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(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. [ABSTRACT FROM AUTHOR]

Abstract: 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+CD25int 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 &y& Elsevier]

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.

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.

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.

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.