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Background Nailfold videocapillaroscopy (NVC) is the primary diagnostic tool for the assessment of microcirculation in the pediatric population. Objective To define and standardize age-specific normal NVC patterns in healthy children and adolescents. Methods A cross-sectional observational multicentric study was conducted in 564 participants aged 5–17 years. Dino-Lite CapillaryScope 200 Pro Model MEDL4N Pro was performed at 200× magnification. Quantitative and qualitative NVC parameters were analysed separately for each age group and divided into four groups based on age categories. Results Of the 564 healthy participants, 54.9% were female. A total of 1184 images and 3384 capillaries were analysed. Positive correlations were observed between age and capillary density (P  < 0.001, R = 0.450, CI95% 0.398–0.503). There was also a positive correlation between age and arterial/venous, loop diameter and capillary length, whereas there was a weak negative correlation between intercapillary distance. However, no correlation was found between age and capillary width. In addition, capillary density was significantly lower in the 5–7 age group compared with the other patient groups. Arterial limb diameter was lower in the 5–7 age group, while venous limb diameter was significantly wider in the 15–17 age group compared with the other patient groups. Dilated capillaries (8.7%), capillary tortuosity (14.4%), crossed capillaries (43.1%), micro-haemorrhages (2.7%) and avascular area (4.8%) were present in all age groups. Excellent intra- and interobserver ICC values were obtained for all parameters. Conclusion These findings hold potential significance for future studies, aiding in the analysis and differentiation of children suspected of rheumatological diseases with potential microangiopathy. [ABSTRACT FROM AUTHOR]

Methotrexate is a widely used drug in clinical practice for the treatment of collagen vascular diseases and malignant tumors. It has good anti-inflammatory and anti-proliferative effects, but the cytotoxicity of methotrexate can cause various adverse reactions in patients. Studies have shown that the sensitivity and tolerance of different individuals to methotrexate is different. There are many reasons for this difference. Among them, genetic polymorphism is one of the main factors that cause individual differences. This article provides an overview of the genetic polymorphisms of key proteins involved in methotrexate metabolism and transport, such as MTHFR, FPGS, γ-GGH, ABC transporter, OATPs, SLC, TS and DHFR, are related to their efficacy and adverse reactions. The aim is to clarify the impact of genetic polymorphisms on the efficacy and adverse effects of methotrexate at the pharmacogenomic level, in order to provide a basis for the clinical application of methotrexate. [ABSTRACT FROM AUTHOR]

Background: Acquired reactive perforating collagenosis (ARPC) poses a clinical challenge with an unclear pathogenesis. This disease has been frequently proven resistant to immunosuppressive treatments, significantly affecting the quality of life of patients. In this report, we highlight the efficacy of baricitinib as a viable option for maintenance therapy in ARPC. Case summary: An 81-year-old woman presented to our hospital with recurrent pruritus and cup-like ulcerated lesions on her trunk and limbs persisting for 1 year. She exhibited limited response to oral antihistamines and topical steroids. Past medical history revealed a prolonged history of coronary heart disease and type 2 diabetes spanning several years to decades. Histopathological examination revealed cup-shaped depressions filled with necrotic inflammatory debris. In the dermis, a mixed inflammatory infiltrate composed of lymphocytes and histiocytes was observed. Van Gieson staining indicated the elimination of fibrous tissue extending from the dermis into the epidermis. Consequently, a diagnosis of ARPC was established. Due to the inadequate response to conventional treatments and the severe itching, we initiated baricitinib therapy for ARPC, resulting in gradual symptom improvement. Follow-up assessments showed no adverse reactions and normal laboratory findings. Conclusion: The case report suggests that baricitinib might offer significant therapeutic benefits for ARPC. [ABSTRACT FROM AUTHOR]

Introduction: Juvenile-onset systemic lupus erythematosus (jSLE) is a rare chronic autoimmune disease with multisystemic involvement that can cause significant damage, disability and death. jSLE has a more severe disease presentation than lupus in adults, with a higher incidence of major organ involvement. Aim of the study is to determine the presenting clinical and laboratory characteristics of jSLE patients and to compare symptoms at disease onset from our cohort through a review of the literature. Materials and methods: rretrospective review of medical records encompassing all juvenile systemic lupus erythematosus (jSLE) patients treated in department from July 2007 to July 2024. Upon disease presentation, we abstracted array of clinical and laboratory indices: constitutional symptoms, skin and mucosal changes, arthritis, renal and CNS involvement, CRP, ESR, WBC, platelet count, DCT, ANA, anti-DsDNA, anti-Sm, antiphospholipid antibodies, C3, C4, and RF. Results:. Nineteen children (15 girls and 4 boys) were diagnosed with jSLE. The mean age was 11 years. Arthralgia was the most common complaint (78%), followed by fever, (47%). The most common clinical manifestation was arthritis (62%), followed by renal disease (68%), leukopenia (57%), oral ulcers and thrombocytopenia (42%), malar rash (31%), serositis (21%), and CNS symptoms (16%). Four patients (21%) had stage 2 nephritis, one had stage 3 nephritis, and three (16%) had stage 4 nephritis. All exhibited elevated ESR and positive ANA. Majority of patients had elevated anti-DsDNA (83%), anti-Sm (52%), antiphospholipid antibodies(62%), and anti-ribonucleoprotein antibodies in 9 patients (47%). Low C3 levels were observed in 14 patients (73%) and low C4 levels in 17 patients (88%). Conclusion: (jSLE) is a challenging disease in terms of both diagnosis and treatment. Any case of fever of unknown origin, particularly when accompanied by joint pain, should prompt an evaluation for jSLE. In our cohort, the most common presenting symptoms were fever, arthritis, and arthralgia. [ABSTRACT FROM AUTHOR]