Giant cell arteritis (GCA) is a medium and large vessel vasculitis. Vision loss is considered the most serious complications traditionally attributed to untreated disease. Urgent corticosteroids (CS) therapy is the standard of care and is considered adequate to prevent blindness. However, in some rare cases blindness may occur despite implementation of the appropriate treatment. We aimed to find patients who went blind despite high dose CS therapy and identify unique features of them together with conducting narrative review of previous case reports to characterize this group in search of common potential risk factors. Cases of blindness prior to treatment induction were not analyzed here. We identified 2 patients in our records who went blind despite high dose CS therapy. We found some repeated common features in patients that went blind in spite of treatment: advanced age, preexisting pronounced arteriosclerosis, thrombocytosis, contraindication to full dose CS therapy resulting in lower doses of CS within the recommended ranges. Defining the subgroup of GCA patients that went blind in spite of proper CS treatment requires further attention as they might potentially benefit from more aggressive therapy (e.g. early introduction of disease-modifying antirheumatic drugs).

Predicting the clinical outcomes in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) remains a challenge and early identification of patients who are at risk of severe disease course is crucial. To address this, we applied machine learning (ML) and federated learning (FL) techniques to the POLVAS dataset – the largest multicenter clinical database of vasculitis cases in Poland and one of the largest AAV datasets in Europe. Our goal was to predict the key outcomes: increased risk of death and the need for renal replacement therapy (RRT), an independent risk factor of death. We also analysed the significance of individual input features for the predictive capabilities of our models. Furthermore, we compared the performance of centralized model with FL models, which allow for data privacy preservation – a key factor given the highly sensitive medical data involved. We achieved a prediction performance of 0.86 AUC for RRT prediction, and 0.81 AUC for death prediction using a centralized approach and 0.86 weighted mean AUC for RRT prediction and 0.80 for death prediction with the FL approach. The presented results show that FL can effectively predict the risk of RRT and mortality in AAV patients, addressing privacy concerns without compromising the accuracy.

To estimate the actual incidence and prevalence of psoriatic arthritis (PsA) within a 9-year timeframe in Poland. Patients were defined as having PsA if they had at least two visits more than 90 days apart with ICD-10 codes M07.0, M07.1, M07.2, M07.3, or L40.5 and filled at least one reimbursed prescription for peripheral or axial PsA-specific treatments during this period (including methotrexate, sulfasalazine, ciclosporin, leflunomide, biologics, targeted synthetic drugs, or non-steroidal anti-inflammatory drugs). Data was obtained from the nationwide public payer database, considering gender, age, and region of residence. We observed an incidence rate of 1.1 per 100,000 inhabitants in 2021, compared to 13.2 in 2013. Regarding the age of the first diagnosis, the peak incidence rate decreased, with a more pronounced decline in men. The prevalence of PsA rose from 72.5 individuals per 100,000 in 2013 to 95.5 in 2021, representing approximately 0.1% of the total population in Poland, with a significant predominance of women among patients over 55 years of age. The decline in PsA incidence may be influenced by a strict case definition and improved access to treatment. Higher prevalence in older women suggests potential gender-related differences. The lower peak incidence and younger diagnosis age in men raise questions about whether lower PsA prevalence in older males is linked to higher mortality due to longer disease duration and comorbidities. Further research is needed to clarify these findings.

Diagnostic pathways for patients with juvenile idiopathic arthritis (JIA) have gradually improved over time. Provider practice has also shifted towards goal-oriented treatment with disease-modifying drugs (DMARDs) that together may have changed the epidemiologic landscape of JIA. Public healthcare utilization records from the National Health Fund (NHF) were screened between 2010 and 2022. For individuals aged < 16 years, we utilized a narrow JIA case definition combining repeat ICD-10 encoding with DMARDs prescription based on ATC codes. In 2022, we identified 1,625 incident and 29,758 prevalent JIA cases (< 16 years), which corresponds to incidence (IRs) and prevalence rates of 4.30 and 78.80 per 100,000 persons of the general population. For the pediatric population, annual IRs for JIA (< 16 years) ranged between 24.0 (95 • This nationwide, healthcare system analysis examines temporal changes in JIA burden of the Polish population, including co-morbidities and direct costs of ambulatory care. • Cyclic and age-dependent trends in incidence rates were observed, with significant comorbidity recorded for every fourth patient with JIA.

OBJECTIVES:To investigate the association between risk of different concurrent disorders and diagnosis of rheumatoid arthritis (RA) using a temporal approach. METHODS:Retrospective, case-control study. Data were extracted from all available healthcare claims for Poland between 2011-2021. Prevalent RA (n = 262 265) patients were examined to evaluate morbidity patterns. Incident RA (n = 15 879) and age-, sex- and region-matched controls were sampled 1:10 from the general population (GP). Exposure was new-onset RA identified by interspaced, repeat claims and prescription. Follow-up was performed in a bidirectional, five-year timeframe from RA diagnosis (Dx). RESULTS:RA is associated with enhanced risk of multiple concomitant disorders both pre- and post-Dx, especially hematologic (incidence rate ratio (IRR) 1.80, 95% CI 1.58, 2.05), pulmonary (IRR 1.57, 95% CI 1.53, 1.62), gastrointestinal (IRR 1.53, 95% CI 1.46, 1.60) and cardiovascular (IRR 1.25, 95% CI 1.22, 1.27) conditions. RA appears strongly associated with interstitial lung disease (pre-Dx IRR 2.97, 95% CI 2.20, 4.02; post-Dx IRR 4.66, 95% CI 3.91, 5.56) and inflammatory bowel disease (pre-Dx IRR 1.61, 95% CI 1.25, 2.07; post-Dx IRR 2.12, 95% CI 1.70, 2.63). Enhanced post-Dx risk of thyroid cancer (IRR 1.29, 95% CI 1.00, 1.66) and lymphoma (IRR 1.50, 95% CI 1.20, 1.88) was observed, in contrast to reduced risk of colon cancer (IRR 0.77, 95% CI 0.62, 0.94). CONCLUSION:Enhanced risk of multiple concurrent disorders was observed both pre- and post-RA Dx, with varying patterns across different disease groups. These data highlight the multisystemic nature of RA and warrant further research into causal, bidirectional relationships.

Background/Objectives: This study was conducted to analyze the associations between vascular endothelial growth factor (VEGF) serum concentrations and immunological biomarkers, inflammatory parameters, classical atherosclerosis risk factors, and cardiovascular manifestations in systemic lupus erythematosus (SLE) patients. Methods: The project included 83 individuals suffering from SLE, with 20 healthy individuals as controls. The serum levels of VEGF were determined through the ELISA method using R&D Systems tests. Laboratory markers, autoantibody profiles, traditional atherosclerotic risk factors, and organ manifestations were evaluated. Atherosclerotic changes were determined based on several indices including carotid intima-media thickness, ankle-brachial index and high resistance index assessments. Results: The reference range of serum VEGF concentrations was established based on the 25th and 75th percentiles obtained in the controls. High VEGF levels were significantly correlated with the presence of selected anti-phospholipid antibodies such as anti-prothrombin (OR = 10.7; 95%CI: 2.1-53.4) and anti-beta2 glycoprotein I (OR = 3.5; 95%CI: 1.1-10.8), as well as cardiac disorders (OR = 8.0; 95%CI: 1.6-39.5). On the other hand, low concentrations of VEGF were significantly related to lower frequencies of anti-double-stranded DNA antibodies (OR = 0.31; 95%CI: 0.11-0.91) and anti-endothelial cell antibodies (OR = 0.30; 95%CI: 0.11-0.85). Patients with low VEGF levels showed significantly reduced risks of atherosclerotic lesions (OR = 0.24; 95%CI: 0.04-0.99) and vasculitis development (OR = 0.17; 95%CI = 0.03-0.91). Conclusions: In conclusion, VEGF's pathogenetic role in SLE and SLE-related atherothrombosis is manifested in close correlation with aPLs which may enhance their direct impact on endothelium. High VEGF levels are helpful for identifying cardiovascular risk in patients, while low concentrations indicate lower disease activity, as well as a lower risk of organ involvement.

BACKGROUND:Diagnosing central nervous system (CNS) involvement in patients with autoimmune diseases remains a significant clinical challenge. Magnetic resonance imaging (MRI) often fails to reveal abnormalities in patients presenting with CNS-related symptoms. Despite the increasing interest in brain perfusion imaging in autoimmune diseases, studies assessing the utility of scintigraphy in this context remain limited. A review of the existing literature indicates a lack of comparative studies evaluating scintigraphy, MRI, and clinical symptoms in the early diagnosis and monitoring of CNS involvement in rheumatic diseases. This study aimed to assess the utility of brain single photon emission computed tomography (SPECT) scintigraphy with hexamethylpropyleneamine oxime labeled with technetium-99m ([99mTc]Tc-HMPAO) and MRI in visualizing cerebral perfusion disturbances in patients with rheumatic diseases presenting CNS-related symptoms. MATERIAL AND METHODS:A total of 204 brain perfusion SPECT scintigraphies using [99mTc]Tc-HMPAO from 71 patients (10 men and 61 women, aged 20-69 years) were retrospectively analyzed. The study included patients presenting with clinical symptoms such as severe headaches, memory impairment, and cognitive dysfunction who were diagnosed with systemic lupus erythematosus (n = 52), antiphospholipid syndrome (n = 9), Sjögren's syndrome (n = 5), and undifferentiated connective tissue disease (n = 5). Analysis was done in patients who underwent a minimum of two brain scintigraphies at intervals of at least six months (mean interval: 1 year ± 2 months). Due to the absence of a universally accepted diagnostic gold standard, initial SPECT scans were compared with follow-up images and current clinical symptoms by two independent physicians. Image analysis was conducted using dedicated software for the processing and interpretation of brain SPECT perfusion studies. In 65 patients, a brain MRI scan had been performed and compared to the brain scintigraphy results. RESULTS:In 24 patients, regression of the disease in the brain perfusion scintigraphy was observed, while 31 patients showed progression of scintigraphic changes. In 16 patients, no differences were detected compared to the initial imaging. In 24/65 patients, brain MRI did not reveal any abnormalities, while in the scintigraphy, perfusion defects were visible. In 6 patients, no changes were seen in either brain MRI or scintigraphy. The analysis of results using Cohen's Kappa demonstrated a good correlation: 0.78 between cerebral perfusion scintigraphic changes observed in [99mTc]Tc-HMPAO SPECT and the clinical symptoms of patients. CONCLUSIONS:Brain perfusion scintigraphy using [99mTc]Tc-HMPAO shows better correlation than brain MRI with the CNS clinical symptoms of patients with rheumatic diseases. This method appears to be a useful diagnostic tool for evaluating CNS involvement in patients presenting with neurological symptoms and normal findings on MRI.

Slavic populations, such as those in Poland, are considered to have a low prevalence of giant cell arteritis (GCA), although epidemiological data are sparse. The study aimed to compare the reported frequency of GCA in various regions of Poland and analyze the differences between them. We conducted a multicenter, retrospective study of all GCA patients included in the POLVAS registry—the first large multicenter database of patients with vasculitis in Poland. The data from the POLVAS registry were compared with the reported prevalence provided by national insurers from the corresponding regions. A 10-fold increase in the diagnostic rates of GCA was observed in Poland between 2008 and 2019, reaching 8.38 per 100,000 population > 50 years old. It may be attributed to increased interest accompanied by improved diagnostic modalities with the introduction of ultrasound-based, fast-track diagnostic pathways in some centers. However, regional inequities are present, resulting in 10-fold differences (from 2.57 to 24.92) in reported prevalence between different regions. Corticosteroid (CS) monotherapy was the main stem of treatment. Further cooperation and education are needed to minimize regional inequities. This observational study suggests some potential for further increase of the recognizability of GCA and wider use of other than CS monotherapy treatment regimens. We hope that the Polish experience might be interesting and serve as some guidance for the populations where GCA is underdiagnosed.

BackgroundThe anti-Nucleolar Organizer Region 90 antibodies (NOR90) are rare antinuclear antibodies (ANA) reported in systemic sclerosis (SSc). Especially due to low prevalence, the clinical relevance of NOR90 in SSc remains uncertain.ObjectivesTo analyze the clinical associations of NOR90 in patients with SSc in a multicentric cohort.MethodsPost-hoc, cross-sectional study of prospectively collected data from the European Scleroderma Trials and Research (EUSTAR) database, with additional information on NOR90.Further, we performed a systematic literature search, using the terms “systemic sclerosis” and “NOR90” across three databases: Medline via PubMed, Scopus, and Thomson Reuters’ Web of Science Core Collection, from inception to November 1st, 2023.ResultsOverall, 1318 patients with SSc were included (mean age 58.3 ± 13.7 years, 81.3 % female), of whom 44 (3.3 %) were positive for NOR90. Of these, 32 were also positive for one of the SSc-criteria antibodies: 9/44 (20.5 %) for anti-topoisomerase I, 18/42 (42.9 %) for anti-centromere, and 5/40 (12.5 %) for anti-RNA polymerase III. NOR90-positive patients were more frequently female, had lower modified Rodnan skin score (mRSS), and lower prevalence of upper and lower gastrointestinal (GI) symptoms compared to NOR90-negative patients. In multivariable analysis, NOR90 remained significantly associated with lower mRSS and less frequent GI symptoms.The literature search identified 17 articles, including a total number of 87 NOR90-positive out of 3357 SSc patients, corresponding to an overall prevalence of 2.6 %.ConclusionTo our best knowledge, this is the largest SSc cohort tested for NOR90 to date, confirming the NOR90 prevalence in SSc patients is around 3 %.

To assess the incidence and prevalence of rheumatoid arthritis (RA) in Poland for the period 2013–2021, total and dependent on gender, age, region and serological status. Information on reported National Health Fund (NHF) health services and reimbursed prescriptions were used, defining an RA patient as a person who had at least two visits in different quarters with ICD-10 code M05 or M06 and at the same time filled at least one reimbursed prescription for a drug whose active substance is methotrexate, sulfasalazine, leflunomide or was treated with biologic disease-modifying anti-rheumatic drugs (bDMRDs) or targeted synthetic DMARDs (tsDMARDs) as part of a drug program financed by the National Health Fund. The nationwide standardised incidence rate of RA in 2021 was 29 persons per 100,000 population (18 per 100,000 population of seropositive vs. 11 per 100,000 population of seronegative RA). The prevalence of RA in Poland in 2021 was 689.0 people per 100,000 population, a total of 0.7

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. Individual disease course prediction, prognosis, and maintenance treatment regimen selection create difficulties due to the heterogeneity of the AAV. The ability to predict the risk of relapse in AAV course is crucial for decision about maintenance therapy duration. It also forms an important unmet need in actual guidelines [1]. Objectives: To identify risk factors of AAV relapse and build a model for personalized prediction of AAV exacerbation. Methods: We conducted a national multicenter study of adult patients diagnosed with AAV (648–GPA, 170–MPA) [2]. Their clinical and laboratory data were collected in the POLVAS registry by 12 referral centers. Cox proportional hazards analyses were applied to calculate hazard ratios for the first relapse as the main endpoint. First, one-dimensional models were used to identify potentially relevant variables. Then, using stepwise regression with different order of inclusion and exclusion of variables, a multidimensional model was obtained. Results: Data analysis from 818 patients identified seven significant risk factors of AAV relapse: gender, skin, ENT or eye involvement, maximal ever creatinine < 475 umol and CRP > 10 ng/ml at baseline (Table 1).In the next step AAV patients were divided into 5 groups with different risk of relapse (HP) over time (Figure 1) using following algorithm: hi,i ∈ {1,...7} are hazard ratios for risk factors obtained in analysis. HP value determines to which group the patient belongs:: Group 1: HP≤1.5, Group 2: 1.55.The Relapse free survival rate for all five groups is shown in Figure 1. These results suggest the in the groups 4 and 5 over 80 % of cases will have AAV relapse, whereas in the groups 1 and 2 less than 60%. If we look at the time course of relapse occurrence we can observe the fast decrease of relapse free survival in the first 36-48 months, later on the dynamic of relapse rate in all groups is similar. Comparison of different groups suggests that in group 1 the first 24 months are crucial with ca 50% of cases having no relapse during next 240 months. In groups 2 and 3 the highest number of relapses occur in the first 36 months. In groups 4 and 5 patients have the highest risk of relapse and what is more almost all of them will experience the AAV relapse in long term perspective. Conclusion: POLVAS registry data analysis identified AAV relapse risk factors for and allowed to build a model able to define AAV patients’ subsets characterized by different probability of disease relapse which may help to guide personalized decisions about the duration and type of maintenance therapy. REFERENCES: [1] Hellmich B et al. Ann Rheum Dis. 2023 Mar 16:ard-2022-223764. doi: 10.1136/ard-2022-223764.[2] Wójcik K et al. Clin Rheumatol. 2019 Sep;38(9):2553-2563. Acknowledgements: This work is supported by the Jagiellonian University Medical College under Grant No. N41/DBS/001188. Disclosure of Interests: None declared.Figure 1Relapse free survival rates for 5 AAV subgroups Table 1Multidimensional risk factors of AAV relapsep-valueHazard ratio95% lowerbound95% upperboundMale Gender0.0486161.2192981.0098651.507028Skin inv0.0293271.2838391.0254401.607351Eye inv0.0187041.3316181.0488281.690654ENT inv0.0268461.3445151.0345191.747403cANCA or PR30.0361121.3113241.0177061.689653max_crea ever<475 [umol]0.0037521.5850091.1608062.164232max_CRP at baseline >10 [mg/l]0.0141551.3962191.0694231.822878