This study aims to analyze the clinical and immunologic features of SLE in Jordan, while also investigating the impact of age and gender on disease presentation. The study included 275 patients diagnosed with SLE. Data were collected through meticulous patient interviews and thorough examination of patient hospital records. The cohort exhibited a mean age of 36.8 ± 12.9 years, with an average disease duration of 7.0 ± 7.8 years. The mean age at diagnosis was 29.9 ± 12.1 years, and the female to male ratio was 7.8:1. The most frequently observed symptoms were arthralgia (90.2%), fatigue (80.7%), hematologic manifestations (62%), photosensitivity (60.7%), Raynaud\'s phenomenon (53.5%), and malar rash (50.9%). The frequencies of various autoantibodies were as follows: ANA (96.7%), anti-dsDNA (39.6%), anti-SSA/Ro (32.8%), anti-Sm (21.8%), anti-U1-RNP (20.6%), and anti-SSB/La (15.5%). Male patients tended to receive a diagnosis at a younger age and exhibited a higher likelihood of experiencing severe manifestations compared to females. Additionally, juvenile onset patients demonstrated an increased likelihood of fever, photosensitivity, myositis, and anti-dsDNA autoantibodies, while adult onset patients were more predisposed to having anti-Ro, anti-La, and RF autoantibodies. This study reveals that the most prevalent manifestations of SLE in the Jordanian cohort encompassed arthralgia, fatigue, and hematologic manifestations. The prevalence of alopecia and Raynaud\'s phenomenon exceeded that observed in other published cohorts, while arthritis and discoid rash were less frequently encountered. The study highlights that males are more susceptible to developing severe manifestations of SLE compared to females.

UNASSIGNED: Primary Raynaud\'s phenomenon (pRP) is difficult to distinguish from secondary (sRP). Although nailfold capillaroscopy (NFC) may detect early alterations, no universal criteria yet discriminate between pRP from sRP.
UNASSIGNED: To create and validate two NFC scores that could distinguish pRP from sRP and that could predict systemic sclerosis (SSc), respectively.
UNASSIGNED: We performed NFC on two separate cohorts with isolated RP, and recorded number of capillaries per field, enlarged/giant capillaries, crossed/bizarre patterns, microhemorrhages, neoangiogenesis, rarefaction, edema, blood flow velocity, stasis. By multivariate regression analysis, we evaluated the adjusted prognostic role of these features in a derivation cohort of 656 patients. Results were used to construct algorithm-based prognostic scores (A and B). These scores were then tested on a confirmation cohort of 219 patients.
UNASSIGNED: Score A was unable to discriminate sRP from pRP (low negative predictive values with high positive predictive values for any cut-point); score B was unable to discriminate progression to SSc or a SSc-spectrum disorder (low positive predictive values with high negative predictive values for lower cut-points).
UNASSIGNED: NFC patterns, believed as specific, showed low discriminatory power and on their own are unable to reliably discriminate sRP from pRP or predict evolution to SSc.

OBJECTIVE: To explore, from patients\' perspectives, the symptoms and impact of Raynaud\'s phenomenon (RP) on the feet of patients with systemic sclerosis (SSc-RP), and to identify which foot-related domains are important to patients.
METHODS: Forty participants (34 women) with SSc-RP took part in one of six focus groups held in the United Kingdom or United States. Participants were purposively sampled to ensure diversity in disease type, duration, and ethnicity. The topic guide included questions on RP impact, self-management, and treatment expectations. Qualitative content analysis was employed to identify key concepts in the data relating to foot-specific symptoms and their impact. Themes were organized by corresponding domains of potential importance.
RESULTS: Twenty-eight participants (70 %) reported experiencing RP in their feet. Five themes were identified corresponding to domains of potential importance: temperature changes, pain, cramping and stiffness, numbness, and color changes. These issues negatively affected participants\' lives, impairing walking, driving, and socializing, and causing issues with footwear and hosiery.
CONCLUSIONS: This large qualitative study exploring the experiences of patients with SSc-RP in the feet identified several key domains of high importance to patients. SSc-RP is common in the feet, presents in several patterns, and impacts multiple aspects of patients\' lives. These findings indicate where future foot-specific interventions for RP could be targeted. Findings from this study improve understanding of what domains are important to patients with SSc-RP affecting the feet and will contribute to the development of a core outcome set for foot and ankle disorders in rheumatic and musculoskeletal diseases.

UNASSIGNED: To evaluate the clinical and laboratory characteristics, therapeutic drugs, and prognosis of juvenile systemic sclerosis (JSSc) at a single center in Korea.
UNASSIGNED: This study was a retrospective analysis of patients with JSSc aged <16 years at disease onset and who were treated at our hospital between January 1992 and April 2023. All patients met the Pediatric Rheumatology European Society/American College of Rheumatology/European League against Rheumatism provisional classification criteria for JSSc, and those with localized scleroderma (morphea) were excluded.
UNASSIGNED: Among the 13 patients, proximal skin sclerosis (100%), Raynaud\'s phenomenon (RP) (84.6%), and sclerodactyly (69.2%) were present at the time of diagnosis. The most common symptom before diagnosis was RP, which was present in 10 patients (76.9%), whereas proximal skin sclerosis was observed in only five patients (38.5%). Thirteen patients had positive anti-nuclear antibody (ANA). At the time of diagnosis, five individuals had findings suggestive of interstitial lung disease (ILD) on a pulmonary function test (PFT) or chest computed tomography (CT), two of whom were asymptomatic. During follow-up, three patients developed ILD, one developed renal dysfunction, one developed heart disease, and none died.
UNASSIGNED: This study was the first descriptive analysis of clinical features of JSSc in South Korea. Clinical suspicion is essential for diagnosing JSSc in patients with RP, especially if ANA is positive; however, proximal skin sclerosis, which is crucial for diagnosing JSSc, was unrecognized in the early phase of the disease. PFT should be considered even if a patient is asymptomatic or has normal chest CT.

暂无摘要。

This cross-sectional study aimed to describe exposure to cold climate and hand-arm vibration (HAV) as well as neurosensory and vascular symptoms and clinical findings among open-pit Arctic miners. It was based on data from questionnaires and physical examinations, including 177 men and 75 women from two open-pit mines in Sweden and Norway (response rate 54%). Working outdoors or in an unheated building or machine for at least two hours per day was reported by 44% and HAV exposure of the same duration by 10%. Neurosensory symptoms (e.g. reduced perception of touch) in the hands were reported by 47% and Raynaud\'s phenomenon by 14%. In brief conclusion, the study showed that Arctic miners were commonly exposed to both cold temperatures and HAV. They also reported a broad range of neurosensory and vascular symptoms in their hands and had abnormal clinical findings related to the symptoms. The results emphasise the need for additional preventive measures in this occupational setting.

Systemic Sclerosis in the hand is characteristically evidenced by Raynaud\'s phenomenon, fibrosis of the skin, tendons, ligaments, and joints as well as digital ulcers with prolonged healing. Current medical treatment does not always cure these complications. Local adipose-derived stromal vascular fraction administration into the hands has been proposed as an emerging treatment due to its regenerative properties. The objective of this randomized controlled clinical trial was to evaluate the safety and clinical effects of fat micrografts plus adipose derived-stromal vascular fraction administration into the hands of patients with systemic sclerosis.
This was an open-label, monocentric, randomized controlled study. Twenty patients diagnosed with systemic sclerosis were assigned to the experimental or control group. Fat micrografts plus the adipose derived-stromal vascular fraction were injected into the right hand of experimental group patients. The control group continued to receive only medical treatment. Demographic, serologic data and disease severity were recorded. Digital oximetry, pain, Raynaud phenomenon, digital ulcers number, mobility, thumb opposition, vascular density of the nail bed, skin affection of the hand, serologic antibodies, hand function, and quality of life scores were evaluated in both groups.
The results of the intervention were analyzed with the Wilcoxon rank test, and the differences between the control and experimental groups at 0 days and 168 days were analyzed with the Mann-Whitney U test. Adverse events were not observed in both groups. At the end of the study, statistically significant improvements were observed in pain levels (p<0.05) and number of digital ulcers (p<0.01) in the experimental vs control group.
The injection of adipose derived-stromal vascular fraction plus fat micrografts is a reproducible, and safe technique. Pain and digital ulcers in the hands of patients with systemic sclerosis can be treated with this technique plus conventional medical treatment.

Juvenile systemic sclerosis (SSc) is an orphan disease, associated with high morbidity and mortality. New treatment strategies are much needed, but clearly defining appropriate outcomes is necessary if successful therapies are to be developed. Our objective here was to propose such outcomes.
This proposal is the result of 4 face-to-face consensus meetings with a 27-member multidisciplinary team of pediatric rheumatologists, adult rheumatologists, dermatologists, pediatric cardiologists, pulmonologists, gastroenterologists, a statistician, and patients. Throughout the process, we reviewed the existing adult data in this field, the more limited pediatric literature for juvenile SSc outcomes, and data from 2 juvenile SSc patient cohorts to assist in making informed, data-driven decisions. The use of items for each domain as an outcome measure in an open label 12-month clinical trial of juvenile SSc was voted and agreed upon using a nominal group technique.
After voting, the domains agreed on were global disease activity, skin, Raynaud\'s phenomenon, digital ulcers, musculoskeletal, cardiac, pulmonary, renal, and gastrointestinal involvement, and quality of life. Fourteen outcome measures had 100% agreement, 1 item had 91% agreement, and 1 item had 86% agreement. The domains of biomarkers and growth/development were moved to the research agenda.
We reached consensus on multiple domains and items that should be assessed in an open label, 12-month clinical juvenile SSc trial as well as a research agenda for future development.

The purpose of this qualitative study was to provide insight into the changes and barriers to leisure experienced by people with scleroderma.
Twenty-five people with scleroderma consented to be interviewed via telephone using a semi-structured interview. The conversations were audio recorded and transcribed verbatim. Thorne\'s Interpretive Description informed the analysis process.
Three themes emerged from the analysis: barriers to leisure participation (impact of Raynaud\'s Phenomenon symptoms, fatigue, changes in body structures and functions, unpredictability of daily symptoms and selection of leisure activities), decreases in leisure participation (less time outdoors, reduction in time spent in active leisure, more time spent in passive leisure) and experiences of losing a valued leisure activity (depressed mood, identity change, fear of loss and sense of isolation).
The changes in leisure participation found in this study were reported to be due to the rate of disease progression and the severity of disease symptoms, particularly, Raynaud symptoms and fatigue. Health professionals should work with people with SSc to facilitate participation in valued leisure activities.

OBJECTIVE: To determine diagnostic accuracy and evaluate the predictive value of autoantibody profiles in patients with systemic sclerosis (SSc).
METHODS: A total of 140 patients with SSc (125 female, mean age 54.2 ± 14.2 years) were analyzed by a multiplex line immunoassay (Euroimmun) for autoantibodies against 12 SSc-related antigens. Associations between the presence of the autoantibodies and demographic clinical manifestations of patients with SSc were investigated.
RESULTS: The sensitivity and specificity of this assay were as follows: 32.9% and 99.4% for anti-Scl-70, 29.3% and 88.9% for anti-CENP A, 28.6% and 87.8% for anti-CENP B, 7.1% and 97.8% for anti-RP11, 5.7% and 100% for anti-RP155, 2.9% and 99.4% for anti-NOR 90, 2.9% and 98.9% for anti-Th/To, 1.4% and 96.7% for anti-PM-Scl-100, 5.0% and 98.3% for anti-PM-Scl-75, and 2.9% and 97.2% for anti-Ku, respectively. Anti-Scl-70 was significantly associated with sine scleroderma (P = 0.003), digital ulcers (P = 0.047), and Raynaud\'s phenomenon as the first clinical manifestation of onset (P = 0.017). SSc-ILD was more common in patients with anti-Scl-70 (P = 0.029) and less frequent in patients with anti-CENP A (P < 0.001) and anti-CENP B (P < 0.001). There was a significant association between PAH with anti-CENP A (P = 0.008) and anti-CENP B (P = 0.025). Renal involvement was significantly related to anti-NOR90 (P = 0.026) and anti-Th/To (P = 0.026).
CONCLUSIONS: This study confirmed the important role of autoantibodies in accurately diagnosing SSc. The autoimmune profile of patients with SSc was related to specific disease manifestations. Key Points • Autoantibody profiles were useful for diagnosing SSc and predicting clinical features of patients.