Impact of connective tissue diseases on complications following aesthetic surgery: A matched cohort study.

BACKGROUND: The association between connective tissue diseases (CTDs), including rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and scleroderma, and complications following aesthetic surgery is under-investigated. We hypothesized that the risk of complications following aesthetic surgery was higher in patients with these connective tissue disorders compared to matched non-CTD patients. METHODS: All patients diagnosed with RA, SLE, and scleroderma who underwent aesthetic surgery at our institution from 2003-2022 were reviewed. Demographic data, comorbidities, medications, procedures, and postoperative complications were collected. Non-CTD controls were identified for each procedure and matched 1:1 based on propensity scores derived from race, sex, body mass index, smoking status, and comorbidities. RESULTS: Six hundred 38 patients were included, comprising 319 (50%) patients diagnosed with CTD and 319 (50%) controls. The average age at surgery was 56.3 years. There were 129 complications. There were no differences between the CTD and non-CTD patients in number of total complications (69 versus 60, p = 0.38), major complications (23 versus 16, p = 0.25), or minor complications (46 versus 44, p = 0.73). Complications were not significantly different between CTD patients and controls who underwent blepharoplasty (p = 0.38), breast reduction (p = 0.91), abdominoplasty (p = 0.46), or rhytidectomy (p = 0.50). CTD patients who underwent breast augmentation had significantly more complications than matched non-CTD patients in bivariate analysis (7 versus 0, p = 0.018*) and multivariable logistic regression (OR: 10.2, 95% CI: 1.21 to 93.3, p = 0.039*). CONCLUSIONS: Most aesthetic surgeries can safely be performed in patients with CTDs. Patients seeking breast augmentation should be counseled on a potentially increased risk of postoperative complications.

Changes in work and adequacy of financial resources during COVID-19 among people with systemic sclerosis: A Scleroderma Patient-centered Intervention Network study.

Introduction/objective: We investigated (1) work status changes during COVID-19, (2) financial resource adequacy, (3) preferences for work requirements (e.g. remote, workplace, mixed) and (4) work requirements versus preferences, among people with systemic sclerosis. Methods: This was a cross-sectional study of participants in the Scleroderma Patient-centered Intervention Network COVID-19 Cohort, which enrolled participants from the ongoing Scleroderma Patient-centered Intervention Network Cohort and externally in April 2020. In August 2022, participants completed questions on work status, financial well-being using the Consumer Financial Protection Bureau Financial Well-Being Scale, work requirements and work requirement preferences. Results: A total of 298 participants with systemic sclerosis were included. Mean age was 58.6 years (SD = 11.4). There were 101 (34%) participants working at the start of the pandemic and still working in August 2022, 179 (60%) not working at the start of the pandemic and still not working, 10 (3%) who stopped working after April 2020 and 8 (3%) who started working. Mean financial well-being did not change from April 2020 to August 2022 (difference: 0.2 points; 95% confidence interval: -1.1 to 0.7). Working participants (N = 109) preferred flexible work requirements (N = 34, 31%) or working entirely remotely (N = 32, 29%), but most were required to work entirely at a workplace (N = 35, 32%) or combined workplace and remotely with a fixed schedule (N = 31, 28%). Conclusion: Work status and financial well-being did not change substantively among people with systemic sclerosis during the pandemic. Flexible work policies may support people with systemic sclerosis to work.

Atypical morphoea, a herald of two malignancies: Lung adenocarcinoma and a neuroendocrine tumour.

Introduction: Morphoea is a disorder characterised by fibrosis and inflammation of the skin and on rare occasions can be precipitated by malignancy. Here, we describe a case of morphoea unmasking two malignancies. Case description: A 73-year-old woman presented with circumferential lower limb skin thickening, associated with violaceous, doughy oedema and significantly impaired mobility. Histology confirmed dermal sclerosis with no increased mucin and broader investigations excluded systemic sclerosis, scleromyxoedema and scleroedema. An atypical morphoea was diagnosed. In the context of atypical and subsequently treatment-resistant disease, further imaging uncovered a lung adenocarcinoma which was promptly treated. Despite this, the patient's atypical oedematous skin sclerosis continued to progress proximally, and she developed flatulence, bloating and atypical flushing. This prompted further investigation, which revealed a metastatic neuroendocrine tumour. The patient was commenced on octreotide, with rapid improvement in all her cutaneous and systemic symptoms. Conclusion: Atypical morphoea can be a herald for an underlying malignancy, representing a paraneoplastic presentation. Progressive treatment-resistant morphoea may be an indicator of metastatic disease, or in our case a second malignancy.

Within-person fluctuations of fatigue in patients with a clinical diagnosis of systemic sclerosis and its relationship with mood, pain, sleep and physical activity.

Objectives: To explore the within-person fluctuations of fatigue in systemic sclerosis and its association with negative affect, positive affect, pain, perceived exertion of physical activity and quality of sleep. Methods: We performed an ecological momentary assessment study in adult patients with a clinical diagnosis of systemic sclerosis. During 14 days, patients completed daily assessments of fatigue severity, negative affect, positive affect, pain, quality of sleep and perceived exertion of physical activity at four fixed time points. The day-to-day fluctuations in fatigue were quantified by the intra-individual variance and probability of acute change, capturing the magnitude and frequency of clinical relevant within-person day-to-day fluctuations, respectively. Using multilevel models, the within-person association between fatigue and the daily assessments were analysed. Results: Fifty-seven patients with systemic sclerosis participated. The mean (standard deviation) intra-individual variance was 1.08 (0.42) and the probability of acute change was mean (standard deviation) 0.40 (0.14), ranging from 0.08 to 0.77. For fatigue, a within-person variation of 51% was observed. Multilevel analyses showed that higher average levels and daily increases in negative affect, pain and perceived exertion of physical activity were associated with more fatigue, while the opposite was observed for positive affect and quality of sleep. Positive affect demonstrated the strongest association with fatigue fluctuations. Conclusion: This is the first quantitative study showing that fatigue in systemic sclerosis is characterized by a dynamic course and that approximately half of the day-to-day fluctuations within persons are clinically meaningful. Furthermore, our results indicate that integrating activities with positive impact on mood into fatigue treatment strategies might reduce the frequency of fatigue fluctuations.

An overview of the role of chemokine CX3CL1 (Fractalkine) and CX3C chemokine receptor 1 in systemic sclerosis.

INTRODUCTION: Systemic sclerosis (SSc) is a complex autoimmune disease characterized by fibrosis, vascular damage, and immune dysregulation. Fractalkine or chemokine (C-X3-C motif) ligand 1 (CX3CL1), a chemokine and adhesion molecule, along with its receptor CX3CR1, have been implicated in the inflammatory processes of SSc. CX3CL1 functions as both a chemoattractant and an adhesion molecule, guiding immune cell trafficking. This systematic review examines the role of CX3CL1 and its receptor CX3CR1 in the pathogenesis of SSc, with a focus on pulmonary and vascular complications. METHODS: A systematic literature search was conducted across databases including PubMed, Scopus, and Web of Science from inception to November 2020. The search focused on studies investigating the CX3CL1/CX3CR1 axis in the context of SSc. RESULTS: The review identified elevated CX3CL1 expression in SSc patients, particularly in the skin and lungs, where CX3CR1 is expressed on infiltrating immune cells. Higher levels of CX3CL1 were correlated with the severity of interstitial lung disease in SSc patients, indicating a potential predictive marker for disease progression. CX3CR1-positive monocytes and NK cells were recruited to inflamed tissues, contributing to fibrosis and tissue damage. Animal studies showed that inhibition of the CX3CL1/CX3CR1 axis reduced fibrosis and improved vascular function. CONCLUSION: The CX3CL1/CX3CR1 axis plays a key role in immune cell recruitment and fibrosis in SSc. Elevated CX3CL1 levels are associated with lung and vascular complications, making it a potential biomarker for disease progression and a promising therapeutic target.

Demographics and Clinical Features Associated with Abnormal Small Bowel Motility in Systemic Sclerosis.

OBJECTIVE: The small bowel is affected in up to 50% of systemic sclerosis (SSc) patients, and some patients experience severe complications. Our aim was to use specific statistical methods to compare demographic and clinical features of SSc patients with and without abnormal small bowel to better characterize patients at risk for this complication. METHODS: SSc patients with gastrointestinal symptoms were prospectively enrolled and underwent a scintigraphy-based whole gut transit (WGT) study. A cross-sectional analysis was performed comparing clinical features between patients with and without abnormal small bowel transit by WGT. Univariate logistic regression models and multivariable models were used to examine the relationship between clinical features and abnormal small bowel transit. RESULTS: Of 130 patients enrolled in this study, 22 had abnormal small bowel transit. SSc patients with abnormal small bowel transit were more likely to be male [Odds Ratio(OR)=3.70, Confidence Interval(CI) 1.07-12.50, p= 0.038], and have more severe cardiac involvement (OR = 3.98, CI 1.10-14.38, p= 0.035), while they were less likely to have sicca symptoms (OR = 0.30, CI 0.10-0.94, p= 0.039). In multivariable analyses, sicca symptoms (OR = 0.28, CI 0.08-0.96, p= 0.043) remained negatively associated with abnormal small bowel transit. Additionally, SSc patients with abnormal small bowel transit had higher mortality than patients with normal small bowel transit [Hazard ratio(HR)=4.57, CI 1.58-13.24, p= 0.005]. CONCLUSIONS: These findings suggest that patients with abnormal small bowel transit in SSc are more likely to be male, have more severe cardiac involvement, higher mortality, and less sicca symptoms. Recognizing this patient subgroup is essential for risk stratification and optimizing clinical care.

Coccoloba uvifera L. associated with Scleroderma Bermudense Coker: a pantropical ectomycorrhizal symbiosis used in restoring of degraded coastal sand dunes.

Coccoloba uvifera L. (Polygonacaeae), named also seagrape, is an ectomycorrhizal (ECM) Caribbean beach tree, introduced pantropically for stabilizing coastal soils and producing edible fruits. This review covers the pantropical distribution and micropropagation of seagrape as well as genetic diversity, functional traits and use of ECM symbioses in response to salinity, both in its native regions and areas where it has been introduced. The ECM fungal diversity associated with seagrape was found to be relatively low in its region of origin, with Scleroderma bermudense Coker being the predominant fungal species. In regions of introduction, seagrape predominantly associated with Scleroderma species, whereas S. bermudense was exclusively identified in Réunion and Senegal. The introduction of S. bermudense is likely through spores adhering to the seed coats of seagrape, suggesting a vertical transmission of ECM colonization in seagrape by S. bermudense. This ECM fungus demonstrated its capacity to enhance salt tolerance in seagrape seedlings by reducing Na concentration and increasing K and Ca levels, consequently promoting higher K/Na and Ca/Na ratios in the tissues of ECM seedlings vs. non-ECM plants in nursery conditions. Moreover, the ECM symbiosis positively influenced growth, photosynthetic and transpiration rates, chlorophyll fluorescence and content, stomatal conductance, intercellular CO2, and water status, which improved the performance of ECM seagrape exposed to salt stress in planting conditions. The standardization of seagrape micropropagation emerges as a crucial tool for propagating homogeneous plant material in nursery and planting conditions. This review also explores the use of the ECM symbiosis between seagrape and S. bermudense as a strategy for restoring degraded coastal ecosystems in the Caribbean, Indian Ocean, and West African regions.

Analysis of nailfold capillaroscopy images with artificial intelligence: Data from literature and performance of machine learning and deep learning from images acquired in the SCLEROCAP study.

OBJECTIVE: To evaluate the performance of machine learning and then deep learning to detect a systemic scleroderma (SSc) landscape from the same set of nailfold capillaroscopy (NC) images from the French prospective multicenter observational study SCLEROCAP. METHODS: NC images from the first 100 SCLEROCAP patients were analyzed to assess the performance of machine learning and then deep learning in identifying the SSc landscape, the NC images having previously been independently and consensually labeled by expert clinicians. Images were divided into a training set (70 %) and a validation set (30 %). After features extraction from the NC images, we tested six classifiers (random forests (RF), support vector machine (SVM), logistic regression (LR), light gradient boosting (LGB), extreme gradient boosting (XGB), K-nearest neighbors (KNN)) on the training set with five different combinations of the images. The performance of each classifier was evaluated by the F1 score. In the deep learning section, we tested three pre-trained models from the TIMM library (ResNet-18, DenseNet-121 and VGG-16) on raw NC images after applying image augmentation methods. RESULTS: With machine learning, performance ranged from 0.60 to 0.73 for each variable, with Hu and Haralick moments being the most discriminating. Performance was highest with the RF, LGB and XGB models (F1 scores: 0.75-0.79). The highest score was obtained by combining all variables and using the LGB model (F1 score: 0.79 ±â€¯0.05, p < 0.01). With deep learning, performance reached a minimum accuracy of 0.87. The best results were obtained with the DenseNet-121 model (accuracy 0.94 ±â€¯0.02, F1 score 0.94 ±â€¯0.02, AUC 0.95 ±â€¯0.03) as compared to ResNet-18 (accuracy 0.87 ±â€¯0.04, F1 score 0.85 ±â€¯0.03, AUC 0.87 ±â€¯0.04) and VGG-16 (accuracy 0.90 ±â€¯0.03, F1 score 0.91 ±â€¯0.02, AUC 0.91 ±â€¯0.04). CONCLUSION: By using machine learning and then deep learning on the same set of labeled NC images from the SCLEROCAP study, the highest performances to detect SSc landscape were obtained with deep learning and in particular DenseNet-121. This pre-trained model could therefore be used to automatically interpret NC images in case of suspected SSc. This result nevertheless needs to be confirmed on a larger number of NC images.

Nodular Scleroderma: Characterization of a Distinct Clinical Phenotype.

Nodular scleroderma is a rare variant of systemic sclerosis (SSc) characterized by fleshy, indurated nodules commonly distributed over the upper and lower extremities and in the trunk. Most scientific publications of the nodular and keloid variants of scleroderma use the terms interchangeably. However, nodular scleroderma has been recently differentiated from keloid forms. Although few cases of isolated local involvement have been reported, nodular scleroderma more commonly presents in conjunction with other manifestations of SSc. We performed a review of all cases of nodular scleroderma reported in the literature to characterize their clinical features. This review indicated that Nodular Scleroderma is usually associated with a Diffuse SSc phenotype and develops during the early progressive skin involvement. Patients with the Nodular Scleroderma phenotype display antinuclear antibodies with speckled or nucleolar patterns, a low frequency of positive SSc-specific antibodies, and typical SSc multiorgan involvement. However, a very low frequency of pulmonary hypertension was found in these patients. Although immunosuppressive or antifibrotic treatment may improve skin thickening and organ involvement, the characteristic nodules are refractory to treatment with these agents. This is the first review, to our knowledge, characterizing the nodular phenotype in patients with SSc.

Evaluation of the miRNA-126 and VCAM-1 in scleroderma patients and its association with clinical characteristics.

BACKGROUND: Systemic sclerosis (SSc) has the highest level of mortality and disability among all rheumatological diseases. Being heterogenous leads to no predictable method for clinical courses. The aim of this study was to evaluate the levels of miRNA-126 and soluble VCAM-1 protein markers in patients with SSc, and to examine the assossiation of their levels with the severity of clinical and paraclinical parameters in patients with SSc. METHOD: In current study tweny six patients with SSc along with twenty-three SSc-free controls were recruited. Enzyme-linked immunosorbent assay (ELISA) was performed to measure the VCAM-1 protein. MiRNA-126 amounts in serum were detected by quantitative real-time polymerase chain reaction (PCR). RESULT: SSc patients' average age was 45.42 years and control group 49.85. The mean±SD for circulating miR-126 levels were significantly lower in SSc patients compared with healthy donors (p = 0.02), 0.48 ± 0.72 vs 1.11 ± 0.61 respectively. A significant difference was also observed in the serum level of miRNA-126 in SSc patients who suffer from pulmonary artery hypertension (P = 0.03) and pulmonary fibrosis (P = 0.04). In contrast, analysis of the serum VCAM-1 levels in the study groups uncovered a significant increase in SSc patients (5.92 ± 3.52 µg/ml) compared to control group (2.62 ± 1.2 µg/ml) (P value < 0.001). CONCLUSION: Significant change in circulating levels of miR-126 and VCAM-1 in the SSc patients supporting its role in the pathogenesis of the disease. It could also proposed potential role as a predictor of pulmonary complications for miRNA-126.

The Effect of Telerehabilitation-Based Self-Management Programme on Individuals With Scleroderma.

OBJECTIVES: Our study endeavoured to develop an online self-management programme facilitated by an interdisciplinary team and to assess its impact on the quality of life, sleep patterns, pain perception, and fatigue levels among individuals diagnosed with scleroderma. METHODS: Twenty-nine individuals with scleroderma completed the programme. The study spanned 8 weeks during which participants received weekly 45-min video sessions. Assessment tools included the Scleroderma Health Assessment Questionnaire (SHAQ) for disease severity and pain intensity, the Self-Efficacy Scale for Chronic Disease Patients for self-management evaluation, the Pittsburgh Sleep Quality Index (PSQI) for assessing sleep quality, the Fatigue Severity Scale for measuring fatigue severity, and the Short Form-12 Quality of Life Scale (SF-12) for evaluating quality of life. RESULTS: Self-management score increased significantly, while fatigue score decreased significantly. Feedback from participants indicated a positive perception of the programme and its content, suggesting its usefulness in managing their condition. Feedback from participants indicated a positive perception of the programme and its content, suggesting its usefulness in managing their condition. CONCLUSIONS: The self-management programme, developed collaboratively by an interdisciplinary team and implemented via telerehabilitation, yielded beneficial outcomes concerning self-management skills and fatigue severity among individuals with scleroderma. Strengthening the interdisciplinary composition of the study team by incorporating diverse healthcare professionals may enhance future investigations. Additionally, we advocate for the repetition of the study employing randomised methodologies and implementing long-term follow-up assessments to further elucidate the programme's efficacy and sustainability over time.

Ayurvedic management of systemic sclerosis - A case report.

Scleroderma, an autoimmune connective tissue disorder, leads to skin and tissue thickening. In this autoimmune disease condition, the defense mechanism works against the body itself and mistakenly attacks normal cells. Ayurveda links it to vatarakta, initially affecting tvak, rakta, and mamsa. Vata is primarily involved, but as the disease progresses, all doshas and dhatu get involved. A 45-year-old woman presented with skin hardening, skin depigmentation all over her body, limb stiffness, weakness, arthralgia, anorexia, constipation and burning in the chest region for the last three years. She was diagnosed with diffuse systemic sclerosis (SSc) but as per Ayurveda we can correlate with aamvata and vatarakta based on her lakshanas(symptoms). Ayurvedic treatment commenced with vardhamana pippali(Piper longum)rasayan, svedana karma, and virechan karma, followed by basti(enema) chikitsa (pathyadi kvath niruh basti) and oral ayurvedic medication viz., Manjisthadi kvatha, kaishora guggulu, Jatamansi(Nardostachys Jatamansi) churna, mishreya(Foeniculum vulgare) arka, dashang churna with water for local application and daily shamanarth panchtikta ghrit. After 8 weeks, depigmentation was reduced, new hair growth emerged, mild skin softening occured, chest burning, anorexia, sleeplessness was decreased and enhanced mental well-being. The treatment aimed to balance vitiated doshas and dhatu while alleviating symptoms, and enhancing overall well-being, demonstrating the efficacy of the holistic approach in managing scleroderma through Ayurveda. Auto-immune disorder, scleroderma, chronic complications, skin tightness, salt pepper depigmentation, sclerodactyly.

Human hypofunctional NCF1 variants promote pulmonary fibrosis in the bleomycin-induced mouse model and patients with systemic sclerosis via expansion of SPP1+ monocytes-derived macrophages.

OBJECTIVE: We assessed the role of a systemic lupus erythematosus causal hypofunctional variant, neutrophil cytosolic factor 1 (NCF1)-p.Arg90His (p.R90H) substitution, in systemic sclerosis (SSc). METHODS: Association of NCF1-H90 with SSc was performed in case-control cohorts, bleomycin (BLM)-treated Ncf1-R90 C57BL/6 wildtype and Ncf1-H90 knock-in (KI) littermates. Peripheral blood mononuclear cell (PBMC) subsets were analysed by cytometry by time-of-flight. RESULTS: The NCF1-H90 allele is associated with risk for diffuse cutaneous SSc (dcSSc) in Chinese and European Americans, and lung fibrosis in Chinese patients with SSc (OR=2.09, p=7.96E-10). Low copy number of NCF1 associated with lung fibrosis in European Americans (OR=4.33, p=2.60E-2). BLM-treated KI mice demonstrated increased pulmonary fibrosis, exhibiting activated type I interferon signature, elevated Spp1, Ccl2, Arg1, Timp1 and Il6 expression, enriched macrophage scores in lung tissues. In a longitudinal observation cohort, homozygous H90 patients with SSc at baseline had increased anti-nuclear antibody titres, anti-topoisomerase antibody seropositivity and anti-centromere antibody seronegativity, increased incidence of lung fibrosis and Gender-Age-lung Physiology index, elevated modified Rodnan Skin Score (mRSS) and elevated plasma osteopontin (OPN, SPP1), CCL2, ARG1, TIMP-1 and IL-6. These H90 patients with SSc sustained elevated mRSS during follow-up years with decreased survival. The 0, 1 and 2 copies of H90 carriage in SSc PBMCs exhibited dose-dependent increases in profibrotic CD14+CD68+CD11b+Tim3+monocytes. Elevated OPN, CCL2 and ARG1 in CD68+CD11b+monocyte-derived macrophages from H90 patients were decreased after co-culturing with anti-CCL2 antibody. CONCLUSION: Low NCF1 activity increases the risk for the development of dcSSc and lung fibrosis via expanding profibrotic SPP1+MoMs in a CCL2-dependent manner, contributing to the severity of lung fibrosis in both BLM-treated mice and patients with SSc.

Autoantibodies, cutaneous subset and immunosuppressants contribute to the cancer risk in systemic sclerosis.

OBJECTIVE: Systemic sclerosis (SSc) is associated with an increased risk of cancer. We aimed to assess the prevalence of cancer in our cohort and to explore possible associations with clinical, immunological and treatment characteristics. METHODS: Our retrospective monocentric cohort study of patients with SSc recorded prevalent and incident cases of malignancy, including those diagnosed within 3 years of the SSc onset (defined as cancer-associated scleroderma) and sought associations with the clinical characteristics and the serum autoantibody profiling performed using RNA and protein immunoprecipitation, Western-blot, immunoblot and ELISA at the time of SSc diagnosis, prior to any specific treatment. RESULTS: Among 290 patients with SSc, the overall prevalence of cancer was 20%, with 8% of cases being cancer-associated scleroderma. Both conditions were more frequent in elderly patients and in patients with positive anti-Ro52 or anti-U3-RNP. Cancer-associated scleroderma was significantly more prevalent among patients negative for both anti-centromere (ACA) and anti-topoisomerase-1 (TOPO1) antibodies, especially in the case of diffuse SSc. Immunosuppressants were not significantly associated with cancer. Patients triple negative for ACA, TOPO1 and anti-RNA polymerase III antibodies had a significantly higher risk of breast cancer. CONCLUSIONS: Cancer surveillance should be particularly careful in patients with diffuse SSc, increased age at disease onset and without classical SSc-related autoantibodies.

Nailfold Capillaroscopy Changes in Patients with Idiopathic Inflammatory Myopathies.

Background/Objectives: Idiopathic inflammatory myopathies (IIMs) are rare autoimmune disorders characterized by progressive proximal muscle weakness and varying extra-muscular manifestations. The latest 2017 EULAR/ACR criteria classify them into subgroups. This study aims to evaluate the role of nailfold capillaroscopy (NFC) as a diagnostic and prognostic tool in IIMs by comparing capillaroscopic patterns across different IIM subtypes. Methods: We conducted an observational, cross-sectional study at the Institute of Rheumatology in Belgrade, analyzing 90 patients diagnosed with IIMs per the 2017 EULAR/ACR criteria. Patients were categorized into dermatomyositis (DM) (n = 37), polymyositis (PM) (n = 35), amyopathic dermatomyositis (ADM) (n = 13), and juvenile dermatomyositis (JDM) (n = 5). A control group of 35 patients with primary Raynaud's phenomenon was also included. NFC findings, clinical manifestations, and laboratory data were compared across the groups. Results: In DM, 81.9% exhibited a scleroderma capillaroscopic pattern, which was also present in 76.9% of ADM patients. In PM, the most common pattern was nonspecific changes (48.6%). JDM patients showed a high prevalence of scleroderma changes (n = 4 (80%)). Scleroderma patterns correlated with Gottron's papules, heliotrope rash, periungual erythema, Raynaud's phenomenon, and interstitial lung disease (ILD). No significant differences were found in laboratory parameters across capillaroscopic groups, except for a higher prevalence of anti-Jo1 antibodies in patients with nonspecific capillaroscopic changes. Conclusions: NFC is a valuable tool for differentiating IIM subtypes and correlating clinical manifestations with specific capillaroscopic patterns. The high prevalence of scleroderma changes in DM and ADM suggests their potential as a diagnostic and prognostic marker in IIMs. Further research with larger cohorts is warranted to validate these findings.

Pathogenesis of Inflammation in Skin Disease: From Molecular Mechanisms to Pathology.

Many skin diseases begin with inflammatory changes on a molecular level. To develop a more thorough understanding of skin pathology and to identify new targets for therapeutic advancements, molecular mechanisms of inflammation in the context of skin disease should be studied. Current research efforts to better understand skin disease have focused on examining the role of molecular processes at several stages of the inflammatory response such as the dysregulation of innate immunity sensors, disruption of both transcriptional and post-transcriptional regulation, and crosstalk between immune and neuronal processes (neuro-immune crosstalk). This review seeks to summarize recent developments in our understanding of inflammatory processes in skin disease and to highlight opportunities for therapeutic advancements. With a focus on publications within the past 5 years (2019-2024), the databases PubMed and EBSCOhost were used to search for peer-reviewed papers regarding inflammatory molecular mechanisms and skin disease. Several themes of research interest regarding inflammatory processes in skin disease were determined through extensive review and were included based on their relative representation in current research and their focus on therapeutic potential. Several skin diseases such as psoriasis, atopic dermatitis, hidradenitis suppurativa, and scleroderma were described in the paper to demonstrate the widespread influence of inflammation in skin disease.

Exploring the relationship between morphea and malignancy: a decade-long single-center study of 204 patients.

The association between systemic scleroderma and malignancy is well-documented, but there is limited data on the relationship between morphea and malignancy. This study aims to assess the incidence and types of malignancies in morphea patients, comparing demographics, clinical characteristics, treatments, and outcomes between those with and without malignancy. We conducted a retrospective study of 204 morphea patients treated at Rabin Medical Center between 2012 and 2023. Data on demographics, clinical subtypes, comorbidities, treatments, and outcomes were collected. Patients were categorized based on malignancy status and the timing of malignancy relative to their morphea diagnosis. Among the 204 patients (154 women and 50 men, mean age 53.7 ± 20 years), 47 (23%) developed malignancies. In 29 patients (61.7%), malignancy occurred before the onset of morphea; in 23 patients (48.9%), it occurred after morphea. Five patients (10.6%) had malignancies both before and after the diagnosis of morphea. Patients with malignancy were significantly older than those without (64.7 ± 15.1 years vs. 50.3 ± 20 years, p < 0.0001). The all-cause mortality rate was higher in the malignancy group compared to those without malignancy (23.4% vs. 3.8%, p = 0.00002). Moreover, mortality was higher in patients whose malignancy occurred after morphea than in those whose malignancy preceded morphea (26% vs. 17.2%). The most common post-morphea malignancies in our cohort included non-melanoma skin cancer, cervical cancer, breast cancer, stomach cancer, and lung cancer. The most common pre-morphea malignancies included breast cancer, non-melanoma skin cancer, colon cancer, prostate cancer, and testicular cancer. This study suggests potential associations between morphea and malignancies, influenced by patient age, sequence of diagnosis, and treatment regimens. Further control studies are needed to explore these relationships more definitively.

Characterizing the Links Between Systemic Sclerosis and Breast Cancer.

Systemic sclerosis (SSc) is a complex, autoimmune connective tissue disease that affects multiple organs in the body, culminating in a variance of severity and a reduced quality of life. Breast cancer (BC) also affects patients with SSc, and these two conditions affect a similar demographic. With this systematic review, we aim to characterize the links between SSc and BC. Characterizing possible links between SSc and breast malignancies is important for advancing the understanding of SSc management and comorbidities. In this systematic meta-analysis, a comprehensive literature search was conducted in PubMed using relevant keywords and MeSH terms. The inclusion criteria included an English-language retrospective analysis that characterized patients with SSc with or without BC. Two independent reviewers assessed the study's eligibility based on predetermined criteria. Data extraction included patient antibody measurements, demographics (age and gender), family history, social behaviors (alcohol use and smoking history), concurrent condition treatments, and adverse effects following treatment. Thirteen articles were identified in the literature with relevant data on SSc and BC patients. Studies encompassed research about SSc patients with or without BC and relevant risk factors being measured. SSc was found to have a link to antibodies widely associated with cancer. Adverse treatment outcomes and concurrent conditions of BC were found when patients had a family history of SSc, BC, or an alcohol or smoking history. Our results suggest that the presence of antinuclear antibodies, anti-centromere antibodies, or anti-topoisomerase antibodies in SSc patients is correlated with BC. Out of the three antibodies, ATA seemed to be found more commonly in patients with SSc and malignancy across the studies. This systematic review discusses the link between SSc and BC through patients with relevant clinical markers, medical histories, and treatments. However, further research is necessary to advance the linkage between SSc and BC and determine whether management of one condition may prevent or alleviate the other.

Color changes in the feet: a sign of autonomic symptoms in systemic sclerosis.

OBJECTIVES: Patients with autonomic dysfunction, or dysautonomia, often report discoloration of their dependent extremities, which is thought to be from venous pooling or acrocyanosis. A subset of patients with systemic sclerosis (SSc) are affected by dysautonomia but may be challenging to identify. We sought to determine whether patients with SSc who report discoloration in their feet have a higher burden of autonomic symptoms, including orthostatic, gastrointestinal (GI), urinary, secretomotor, and pupillomotor. METHODS: 167 patients with SSc completed the COMPASS-31 survey, which queries whether the patient experiences discoloration of the feet or hands. We compared the COMPASS-31 subdomain scores between SSc patients with and without foot discoloration. 79 patients with postural orthostatic tachycardia syndrome (POTS) also completed the COMPASS-31 questionnaire for comparison. RESULTS: We found that extremity discoloration is common in POTS and more often affects the feet, whereas in SSc the hands are more frequently involved. 62% of SSc patients report colour changes in their feet. These patients are more likely to have other autonomic symptoms, including orthostatic (11.7 ± 10.6 vs 8.6 ± 9.9, p= 0.06), GI (11.3 ± 4.3 vs 8.8 ± 4.3, p= 0.0003), urinary (1.4 ± 1.5 vs 0.8 ± 1.3, p= 0.002) and secretomotor (7.0 ± 3.8 vs 5.9 ± 3.8, p= 0.06) symptoms. These associations persist in a multivariable model after adjusting for potential confounders. CONCLUSION: Dependent extremity discoloration is common in dysautonomia. Patients with SSc who report colour changes in their feet are more likely to report other symptoms of autonomic dysfunction.