Systemic lupus erythematosus (SLE) patients have frequently neuropsychiatric manifestations. From the first description of coma in 1875, a variety of manifestations has been described to occur in SLE. However, the lack of standardization reduced the comparability of published studies. In 1999, the American College of Rheumatology published guidelines to define neuropsychiatric nomenclature in SLE. This was the first step toward uniform diagnostic criteria. Several studies have been published since then applying the ACR criteria and frequencies of different manifestations can now be compared between cohorts. Although these criteria are diagnostic, therapeutic approach to different manifestations varies according to nature and severity of the manifestations. Herby, we will review the different definition for NPSLE published, and determine advantages and limitation.

Fabry disease (FD) is a rare, recessive X-linked, multisystemic lysosomal storage disorder (LSD) that results from a deficiency in the hydrolase alpha-galactosidase A (α-GalA) caused by a GLA gene variant. The progressive accumulation of the glycosphingolipid globotriaosylceramide (Gb3) in organs such as skin, kidney, brain, joints, vascular walls and eyes are responsible for the wide spectrum of clinical manifestations, often unspecific. In result, clinically relevant and life-threatening complications, such as malignant ventricular arrhythmia, sudden cardiac death, end stage kidney failure and stroke may occur. In this review, we will describe the clinical features and the current perspectives in the multidisciplinary management Of FD patients.

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This cross-sectional study aimed to evaluate the impact of physical activity and physical fitness on the health-related quality of life (HQoL) of adult patients with Juvenile Idiopathic Arthritis (JIA). Fifty-nine JIA patients and sixty healthy individuals participated in this study. All individuals had the following evaluations performed: body composition (electrical bioimpedance), physical fitness (6 min walk test (6MWT)), physical activity level (International Physical Activity Questionnaire (IPAQ)), and HQoL (Quality of Life Questionnaire in relation to Health—Short Form (SF36)). Thirty-nine (66%) JIA patients were considered sedentary compared with 15 (25%) in the control group (p < 0.01). JIA patients had a lower HQoL compared with the control group in all variables studied (p < 0.05). JIA patients who were very physically active had better HQoL conditions in the categories of functional capacity (p = 0.001), limitations by physical aspects (p = 0.003), and emotional aspects (p = 0.002) compared with sedentary patients. JIA patients had more cardiovascular abnormalities and walked shorter distances compared with healthy controls in the 6MWT. In conclusion, we observed that HQoL was reduced in adults with JIA. A high percentage of JIA patients were sedentary with lower physical fitness, but physically active patients had a better HQoL than sedentary patients. The duration of physical activity, rather than intensity, influenced the mental aspects of HQoL.

BACKGROUNDWith improvement of treatment, long-term factors influencing quality of life have to be addressed in the care of juvenile idiopathic arthritis (JIA) patients. The aim of our study was to compare body composition, level of physical activity and self-esteem of adult patients with JIA. METHODSThe levels of physical activity were analyzed using the International physical activity questionnaire (IPAQ) short version. The body composition data were collected using the Omron HBF 514 C vertical electric bioimpedance device. The Rosenberg self-esteem scale was used to assess positive and negative attitude and feelings about themselves, where the lower the score, the higher the individual's self-esteem. For the statistical analysis of the data, the IBM SPSS software was used with a significance index of p < 0.05. RESULTSWe included 81 individuals > 18 years, 38 with JIA and 43 healthy people (control group, CG). The female sex was the largest participant in this research. There was no significant difference regarding sex (female = 28 [73.7%] in JIA and 26 [60.5%] in CG) and age of the groups (29.39 ± 7.6 vs. 31.1 ± 3.1, p = 0.18). The CG showed greater height and body weight (163.5 ± 9.8 vs. 169.6 ± 8.0, p < 0.001; 63.07 ± 16.65 vs. 69.33 ± 6.88, p = 0.003, respectively), but no difference in the body mass index (BMI) was observed (24.32 ± 4.9 vs. 24.12 ± 2.09, p = 0.8). The JIA group had a higher percentage of total fat mass (31.65 ± 10.20 vs. 28.47 ± 4.9, p = 0.07), while the CG had a higher percentage of total muscle mass (29.7 ± 6.5 vs. 35.86 ± 6.05, p < 0.001). The self-esteem of JIA patients was lower than in the CG (21 ± 3.9 vs. 16.26 ± 4.3, p < 0.001). In the IPAQ classifications, JIA patients were classified as less physically active than the CG (2.63% vs. 20.93%, p = 0.022), less irregularly active (5.26% vs. 23.25, p = 0.023) and a higher level of sedentary lifestyle (65.78% vs. 25.58%, p < 0.001). A higher percentage of muscle mass was associated with greater self-esteem (r = -0.3; p = 0.01). CONCLUSIONThe low practice of physical activities by patients with JIA seems to directly influence both, their body composition and their self-esteem. These patients should be encouraged to increase physical activity from an early age by a multiprofessional healthcare team to seek improvement their quality of life.