis (SSc) is an autoimmune disease caused by the imbalance between the activity of angiotensin II and angiotensin-(1–7). Their balance should be controlled by angiotensin-converting enzyme 2 (ACE2), which degrades angiotensin II into angiotensin-(1–7). Previously, autoantibodies to ACE2 (anti-ACE2) were identified in patients with vasculopathy due to different connective tissue diseases, including SSc, but their frequency in SSc was not further analyzed. The aim of the research was to investigate the prevalence and potential role of those anti-ACE2 antibodies in SSc patients. There were enrolled 27 patients with SSc and 23 healthy donors. ELISA assay determined the presence of anti-ACE2 autoantibodies in serum samples. The results were compared to plasma measurements of angiotensin-(1–7) level via commercial ELISA. The presence of anti-ACE2 autoantibodies was confirmed in five patients with SSc and two healthy controls. Two of those SSc subjects were anti-Scl70+, another two were double anti-Scl70+ and anti-Ro/SSA+, and anti-PM/Scl antibodies were detected in one patient. Median plasma level of Ang-(1–7) in anti-ACE2 negative patients was 47.4 pg/ml and stayed below the detection level in anti-ACE2 positive subjects. The plasma level of Ang-(1–7) was undetectable in four SSc patients, and three of them were anti-ACE2 positive. Anti-ACE2 antibodies appear to be other functional autoantibodies with the potential to dysregulate the balance between Ang II and Ang-(1–7). They are non-specific for SSc and probably result from polyautoimmunity which affect some of SSc patients. Their occurrence in SSc settings may be associated with a severe depletion of plasma Ang-(1–7).

Systemic connective tissue diseases (CTDs) comprise a large group of diseases that are auto-immune in nature and characterized by the involvement of multiple systems and organs. Pul-monary hypertension (PH) of various etiologies may develop in the course of CTD, including pulmonary arterial hypertension (PAH), PH secondary to the lung disease, postcapillary PH in the course of left heart disease, and chronic thromboembolic pulmonary hypertension (CTEPH). In addition, the different forms of PH may coexist with each other. Among patients with CTD, PAH occurs most commonly in those with systemic sclerosis, where it affects ap-proximately 8%-12% of patients. The prognosis in patients with untreated PAH is very poor. It is particularly important to identify the high-risk CTD-PAH population and to perform effi-cient and accurate diagnostics so that targeted therapy of the pulmonary arteries can be intro-duced. Echocardiography is used to screen for PH, but clinical and echocardiographic suspicion of PH always requires confirmation by right heart catheterization. Confirmation of PAH ena-bles the initiation of life-prolonging pharmacological treatment in this group of patients, which should be administered in referral centers. Drugs available for pharmacological management include endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and prostacyclins.

Coronavirus disease 2019 (COVID-19) is an infectious disease that became a global health emergency. The paper reviews aspects of COVID-19 that pertain to rheumatology, including symptoms and signs akin to those observed in rheumatic disorders, risk of infection or severe course of the disease in patients with a pre-existing rheumatic disease and those receiving antirheumatic or immunosuppressive medication as well as potential applications of antirheumatic or anticytokine therapeutic strategies that are already applied in rheumatology (including chloroquine, hydroxychloroquine, tocilizumab, baricitinib, and others) for patients with COVID-19.

This study was performed to evaluate the effects of 15-month anti-tumor necrosis factor &alpha, (anti-TNF-&alpha, ) therapy on the aggrecan turnover of female rheumatoid arthritis (RA) patients. Serum was obtained from healthy subjects and female RA patients treated with TNF-&alpha, inhibitors (TNF&alpha, I) in combination with methotrexate. We measured serum levels of aggrecan chondroitin sulfate 846 epitope (CS846), aggrecan fragments (AGC), disintegrin and metalloproteinase with thrombospondin motifs-4 (ADAMTS-4) and 5 (ADAMTS-5), as well as their natural inhibitor, known as tissue inhibitor of matrix metalloproteinase-3 (TIMP-3), using immunoassay methods. Serum levels of CS846, AGC, ADAMTS-4, ADAMTS-5 and TIMP-3 were higher in female patients with RA before the treatment in comparison to healthy subjects. Ratio of ADAMTS-5 to TIMP-3 was significantly higher in RA women than in controls, whereas ADAMTS-4/TIMP-3 ratio did not differ from that in controls. During the anti-TNF-&alpha, therapy, the serum levels of 846 epitope increased, whereas levels of AGC decreased in female RA patients. Furthermore, 15 months of treatment with TNF&alpha, I downregulated serum levels of both ADAMTS, without any effect on TIMP-3 levels. These changes were accompanied by significantly reduced ratios of ADAMTS to TIMP-3. According to our results, anti-TNF-&alpha, therapy has a beneficial impact on aggrecan remodeling during RA.

INTRODUCTION ANCA-associated vasculitides (AAV) is a group of rare disorders where inflammation and damage of the small blood vessels lead to dysfunction of the supplied organs. In severe flares of the disease patients may require intensive care unit (ICU) admission and treatment. The study aims to characterize Polish patients with AAV who were admitted to the ICU and compare them to the others. MATERIAL AND METHODS An observational, retrospective study based on the POLVAS - registry of Polish adult patients with AAV was carried out. Patients admitted to the ICU (ICU group) were identified and compared with the patients who did not require ICU admission (non-ICU group). Characteristics and comparison between groups were made using standard statistic descriptive methods. RESULTS 30 patients admitted to the ICU were identified among 573 cases included in the registry. All patients in the ICU group with available data were ANCA positive. The clinical manifestations related to the ICU admission were respiratory, renal and central nervous system involvement. The treatment regimen for remission induction was similar in both groups. Almost half of the patients in the ICU-group (48.3%) required dialysis, whereas in the non-ICU group it was 21.8% (P = 0.01). Infections were also more frequent in the ICU group (72.4% vs. 36.9% P < 0.001). The mortality rate among patients who needed ICU treatment was significantly higher when compared to the rest of the patients (53.6% vs. 7.8%; P < 0.001). CONCLUSIONS In the Polish AAV cohort one in twenty patients required ICU admission. This group was characterized by multiple organ involvement and high mortality.

ObjectivesInflammation has been revealed to be associated with angiogenesis. Granulomatosis with polyangiitis (GPA) and immune complex small vessel vasculitis (ICSVV) are forms of systemic vasculitides of different pathogenesis. GPA is a necrotizing granulomatosis and ICSVV is associated with inflammation of postcapillary venules induced by deposits of immune complexes. The aim of the study was to determine serum levels of angiostatin and endostatin, natural angiogenesis inhibitors, in patients with GPA and ICSVV as well as healthy individuals.Material and methodsTwo groups of patients with GPA (20 patients) and ICSVV (20 patients) as well as 20 controls were investigated. All patients were investigated before initiation of immunosuppressive therapy or administration of corticosteroids. Angiostatin and endostatin levels were assayed with the ELISA method.ResultsEnhanced serum levels of angiostatin and endostatin were found in patients with GPA but not in those suffering from ICSVV. In patients with GPA increased levels of angiogenesis inhibitors correlated with the disease activity. A correlation between angiostatin and endostatin levels was observed in all groups of investigated individuals.ConclusionsIt is suggested that formation of necrotizing granulation is associated with profound activation of angiogenesis and an increase in serum levels of inhibitors is a phenomenon occurring during blood vessel formation in the granulation tissue. The obtained results confirm involvement of angiogenesis in pathogenesis of at least some forms of vasculitides and suggest the need for continuation of investigations in this field.

Evidence from over 10 years of clinical experience demonstrates that biosimilar medicines approved in the European Union can be used for all their registered indications as safely as their originators and with no negative impact on therapeutic efficacy. The debate on the use of biosimilars in rheumatology focuses specifically on the safety of switching between biosimilars and reference products. Studies conducted to date, including randomised double-blind and open-label extension trials, have not demonstrated any significant differences in therapeutic efficacy or safety between patients switched from one medicine to another and those who were continued on a single medicine. According to the latest recommendations for the use of biosimilars in rheumatic diseases, developed by an international task force in 2017, there is no clinical evidence that a single switch from an originator to a biosimilar medicine is associated with any significant risk for patient safety or reduction in therapeutic efficacy.

Tofacitinib is a newly approved small-molecule targeted synthetic disease-modifying antirheumatic drug. The drug was designed as a selective and specific inhibitor of pro-inflammatory receptor signalling. Tofacitinib inhibits the process of intracellular signalling from the receptor to the cellular nucleus and inhibits the inflammation process via a new pathway (inhibition of the Janus kinases), which is unavailable to biological medicines. Tofacitinib has been approved for use in the treatment of patients with moderate to severe active RA. The drug may be used in combination with methotrexate or another conventional synthetic disease-modifying antirheumatic drug or in monotherapy. The efficacy of tofacitinib has been confirmed in several clinical trials. The drug inhibits radiographic progression of the disease. The innovative mechanism of action of tofacitinib is a noteworthy feature because it offers hope of effective treatment for patients who fail to respond to other drugs. The presented article discusses the mechanism of action and the clinical application of tofacitinib. Tofacitinib represents a new group of disease-modifying antirheumatic drugs that can be placed on an equal footing with biological drugs already available.

Introduction Tumour necrosis factor (TNF-α) is one of the main cytokines participating in inflammation and immune response. Biological effects of the cytokine action, mediated by two receptors: TNFRSF1A and TNFRSF1B involve activation of many signal paths, thus change the transcription activity of many genes. The mechanism of action of an anti-TNF medicine consists in blocking TNF-α though preventing activation of signal paths. Aim To single out mRNA and microRNA genes relating to TNF-α signal paths, the expression of which could indicate sensitivity of cells to the medicine in question. Material and methods The material used in the research consisted in the cell line of regular human skin fibroblasts NHDF (CC-2511 Lonza, Basel, Switzerland) exposed to adalimumab with a concentration of 8.00 μg/ml of the medium for 2, 8 and 24 h, compared with the control material, i.e. non-stimulated cells. Molecular analysis was performed using the oligonucleotide expressive micro-matrices technology HG-U133A, miRNA 2.0 Array micro-matrices and RTqPCR. Results mRNA: BIRC5, MAP3K4, ZFAND5, JUN differentiate cells exposed to the anti-TNF medicine, regardless of the time of cell/medicine incubation. TNF-α transcription activity is reduced during exposure of NHDF cells to adalimumab. miRNA regulating transcription activity of the said 4 mRNA and miRNA related to TNF-α and its receptors was also singled out. Conclusions It was ascertained that adalimumab has therapeutic potential and affects genes engaged in signal paths activated by TNF-α. The results indicate the TNF-α usefulness as the molecular, supplementary marker in diagnostics and control of treatment effects.

Autologous hematopoietic stem cell transplantation (AHSCT) is thought to be effective therapeutic approach in patients with poor prognosis systemic sclerosis; however, the toxicity remains a challenge. Between years 2003 and 2016, we enrolled 18 patients with systemic sclerosis at median age at transplant of 52 years (range 24–68). The median duration of disease before AHSCT was 14 months (range 2–85). Peripheral blood stem cells were mobilized with cyclophosphamide (CY) and granulocyte colony-stimulating factor. Conditioning regimen included CY (200 mg/kg) and alemtuzumab (median dose, 60 mg) [n = 11], melphalan (MEL; 140 mg/m2) and alemtuzumab [n = 2], CY and rabbit anti-thymocyte globulin (rATG; 7.5 mg/kg) [n = 4], and CY alone (n = 1). Four deaths occurred early after transplant. There were three males and one female at median age at death of 51 years (range 24–68). The AHSCT-related deaths have been observed on days + 1, + 4, + 9, and + 15 after procedure. The causes of death included bilateral pneumonia followed by multi-organ failure in three patients and myocardial infarction in one. Three patients expired late during post-transplant follow-up, after 5, 21, and 42 months. The causes of death were disease progression in two patients and sudden heart attack in one. Eleven patients are alive after median follow-up after AHSCT of 42.0 months (range 0–95). Before proceeding to AHSCT in systemic sclerosis, there is a strong need to optimize patient selection to reduce toxicity. The administration of alemtuzumab should be avoided due to high risk of life-threatening infectious complications.

Background:ANCA associated vasculitides (AAV) are a heterogeneous group of rare diseases with unknown etiology and the broad clinical spectrum ranging from life-threatening systemic disease, through single organ involvement to minor isolated skin changes. Unfortunately the clinical classification, ANCA specificity or genetic characteristics alone is not able to categorize AAV patients in a satisfactory manner. As a consequence advanced statistical techniques were used to identify and stratify AAV subphenotypes [1, 2]. Here we have analyzed influence of the ANCA type on clinical manifestations and demographic characteristics in various types of AAV, based on data from the POLVAS registryObjectives:We decided to retrospectively analyze a large cohort of Polish AAV patients deriving from several referral centers – members of the Scientific Consortium of the Polish Vasculitis Registry (POLVAS) – and concentrate on demographic and clinical characteristics of anti-PR3 and anti-MPO positive patients regardless of their clinical diagnosis.Methods:We conducted a systematic multicenter retrospective study of adult patients diagnosed with AAV between Jan 1990 and Dec 2016. Patients were enrolled by 9 referral centers. We analyzed dichotomous variables: gender; ANCA status – anti-PR3+ or anti-MPO+, ANCA negative; organ involvement - skin, eye, ENT, respiratory, heart, GI, renal, urinary, CNS, peripheral nerves and polytomous variable (number of relapses), supported by quantitative covariates (e.g., age at diagnosis, CRP at diagnosis, maximal serum creatinine concentration ever)[3].Results:MPO-positive patients (both GPA and EGPA phenotype) were older at the time of diagnosis with a substantial percentage diagnosed > 65 years of age, and with high rate of renal involvement. Interestingly, while in the whole group of patients diagnosed with EGPA male to female ratio was 1:2, the MPO+ EGPA patients showed M:F ratio of 1:1.The analysis of ANCA negative AAV reveled significant differences in GPA, ANCA negative group is characterized with significantly lower frequency of renal involvement compared to rest GPA (11,5% vs 63,7%) pConclusion:ANCA specificity is indispensable as a separate variable in any clinically relevant analysis of AAV subcategories. MPO+ group is characterized by older age at time of diagnosis, male to female ration 1:1, kidney involvement, and shows more homogenous clinical phenotype than PR3+ AAV patients. In our group ANCA negative AAV never lead to ESRD (end stage renal disease) or even transient dialysis.References:[1]Mahr A, Specks U, Jayne D. Subclassifying ANCA-associated vasculitis: a unifying view of disease spectrum. Rheumatol Oxf Engl 2019;58:1707–9. https://doi.org/10.1093/rheumatology/kez148.[2]Wójcik K, Biedroń G, Wawrzycka-Adamczyk K, Bazan-Socha S, Ćmiel A, Zdrojewski Z et al. Subphenotypes of ANCA-associated vasculitis identified by latent class analysis. Clin Exp Rheumatol. 2020 Sep 1. Epub PMID: 32896241.[3]Wójcik K, Wawrzycka-Adamczyk K, Włudarczyk A, Sznajd J, Zdrojewski Z, Masiak A, et al. Clinical characteristics of Polish patients with ANCA-associated vasculitides—retrospective analysis of POLVAS registry. Clinical Rheumatology. 1 wrzesień 2019;38(9):2553–63.Disclosure of Interests:None declared

Systemic sclerosis is a rare generalized disease with scleroderma, i.e. skin thickening as one of the most common symptoms. The disease has 2 main subsets, diffuse and limited forms. The subset known as systemic sclerosis sine scleroderma (ssSSc) is a very rare subset characterized by the total or partial absence of cutaneous manifestations of systemic sclerosis with the occurrence of internal organ involvement and serologic abnormalities. The classification of ssSSc into 3 groups was proposed. Type I (complete) is characterized by the lack of any cutaneous changes typical for the disease at least until systemic sclerosis-related insufficiency of any internal organ occurs. Type II (incomplete) is characterized by the absence of sclerodactyly, but other cutaneous involvements (e.g. calcifications, telangiectasies, pitting scars) can be found. Type III (delayed) is characterized by clinical internal organ involvement typical for systemic sclerosis that has appeared before skin changes (complete or incomplete). In general, the demographic and clinical characteristics of the ssSSc patients suggest that they are similar to those with diffuse or limited form of the disease. Diagnosis of ssSSc still remains difficult and this disease form should be considered in all cases of unexplained fibrotic involvement of the internal organs.

Edmund Faustinus Biernacki (1866–1911) was a Polish physician and philosopher of medicine. He described erythrocyte sedimentation, designed equipment to measure the erythrocyte sedimentation rate, and applied it to clinical practice. His contribution to the development of one of the most commonly used medical laboratory tests is forgotten, and the test is attributed to other scientists.

Calprotectin also known as MRP8/14 or S100A8/A9 is a heterodimeric complex of two S100 calcium-binding proteins: myeloid-related protein 8 (MRP-8 or S100A8) and MRP-14 (or S100A9). At present, according to many authors, it is considered that calprotectin MRP8/14 is a potentially more sensitive biomarker of disease activity in rheumatoid disease than conventional inflammatory indices such as the erythrocyte sedimentation rate, C-reactive protein and others. A review of the literature on concentration of calprotectin in patients with some rheumatic diseases (rheumatoid arthritis, juvenile idiopathic arthritis, adult-onset Still’s disease, systemic vasculitis, polymyalgia rheumatica, ankylosis spondylitis, systemic lupus erythematosus, and primary Sjögren’s syndrome) is presented.

We present the case of a male patient admitted to the Department of Orthopaedics and Musculoskeletal Traumatology, Medical University of Silesia (MUS) in Katowice, due to a right hindfoot abscess with calcaneal infiltration and pain in the forehead and the back, with evidence of local inflammation. The patient had a history of ulcerative colitis and sclerosing cholangitis was suspected. During the hospital stay, the patient underwent calcaneal CT, surgery (resection of an inflammatory focus) and MRI of the thoracic and lumbosacral spine, which revealed inflamed spinous processes of the Th10, Th11 and Th12 vertebrae. Numerous cultures and histo-pathological examinations were performed. Specialist consultations were obtained. The differential diagnosis included myeloproliferative and lymphoproliferative processes as well as skin and soft tissue neoplasms. The clinical presentation and examination results led to a diagnosis of pyoderma gangrenosum and the patient was referred to the Department of Internal Medicine and Rheumatology of the MUS to undergo further treatment.

BACKGROUND Subcutaneous methotrexate (sMTX) administration is considered more effective than the oral route due to better bioavailability and a lower rate of adverse drug reactions (ADRs); however, clinical data supporting this hypothesis is scarce. OBJECTIVES The aim of the study was to evaluate the efficacy and tolerability of sMTX in patients with active rheumatoid arthritis (RA), including a subset classified as an early stage of RA. MATERIAL AND METHODS A post-marketing, multicenter, open-label, non-randomized, non-interventional study enrolled 771 adult patients with active RA treated with sMTX (Metex®) for 2-6 weeks. The evaluation of therapy effectiveness (DAS28-ESR or DAS28-CRP) and monitoring of ADRs was an element of routine patient management. Therapy effectiveness was scored as the achievement of remission or response (according to European League Against Rheumatism (EULAR)). RESULTS Among 761 (98.7%) patients that continued sMTX (after 25-31 weeks), clinical response was achieved by 69.5%, remission by 19.2% and low disease activity by 34.2%. Patients aged >60 years were less likely to achieve both remission (odds ratio (OR) = 0.61 (95% confidence interval (95% CI) = 0.39-0.93)) and clinical response (OR = 0.82 (95% CI = 0.71-0.95)), while overweight/obese patients (OR = 1.11 (95% CI = 1.00-1.24)) and those with early RA had greater chance to reach a clinical response (OR = 1.18 (95% CI = 1.03-1.34)). There were 16 ADRs (no serious or severe). In addition, at least 2-fold increase in alanine transaminase (ALT) activity was noted in 10 patients (1.3%). CONCLUSIONS After 6-month therapy with sMTX, about 70% of patients with RA achieve a clinical response, and remission was observed in 20%. Younger age, overweight/obesity and an early stage of the disease are factors increasing therapy effectiveness; sMTX is well tolerated.

BACKGROUND Systemic sclerosis (SSc) is a multisystemic disease with an extensive microvasculopathy. Previously, disturbances in plasma levels of angiotensin II (Ang II) and its antagonistic angiotensin-(1-7) (Ang-(1-7)) were found in patients with SSc. Their significance in a pathogenesis of SSc stays unclear due to discrepancies of earlier studies. OBJECTIVES To evaluate a significance of disturbances in production pathway of angiotensins in a development of SSc. METHODS There were enrolled 27 patients with established SSc, 23 subjects with very early SSc and 23 healthy controls. The diagnosis of SSc was established in patients who met EULAR/ACR 2013 classification criteria. Very early SSc described patients with Raynaud's phenomenon having SSc-specific antinuclear antibodies and SSc-like abnormalities in nailfold videocapillaroscopy. Patients were submitted to evaluation of internal organ involvement and blood sampling to assay plasma levels of angiotensin I, angiotensin II and angiotensin-(1-7) with ELISA technique. RESULTS Plasma level of angiotensin-(1-7) was significantly reduced in both SSc group (median = 47.2 pg/mL; P

Background Sandoz etanercept (SDZ ETN; GP2015) is an etanercept biosimilar with equivalent efficacy and comparable safety and immunogenicity to reference etanercept (ETN) in patients with moderate-to-severe chronic plaque-type psoriasis. Methods EQUIRA was a phase III, double-blind study conducted in patients with moderate-to-severe rheumatoid arthritis and inadequate response to disease-modifying anti-rheumatic drugs. Eligible patients were randomized 1:1 to receive subcutaneous 50 mg SDZ ETN or ETN, once-weekly, for 24 weeks. At week 24, patients with at least moderate EULAR response in the SDZ ETN group continued SDZ ETN treatment, and those in the ETN group were switched to receive 50 mg SDZ ETN, for up to 48 weeks. Patients received concomitant methotrexate at a stable dose (10–25 mg/week) and folic acid (≥ 5 mg/week). Equivalence between SDZ ETN and ETN for change from baseline in disease activity score including 28 joint count C-reactive protein (DAS28-CRP) at week 24 (primary endpoint) and comparable safety and immunogenicity profile of SDZ ETN and ETN have previously been demonstrated at week 24. Herein, we present the 48-week results of the study after a single switch from ETN to its biosimilar at week 24. Results The least squares mean (standard error) change in DAS28-CRP from baseline up to week 48 was comparable between “continued SDZ ETN” (− 2.90 [0.12], n = 148) and “switched to SDZ ETN” (− 2.78 [0.13], n = 131) groups. The proportion of patients achieving EULAR good/moderate responses based on DAS28-erythrocyte sedimentation rate and ACR20/50/70 response rates were comparable between the two groups. The proportion of patients with at least one treatment-emergent adverse event was 42.9% in the “continued SDZ ETN” and 38.0% in the “switched to SDZ ETN” groups. Serious adverse events occurred in 4 patients in each of the two groups. After week 24, none of the patients in the switched group developed anti-drug antibodies (ADAs), while 4 patients in the continued SDZ ETN group had single-event, very low titer, non-neutralizing ADAs detected. Conclusions The 48-week results from the EQUIRA study demonstrate that switch from ETN to SDZ ETN in patients with moderate-to-severe rheumatoid arthritis does not impact the efficacy, safety, or immunogenicity of etanercept. Trial registration EudraCT number 2012-002009-23, Registered 19 April 2012—prospectively registered. Electronic supplementary material The online version of this article (10.1186/s13075-019-1907-x) contains supplementary material, which is available to authorized users.

Purpose The aim of this study is to present the treatment modalities and associated side effects in a Polish nation-wide ANCA-associated vasculitides (AAV) patients’ cohort. Materials and methods Retrospective analysis of patients diagnosed with AAV between 1990 and 2016, included in the POLVAS registry was performed. Standard descriptive statistic methods were used with an emphasis on the treatment modalities. Results There were 625 patients diagnosed with AAV included in this study: 417 cases of granulomatosis with polyangiitis (GPA; 66.7%), 106 cases of microscopic polyangiitis (MPA; 17.0%) and 102 cases of eosinophilic granulomatosis with polyangiitis (EGPA; 16.3%). The mean age at the date of diagnosis was 50.4 (±15.7) years and the median observational period amounted to 4.0 (2.0–8.0) years. Glucocorticosteroids (GCs) were the medicaments most frequently used for remission induction (593/622; 95.3%), followed by cyclophosphamide (487/622; 78.3%), rituximab (44/622; 7.1%), and methotrexate (39/622; 6.3%). GCs were also most frequently administered for maintenance therapy (499/592; 84.3%), followed by azathioprine (224/592; 37.8%), methotrexate (136/592; 23.0%) and mycophenolate mofetil (99/592; 16.7%). The median cumulative doses of cyclophosphamide and rituximab equalled 7.99 g (4.18–14.0) and 2000 mg (1500–2800), respectively. The most commonly observed adverse events included: infections - 214/551 cases (38.8%), which were associated with the time of observation (OR = 1.05; 95% CI 1.01–1.10), the use of GCs intravenous pulses (OR = 2.76; 95% CI 1.68–4.54) and need for haemodialysis (OR = 1.73; 95% CI 1.10–2.71). Conclusions Polish patients with AAV were predominantly treated according to appropriate guidelines. The most frequent adverse events were typical for usually administered immunosuppressive treatment.

Objective Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare small to medium-size vessel systemic diseases. As their clinical picture, organ involvement, and factors influencing outcome may differ between countries and geographical areas, we decided to describe a large cohort of Polish AAV patients coming from several referral centers-members of the Scientific Consortium of the Polish Vasculitis Registry (POLVAS). Methods We conducted a systematic multicenter retrospective study of adult patients diagnosed with AAV between Jan 1990 and Dec 2016 to analyze their clinical picture, organ involvement, and factors influencing outcome. Patients were enrolled to the study by nine centers (14 clinical wards) from seven Voivodeships populated by 22.3 mln inhabitants (58.2% of the Polish population). Results Participating centers included 625 AAV patients into the registry. Their distribution was as follows: 417 patients (66.7%) with GPA, 106 (17.0%) with MPA, and 102 (16.3%) with EGPA. Male-to-female ratios were almost 1:1 for GPA (210/207) and MPA (54/52), but EGPA was twice more frequent among women (34/68). Clinical manifestations and organ involvement were analyzed by clinical phenotype. Their clinical manifestations seem very similar to other European countries, but interestingly, men with GPA appeared to follow a more severe course than the women. Fifty five patients died. In GPA, two variables were significantly associated with death: permanent renal replacement therapy (PRRT) and respiratory involvement (univariate analysis). In multivariate analysis, PRRT (OR = 5.3; 95% confidence interval (CI) = 2.3–12.2), respiratory involvement (OR = 3.2; 95% CI = 1.06–9.7), and, in addition, age > 65 (OR = 2.6; 95% CI = 1.05–6.6) were independently associated with death. In MPA, also three variables were observed to be independent predictors of death: PRRT (OR = 5.7; 95% CI = 1.3–25.5), skin involvement (OR = 4.4; 95% CI = 1.02– 19.6), and age > 65 (OR = 6.3; 95% CI = 1.18–33.7). Conclusions In this first multicenter retrospective study of the Polish AAV patients, we have shown that their demographic characteristics, disease manifestations, and predictors of fatal outcome follow the same pattern as those from other European countries, with men possibly suffering from more severe course of the disease.

Low back pain (LBP) or pain of the lumbo-sacral region of the vertebral column is a polyetiological clinical state. LBP is a common medical condition and has a recurrent nature. The incidence of the first LBP episode in life varies from 6.3 to 15.4% a year, while the total annual LBP incidence is estimated at up to 36%. The cause of LBP can be located in the osseous structures and the joints of the vertebral column, the intervertebral discs, as well as the muscles, ligaments and nerves of the lumbo-sacral region. Commonly, the disease mechanism is complex. Differential diagnosis is an important part of the management of LBP patients due to a number of potential causes. Some forms of LBP need emergency management, and their features are known as “red flag symptoms”. Such LBP forms occur at night and lack any connection with physical activity (especially in the early stage of the disease). Rapidly deteriorating LBP also needs to be considered as an emergency. Inflammatory LBP occurs in patients with inflammatory spondyloarthropathies (in 70–80% of the patients). The presented recommendations are designed to facilitate the identification of patients with inflammatory LBP. They also describe rules of referring to a rheumatologist, as well as focus on the cooperation of a family doctor and a rheumatologist in the treatment of these patients. In most patients LBP is the first symptom of inflammatory spondyloartropathy. The classification criteria of inflammatory LBP are as follows: 1) onset at the age below 40; 2) insidious onset; 3) an improvement after physical exercise; 4) the lack of improvement after rest; 5) pain at night with improvement after getting up from bed. Non-pharmacological (kinesiotherapy, patient education) and pharmacological methods (non-steroidal anti-inflammatory drugs, TNF -alpha blockers) are used in the management of patients with inflammatory spondyloartropathy.

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Background:ANCA associated vasculitides (AAV) are a heterogeneous group of rare diseases with unknown etiology and the clinical spectrum ranging from life-threatening systemic disease, through single organ involvement to minor isolated skin changes. Thus there is an unmet need for phenotype identification especially among patients with granulomatosis with polyangiitis GPA, patients with microscopic polyangiitis MPA group seems to be more uniform. Recently, based on previous clustering analysis and clinical, histopathological, serological and prognostic aspects three subcategories of AAV have been proposed and named as: non-severe AAV, severe PR3-AAV and severe MPO-AAV [1].Objectives:In line with these attempts to subcategorize AAV we decided to use latent class analysis (LCA) on a large multicenter cohort of polish AAV patients from POLVAS [2] registry to identify potential new subphenotypes or confirm already proposed ones.Methods:Latent Class Analysis (LCA) approach was used as a model based clustering method of objects described by dichotomous (e.g., gender; ANCA status – cANCA, pANCA; organ involvement - skin, eye, ENT, respiratory, heart, GI, renal, urinary, CNS, peripheral nerves) and polytomous (number of relapses) variables supported by quantitative covariates (e.g., age at diagnosis, CRP at diagnosis, maximal serum creatinine concentration ever).Results:Results of LCA on our AAV group returned four class model of AAV subphenotypes, confirming existence of the previously proposed by Mahr at al. [1] and revealed fourth – previously not described clinically relevant subphenotype. To this fourth class - belong patients only with GPA, diagnosed at young age, with multiorgan involvement, high relapse rate and relatively high risk of death.Table 1.AAV subcategorization – summary of clinical characteristics and ANCA specificityLCA Class 1LCA Class 2LCA Class 3LCA Class 4No of patients13019410297AAV typeMainly GPAMainly GPAmainly MPAOnly GPAAge at diagnosisMiddle ageMiddle ageOldYoungMale/female ratio1:22:11:11:1Main organ involvementENT, respiratory, eyeRenal, respiratory, ENTRenal, respiratory, skinMultiorgan involvementRelapse rateintermediateintermediatelowhighModified class description (based on ref. [1])Non severe AAVSevere PR3 AAVSevere MPO AAVSevere non-renalPR3 AAVConclusion:Based on multiple clinical and serological variables LCA methodology identified 4-class subphenotypes model of AAV. Fourth-class is a new clinically important subphenotype including exclusively PR3-positive young AAV patients with multiorgan involvement, high risk of relapse and distinct mortality.References:[1]Mahr A, Specks U, Jayne D. Subclassifying ANCA-associated vasculitis: a unifying view of disease spectrum. Rheumatol Oxf Engl 2019;58:1707–9.https://doi.org/10.1093/rheumatology/kez148.[2]Wójcik K, Wawrzycka-Adamczyk K, Włudarczyk A, Sznajd J, Zdrojewski Z, Masiak A, i in. Clinical characteristics of Polish patients with ANCA-associated vasculitides—retrospective analysis of POLVAS registry. Clinical Rheumatology. 1 wrzesień 2019;38(9):2553–63.Disclosure of Interests:Krzysztof Wójcik: None declared, Adam Ćmiel: None declared, Anna Masiak: None declared, Zbigniew Zdrojewski: None declared, Radoslaw Jeleniewicz: None declared, Maria Majdan Consultant of: Roche, Amgen, Speakers bureau: Roche, Amgen, Iwona Brzosko: None declared, Marek Brzosko: None declared, Piotr Głuszko: None declared, Małgorzata Stasiek: None declared, Małgorzata Wisłowska: None declared, Joanna Kur-Zalewska: None declared, Marta Madej: None declared, Anna Hawrot-Kawecka: None declared, Hanna Storoniak: None declared, Barbara Bułło-Piontecka: None declared, Alicja Dębska-Ślizień: None declared, Eugeniusz Kucharz: None declared, Katarzyna Jakuszko: None declared, Jacek Musiał: None declared

Background:Orbital inflammatory masses have been described as the common manifestation of granulomatosis with polyangiitis (GPA) occuring in 7- 45% of patients.Objectives:Identification and characterization of patients with orbital pseudotumor among Polish patients based on the national vasculitis registry, POLVAS.Methods:Clinical presentation and management of all GPA patients fulfilling ACR criteria or Chapel Hill Consensus Conference definition included to the Polish registry POLVAS who developed orbital masses in the course of GPA were evaluated.Results:Ocular involvement was found in 114 (27%) of 417 GPA patients registered in POLVAS, 34 (8%) developed orbital masses. Mean patients’ age was 47.8 (range from 19-75) yrs., 23 (67%) were women. Forty four per cent of the patients developed tumor at the beginning of the disease, 56% during relapse. Patients’ characteristics on diagnosis of orbital mass: 24 cANCA, 2 pANCA, and 8 ANCA negative, 9% active smokers and 31% past smokers, 29% had localized disease, 21% early systemic and 50% systemic with organ involvement, 29% had other type of ophthalmological involvement before pseudotumor occurred, 88% had active paranasal sinus involvement, 41% lungs, 15% CNS, 15% skin and 6% heart manifestations. Thirty seven per cent of patients had positive nasal swabs cultures, 50% of which were positive for Staphylococcus aureus. In 65%, tumor occurred during steroid therapy (46% had prednisone more than 5mg/d) and 45% on immunosuppressive treatment (19% when treated with AZA, 16% MTX, 6,5% MMF and 3,5% CYC). Due to orbital mass 86.5% were treated with CYC and 13.5% with RTX. Twenty one per cent had complete remission of the pseudotumor, 76% partial remission and in 3% patients there was no response to the treatment; 43% developed visual impairment, 20% suffered from blindness.Conclusion:Orbital inflammatory mass was not common manifestation of GPA among our patients. The mass developed at the beginning or in the course of the disease, even during immunosuppressive treatment. Orbital masses have been resistant to therapeutic interventions and were accompanied by high risk of visual impairment.Disclosure of Interests:Anna Masiak: None declared, Marcin Ziętkiewicz: None declared, Krzysztof Wójcik: None declared, Katarzyna Wawrzycka-Adamczyk: None declared, Radoslaw Jeleniewicz: None declared, Marta Madej: None declared, Joanna Kur-Zalewska: None declared, Katarzyna Jakuszko: None declared, Małgorzata Wisłowska: None declared, Hanna Storoniak: None declared, Michał Komorniczak: None declared, Barbara Bułło-Piontecka: None declared, Iwona Brzosko: None declared, Małgorzata Stasiek: None declared, Eugeniusz Kucharz: None declared, Alicja Dębska-Ślizień: None declared, Maria Majdan Consultant of: Roche, Amgen, Speakers bureau: Roche, Amgen, Jacek Musiał: None declared, Zbigniew Zdrojewski: None declared

ObjectivesTo demonstrate the equivalent efficacy and compare the safety and immunogenicity of an etanercept biosimilar, GP2015, with reference etanercept (ETN) in patients with moderate-to-severe, active rheumatoid arthritis (RA), characterised by an inadequate response to synthetic or biologic disease-modifying antirheumatic drugs (DMARDs).MethodsIn the EQUIRA study, eligible patients (n=376) were randomised 1: 1 to 50 mg GP2015 or ETN subcutaneously, once weekly, for 24 weeks (treatment period 1). Patients from both groups, with at least moderate European League Against Rheumatism response at week 24, received GP2015 up to week 48 (treatment period 2). All patients continued to receive concomitant methotrexate at a stable dose (10–25 mg/week) until end of the study. The 24-week results are presented here.ResultsEquivalent efficacy between GP2015 and ETN was demonstrated if the 95% CI for the difference in disease activity score 28-joint count C reactive protein (DAS28-CRP) change from baseline to week 24 between treatment arms was contained within the prespecified equivalence margin range of −0.6 to 0.6. The least squares mean difference (GP2015–ETN) in change from baseline in DAS28-CRP up to week 24 was −0.07 (95% CI −0.26 to 0.12 [primary endpoint)]. The incidence of treatment-emergent adverse events was comparable between GP2015 (43.5%) and ETN (49.5%). None of the GP2015-treated patients developed neutralising anti-drug antibodies (NAbs) whereas 1.6% and 0.6% of patients in ETN group were NAb positive at weeks 4 and 12, respectively.ConclusionIn patients with RA who had an inadequate response to DMARDs, GP2015 demonstrated a similar efficacy and a comparable safety and immunogenicity profile with ETN.Trial registrationNCT02638259.

Background GP2015 is an etanercept biosimilar. It has shown an equivalent efficacy, and comparable safety and immunogenicity to ETN in patients with chronic plaque-type psoriasis.1 Objectives To compare the efficacy and safety of GP2015 versus ETN in patients with moderate-to-severe rheumatoid arthritis (RA) and evaluate the effects of switching from ETN to GP2015. Methods EQUIRA was a 48 week, randomised, double-blind, Phase 3 study. The primary endpoint was equivalent change from baseline (BL) in DAS28-CRP at Week 24. Patients≥18 years with active RA ACR 1987 or ACR/EULAR 2010 criteria for ≥6 months before BL and active disease defined as DAS28-CRP≥3.2 and CRP >5 mg/L or ESR ≥28 mm/h) and inadequate response to methotrexate (MTX) were randomised 1:1 to 50 mg GP2015 or ETN subcutaneously once weekly for 24 weeks (Treatment period 1). Patients with at least moderate EULAR response at Week 24 either continued GP2015 treatment or, in the ETN group, were switched to receive 50 mg GP2015 up to 48 weeks (Treatment period 2 [TP2]). All patients continued to receive concomitant MTX (10–25 mg/week) at a stable dose and folic acid. Efficacy outcome measures included change in DAS28-CRP, EULAR and ACR20/50/70 responses. Results Baseline characteristics were comparable between the GP2015 (n=186) and ETN (n=190) groups. The primary endpoint for equivalence was met.2 The mean change in DAS28-CRP from BL to Week 48 was comparable between the continued and switched to GP2015 groups (TP2 per-protocol set; figure 1). At Week 48, the EULAR and ACR 20/50/70 response rates were comparable between the two groups (table 1). In TP2, treatment-emergent adverse events (AEs) occurred in 42.9% vs 38.0% patients in the continued GP2015 (n=175) vs the switched (n=166) groups; serious AEs occurred in 2.3% vs 2.4% patients (TP2 safety set). Injection site reactions occurred in 6 (3.6%) patients in the switched group but none in the continued GP2015 group. In TP2, 4 (2.4%) patients in the continued GP2015 group had single-event, very low titer, non-neutralising antidrug antibodies detected. Conclusions The efficacy of GP2015 was comparable to that of ETN. Moreover, the switch from ETN to GP2015 did not impact on efficacy and safety of etanercept in patients with moderate-to-severe RA. References [1] Griffiths CEM, et al. Br J Dermatol2017;176:928–38. [2] Kavanaugh A, et al. Arthritis Rheumatol2017;69(suppl 10). Disclosure of Interest M. Matucci-Cerinic Grant/research support from: Actelion, Beyer, BMS, Chemomab, Inventiva, Pfizer and Sandoz, H. Schulze-Koops: None declared, M. Buch Grant/research support from: Abbvie, AstraZeneca, Eli Lilly, Pfizer, Roche, Sandoz and UCB, Consultant for: Abbvie, AstraZeneca, Eli Lilly, Pfizer, Roche, Sandoz and UCB, A. Kavanaugh Consultant for: Sandoz, Merck and Boehringer-Ingelheim, Y. Allanore Grant/research support from: Pfizer and Sandoz, Consultant for: Pfizer and Sandoz, E. J. Kucharz Consultant for: AbbVie, Berlin Chemie, Biogen, Celgene, Egis, Eli Lilly Polska, MSD, Novartis, Pfizer, Polpharma, Roche, Sandoz and UCB Biopharma, G. Babic Employee of: Hexal AG

Background In this study, the effect of 15-month anti-tumor necrosis factor alpha (TNF-α) treatment on circulating levels of plasma sulfated glycosaminoglycans (GAGs) and the nonsulfated GAG hyaluronic acid (HA) in female rheumatoid arthritis (RA) patients was assessed. Methods Plasma was obtained from healthy subjects and RA women treated with TNF-α antagonists (etanercept or adalimumab or certolizumab pegol) in combination with methotrexate. GAGs were isolated from plasma samples using ion exchange low-pressure liquid chromatography. Total sulfated GAGs were quantified using a hexuronic acid assay. Plasma levels of keratan sulfate (KS) and HA were measured using immunoassay kits. Results Total sulfated GAGs and HA levels were higher in female RA patients before treatment in comparison to healthy subjects. KS levels did not differ between RA women and controls. Anti-TNF-α treatment resulted in normalization of plasma total GAG and HA levels in RA patients, without any effect on KS levels. Conclusions Our results suggest that anti-TNF-α therapy has a beneficial effect on extracellular matrix remodeling in the course of RA.

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).

Objectives: Erythrocyte sedimentation rate (ESR) and serum level of C-reactive protein (CRP) are the acute phase reactants most commonly determined in patients with rheumatic diseases. The indices are affected by different factors, but both of them are applied for evaluation of the disease activity in patients with inflammatory disorders of the musculoskeletal system. Material and methods: The authors compared the results of ESR and CRP, which were carried out during routine diagnosis in 200 patients admitted to the Department of Rheumatology. Results: A significant correlation between ESR and CRP was found (ESR after 1 h/CRP: correlation coefficient 0.6944, ESR after 2 h/CRP: correlation coefficient 0.6126). There was no difference in ESR or CRP between male and female patients, and patients older than 40 years had higher ESR and CRP. Conclusions: The obtained results support the usefulness of both indices in the clinical practice of rheumatologists.

Background Systemic sclerosis (SSc) is a connective tissue disease characterized by an excessive collagen production, fibrotic changes in the skin, internal organs, and vascular involvement. Patients with SSc may have pathological changes in their joints, especially in feet that enclosed: flat feet, valgus or varus foot, hallux valgus, tendinopathy, foot ulcers, joint space narrowing, joint subluxation and degenerative changes [1, 2]. Objectives The aim of the study was to assess occurrence and the severity of lesions and deformities of spine and joints of lower limbs, especially of feet, in patients with SSc, in comparison to the healthy volunteers. Methods All subject have anamnesis, anthropometric tests, and feet assessment with the computer podometric measuring system. Results The study enclosed 53 patients with SSc (69.8% female) in mean age 55±12 and 65 healthy volunteers (53.8% female) in mean age 45±15. Disease duration median was 3 years (IQR =6). In comparison to healthy volunteers, patients with SSc have more frequent flat feet and pes valgus (p Conclusions Patients with SSc have a relatively high prevalence of feet pathological deformities and a smaller range of flexion of the joints than the lowest normal range, but mostly normal curvature of the spine. References La Montagna G, Baruffo A, Tirri R, Buono G, Valentini G: Foot involvement in systemic sclerosis: A longitudinal study of 100 patients. Semin Arthritis Rheum 2002, 31(4): 248–255. Sari-Kouzel H, Hutchinson CE, Middleton A, Webb F, Moore T, Griffin K, Herrick AL: Foot problems in patients with systemic sclerosis. Br Soc for Rheumatol 2001, 40: 410–413. Disclosure of Interest None declared

Background Biologics are still considered by patients as a new and partially enigmatic tool for management of rheumatic diseases. Objectives The study was designed to evaluate educational needs and sources of knowledge in patients with rheumatic diseases treated with biologics. Methods Anonymous questionnaires were distributed in 23 Polish rheumatological centers involved in the treatment, 1231 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-year-old), as well as 19 ones receiving the drugs due to other musculoskeletal disorders. The investigated group constituted about one-fifth of all rheumatic patients on biologics in Poland. Results Almost all the patients had learnt for the first time on biologics from their rheumatologist (93%). Few only patients had got such data from internet or from other patients. Likewise, most of the patients got majority of educational data on treatment with biologics from rheumatologist who was supervising the therapy (82%). Remaining sources included internet (8%) and other patients (5%). Relative low number of patients was educated by nurses (2%). Most of the patients (87%) were looking for more details on biological treatment. The patients with rheumatic disease lasting less than 10 yrs. were more interested in the management than those suffering longer. Most of the patients (94%) considered their rheumatologist as the main person responsible for their education on biologics. There was no difference between patients with various rheumatic diseases as well as no difference was found between female and male patients. Biological treatment attracted more interest in younger than older patients. Conclusions Education is still a challenge in patients receiving biologics. Most of the patients represented traditional attitude to health education, expecting almost all educational data to be provided by their physician. We were surprised that role of the nurses was found to be rather low. An increase in role of nurses seems to be the future aim of the educational efforts in Polish rheumatology. Disclosure of Interest None declared

W pracy przedstawiono epidemiologię i aspekty socjoekonomiczne trzech najczęstszych chorób reumatycznych: reumatoidalnego zapalenia stawów (RZS), tocznia rumieniowatego układowego (TRU) oraz twardziny układowej (TU). Częstość występowania chorób reumatycznych w populacji szacuje się na 4–5%. Współczynnik chorobowości dla RZS w Polsce wynosi 0,45% populacji dorosłej i jest zbliżony do współczynnika w Unii Europejskiej, który wynosi 0,49%. Szacuje się, że średnia częstość występowania tocznia rumieniowatego układowego wynosi 40–55/100 tys. osób. Roczna zapadalność na TU wynosi 19–35 przypadków na milion mieszkańców (w zależności od kraju, w którym przeprowadzono badania). W Polsce blisko 18% wszystkich hospitalizacji jest związanych ze schorzeniami reumatycznymi. Wprowadzenie nowych kryteriów klasyfikacyjnych dla RZS, umożliwiających klasyfikowanie wczesnych postaci choroby i szersze ich zastosowanie w praktyce klinicznej, wpłynie zapewne na zmianę oceny częstości występowania tej choroby w populacji.

Aim The relationship between adiponectin, leptin, insulin-like growth factor-1 (IGF-1) and total lipid peroxide (TLP) concentrations, and its possible role in the development of diffuse cutaneous systemic sclerosis (dcSSc), were evaluated in this study. Methods, results Plasma adipokines and IGF-1 levels were determined using the enzyme-linked immunosorbent assay method, whereas TLP levels were determined using a photometric test, in 36 dcSSc patients and 40 healthy controls matched by age, sex and body mass index (BMI). Plasma levels of adipokines were significantly lowered, while TLP and IGF-1 were increased in dcSSc patients compared to controls. Adiponectin correlated significantly with leptin (r = 0.44), TLP (r = −0.54), CRP (r = −0.47), erythrocyte sedimentation rate (ESR) (r = −0.40) and duration of disease (r = −0.44). A significant relationship was found between leptinemia and IGF-1 (r = −0.40), TLP (r = 0.44), duration of disease (r = −0.38) and BMI (r = 0.65). TLP correlated with IGF-1 (r = −0.43), C-reactive protein (r = 0.47), ESR (r = 0.49) and duration of disease (r = 0.46), while IGF-1 correlated with ESR (r = −0.40). Conclusions Adipose tissue may play a complex role in the development of dcSSc, affecting both the metabolic state of the organism, as well as free radical-induced connective tissue degradation. Although, leptin seems to exert a pro-oxidative effect and both adiponectin and IGF-1 appear to prevent free radical damage, confirmation of the above effects requires further research.

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Choroby autoinflamatoryjne (autoinflammatory diseases) to grupa chorób zapalnych uwarunkowanych genetycznie, w których patogenezie uczestniczy odporność wrodzona. Jest to cecha, która odróżnia je od chorób autoimmunologicznych związanych z zaburzeniami tolerancji wobec własnych antygenów w ramach odporności nabytej. Choroby autoinflamatoryjne i autoimmunologiczne są powiązane, stanowią jednak kontinuum. Wiele objawów chorób autoinflamatoryjnych występuje u chorych leczonych przez reumatologa i wydaje się, że choroby te nie są takie rzadkie, jak rzadko są rozpoznawane.

Cel pracy: Charakterystyka grupy polskich chorych na toczeń rumieniowaty układowy (TRU). W opracowaniu skupiono się na charakterystyce społeczno-demograficznej tej grupy, obrazie klinicznym w chwili zakwalifikowania do badania oraz dostępie do pomocy społecznej. Materiał i metody: Badaniem objęto 269 kolejnych pacjentów z sześciu polskich ośrodków reumatologicznych: w Białymstoku, Katowicach, Lublinie, Poznaniu, Warszawie i Wrocławiu, zakwalifikowanych do międzynarodowego wieloośrodkowego badania SESAME, dotyczącego epidemiologii TRU w trzech krajach Europy Środkowej: w Rumunii, Polsce i na Węgrzech. Wyniki: Wśród uczestników badania przeważały kobiety (n = 234; 87%). Zaledwie 28,6% chorych na TRU było zatrudnionych w pełnym wymiarze czasu pracy, a ok. 1/3 pobierała rentę zdrowotną. W badanej próbie znalazło się 12% chorych z SELENA SLEDAI score wynoszącym co najmniej 6 pkt, u których stwierdzono zmniejszone stężenie dopełniacza oraz obecność przeciwciał przeciw natywnemu DNA. U pacjentów, u których wartości SELENA SLEDAI score były wyższe niż 10 pkt (42%), istotnie częściej niż w pozostałych grupach stwierdzano aktywne zajęcie wszystkich układów i narządów uwzględnionych w ocenie SLE Activity Index, z wyjątkiem skóry i błon śluzowych. Zajęcie układu mięśniowo-szkieletowego znamiennie częściej dotyczyło chorych o wartościach SELENA SLEDAI score mieszczących się w zakresie od 6 do 10 pkt (30,5%). Zaobserwowano istotny związek pomiędzy wyższą aktywnością choroby a częstością występowania różnego stopnia niepełnosprawności oraz korzystaniem z pomocy pracownika socjalnego. Wnioski: W populacji polskich chorych na TRU znajduje się grupa osób, które z uwagi na wysoką aktywność choroby potwierdzoną zarówno w badaniu klinicznym, jak i w badaniach laboratoryjnych wymagają intensyfikacji postępowania terapeutycznego. Wydaje się, że grupa ta może być identyfikowana na podstawie aktywności choroby określonej przy użyciu skali SELENA SLEDAI score. Znamiennie wyższą aktywnością choroby charakteryzują się również chorzy o znacznym stopniu niepełnosprawności. Nie można wykluczyć, że optymalna kontrola terapeutyczna aktywności TRU w tej grupie mogłaby przełożyć się na mniejsze zapotrzebowanie na świadczenia opieki społecznej oraz na poprawę jakości życia.