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Background Suicidal ideation is observed in patients with systemic lupus erythematosus (SLE). No study on this notable phenomenon in neuropsychiatric SLE (NPSLE) is available so far. Methods Participants were 53 consecutive outpatients with NPSLE (48 women; mean age 43.8 years) diagnosed according to the American College of Rheumatology nomenclature for SLE neuropsychiatric syndromes. A Neuropsychiatric Questionnaire (NP-Q) concerning 45 neurological, cognitive and psychiatric symptoms was used to assess the prevalence of self-perceived neuropsychiatric symptoms. The Modified Hospital Anxiety and Depression Scale (HADS-M) was used to assess the level of anxiety, depression and irritability. Formal neuropsychological examination was performed. Clinical data were collected by means of medical charts review and structured interview. Results Suicidal thoughts were present in 25% of patients with NPSLE, irrespective of sex, age, education, work status, disease duration and steroid treatment. Suicidal ideation was connected with elevated levels of depression, anxiety and irritability. In patients with suicidal ideation the prevalence of cognitive, psychiatric and neurological self-perceived problems was significantly higher. Conclusions Suicidal thoughts are common in patients with NPSLE. Neuropsychiatric manifestation per se, depression, anxiety and patients’ subjective complaints can be risk factors for suicidal ideation. Screening for suicidal thoughts is vital in routine care of SLE patients. [ABSTRACT FROM AUTHOR]

(1) Background: Impaired nutritional status in systemic sclerosis (SSc) is prevalent. (2) Objective: This study aimed to identify pre-cachexia and malnutrition in SSc patients and to estimate the effectiveness of a high-protein oral nutritional supplement (ONS) in improving their nutritional status. (3) Materials and methods: The SSc population comprised 56 patients and a control group of 49 healthy persons. After a baseline clinical evaluation, bioelectrical impedance analysis (BIA), and laboratory tests, SSc patients were divided into well-nourished, pre-cachectic, and malnourished categories. SSc patients with a nutritional disbalance received a high-protein ONS once daily for 3 months. Patients were reassessed at 3 and 12 months after inclusion in the study. (4) Results: SSc patients, in comparison to the control group, had a significantly lower seven-point SGA value [6(0) vs. 7(1), p < 0.001)], lean tissue mass [LTM, 35.1 (10.5) vs. 40.1 (10.10), p = 0.008], and lean tissue index [LTI, 13.5 (3) vs. 14.9 (4), p = 0.009]. Of the 56 SSc patients, 40 (71.4%) were well nourished, 5 (8.9%) had pre-cachexia, and 11 (19.7%) were malnourished. A high-protein ONS in the pre-cachexia group stabilized the SGA value, anthropometric measurements, and BIA after 3 and 12 months. In malnourished patients, it significantly improved the SGA value [5(0) vs. 6(0), p = 0.002], LTI [12.1 (2.1) vs. 12.7 (3.2), p = 0.021] and LTM [31.1 (7.7) vs. 35.1 (9.1), p = 0.021], and that effect remained stable at 12 months. (5) Conclusion: Malnutrition is a common complication of SSc that can be improved with nutritional intervention.

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/ μ l) (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/ μ l, 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., Competing Interests: All authors have declared no conflicts of interest., (Copyright © 2024 Anna Drynda et al.)

Mixed connective tissue disease (MCTD) is a very rare disorder that belongs in the rare and clinically multifactorial groups of diseases. The pathogenesis of MCTD is still unclear. The best understood epigenetic alteration is DNA methylation whose role is to regulate gene expression. In the literature, there are ever-increasing assumptions that DNA methylation can be one of the possible reasons for the development of Autoimmune Connective Tissue Diseases (ACTDs) such as systemic sclerosis (SSc) and systemic lupus erythematosus (SLE). The aim of this study was to define the global DNA methylation changes between MCTD and other ACTDs patients in whole blood samples. The study included 54 MCTD patients, 43 SSc patients, 45 SLE patients, and 43 healthy donors (HC). The global DNA methylation level was measured by ELISA. Although the global DNA methylation was not significantly different between MCTD and control, we observed that hypomethylation distinguishes the MCTD patients from the SSc and SLE patients. The present analysis revealed a statistically significant difference of global methylation between SLE and MCTD ( p < 0.001), SLE and HC ( p = 0.008), SSc and MCTD ( p ≤ 0.001), and SSc and HC ( p < 0.001), but neither between MCTD and HC ( p = 0.09) nor SSc and SLE ( p = 0.08). The highest % of global methylation (median, IQR) has been observed in the group of patients with SLE [0.73 (0.43, 1.22] and SSc [0,91 (0.59, 1.50)], whereas in the MCTD [0.29 (0.20, 0.54)], patients and healthy subjects [0.51 (0.24, 0.70)] were comparable. In addition, our study provided evidence of different levels of global DNA methylation between the SSc subtypes ( p = 0.01). Our study showed that patients with limited SSc had a significantly higher global methylation level when compared to diffuse SSc. Our data has shown that the level of global DNA methylation may not be a good diagnostic marker to distinguish MCTD from other ACTDs. Our research provides the groundwork for a more detailed examination of the significance of global DNA methylation as a distinguishing factor in patients with MCTD compared to other ACTDs patients.

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.

Introduction: Mixed connective tissue disease (MCTD) is a rare disease with clinical picture consisted of multiple organ manifestations, including skin changes resembling systemic lupus erythematosus (SLE), systemic sclerosis (SSc), or dermatomyositis (DM). On the background of these manifestations are microvascular changes - alteration of endothelial function and impairment of endothelial progenitor cell. Nailfold capillaroscopy (NFC) is a simple, non-invasive technique for investigating microvascular involvement in rheumatic diseases., Objectives: To describe the relationship between type of skin lesions and NFC pattern in MCTD patients., Methods: We analyzed the clinical picture and NFC patterns in 79 patients with MCTD. The NFC changes were classified into Normal, "Early," "Active," and "Late" scleroderma-like patterns (SD-like pattern) based on Cutolo classification. In all patients, subjective and physical examinations were carried out, specifically the occurrence of skin lesions in the course of MCTD was assessed (systemic sclerosis-like (Ssc-like), systemic lupus erythematosus-like (SLE-like), dermatomysitis-like (DM-like))., Results: Skin changes were present in 64 (81%) patients, involving 43 (54%) SLE-like, 48 (61%) SSc-like, and 4 (5.1%) DM-like. NFC changes were observed in a total of 55 (69.6 %) patients with predominance of the "Early" pattern - 41 (51.9 %) patients. According to skin change phenotypes, NFC changes were observed in 31 (72%) patients with SLE-like and in 32 (66.7%) patients with SSc-like skin phenotypes. The "early" pattern predominated in both group., Conclusions: We did not find any correlation between NFC pattern and the type skin changes. Key Points • The study did not show a correlation between the presence and absence of skin lesions and NFC pattern. • Scleroderma-like patterns were found in over 60% of patients with mixed connective tissue disease. • The "early" pattern is dominant regardless of the occurrence or absence of skin lesions in patients with MCTD. • Skin lesions, regardless of their type (SLE or SSc), do not correlate with type of lesion found in the NFC examination., (© 2021. The Author(s).)

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.

Objectives: Sjögren's syndrome (SS) is a chronic inflammatory disease with an autoimmune background with possible complications from peripheral (PNS) and central nervous system (CNS). The aim of this study was to assess the prevalence and to describe the phenotype of peripheral neuropathies in patients with SS., Materials & Methods: We studied fifty patients with primary Sjögren's syndrome for peripheral nervous system involvement. All patients underwent neurological and rheumatological examination followed by nerve conduction studies (NCS) of nine peripheral nerves., Results: Thirty-six patients (72%) fulfilled the criteria for the diagnosis of neuropathy. Carpal tunnel syndrome (54%) and axonal sensorimotor neuropathy (22%) were the most common. Neurological symptoms preceded the diagnosis of SS in eight patients., Conclusions: Peripheral neuropathies are frequent in SS patients. Neurologists should be aware of possible autoimmune causes of neuropathies because clinical manifestations of neuropathy may precede the development of other symptoms of the autoimmune disease., (© 2020 The Authors. Brain and Behavior published by Wiley Periodicals LLC.)

Sjögren's syndrome (SS) is a chronic autoimmune disease with a wide spectrum of possible organ involvement. Peripheral (PNS) and central nervous system (CNS)-related symptoms may occur in the course of the disease. The aim of this study was to compare the health-related quality of life (HR-QOL) in SS patients with and without peripheral neuropathy. The study involved 50 patients with primary Sjögren's syndrome (pSS). All patients underwent neurological clinical examination followed by nerve conduction studies (NCS) and rheumatological examination. Thirty-six-item Short Form Health Survey (SF-36) was used for evaluating HR-QOL. To assess pSS activity, the EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) and EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI) were used. For the assessment of clinical disability due to peripheral neuropathy, the Overall Disability Sum Score scale (ODSS) was used. Additional evaluation of pain was performed with the use of the Visual Analogue Scale (VAS) and a semistructured interview. Twenty-three (46%) patients were diagnosed with peripheral neuropathy. The most common PNS manifestation was sensorimotor neuropathy (47%). Neurological symptoms preceded the diagnosis of pSS in eight patients. The following domains of the SF-36 form were significantly lower scored by patients with peripheral nervous system involvement: role-physical [0 (0-100) vs. 75 (0-100)], role-emotional [67 (0-100) vs. 100 (0-100)], vitality [40 (10-70) vs. 50 (20-75)], bodily pain [45 (10-75) vs. 55 (0-100)], and general health [20 (5-50) vs. 30 (0-50)] (p ≤ 0.05). Our study showed that peripheral neuropathy was a common organ-specific complication in SS patients. In pSS patients, coexisting neurological involvement with symptoms such as pain and physical disability may be responsible for diminished HR-QOL.

Purpose: The aim of this study is to present the treatment modalities and associated side effects in a Polish nation-wide ANCA-associated vasculitides (AAV) patients' cohort., Materials and Methods: Retrospective analysis of patients diagnosed with AAV between 1990 and 2016, included in the POLVAS registry was performed. Standard descriptive statistic methods were used with an emphasis on the treatment modalities., Results: There were 625 patients diagnosed with AAV included in this study: 417 cases of granulomatosis with polyangiitis (GPA; 66.7%), 106 cases of microscopic polyangiitis (MPA; 17.0%) and 102 cases of eosinophilic granulomatosis with polyangiitis (EGPA; 16.3%). The mean age at the date of diagnosis was 50.4 (±15.7) years and the median observational period amounted to 4.0 (2.0-8.0) years. Glucocorticosteroids (GCs) were the medicaments most frequently used for remission induction (593/622; 95.3%), followed by cyclophosphamide (487/622; 78.3%), rituximab (44/622; 7.1%), and methotrexate (39/622; 6.3%). GCs were also most frequently administered for maintenance therapy (499/592; 84.3%), followed by azathioprine (224/592; 37.8%), methotrexate (136/592; 23.0%) and mycophenolate mofetil (99/592; 16.7%). The median cumulative doses of cyclophosphamide and rituximab equalled 7.99 g (4.18-14.0) and 2000 mg (1500-2800), respectively. The most commonly observed adverse events included: infections - 214/551 cases (38.8%), which were associated with the time of observation (OR = 1.05; 95% CI 1.01-1.10), the use of GCs intravenous pulses (OR = 2.76; 95% CI 1.68-4.54) and need for haemodialysis (OR = 1.73; 95% CI 1.10-2.71)., Conclusions: Polish patients with AAV were predominantly treated according to appropriate guidelines. The most frequent adverse events were typical for usually administered immunosuppressive treatment., Competing Interests: Declaration of competing interest The authors declare no conflict of interests., (Copyright © 2020 The Authors. Published by Elsevier B.V. All rights reserved.)

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.

Introduction: Granulomatosis with polyangiitis (GPA) is a rare, ANCA-associated, systemic disease characterized by necrotizing small and medium vessel vasculitis of unknown etiology associated with granulomatous inflammation affecting the renal, pulmonary, upper airways, ocular systems and other tissues. Histological proof of the granulomatosis with polyangiitis (GPA) can be obtained by biopsy of clinically involved sites. The main purpose of this study was to examine histopathological changes in non-renal biopsies from patients with established diagnosis of GPA and evaluated the histological confirmation at diagnosis of this disease., Material and Methods: A retrospective analysis was performed in patients with GPA diagnosed and treated in clinics of the University Clinical Center (UCK) in Gdansk in 1988-2009., Results: In the analyzed group of GPA patients the histopathological examination of biopsies taken from involved tissues (except kidney) was performed in 60% of patients. Thirty-six out of 93 biopsies (39%) were diagnosed as typical of GPA, 10 (10.7%) were suggestive and 51 (54.8%) were non-specific. Considering all biopsies, the diagnosis was confirmed in 24 patients (57% of patients in whom biopsies were taken). Epitheloid cell granulomas were present in 33 biopsies (43%), characteristic necrosis in 27 biopsies (35%), small vessel vasculitis in 18 biopsies (23%), while multinucleated giant cells were identified only in 9 biopsies (12%)., Conclusions: Histopathological examination of the affected tissues remains the gold standard of the diagnosis of GPA. Its usefulness increases, particularly in ANCA-negative patients, in the initial phase of the disease, or in patients with atypical clinical presentation. In many cases, it is necessary to repeat biopsy to establish the diagnosis. The role of the histopathological examination seems to be particularly important when ANCA is negative or clinical symptoms are atypical of GPA., Competing Interests: The authors declare no conflict of interest.

The study evaluates the psychometric properties of a Polish translation of the Brief Illness Perception Questionnaire. A total of 276 patients with chronic conditions (58.7% women) completed the Brief Illness Perception Questionnaire and the Hospital Anxiety and Depression Scale. The internal consistency of the Polish Brief Illness Perception Questionnaire measured with Cronbach's alpha was satisfactory (α = 0.74). Structural validity was demonstrated by significant inter-correlations between the Brief Illness Perception Questionnaire components. Discriminant validity was supported by the fact that the Brief Illness Perception Questionnaire enables patients with various conditions to be differentiated. Significant correlations were found between Brief Illness Perception Questionnaire and depression and anxiety levels. The Polish Brief Illness Perception Questionnaire thus evaluated is a reliable and valid tool., (© The Author(s) 2015.)

The aim of the present study was to assess and compare illness perception of systemic lupus erythematosus (SLE) held by 6th-year medical students and patients suffering from SLE. The study group consisted of 104 students (66 women; 63.5 %), mean age 24.7 (±1), and 64 outpatients with SLE (60 women; 93.7 %). All patients were treated at a university rheumatology outpatient clinic. Mean patients' age was 44.3 years (±12.5). Mean duration of the disease was 11 years (±6.8). The Polish version of Brief Illness Perception Questionnaire (B-IPQ) was used to assess five dimensions of illness perception. The students were asked to complete a modified version of B-IPQ designed to measure health professionals' illness perception. Significant differences were found in all but one B-IPQ scores. The students obtained significantly higher scores than the SLE patients in consequences, identity, concern and emotional response, whereas significantly lower scores in personal control, treatment control and understanding were noted among students. No differences were found in timeline scores. Medical students' perception of SLE is more threatening and more negative than that of patients'. Doctors-to-be perceive SLE as being less controllable, more burdensome and having more consequences than patients do. Additionally, they believe the disease causes more emotional concern. The article discusses possible explanations together with positive and negative aspects of the discrepancies.

Objectives: Inflammatory myopathies are a group of idiopathic, heterogeneous systemic diseases affecting predominantly skeletal muscles, though they can also involve the skin and internal organs. The association between cancer and idiopathic inflammatory myopathies, particularly dermatomyositis, which is termed cancer-associated myositis (CAM), has been reported in the medical literature. A newly described autoantibody to a 155-kDa nuclear protein, identified as transcription intermediary factor 1-gamma (TIF1-γ), has proven useful for cancer screening in patients with dermatomyositis., Material and Methods: Based on our database of laboratory results, between November 2014 and January 2016, we found 80 patients with a positive autoimmune inflammatory myopathy immunoblot profile., Results: Eleven of 80 patients revealed the presence of anti-TIF1-γ antibodies: 8 women and 3 men with average age 54.2 years. Dermatomyositis (DM) was diagnosed in 6 cases, polymyositis in 1 case, myositis limited to ocular muscles and rhabdomyolysis in 1 case each, and undifferentiated connective tissue disease in 2 cases. Neoplasm was found in 4 cases. All of those patients had DM. The average time between DM and diagnosis of neoplasm was 7.5 months (from 1 to 18 months)., Conclusions: The association between cancer and idiopathic inflammatory myopathies, particularly DM, is well known, and cancer screening should be obligatory in such patients. So far there is no consensus as to the method or frequency with which patients with an idiopathic inflammatory myopathy should be tested to rule out neoplasm. Detection of anti-TIF1-γ antibodies in patients with DM gives the clinicians the very important suggestion of CAM. It seems reasonable that these patients should have more detailed and often repeated differential diagnostics.

The macrophage activation syndrome (MAS) is a rare and potentially fatal disease. This syndrome is founded on congenital or acquired dysfunction of NK cells resulting in secondary activation and proliferation of macrophages with excessive cytokine production and organ infiltration. Causes of acquired MAS include viral infections (chiefly EBV and CMV), malignancies, and autoimmune diseases. The macrophage activation syndrome is usually associated with juvenile idiopathic arthritis and adult-onset Still's disease and rarely with rheumatoid arthritis, systemic lupus erythematosus, dermatomyositis, and systemic sclerosis. Fever, hepatosplenomegaly, lymphadenopathy, and bi- or pancytopenia in peripheral blood represent typical symptoms of MAS. Hyperferritinemia, hypertriglyceridemia, hypertransaminasemia, and hypofibrinogenemia are among the common laboratory findings. The macrophage activation syndrome is a life-threatening condition requiring aggressive therapy due to multiple organ dysfunction. Treatment also includes elimination of the triggering infection and high-dose glucocorticosteroids. Second-line therapy is based on cyclosporin, intravenous immunoglobulins, and etoposide. The present work focuses on diagnostic and therapeutic difficulties in three patients with the macrophage activation syndrome.

Global disease activity measurement in systemic lupus erythematosus (SLE) patients is important for the clinical estimation and adjustment of therapy. By contrast, immune system activation plays a significant role in disease pathogenesis, with CD4+ lymphocytes acting as central cells in the immune response. We investigated which scale better correlates with immunologic changes in the blood of SLE patients, the SLE Disease Activity Index (SLEDAI) or the Systemic Lupus Activity Measure (SLAM) scale. Samples of peripheral blood were obtained from 45 SLE patients with different disease activity as assessed by the SLEDAI and the SLAM scales on the same day. We assessed the percentage of CD4+ T cells with activation-associated receptors: CD69, CD25int, CD95, HLA-DR, and CD4+ T cells with killing properties containing perforin and granzyme B. Our results indicated that the percentage of CD4+CD69+ and CD4+CD25(int) cells did not correlate with either the SLEDAI or the SLAM scale. Significant and positive correlations were observed between percentages of CD4+CD95+ and CD4+HLA-DR+ lymphocytes and SLE activity, but only when activity was measured using the SLAM scale, not with the SLEDAI scale. The percentage of CD4+perforin+ and CD4+granzyme B+ cells also strongly correlated with disease activity measured only with the SLAM scale. We conclude that the SLAM scale better reflects changes of immune system activity in SLE patients compared with the SLEDAI scale., (Copyright © 2011 American Society for Histocompatibility and Immunogenetics. Published by Elsevier Inc. All rights reserved.)

Systemic lupus erythematosus and Still's disease are chronic autoimmune disorders of unknown etiology. Symptomatology of these diseases may be similar causing diagnostic difficulties. Long-term observation and immunological studies are essential to identify the definite disorder. We present a case of a 24-year-old patient with high fever, sore throat and arthritis. During hospitalization rash accompanying fever, nodular erythema, pulmonary changes, liver damage and splenomegaly were observed. Although initially adult-onset Still's disease was diagnosed according to the Yamaguchi criteria, the diagnosis of systemic lupus erythematosus was made after re-analysis of the clinical course and immunological tests.

Introduction: Takayasu's arteritis (TA) is a granulomatous inflammation of the aorta and its major branches, usually occurring in young women. The disease is rare in Europe. The aim of this study was to describe clinical manifestations of TA among patients at the Department of Connective Tissue Diseases, University Medical Center in Gdańsk., Material and Methods: We conducted a retrospective data analysis and performed a clinical examination of the patients. All patients met ACR criteria for TA. The mean age at disease onset was 26 years and the mean diagnostic delay was 19 months. Four out of 12 patients had a history of extensive stroke. Bypass surgery or balloon angioplasty was done in four patients. One patient underwent aortic valve replacement., Conclusion: Early diagnosis and pharmacologic treatment may significantly improve the prognosis in TA.

Background: The aim of the study was to assess myocardial perfusion in women with systemic lupus erythomatosus (SLE) and no symptoms of coronary artery disease (CAD)., Material and Methods: Twenty two women with SLE of mean age 40.5 +/- 7.2 were enrolled in the study. The average duration time of the disease was from 2 to 19 years, mean 8 +/- 4.6 years. The inclusion criterion was the absence of stenocardial symptoms. The myocardial perfusion was studied by using Single Photon Emission Computerized Tomography (SPECT) utilising (99m)Tc-MIBI and a triple-head gamma-camera. We also analyzed risk factors of heart ischemic disease in our group., Results: Myocardial perfusion stress scanning showed abnormal perfusion in 12 patients, 54.5% of the whole group, mostly in the anterior wall. At rest hypoperfusion abnormalities were found in 7 individuals. In patients with positive myocardial perfusion, out scanning, risk factors of CAD were more pronounced than in a sub-group with a negative result of myocardial perfusion scanning., Conclusions: In young women with SLE and no symptoms of coronary artery disease, myocardial perfusion defects may be detected by means of myocardial perfusion scintigraphy. Exercise and resting electrocardiography tests could be not sufficient for CAD diagnosis in women with SLE. The presence of coronary artery disease risk factors in women with SLE could be an indication to perform myocardial perfusion SPECT scanning.

Purpose: As Sjögren syndrome is regarded by ophthalmologists as a synonim of dry eye syndrome, we decided to evaluate how often these patients demonstrate other than ocular symptoms, especially systemic complications., Material and Methods: We examined 30 patients with primary (PZS) and 45 patients with secondary Sjögren syndrome (WZS)., Results: Pathological findings in course of Sjogren syndrome concerned predominantly eyes (94%) and mucous membrane of the mouth (91%), salivary glands (65%) and skin. The most frequent complaint was fatigue (91%)., Conclusions: Sjogren syndrome is a chronic systemic disease, in which predominant symptoms are: dryness of mucous membranes, chronic fatigue, painful joints and stomach disturbances.

Takayasu's arteritis (TA) is a chronic inflammatory arteritis, preferentially affecting the major arteries such as the aorta and its main branches. Its course may be biphasic, with an early systemic phase characterised by non-specific inflammatory features and a later stage with occlusive lesion of the affected vessels. We reported 3 patients with TA, who presented very varied symptoms. These cases emphasise the importance of ultrasound image, which can help to establish the diagnosis at very early stage of vasculitis and improve prognosis.

A case of multisymptomatic relapsing polychondritis in a 22-year-old woman. We report a case of a 22-year-old woman with relapsing polychondritis (RP)--a rare and little known systemic autoimmune disease characterised by episodic inflammation of cartilaginous structures (ear, nose, bronchi, trachea, larynx, ribs, cardiovascular system). This patient presents with a seven-year history, initiated by the saddle nose. The patient developed a multitude of symptoms: auricular chondritis, ocular symptoms, recurrent arthritis, respiratory complications (laryngotracheomalacia, bilateral vocal cord palsy), sensorineural hearing loss and enchondroma of the humeral bone. The examination of an auricle biopsy by an immunofluorescent method and a positive serum reaction from the patient to normal cartilage supported the immunological nature of relapsing polychondritis. Treatment consisted of orally administered prednisone and diaminodiphenylsulfone (Dapsone).