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Purpose: The efficacy of adjunctive ambrisentan treatment in patients with systemic sclerosis (SSc) suffering from digital ulcers (DUs) was investigated.Material and methods: Patients (4 males, 7 females) diagnosed with SSc at our hospital between 2017 and 2022 were enrolled. Ten of them had diffuse SSc, while one had limited SSc. These patients received daily 5 mg doses of ambrisentan in addition to their regular SSc treatment for 16 weeks. Parameters including the total number and size of existing and new DUs, Visual Analog Score (VAS), frequency of Raynaud\'s phenomenon (RP) attacks, and any adverse effects were assessed.Results: At baseline, the median number and size of DUs was 3.0 (interquartile range (IQR): 2.0-4.0 cm) and 0.4 cm (IQR: 0.3-0.5 cm), respectively. Following the intervention, seven patients with a median of 2.0 DUs and a size of 0.35 cm (IQR: 0.15-0.45 cm) at baseline achieved complete healing. Significant improvements were also observed in other patients. VAS scores decreased from a baseline median of 5.0-0.0 (IQR: 0.0-1.0), and both the frequency and duration of RP attacks notably reduced.Conclusion: Adjunctive ambrisentan therapy proved effective in promoting DU healing and preventing new DUs in SSc patients.

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Systemic sclerosis, also known as scleroderma, is a rare multisystem autoimmune disease characterized by vascular lesions caused by collagen deposition in the skin and viscera and damage to the endothelium. Endothelial injury and microvascular occlusion result in Raynaud\'s phenomenon, finger ischemia, pulmonary hypertension, and scleroderma renal crisis. Scleroderma itself is a rare disease with an incidence ranging from 0.1 to 14 per 100,000 people in the general population. Cerebral involvement is not considered a common manifestation of systemic sclerosis, although studies have shown that the brain can be involved. Therefore, to deepen the understanding of this disease, we herein report a case of cerebral infarction associated with systemic sclerosis.

OBJECTIVE: The aim at the current study was to investigate the clinical characteristics and risk factors of Raynaud\'s phenomenon (RP) in patients with primary Sjögren\'s syndrome (pSS).
METHODS: Retrospective analysis of the medical records of 333 new-onset pSS patients was performed. Demographic, clinical, and serological data were compared between individuals with and without RP. Logistic regression analysis was used to identify risk factors.
RESULTS: RP was present in 11.41% of the pSS patients. pSS-RP patients were younger (49.74±14.56 years vs. 54.46±13.20 years, p=0.04) and exhibited higher disease activity (11 [5.75-15] vs. 7 [4-12], p=0.03) than those without. The prevalence of lung involvement was significantly higher in pSS patients with RP (60.53% vs. 17.29%; p<0.001). A significantly higher proportion of patients with pSS-RP tested positive about antinuclear (ANA), anti-RNP, and anti-centromere antibodies (ACA) compared to those without (p=0.003, <0.001, and 0.01, respectively). Multivariate analysis identified lung involvement (odds ratio [OR]=8.81, 95% confidence interval [CI] 2.02-38.47; p=0.04), anti-RNP positive status (OR=79.41, 95% CI 12.57-501.78; p<0.0001), as well as ACA (OR=13.17, 95% CI 2.60-66.72; p=0.002) as prognostic factors for pSS-RP.
CONCLUSIONS: The presence of RP defined a subset of pSS with a unique phenotype, manifesting as increased lung involvement and a higher frequency of anti-RNP antibodies and ACA, as well as greater disease activity. These results suggest that RP has clinical and prognostic value of pSS patients. Further prospective studies with a larger number of subjects are warranted to confirm our findings and assess the prognostic and treatment implications of RP in pSS patients. Key Points • Raynaud\'s phenomenon (RP) was present in 38 (11.41%) of 333 patients with primary Sjögren\'s syndrome (pSS), with patients with RP exhibiting a younger age and higher disease activity. • The presence of RP indicates a subset of pSS with a unique phenotype, with manifestations including increased lung involvement and a higher frequency of anti-RNP antibodies and anti-centromere antibodies. • Patients with pSS and RP need close follow-up and long-term observation (including assessment of microangiopathy), with specific attention paid to the possible development of clinical features of systemic sclerosis.

UNASSIGNED: This study aims to assess the nailfold capillary changes in patients with systemic lupus erythematosus (SLE), particularly among those with Raynaud\'s phenomenon (RP), and the correlation between nailfold capillary changes and autoantibodies and disease activity.
UNASSIGNED: A total of 85 patients (9 males, 76 females; median age 31 years; range, 15 to 58 years) with newly diagnosed SLE were selected between July 2016 and July 2018 from our hospital. Disease activity was scored by the SLE Disease Activity Index. Nailfold capillaroscopy (NFC) was performed in all patients.
UNASSIGNED: Normal pattern, non-specific pattern, and scleroderma pattern were found in 13 (15.3%), 64 (75.3%), and eight (9.4%) patients, respectively. There was no significant difference between anti-double stranded deoxyribonucleic acid, anti-Smith antibodies, and low complements (all p>0.05), while significant differences of NFC pattern were found between low disease activity and high disease activity (p=0.002). RP was present in 31.7% of SLE patients, and the NFC findings in SLE patients with and without RP were significantly different in dilatation (81.5% vs. 14.0%).
UNASSIGNED: The results of our study showed that capillary changes were very common in patients with SLE, which seem to associate with disease activity and RP condition.

Raynaud\'s phenomenon (RP) is a disease that mainly affects human fingertips during cold weather. It is difficult to treat this disease using medicine, apart from keeping the body in a warm environment. In this research, conductive knitted fabrics were fabricated to help relax the vessels of the patient\'s fingertips by providing proper heat, and also serving as a sensor to detect finger motion after relaxation of the blood vessels of patients. Four different structures, termed plain, purl, interlock, and rib were produced using conductive silver/PE (polyethylene) yarn and wool yarn, with a computerized flat knitting machine. The effect of knitted structure on the electro-thermal behavior, sensitivity, and stability of resistance change (∆R/R) under different tensile forces was investigated. By comprehensive comparison, the purl structure was identified as the preferred structure for the heating glove for RP patients, owing to superior electro-thermal behavior. Additionally, the purl structure had a greater capacity to detect different motions with stable resistance change. This potential electro-thermal glove could be used for functional, as well as aesthetic (fashion) purposes, and could be worn at any time and occasion with complete comfort.

Cold and White - Hypothenar Hammer Syndrome Abstract. Hypothenar hammer syndrome (HHS) is a condition caused by digital ischemia as a result of repeated trauma to the little finger. Routine diagnosis should include a detailed medical history and a physical examination including Allen\'s test. Imaging of vascular lesions can be done initially by acral plethysmography and duplex sonography, or directly in the context of angiography (gold standard). Early diagnosis enables effective therapeutic strategies and preventing permanent sequelae. The optimal treatment options are selected depending on the intensity of symptoms, ranging from conservative methods, secondary prevention, through a local thrombolysis up to operational measures.
Zusammenfassung. Das Hypothenar-Hammer-Syndrom (HHS) ist ein Zustand, der durch digitale Ischämie als Folge wiederholter Traumata auf den Kleinfingerballen entsteht. Die Routinediagnostik sollte eine genaue Anamnese sowie eine körperliche Untersuchung einschliesslich Allen-Test umfassen. Bildgebung der Gefässläsionen kann initial mittels akraler Plethysmografie und Duplexsonografie, oder aber direkt im Rahmen einer Angiografie (Goldstandard) erfolgen. Eine frühzeitige Diagnose ermöglicht effektive therapeutische Strategien und Verhinderung permanenter Spätfolgen. Die optimalen Behandlungsmöglichkeiten werden je nach Stärke der Symptomatik ausgewählt und reichen von konservativen Methoden und sekundärer Prävention über eine lokale Thrombolyse bis zu operativen Massnahmen.

UNASSIGNED: Botulinum neurotoxin type A (BoNT/A) is emerging as a treatment modality for Raynaud\'s phenomenon (RP). However, the mechanism of the role of BoNT/A in antagonizing the constriction of arteriola in RP remains unclear.
UNASSIGNED: We tested the constriction of arteriole diameter and the distribution of adrenergic receptors on the rat cremaster modle. Moreover, we measured the secretion of norepinephrine (NE), protein level changes and related receptors on cultured rat superior cervical ganglia neurons(SCGNs), a model of sympathetic neuron.
UNASSIGNED: Based on our results, the inhibition of arteriole vasoconstriction was increased with increasing doses of BoNT/A. BoNT/A, prazosin, and BQ123 treatment can result in significant inhibition of arteriole vasoconstriction with the same electrical stimulation. The inhibition effect of prazosin was equivalent to BoNT/A, while BQ123 has a synergistic effect with BoNT/A. After treating SCGNs using BoNT/A for 30 min, the decrease in fluorescence intensity of FM1-43 slowed down which was correlated with the doses of BoNT/A. Furthermore, release of NE in the supernatant was significantly decreased as measured by enzyme-linked immunosorbent assay, 24 h after a high dose of BoNT/A (25 µ/mL). Cleaved-SNAP-25 was detected by Western blotting 24 h following BoNT/A (50 µ/mL) treatment. Moreover, receptor SV2C, GM1, and FGFR3 were detected on sympathetic neurons, similarly to cholinergic neurons.
UNASSIGNED: Our study showed that BoNT/A could significantly inhibit electrical stimulation-induced arteriole vasoconstriction through the sympathetic pathway. The mechanism was similar to the cholinergic one, in which the vesicle release of sympathetic neurons could be inhibited by cleavage of SNAP-25. The end result was blocked vesicle fusion with the presynaptic membrane after BoNT/A treatment, inhibiting the release of the NE.

OBJECTIVE: The main objective of this study was to summarize the existing evidence and quantitatively evaluate whether serum/plasma levels of homocysteine (Hcy) were associated with sclerosis (SSc) diseases by performing a meta-analysis of previous studies.
METHODS: PubMed, Elsevier ScienceDirect and Cochrane Library databases were used to obtain all relative published literatures. Stata version 11.0 (StataCorp, College Station, TX) was used for statistical analysis. The effect size of each study was calculated by the standardized mean difference (SMD) with 95% confidence interval (CI) or quartiles.
RESULTS: A total of eight studies including 475 cases and 265 controls were finally included in this meta-analysis. We found significant between-study heterogeneity and conducted analyses using random-effects models. No significant association was found between the serum levels of Hcy and SSc (pooled SMD =1.382 μmol/L, 95%CI = -0.442 to 3.206, p = .137), but there are two outlier studies that deviate significantly from most other studies, which made it difficult to generalize these results. After excluding these two studies, six studies were included in the meta-analysis. The results showed that the serum levels of Hcy in SSc were significantly higher than that in healthy controls (pooled SMD = 1.182μmol/L, 95%CI = 0.230-2.134, p = .015).
CONCLUSIONS: Serum/plasma levels of Hcy in SSc diseases were higher than that in healthy controls.