Clinical Characteristics of EGPA Patients in Comparison to GPA Subgroup with Increased Blood Eosinophilia from POLVAS Registry.

Objective: To characterize the eosinophilic granulomatosis with polyangiitis (EGPA) population from the POLVAS registry depending on ANCA status and diagnosis onset, including their comparison with the granulomatosis with polyangiitis (GPA) subset with elevated blood eosinophilia (min. 400/µl) (GPA HE) to develop a differentiating strategy. Methods: A retrospective analysis of the POLVAS registry. Results: The EGPA group comprised 111 patients. The ANCA-positive subset (n = 45 [40.54%]) did not differ from the ANCA-negative one in clinics. Nevertheless, cardiovascular manifestations were more common in ANCA-negative patients than in those with anti-myeloperoxidase (MPO) antibodies (46.97% vs. 26.92%, p = 0.045). Patients diagnosed before 2012 (n = 70 [63.06%]) were younger (median 41 vs. 49 years, p < 0.01), had higher blood eosinophilia at diagnosis (median 4,946 vs. 3,200/µl, p < 0.01), and more often ear/nose/throat (ENT) and cardiovascular involvement. GPA HE comprised 42 (13.00%) out of 323 GPA cases with reported blood eosinophil count. Both GPA subsets had a lower prevalence of respiratory, cardiovascular, and neurologic manifestations but more often renal and ocular involvement than EGPA. EGPA also had cutaneous and gastrointestinal signs more often than GPA with normal blood eosinophilia (GPA NE) but not GPA HE. The model differentiating EGPA from GPA HE, using ANCA status and clinical manifestations, had an AUC of 0.92, sensitivity of 96%, and specificity of 95%. Conclusion: Cardiovascular symptoms were more prevalent in the ANCA-negative subset than in the MPO-ANCA-positive one. Since EGPA and GPE HE share similarities in clinics, diagnostic misleading may result in an inappropriate therapeutic approach. Further studies are needed to optimize their differentiation and tailored therapy, including biologics.

Ebbing Strength, Fading Power: Unveiling the Impact of Persistent Fatigue on Muscle Performance in COVID-19 Survivors.

The total number of confirmed cases of COVID-19 caused by SARS-CoV-2 virus infection is over 621 million. Post-COVID-19 syndrome, also known as long COVID or long-haul COVID, refers to a persistent condition where individuals experience symptoms and health issues after the acute phase of COVID-19. The aim of this study was to assess the strength and fatigue of skeletal muscles in people recovered from COVID-19. A total of 94 individuals took part in this cross-sectional study, with 45 participants (referred to as the Post-COVID Cohort, PCC) and 49 healthy age-matched volunteers (Healthy Control Cohort, HCC). This research article uses the direct dynamometry method to provide a detailed analysis of post-COVID survivors' strength and power characteristics. The Biodex System 4 Pro was utilized to evaluate muscle strength characteristics during the fatigue test. The fatigue work in extensors and flexors was significantly higher in the PCC. The PCC also showed significantly less power in both extensors and flexors compared to the HCC. In conclusion, this study provides compelling evidence of the impact of post-COVID-19 fatigue on muscle performance, highlighting the importance of considering these effects in the rehabilitation and care of individuals recovering from the virus. PCC achieved lower muscle strength values than HCC.

Effect of COVID-19 on Musculoskeletal Performance in Gait and the Timed-Up and Go Test.

INTRODUCTION: The total number of confirmed cases of COVID-19 caused by the SARS-CoV-2 virus infection is over 621 million in the world. In approximately 63% of cases, the patient still experiences persistent symptoms 30 days after the onset of symptoms or hospitalisation, and 45.9% of patients have experienced or will experience symptoms for at least three months. Despite the prevalence of chronic symptoms and pathological changes that may affect gait and functional mobility in people with a history of COVID-19, there are few publications investigating the impact of these abnormalities. This study aims to determine the long-term effects of COVID-19 on gait and the Timed-Up and Go Task. MATERIAL AND METHODS: A total of 30 individuals took part in the experiment. The subjects in the study group were infected with the COVID-19 virus and required hospital treatment. Prior to the study, the subjects had no chronic diseases or other conditions affecting the musculoskeletal system. The non-infected by COVID-19 group was a healthy population with no history of COVID-19 disease. The study used the inertial system wireless motion analysis system based on 15 inertial sensors (inertial measurement units, IMUs). IMU sensors were placed on the following body segments: head, sternum, middle and lower spine, shoulder, arm, forearm, hand, shank, for the left and right limb. Movement task reports generated from the recording were created using myoRESEARCH 3.10. The subjects in the study group were asked to perform a movement task test-the Timed-Up and Go Test (TUG): sit-to-stand, walk (3 m) without change in direction, walk termination, and stand-to-sit. RESULTS: It took 46% longer for those infected by COVID-19 (participants) to complete the entire movement task compared to those in the not-infected by COVID-19 group. Sit-to-Stand Time [s] was greater in the infected by COVID-19 group and was 2.1 ± 0.7. Mean Walking Speed [m/s] was lower than in the not-infected by COVID-19 group and was 0.26 ± 0.07. Walking cadence [steps/min] was lower and was 21.2 ± 1.2. Infected by COVID-19 participants achieved a smaller anterior pelvic tilt angle (p < 0.001) and a smaller hip flexion angle (p = 0.025), with an increase in knee (p < 0.001) and ankle (p < 0.001) flexion angles. CONCLUSIONS: Individuals in the infected by COVID-19 group present changes in the ranges of motion and the time to complete the TUG task, despite the fact that at least eight weeks passed after hospital discharge.

Biological treatment in Felty's syndrome with profound neutropenia.

A triad of symptoms characterises Felty's syndrome: seropositive rheumatoid arthritis (RA), splenomegaly and neutropenia. The treatment of Felty's syndrome is based on using classic synthetic and biological disease-modifying anti-rheumatic drugs (DMARDs). In this article, we present a case of a patient with Felty's syndrome who was treated with biologic treatment. A systematic search of the literature on the electronic medical database was conducted. The drugs from the DMARD group, despite reducing the activity of the disease, may cause significant clinical complications. It is important to know about the diagnosis, differentiation and treatment of neutropenia and the prevention of febrile neutropenia. The article discusses the current therapeutic possibilities using both classical and biologic DMARDs.

Meningitis after elective intracranial surgery: a systematic review and meta-analysis of prevalence.

Meningitis is a potential complication of elective intracranial surgery (EIS). The prevalence of meningitis after EIS varies greatly in the literature. The objective of this study was to estimate the overall pooled prevalence of meningitis following EIS. Four databases (PubMed, Scopus, Web of Science, and Embase) were searched to identify relevant studies. Meta-analyses of proportions were used to combine data. Cochran's Q and I2 statistics were used to assess and quantify heterogeneity. Additionally, several subgroup analyses were conducted to investigate the source of heterogeneity and examine differences in the prevalence based on variables such as geographical regions, income level, and meningitis type. The meta-analysis included 83 studies (30 959 patients) from 26 countries. The overall pooled prevalence of meningitis after EIS was 1.6% (95% CI 1.1-2.1), with high heterogeneity present (I2 = 88%). The pooled prevalence in low- to middle-income countries and high-income countries was 2.7% (95% CI 1.6-4.1) and 1.2% (95% CI 0.8-1.7), respectively. Studies that reported only aseptic meningitis had a pooled prevalence of 3.2% (95% CI 1.3-5.8). The pooled prevalence was 2.8% (95% CI 1.5-4.5) in studies that reported only bacterial meningitis. Similar prevalence rates of meningitis were observed in the subgroups of tumor resection, microvascular decompression, and aneurysm clipping. Meningitis is a rare but not exceptional complication following EIS, with an estimated prevalence of 1.6%.

The Risk of Autoimmunity Development following mRNA COVID-19 Vaccination.

The broad spectrum of interactions between autoimmune diseases and the SARS-CoV-2 vaccination is not fully understood. This study aims to evaluate the prevalence of anti-nuclear antibodies (ANA), anti-ENA, anticardiolipin antibodies (ACL), and anti-beta-2 glycoprotein I antibodies (anti-ß2GPI) before and after the SARS-CoV-2 mRNA vaccination in a real-life setting in healthcare professionals. The identification of risk factors associated with vaccine immunogenicity was evaluated. The study group consisted of employees of two hospitals (354 individuals). Samples for antibody assays were collected before vaccination and at 7-9 months after complete immunisation. There was no significant increase in the prevalence of ANA, ACL or anti-ß2GPI antibodies, or autoimmune diseases in subjects who were vaccinated 7-9 months after complete immunisation. In terms of detected anti-ENA, the anti-DFS70 antibodies were found in 6 times more subjects than before vaccination at the second blood draw (in 18 and 3 subjects, respectively) (p = 0.001). There were no significant relationships between a SARS-CoV-2 infection history, humoral response, cellular response, subject category, smoking, sex, body weight, ANA, anti-ENA, ACL, or anti-ß2GPI. This study revealed a possible association between the severity of vaccine adverse events (VAEs) and ANA titre. Individuals with more severe VAEs (>10 points) after the second dose of the vaccine had significantly higher ANA titre after complete immunization. When analysing the significance of time between the ANA, anti-ENA, ACL, and anti- ß2GPI assays and complete immunisation antibody values, no qualitative result was statistically significant. There was correlation between the time since complete immunization and ANA after.

The Usefulness of the COVID-GRAM Score in Predicting the Outcomes of Study Population with COVID-19.

BACKGROUND: The COVID-GRAM is a clinical risk rating score for predicting the prognosis of hospitalized COVID-19 infected patients. AIM: Our study aimed to evaluate the use of the COVID-GRAM score in patients with COVID-19 based on the data from the COronavirus in the LOwer Silesia (COLOS) registry. MATERIAL AND METHODS: The study group (834 patients of Caucasian patients) was retrospectively divided into three arms according to the risk achieved on the COVID-GRAM score calculated at the time of hospital admission (between February 2020 and July 2021): low, medium, and high risk. The Omnibus chi-square test, Fisher test, and Welch ANOVA were used in the statistical analysis. Post-hoc analysis for continuous variables was performed using Tukey's correction with the Games-Howell test. Additionally, the ROC analysis was performed over time using inverse probability of censorship (IPCW) estimation. The GRAM-COVID score was estimated from the time-dependent area under the curve (AUC). RESULTS: Most patients (65%) had a low risk of complications on the COVID-GRAM scale. There were 113 patients in the high-risk group (13%). In the medium- and high-risk groups, comorbidities occurred statistically significantly more often, e.g., hypertension, diabetes, atrial fibrillation and flutter, heart failure, valvular disease, chronic kidney disease, and obstructive pulmonary disease (COPD), compared to low-risk tier subjects. These individuals were also patients with a higher incidence of neurological and cardiac complications in the past. Low saturation of oxygen values on admission, changes in C-reactive protein, leukocytosis, hyperglycemia, and procalcitonin level were associated with an increased risk of death during hospitalization. The troponin level was an independent mortality factor. A change from low to medium category reduced the overall survival probability by more than 8 times and from low to high by 25 times. The factor with the strongest impact on survival was the absence of other diseases. The medium-risk patient group was more likely to require dialysis during hospitalization. The need for antibiotics was more significant in the high-risk group on the GRAM score. CONCLUSION: The COVID-GRAM score corresponds well with total mortality. The factor with the strongest impact on survival was the absence of other diseases. The worst prognosis was for patients who were unconscious during admission. Patients with higher COVID-GRAM score were significantly less likely to return to full health during follow-up. There is a continuing need to develop reliable, easy-to-adopt tools for stratifying the course of SARS-CoV-2 infection.

Interferon Gamma Targeted Therapy: Is It Justified in Primary Sjögren's Syndrome?

Background: The pathomechanism of primary Sjögren syndrome (pSS) is multifactorial. Many cytokines take part in this process, including interferon. The study aimed to quantify certain cytokines involved in the pathomechanism of primary Sjögren syndrome (IL2, IL5, IL6, IL10, IL13, TNFα, IFNγ) and determine their common clinical correlation. On this basis, we discuss the potential use of anti-cytokine drugs in pSS therapy. Methods: The study group consisted of adult patients with a confirmed diagnosis of pSS. Results: The most frequently detected cytokines were IFNγ (82% of patients), TNFα (70%), IL6 (50%), and IL2 (42.5%). In all patients, except for one patient, IFNγ was found in the presence of other specific cytokines. There was no difference in clinical symptoms, age, and laboratory test results between the group of patients with IL-6 + TNFα + IFNγ positive cytokine, and the group of patients in whom they were not detected. There was no correlation between the presence of IL5, IL13, IL2, IL6, IL10, TNFα and musculoskeletal symptoms, skin lesions, glandular domains, pulmonary neurological, lymphadenopathy, biological and hematological domains in ESSDAI (p > 0.05). Conclusions: IFNγ most likely plays a central role in the pathomechanism of the disease. We have not noticed a clinical correlation between the three most common cytokines (IL6, IFNγ and TNFα), preliminary research results open up the possibility of searching for new treatments for pSS. The lower percentage of patients with detectable levels of TNFα and IL6 may explain the ineffectiveness of drugs targeting cytokines in clinical trials to date.

The Usefulness of the C2HEST Risk Score in Predicting Clinical Outcomes among Hospitalized Subjects with COVID-19 and Coronary Artery Disease.

BACKGROUND: Even though coronary artery disease (CAD) is considered an independent risk factor of an unfavorable outcome of SARS-CoV-2-infection, the clinical course of COVID-19 in subjects with CAD is heterogeneous, ranging from clinically asymptomatic to fatal cases. Since the individual C2HEST components are similar to the COVID-19 risk factors, we evaluated its predictive value in CAD subjects. MATERIALS AND METHODS: In total, 2183 patients hospitalized due to confirmed COVID-19 were enrolled onto this study consecutively. Based on past medical history, subjects were assigned to one of two of the study arms (CAD vs. non-CAD) and allocated to different risk strata, based on the C2HEST score. RESULTS: The CAD cohort included 228 subjects, while the non-CAD cohort consisted of 1956 patients. In-hospital, 3-month and 6-month mortality was highest in the high-risk C2HEST stratum in the CAD cohort, reaching 43.06%, 56.25% and 65.89%, respectively, whereas in the non-CAD cohort in the high-risk stratum, it reached: 26.92%, 50.77% and 64.55%. Significant differences in mortality between the C2HEST stratum in the CAD arm were observed in post hoc analysis only for medium- vs. high-risk strata. The C2HEST score in the CAD cohort could predict hypovolemic shock, pneumonia and acute heart failure during hospitalization, whereas in the non-CAD cohort, it could predict cardiovascular events (myocardial injury, acute heart failure, myocardial infract, carcinogenic shock), pneumonia, acute liver dysfunction and renal injury as well as bleedings. CONCLUSIONS: The C2HEST score is a simple, easy-to-apply tool which might be useful in risk stratification, preferably in non-CAD subjects admitted to hospital due to COVID-19.

The assessment of the risk of COVID-19 infection and its course in the medical staff of a COVID-only and a non-COVID hospital.

BACKGROUND: Medical workers are a group that is particularly vulnerable to infection during the coronavirus disease 2019 (COVID-19) pandemic. OBJECTIVES: The study aimed to assess the risk of COVID-19 infection and its course in the medical staff of a COVID-only and a non-COVID hospital. MATERIAL AND METHODS: The observational study included 732 participants who were medical workers. The study was conducted between June 2020 and December 2020, before widespread COVID-19 immunization was introduced. RESULTS: Of the 732 employees of the hospitals, 377 had a history of COVID-19. The risk of disease was twice as high in the medical staff of the COVID-only hospital compared to the medical staff of the non-COVID hospital (odds ratio (OR) = 2.0; p < 0.001). Among medical personnel, 20.6% of the participants were asymptomatic and 6.4% required hospitalization. For the non-COVID hospital, the employees who were most frequently infected with COVID-19 were nurses/paramedics/medical caretakers. The factor influencing the risk of infection was body mass index (BMI; OR = 1.05; p = 0.004). The risk of COVID-19 infection was lower in the influenza vaccine group (OR = 2.23, p < 0.001). CONCLUSIONS: The study results indicate that employees of the hospital treating only COVID patients have a higher risk of infection. Previous observations on factors predisposing to COVID-19 infection like gender and BMI were confirmed. However, the observations carried out on the studied population did not confirm the influence of other factors, such as the coexistence of chronic diseases (apart from diabetes) on the risk of developing COVID-19. In addition, we noticed that seasonal influenza vaccination has a beneficial effect in patients with COVID-19 infection.

Complete (Humoral and Cellular) Response to Vaccination against COVID-19 in a Group of Healthcare Workers-Assessment of Factors Affecting Immunogenicity.

Vaccination is the best way to limit the extent of the COVID pandemic. Knowledge of the duration of the immune response will allow the planning of a vaccination protocol. This study aims to validate the complete (humoral and cellular) immune responses over time in large population groups following the full vaccination of healthcare professionals in real-life conditions and to assess the relationship between antibody levels and T-cell activity in relation to the characteristics of the study group. The samples for the study were obtained from volunteers (staff of two hospitals) on three occasions: before vaccination, T0, then 4-9 weeks after full vaccination (two doses BNT162b2), T1, and 7-9 months after vaccination, T2. The humoral response was investigated by the titre of anti-SARS-CoV-2 IgG antibodies to S1 protein. Assays were performed three times at intervals. The cellular response was assessed in a subgroup of 189 subjects by QuanT-Cell SARS-CoV-2 (IGRA). The assay was performed once. A group of 344 subjects fully vaccinated with the BNT162b2 vaccine were included in the study. The humoral response was observed in 100% of subjects at both 4-7 weeks and 7-9 months, but antibody titres fell by almost 90% in this interval. The cellular response was observed in 94% (177/189) of subjects 7-9 months after the second dose of vaccine. In subjects with a negative cellular response, eight out of 12 smoked. A factor associated with greater immunogenicity of vaccination was past SARS-CoV-2 infection. The administration of full BNT162b2 vaccination (two doses) induces humoral and cellular responses detectable even more than six months after vaccination. Smoking may be a factor associated with impaired cellular response to vaccination.

Respiratory involvement in antineutrophil cytoplasmic antibody-associated vasculitides: a retrospective study based on POLVAS registry.

OBJECTIVES: The study aimed to characterise the Polish population of (ANCA)-associated vasculitides (AAV) with respiratory involvement (RI), in comparison to the subgroup without lung manifestations and the other cohorts. METHODS: Retrospective analysis of the Polish population of AAV with RI was conducted, based on data from the POLVAS registry. Standard descriptive statistics, χ2 test, and Mann-Whitney U test were used to perform comparisons. RESULTS: Among 461 cases qualified to this study, there were 316 cases with RI (68.5%), 206 with granulomatosis with polyangiitis (GPA) (65.2%), 80 with eosinophilic granulomatosis with polyangiitis (EGPA) (25.3%) and 30 with microscopic polyangiitis (MPA) (9.5%). Proportion of RI in GPA, MPA, and EGPA accounted for 67.8%; 40.0%; 97.6%, respectively. The number of relapses was higher in the RI group (median 1.0 vs. 0.0; p=0.01). In the subgroup of combined GPA and MPA with RI, the trends toward higher proportion of deaths (11.7% vs. 5.7%; p=0.07), relapses requiring hospitalisation (52.2% vs. 42.4%, p=0.07) and relapses requiring admission to the intensive care unit (5.6% vs. 1.4%, p=0.09) were observed, median maximal concentration of CRP was higher (46 vs. 25 mg/l; p=0.01) and more aggressive treatment was administered. CONCLUSIONS: Prevalence of RI in the Polish population of AAV is similar to the values reported in the literature, however, the proportion observed in GPA is closer to those presented in Asian than Western European cohorts. RI seems to be associated with a more severe course of disease and its presence prompts more aggressive treatment.

Association of antineutrophil cytoplasmic antibody (ANCA) specificity with demographic and clinical characteristics of patients with ANCA­associated vasculitides.

INTRODUCTION: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is characterized by the presence of proteinase­3 (PR3) or myeloperoxidase (MPO) ANCA. In over 90% of cases, PR3­ANCA is associated with granulomatosis with polyangiitis (GPA). However, it is also rarely found in microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). On the other hand, MPO­ANCA being characteristic of MPA (>90% of cases), is also found in about 40% of EGPA and 5% of GPA patients. On the ground of this overlap, clinical importance of ANCA specificity identification has been questioned. OBJECTIVES: In this study, we analyzed the clinical and demographic characteristics of AAV subgroups identified by ANCA serotype. PATIENTS AND METHODS: We conducted a multicenter study of AAV patients (417 GPA, 106 MPA, 102 EGPA; diagnosed between 1990 and 2016), included in the POLVAS registry. The data were systematically collected according to a standardized protocol. RESULTS: In the ANCA-positive group (anti­MPO, anti­PR3) a male-to-female ratio was 1:1, whereas in the ANCA-negative group it was 1:2, regardless of AAV diagnosis. Anti­MPO antibodies were present in significantly older patients. Patients with MPO+GPA and MPO+EGPA were older than those with corresponding ANCA­negative GPA and EGPA as well as PR3+AAV. Moreover, ANCA­negative AAV was characterized by a low risk of end­stage kidney disease and death. CONCLUSIONS: The presence and specificity of ANCA in AAV patients are related to sex and age, determine their organ involvement and influence mortality as previously shown. Patients with MPO­ANCA-positive AAV constitute a clinically homogeneous group, whereas PR3­ANCA-positive patients are much more clinically heterogeneous. ANCA-negative AAV patients are characterized by better prognosis. Thus, ANCA identification is an indispensable element and should not be omitted in establishing AAV diagnosis.

Visual Evoked Potentials as Potential Biomarkers of Visual Function in Patients with Primary Sjögren's Syndrome.

Visual evoked potentials (VEP) are changes in potentials that arise in the central nervous system. In the interpretation of the VEP test results, it is assumed that the elongation of the latency time is caused by the demyelination of the nerve fibers, and the axon damage is responsible for the decrease in the amplitude. The observed VEP deviations are not specific for specific diseases, but indicate disturbances in visual conductivity. VEP may play a diagnostic role in the early detection of visual involvement. The aim of the study was the functioning of visual pathway assessment on the basis of visual evoked potentials (VEP) examination, in patients with primary Sjögren's Syndrome (pSS), without focal symptoms of central nervous system disorder. The effect of disease activity, as assessed by clinical parameters and antibody levels (anti-Ro52, SSA, and SSB), on the central nervous system was also evaluated. Thirty-two consecutive patient with pSS (31 females, 1 male) were included in the study. VEP was performed at baseline, and after 6 (T6) years. Their results were compared longitudinally between the baseline and T6, depending on the duration of the disease and treatment. The immunological activity of pSS was also analyzed. The group of patients showed a significant prolongation of the P100 implicit time (105.5 ± 5.1 vs. 100.6 ± 3.9; p = 0.000) and a significant higher the P100-N145 amplitude (12.3 ± 4.1 vs. 9.4 ± 3.0; p = 0.000). Abnormalities in electrophysiological parameters of VEP at baseline correlated with presentation of anti-Ro52 antibodies and aching joints. At baseline, the P100 implicit time was shorter for the patients with pSS than for those at T6 (105.50 ± 5.1 vs. 109.37 ± 5.67; p = 0.002). pSS patients without CNS involvement presented with dysfunction of visual pathway, as revealed by VEP abnormalities. Relationships were found between VEP parameters and with present of anti-Ro52 antibodies and aching joints. VEP may be a useful method for assessment and monitoring of subclinical visual deficit in the course of pSS.

Differences in clinical phenotypes of primary Sjögren's syndrome depending on early or late onset.

BACKGROUND: Previous research suggests that systemic involvement in primary Sjögren's syndrome (pSS) is a marker of disease prognosis. OBJECTIVES: To evaluate pSS disease activity and the clinical phenotype of pSS patients depending on the age at diagnosis with long-term follow-up. MATERIAL AND METHODS: The study group consisted of patients diagnosed with pSS based on the 2016 pSS classification criteria. RESULTS: The study group consisted of 46 patients with early-onset pSS (≤35 years of age) and 32 patients with late-onset pSS (≥65 years of age). The study group was identified from a total of 228 patients diagnosed with pSS. There were no differences regarding the frequency of eye and mouth dryness, focus score (FS) ≥1 or anti-SSA/SSB antibodies depending on age. Rheumatoid factor (RF) was more common among older patients (p > 0.05). In the overall assessment of disease activity using European League Against Rheumatism (EULAR) Sjögren Syndrome Disease Activity Index (ESSDAI), no differences related to age were observed on the first and last visit (after 36 months on average). Lymphadenopathy and changes in the hematology domain (p < 0.05) were more common in patients with the early-onset phenotype. Changes in the lungs and musculoskeletal system occurred regardless of age. CONCLUSIONS: Patients with early-onset pSS differ from those with late-onset pSS in terms of higher incidence of peripheral lymphadenopathy and cytopenia. The involvement of lung tissue and joints as well as dryness symptoms are common in pSS regardless of age. The RF plays a role in the pathomechanism of pSS development.

Cardiovascular Risk and Endothelial Dysfunction in Primary Sjogren Syndrome Is Related to the Disease Activity.

The aim of our study was to evaluate if endothelial-dysfunction (ED) occurs in patients with primary Sjogren syndrome (pSS) and whether it is associated with the disease characteristics and activity. A total of 46 patients with pSS and 30 controls, without known cardiovascular disease, were enrolled in this study. A flow-mediated-dilation (FMD) of the brachial artery, plasma concentrations of the nitric oxide (NO) metabolic pathway (ADMA, L-arginine, SDMA, cGMP), and markers of endothelial inflammatory function (PAI-1, sE-selectin) and angiogenesis (angiostatin, VEGF) were analyzed. The FMD was significantly lower in pSS patients (7.56 ± 3.08 vs. 10.91 ± 1.02%, p = 0.043) and positively correlated with the Ro/SS-A-antibodies (r = 0.34, p = 0.03), pulmonary involvement (r = 0.52, p = 0.001) and inversely with ADMA (r = -0.35, p = 0.04). Plasma ADMA, L-arginine and angiostatin levels were significantly higher in pSS patients (0.39 ± 0.08 vs. 0.36 ± 0.06 µmol/L, p = 0.05; 29.07 ± 6.7 vs. 25.4 ± 5.23 µmol/L, p = 0.01; 152.25 ± 60.99 vs. 120.07 ± 38.7 pg/mL, p = 0.0, respectively). ADMA was associated with ESSDAI (r = 0.33, p = 0.02), SCORE (r = 0.57, p = 0.00003) and focus score (r = 0.38, p = 0.04). In the multiple regression analysis, the ESSDAI was significantly and independently associated with plasma ADMA levels (ß = 0.24, p = 0.04). Moreover, plasma cGMP concentrations were negatively correlated with the disease duration (r = -0.31, p = 0.03). Endothelial function is impaired in patients with pSS and associated with the measures of disease activity, which supports the key-role of inflammation in developing and maintaining accelerated atherosclerosis.

Is it possible to not perform salivary gland biopsy in targeted patients according to unstimulated salivary flow results in patients with suspected Sjögren's syndrome?

INTRODUCTION/OBJECTIVE: Xerostomia is one of the main symptoms of primary Sjögren's syndrome (pSS). The unstimulated salivary flow (UWS) test is one of the objective Sjögren's syndrome classification criteria used to assess xerostomia's severity. The study's objective was to evaluate UWS rate measurements (with a threshold rate of 0.1 mL/min) in the screening of patients suspected with pSS, presenting with xerostomia in whom labial salivary gland biopsy (LSGB) should be performed. We will try to answer whether it is possible not to perform LSGB in targeted patients according to UWS results? We analyze the correlation between UWS value and focus score (FS) and anti-SSA antibodies. METHODS: The study group consisted of subjects above 18 years of age with a subjective feeling of oral dryness. RESULTS: A total of 105 subjects were qualified for the study. The final diagnosis of pSS was made in 44 patients according to the classification criteria from 2016. No age differences were identified between pSS patients and control group subjects (patients with dry mouth without autoimmune background). UWS rates were significantly lower in pSS patients than in the control group. No association was identified between UWS and focus score (FS) ≥ 1 in LSGB. No differences were observed between anti-SSA-positive and anti-SSA-negative patients in terms of age, UWS rates, FS. CONCLUSION: LSGB should be performed in all suspected pSS cases regardless of the UWS rate value, particularly in subjects without specific anti-SSA antibodies. In patients with suspected pSS, only less than one-half of the UWS measurements are below the value of 0.1 mL/min adopted as the threshold in the classification criteria for pSS.

Subphenotypes of ANCA-associated vasculitis identified by latent class analysis.

OBJECTIVES: ANCA-associated vasculitides (AAV) are a heterogeneous group of rare diseases with unknown aetiology 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) seem to be clinically much more uniform. Recently, three subcategories of AAV have been proposed and described as non-severe AAV, severe PR3-AAV, and severe MPO-AAV. METHODS: In line with these attempts, we decided to use an unbiased approach offered by latent class analysis (LCA) to subcategorise GPA and MPA in a large cohort of Polish AAV patients included in a multicentre POLVAS registry. RESULTS: LCA of our AAV group identified a four-class model of AAV, including previously proposed three subphenotypes and revealing a fourth (previously not described) clinically relevant subphenotype. This new subphenotype includes only GPA patients, usually diagnosed at a younger age as compared to other groups, and characterised by multiorgan involvement, high relapse rate, relatively high risk of death, but no end-stage kidney disease. CONCLUSIONS: Based on multiple clinical and serological variables, LCA methodology identified 4-class model of AAV. This newly described fourth class of AAV may be of clinical relevance and may require prompt diagnosis and aggressive treatment due to the multiorgan involvement, high risk of relapse and marked mortality among these relatively young GPA subjects.

The Clinical and Immunological Activity Depending on the Presence of Interferon γ in Primary Sjögren's Syndrome-A Pilot Study.

The upregulation of IFN pathways and their stimulated genes is associated with primary Sjögren's syndrome (pSS). The recent studies also indicate the involvement of interferon γ (IFNγ) in the pathogenesis of pSS. The study aimed to assess the clinical and immunological activity depending on the concentration of IFNγ in the peripheral blood in pSS patients. METHODS: The study group consisted of patients over 18 years of age with a confirmed diagnosis of pSS. Based on the collected data, disease activity was assessed using the EULAR Sjögren's syndrome disease activity index (ESSDAI) and the EULAR Sjögren's syndrome patient reported index (ESSPRI). RESULTS: Among 40 pSS patients, 33 (82%) showed increased levels of IFNγ. The group with positive IFNγ was younger (43 years) than the group with negative IFNγ (57 years) (p < 0.05). In the positive IFNγ group, the time to diagnosis was shorter (p < 0.05). There was a difference in ESSDAI among patients with and without IFNγ (p < 0.05). There were no differences between the groups in ESSPRI and the presence of cryoglobulins, specific anti-SSA, and anti-SSB antibodies and in C3 and C4 hypocomplementemia. RF occurred in both groups with a similar frequency (p = 0.6), but in patients with IFNγ presence, significantly higher RF titers were observed (34.9 vs. 10.5; p < 0.05). CONCLUSION: In the group of patients with positive IFNγ, the mean value of RF and ESSDAI was higher. This group was also younger than patients with pSS without IFNγ.

ANCA-associated vasculitis patients treated in Polish intensive care units - retrospective characteristics based on the POLVAS registry.

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.