Cranial and extracranial manifestations of giant cell arteritis: a single-center observational study.

INTRODUCTION: Giant cell arteritis (GCA) presents two major phenotypes - cranial (cGCA) and extracranial (exGCA). exGCA may be overlooked. The study aimed to compare the clinical characteristics between cGCA and exGCA. METHODS: Electronic medical records of patients treated between January 2015 and July 2023 at the Department of Rheumatology were searched for the diagnosis of GCA. The clinical characteristics of patients with cGCA, exGCA, and overlapping GCA manifestations were compared. RESULTS: Out of 32 patients with GCA, 20 had cGCA, 7 had exGCA, and 5 had overlap manifestations. The groups did not differ significantly in demographics, clinical signs/symptoms, or laboratory test results. Importantly, the combined group of patients with exGCA and overlap GCA had a statistically significant delay in initiating treatment (median 12 weeks) compared to patients with cGCA (median 4 weeks; p = 0.008). CONCLUSION: Our study confirmed the insidious nature of exGCA, which lacks distinctive clinical symptoms and consequently leads to delayed treatment.

Sleep-Specific Processing of Auditory Stimuli Is Reflected by Alpha and Sigma Oscillations.

Recent research revealed a surprisingly large range of cognitive operations to be preserved during sleep in humans. The new challenge is therefore to understand functions and mechanisms of processes, which so far have been mainly investigated in awake subjects. The current study focuses on dynamic changes of brain oscillations and connectivity patterns in response to environmental stimulation during non-REM sleep. Our results indicate that aurally presented names were processed and neuronally differentiated across the wake-sleep spectrum. Simultaneously recorded EEG and MEG signals revealed two distinct clusters of oscillatory power increase in response to the stimuli: (1) vigilance state-independent θ synchronization occurring immediately after stimulus onset, followed by (2) sleep-specific α/σ synchronization peaking after stimulus offset. We discuss the possible role of θ, α, and σ oscillations during non-REM sleep, and work toward a unified theory of brain rhythms and their functions during sleep.SIGNIFICANCE STATEMENT Previous research has revealed (residual) capacity of the sleeping human brain to interact with the environment. How sensory processing is realized by the neural assemblies in different stages of sleep is however unclear. To tackle this question, we examined simultaneously recorded MEG and EEG data. We discuss the possible role of θ, α, and σ oscillations during non-REM sleep. In contrast to versatile θ band response that reflected early stimulus processing step, succeeding α and σ band activity was sensitive to the saliency of the incoming information, and contingent on the sleep stage. Our findings suggest that the specific reorganization of mechanisms involved in later stages of sensory processing takes place upon falling asleep.

Osteoporosis and diabetes - possible links and diagnostic difficulties.

Objectives: In this review, the authors aimed to clarify the relationship between the occurrence of osteoporosis and diabetes, analyze the differences between the pathogenesis of osteoporosis in different types of diabetes and propose the most effective diagnostic strategy and fracture risk assessment in diabetic patients. Material and methods: A analysis of publications in MEDLINE, COCHRANE and SCOPUS databases was performed, searching for reports on the diagnostics, fracture risk assessment, prevention, and treatment of osteoporosis in patients with diabetes mellitus (DM) published in the years 2016-2022. The key words for the search were: diabetes, osteoporosis, and low-energy fracture. Results: Bone complications of T1DM are more severe than T2DM, because of the lack of anabolic effect of insulin on bones. In T2DM the risk of fractures is elevated; however, identifying the mechanisms underlying the increased risk of fractures in T2DM is not clear. The FRAX tool is not appropriate for assessing the fracture risk in young patients with T1DM. It is quite useful in older patients with T2DM, but in these patients the calculated fracture risk may be underestimated. In T2DM the fracture risk often does not correspond to BMD value as measured by dual-energy X-ray absorptiometry (DXA). Diagnostic tools such as the trabecular bone score may play a significant role in this group of patients. Conclusions: Optimal strategies to identify and treat high risk individuals require further research and proper definition. The diagnostic criteria for osteoporosis should be clearly defined as well as fracture risk assessment and choice of anti-osteoporotic medication. In all cases of secondary osteoporosis, treatment of the underlying disease is the most important. The relationship between high risk of fractures and diabetes is inseparable, and its full understanding seems to be the key to effective management.

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.

Comparison of treatment of severe rheumatoid arthritis patients with biological agents and JAK-STAT inhibitors. An observational study.

Introduction: Treatment of severe rheumatoid arthritis (RA) is currently based either on biological agents or Janus kinase/signal transducer and activator of transcription inhibitors, most often in combination with methotrexate (MTX). Aim of the study: The aim of the study was to compare the effectiveness and side effects of bio- logic disease-modifying antirheumatic drugs (bDMARDs) and targeted synthetic (ts) DMARDs treatment. Material and methods: The analysis included 108 RA patients with active disease treated with MTX 25 mg per week. Eighty patients (group I) were treated with bDMARDs and 28 patients (group II) with JAK-STAT inhibitors. The duration of morning stiffness, pain on Visual Analogue Scale (VAS), 28-joints Disease Activity Score (DAS28) and Simplified Disease Activity Score (SDAI) were assessed. Classical radiographic images of patients' hands and feet using the Larsen and Dale's criteria were evaluated. The effects of treatment with bDMARDs and tsDMARDs were analyzed. Results: All studied patients presented at least Larsen and Dale's stage 3 of X-ray changes typical for RA. There were no statistically significant differences in disease duration, ESR, CRP, DAS28 and SDAI values between studied groups. Patients from group II previously used higher numbers of bDMARDs than group I treated with bDMARDs. Low disease activity after treatment was achieved by all patients; therefore patients from group II (treated with tsDMARDs) achieved lower values of patients' global assessment on VAS. Conclusions: The results of the present observational study indicated that treatment with JAK inhibitors is very promising. These drugs are not inferior in effectiveness to bDMARDs. It is important to monitor patients for thromboembolic events before and during JAK treatment.

Heart disease in the course of systemic sclerosis - an observational study.

Introduction: Cardiac involvement is one of the major mortality factors in systemic sclerosis (SSc). This observational study aimed to compare patients with and without heart involvement in the course of SSc. Material and methods: Electronic medical records of patients treated between January 2021 and August 2022 in the Department of Rheumatology were searched for the diagnosis of SSc (ICD-10 code M47). The clinical characteristics of patients with and without heart involvement in the course of SSc were compared. Results: Out of 36 patients with SSc, 7 patients were diagnosed with heart disease in the course of SSc. The major type of cardiac involvement was myocarditis (71%). The majority of patients with heart involvement had elevated troponin (86%) and NT-proBNP (71%) concentrations. The most common finding observed in echocardiography was diastolic failure (71%). The most frequent abnormality found in 24-hour Holter ECG was isolated supraventricular extrasystoles (100%). Risk factors for developing heart disease in the course of SSc were male gender (p = 0.018), diffuse type of SSc (p = 0.03), higher values of modified Rodnan skin score (p < 0.001), gastrointestinal tract involvement (p = 0.027) and myositis (p = 0.018). Conclusions: Optimal screening for heart disease is needed in this group of patients.

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.

Decoding Brain Responses to Names and Voices across Different Vigilance States.

Past research has demonstrated differential responses of the brain during sleep in response especially to variations in paralinguistic properties of auditory stimuli, suggesting they can still be processed "offline". However, the nature of the underlying mechanisms remains unclear. Here, we therefore used multivariate pattern analyses to directly test the similarities in brain activity among different sleep stages (non-rapid eye movement stages N1-N3, as well as rapid-eye movement sleep REM, and wake). We varied stimulus salience by manipulating subjective (own vs. unfamiliar name) and paralinguistic (familiar vs. unfamiliar voice) salience in 16 healthy sleepers during an 8-h sleep opportunity. Paralinguistic salience (i.e., familiar vs. unfamiliar voice) was reliably decoded from EEG response patterns during both N2 and N3 sleep. Importantly, the classifiers trained on N2 and N3 data generalized to N3 and N2, respectively, suggesting similar processing mode in these states. Moreover, projecting the classifiers' weights using a forward model revealed similar fronto-central topographical patterns in NREM stages N2 and N3. Finally, we found no generalization from wake to any sleep stage (and vice versa) suggesting that "processing modes" or the overall processing architecture with respect to relevant oscillations and/or networks substantially change from wake to sleep. However, the results point to a single and rather uniform NREM-specific mechanism that is involved in (auditory) salience detection during sleep.

Standing sentinel during human sleep: Continued evaluation of environmental stimuli in the absence of consciousness.

While it is a well-established finding that subjects' own names (SON) and familiar voices are salient during wakefulness, we here investigated processing of environmental stimuli during sleep including deep N3 and REM sleep. Besides the effects of sleep depth we investigated how sleep-specific EEG patterns (i.e. sleep spindles and slow oscillations [SOs]) relate to stimulus processing. Using 256-channel EEG we studied processing of auditory stimuli by means of event-related oscillatory responses (de-/synchronisation, ERD/ERS) and potentials (ERPs) in N = 17 healthy sleepers. We varied stimulus salience by manipulating subjective (SON vs. unfamiliar name) and paralinguistic emotional relevance (familiar vs. unfamiliar voice, FV/UFV). Results reveal that evaluation of voice familiarity continues during all NREM sleep stages and even REM sleep suggesting a 'sentinel processing mode' of the human brain in the absence of wake-like consciousness. Especially UFV stimuli elicit larger responses in a 1-15 Hz range suggesting they continue being salient. Beyond this, we find that sleep spindles and the negative slope of SOs attenuate information processing. However, unlike previously suggested they do not uniformly inhibit information processing, but inhibition seems to be scaled to stimulus salience.

Better than sham? A double-blind placebo-controlled neurofeedback study in primary insomnia.

See Thibault et al. (doi:10.1093/awx033) for a scientific commentary on this article.Neurofeedback training builds upon the simple concept of instrumental conditioning, i.e. behaviour that is rewarded is more likely to reoccur, an effect Thorndike referred to as the 'law of effect'. In the case of neurofeedback, information about specific electroencephalographic activity is fed back to the participant who is rewarded whenever the desired electroencephalography pattern is generated. If some kind of hyperarousal needs to be addressed, the neurofeedback community considers sensorimotor rhythm neurofeedback as the gold standard. Earlier treatment approaches using sensorimotor-rhythm neurofeedback indicated that training to increase 12-15 Hz sensorimotor rhythm over the sensorimotor cortex during wakefulness could reduce attention-deficit/hyperactivity disorder and epilepsy symptoms and even improve sleep quality by enhancing sleep spindle activity (lying in the same frequency range). In the present study we sought to critically test whether earlier findings on the positive effect of sensorimotor rhythm neurofeedback on sleep quality and memory could also be replicated in a double-blind placebo-controlled study on 25 patients with insomnia. Patients spent nine polysomnography nights and 12 sessions of neurofeedback and 12 sessions of placebo-feedback training (sham) in our laboratory. Crucially, we found both neurofeedback and placebo feedback to be equally effective as reflected in subjective measures of sleep complaints suggesting that the observed improvements were due to unspecific factors such as experiencing trust and receiving care and empathy from experimenters. In addition, these improvements were not reflected in objective electroencephalographic-derived measures of sleep quality. Furthermore, objective electroencephalographic measures that potentially reflected mechanisms underlying the efficacy of neurofeedback such as spectral electroencephalographic measures and sleep spindle parameters remained unchanged following 12 training sessions. A stratification into 'true' insomnia patients and 'insomnia misperceivers' (subjective, but no objective sleep problems) did not alter the results. Based on this comprehensive and well-controlled study, we conclude that for the treatment of primary insomnia, neurofeedback does not have a specific efficacy beyond unspecific placebo effects. Importantly, we do not find an advantage of neurofeedback over placebo feedback, therefore it cannot be recommended as an alternative to cognitive behavioural therapy for insomnia, the current (non-pharmacological) standard-of-care treatment. In addition, our study may foster a critical discussion that generally questions the effectiveness of neurofeedback, and emphasizes the importance of demonstrating neurofeedback efficacy in other study samples and disorders using truly placebo and double-blind controlled trials.

Comorbidities in rheumatic arthritis.

OBJECTIVES: Rheumatoid arthritis (RA) is one of the most common systemic inflammatory diseases, but its etiology is still not fully known. The aim of this preliminary study was to assess what particular comorbidities are involved in the progression of RA and determine the influence that the aforementioned diseases have on each other. MATERIAL AND METHODS: Forty patients with diagnosed RA according to EULAR/ACR criteria from 2010 were included in the study. The majority of the group was female (n = 35; 87.5%). Patients were tested using routine laboratory and imaging methods allowing diagnosis and assessment of disease activity. Dual energy X-ray absorptiometry was also evaluated for mineral density. The activity of the disease was assessed using the disease activity score DAS28 (ESR) and SDAI (Simplified Disease Activity Index). RESULTS: Among studied patients, based on the DAS28 index, 9 patients were in the remission phase (22.5%) and 12 (30%) had high disease activity. Increased values of CRP were observed in the majority of patients (65%). The group analysis demonstrated the most common comorbidities in patients with RA, as follows: hypertension (n = 14; 35%) and osteoporosis or osteopenia (n = 13; 32.6%). CONCLUSIONS: Patients with rheumatoid arthritis (RA) are more susceptible to developing hypertension and osteoporosis. We did not observe a significant association between other comorbidities and activity of RA. The next study will assess a larger number of patients.

Can self-relevant stimuli help assessing patients with disorders of consciousness?

Emotional and self-relevant stimuli are able to automatically attract attention and their use in patients suffering from disorders of consciousness (DOC) might help detecting otherwise hidden signs of cognition. We here recorded EEG in three Locked-in syndrome (LIS) and four Vegetative State/Unresponsive Wakefulness Syndrome (VS/UWS) patients while they listened to the voice of a family member or an unfamiliar voice during a passive. Data indicate that, in a passive listening condition, the familiar voice induces stronger alpha desynchronization than the unfamiliar one. In an active condition, the target evoked stronger alpha desynchronization in controls, two LIS patients and one VS/UWS patient. Results suggest that self-relevant familiar voice stimuli can engage additional attentional resources and might allow the detection of otherwise hidden signs of instruction-following and thus residual awareness. Further studies are necessary to find sensitive paradigms that are suited to find subtle signs of cognition and awareness in DOC patients.

[Catastrophic antiphospholipid syndrome]. / Katastrofalny zespól antyfosfolipidowy.

Catastrophic antiphospholipid syndrome is the most dangerous form of the antiphospholipid syndrome, which is characterized by rapid onset of thrombosis in small vessels of many organs and intravascular coagulation, thrombocytopenia and hemolytic anemia. The syndrome develops over a short period of time with acute multi-organ failure, including kidney, respiratory, cardiovascular, central nervous system and adrenal glands, often associated with disseminated thrombotic microangiopathy. The catastrophic antiphospholipid syndrome involves three or more systems, organs and/or tissues, the development of symptoms must occur within less than one week, it is necessary to confirm the histopathological vascular occlusion in at least one organ or tissue, and laboratory confirmation of the presence of antiphospholipid antibodies in the serum on two occasions over an interval of 12 weeks. This syndrome is characterized by a high mortality despite the use of optimal treatment. Early diagnosis and aggressive treatment of patients with catastrophic antiphospholipid syndrome is essential to save the life of these patients. In the last 10 years, the mortality in this disease decreased from 50% to 30% with simultaneous treatment with anticoagulants, corticosteroids, plasmapheresis and immunoglobulins.

Adult-Onset Still's Disease (AOSD)-On the Basis of Own Cases.

INTRODUCTION: Adult-onset Still's disease (AOSD) is a rare chronic autoinflammatory condition characterized by a spiking fever, arthritis, a rash, hepatosplenomegaly, lymphadenopathy, leucocytosis, and hyperferritinemia. It is sometimes accompanied by life-threatening complications like macrophage activation syndrome/hemophagocytic lymphohistiocytosis (MAS/HLH). Treatment options for AOSD include glucocorticoids (GCs), immunosuppressive drugs, biological medications, and Janus kinase (JAK) inhibitors. The features that differentiate MAS/HLH from AOSD are: in MAS/HLH, a different type of fever, which is persistent, a sharp decrease in the number of leukocytes and thrombocytes, a further increase in the level of transaminases and ferritin, significant hepatosplenomegaly, lymphadenopathy, symptoms of the central nervous system (CNS), disseminated intravascular coagulation (DIC) and hemophagocytosis in the bone marrow. This study aimed to evaluate the course of AOSD, which results in MAS/HLD. PATIENTS AND METHODS: Nine AOSD patients, four of whom developed MAS/HLH, were treated at the Rheumatology Clinic in the Central Clinical Hospital of the Ministry of Interior Affairs from 1 January 2015 to 15 March 2020 and at the Rheumatology Clinic in the National Institute of Geriatric, Rheumatology and Rehabilitation from 1 September 2021 to 1 March 2024. Medical history, clinical data, demographic data, laboratory data, imaging data, Hscore, and treatment data were collected. RESULTS: All the patients with MAS and an Hscore above 150 recovered. DISCUSSION: MAS/HLH requires rapid diagnosis as well as treatment with methylprednisolone pulses, cyclosporine A, and etoposide. When comparing patients who developed MAS/HLH with those who did not, possible risk factors were identified: the presence of pregnancy (two cases) and an aggressive course of AOSD. The Hscore is a useful tool for identifying patients with MAS/HLH.

Time Trend Analysis of Comorbidities in Ankylosing Spondylitis: A Population-Based Study from 53,142 Hospitalizations in Poland.

BACKGROUND: (1) Influence of comorbidities on life expectancy and treatment outcomes is one of the main concerns of modern rheumatology, due to their rising prevalence and increasing impact on mortality and disability. The main objective of our study was to analyze the time trends and shifts in the comorbidity profile and mortality over 10 years in the Polish population with ankylosing spondylitis (AS). (2) Data from 2011-2020 years were acquired from the General Hospital Morbidity Study in the National Institute of Public Health-National Institute of Hygiene (NIH-PIB) as ICD-10 codes. Based on ICD10 codes, we calculated the percentage shares for comorbidities, with the relative risk ratios and odds ratios. We analyzed the hospitalization rates and mortality from the overlapping conditions. Also, we analyzed age and sex related differences in the clinical manifestations of AS patients. (3) Results: From 53,142 hospitalizations of patients with AS, we found that the male population presented higher rates of cardiovascular (2.7% vs. 1.3% p < 0.001) and pulmonary conditions (1.2% vs. 0.8% p < 0.025). Inflammatory bowel diseases were more common in the female population than in males (2.3% vs. 1.7%, p < 0.001). In the years 2011-2020, we observed a decline in the number of hospitalized patients due to cardiovascular (p < 0.001) and respiratory system conditions (p < 0.001), yet the relative risk and odd ratios remained high. In the years 2011-2020, 4056 patients received biological treatment (7%). The number of initiated biological therapies correlated negatively with the number of reported hospitalizations due to ischemic heart diseases (IHD) (p < 0.031, r = -0.8). Furthermore, in the logistic regression model, we found strong collinearity between cardiovascular and pulmonary comorbidities (VIF = 14; tolerance = 0.1); also, the number of reported IHD's correlated positively with the number of pulmonary infections (p < 0.031, r = 0.7) (4). CONCLUSIONS: Cardiopulmonary comorbidities are a main factor associated with increased mortality in patients with AS, especially in hospitalized patients. The mortality rates among patients with AS admitted to hospital due to other conditions other than movement disorders exceed the populational risk. The number of biologically treated patients correlated negatively with hospital admissions due to IHD.

Increased interest with the introduction of fast-track diagnostic pathway is associated with the regionally increased frequency of giant cell arteritis in Poland: a study based on POLVAS registry data.

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.

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.

Preliminary report on a study of health-related quality of life in patients with rheumatoid arthritis.

There are studies about health-related quality of life (HRQoL) in patients with rheumatoid arthritis (RA), but few studies prospectively assessed HRQoL. The main purpose of this study was to analyze HRQoL in patients hospitalized due to RA exacerbation and observed over a planned 2-year follow-up in an outpatient setting. The study involved 42 women and 9 men, at mean age of 62.5 years (SD ± 12.6). The mean duration of the study was 22-23 months. The HRQoL analysis was performed using the SF-36 survey. At the beginning of the study, basic data on age, sex, selected biochemical (ESR, CRP, GFR, hemoglobin, plasma albumin, plasma protein), and clinical parameters (the duration of RA, VAS, DAS28, BMI, the presence of cardiovascular disease, diabetes, osteoporosis, osteoporotic fractures, osteoarthritis, neoplasm) were collected. Questionnaires were completed at the beginning and end of the study. Statistically significant reductions in HRQoL scores were observed in social functioning (SF; 0.42 vs 0.32, P < 0.05), whereas role-emotional health (RE; 0.48 vs 0.59, P < 0.05) and mental health (MH; 0.47 vs 0.54, P < 0.05) scores were increased. A decrease in the SF was positively correlated with the lack of osteoporosis at baseline (r = 0.35, P > 0.02). An increase in the MH was inversely correlated with BMI (r = -0.31, P < 0.05), and the level of hemoglobin (r = -0.32, P < 0.028) and positively correlated with the presence of osteoarthritis at baseline (r = 0.29, P < 0.05). In RA patients, dimensions of HRQoL as SF, RE, and MH could change within 2 years and these changes could be related to comorbidities. Although preliminary findings are promising, further studies are needed.

The Assessment of the Quality of Life in Patients with Rheumatoid Foot.

BACKGROUND: The negative effects of rheumatoid arthritis (RA) are multi-dimensional. Foot deformities lead to disability, pain, and impaired quality of life. OBJECTIVE: Identifying the difficulties in functioning rheumatoid foot and assessing the quality of life in this aspect. MATERIALS AND METHODS: The material included 50 patients of Rheumatology Policlinic of the Central Clinical Hospital of Interior Affairs in Warsaw and a matched control group of 50 individuals without RA. The degree of foot joint damage was assessed using the Manchester scale, lower limb movement and quality of life using the American Orthopedic Foot and Ankle Society Score and HAQ. RESULTS: The duration of symptoms was 16.0 ± 8.9 years. High activity of RA measured by the DAS was observed in 20% of patients, moderate in 26%, and low in 54%. The most common foot deformities were: hammer toes (82%), longitudinal flat feet (74%), and hyperkeratosis (56%). The least frequent were: stiff toe (38%) and overlapping fingers (28%). In the RA group, the outcomes of the FAOS questionnaire were statistically significantly worse than in the control group in all categories (p<0.001). The worst-rated domain was the sport and recreation subscale (median 55.0), the best daily activity (median 86.8). The strongest relationship was demonstrated between the FAOS and HAQ indices. Spearman's HAQ correlation coefficient with the ADL subscale was r=-0.85, p<0.001; with the QOL, sport/recreation and pain subscales moderate, it was r=-0.72; r= 0.71, p <0.001. CONCLUSION: Lower limb movement function and quality of life are worse in RA patients; pain accompanies climbing and descending stairs; running and jumping require effort.

Combination Treatment of Locoregionally Aggressive Granulomatosis with Polyangiitis and Cranial Base Infiltration.

OBJECTIVES: To present a personalized approach in three cases of treatment-resistant, locoregionally aggressive forms of cANCA-positive granulomatosis with polyangiitis (GPA) and skull base involvement. METHODS: Three patients with GPA and skull base involvement were described alongside a critical review of the current literature. RESULTS: All presented patients suffered from GPA with an inflammatory tumor at the skull base, alongside cerebellopontine angle involvement, cranial nerve palsies, cerebellar disorders, concomitant hearing loss, and severe otalgia. Symptoms were associated with progressive granulomatous destruction of the temporal bone, laryngopharynx, and central nervous system infiltration. Treatment with cyclophosphamide and high doses of glucocorticoid steroids were ineffective but subsequent therapy with rituximab was successful in the presented cases. The literature review showed that the course of the disease with skull base involvement is associated with poorer clinical and radiological responses to standard pharmacotherapies. CONCLUSION: Granulomatous inflammation localized in the skull base is associated with a more aggressive disease progression and is less likely to respond to pharmacotherapy. Standard induction therapy with cyclophosphamide and glucocorticoid steroids may be ineffective. A better response may be achieved by using rituximab and concomitant local treatment with glucocorticoid steroid injections.