Journal of Scleroderma and Related Disorders最新文献

Background and aims: Systemic sclerosis is a debilitating inflammatory condition synonymous with gastrointestinal symptoms which have the potential to impact dietary intake and nutritional status. This study aimed to describe symptoms experienced by patients with systemic sclerosis that may impact on dietary intake and assess nutrition education preferences in this cohort.

Methods: A 24-item online qualitative and quantitative survey distributed via REDCap^® was conducted in adult patients (aged ⩾18 years) living with systemic sclerosis and attending outpatient services at a single healthcare setting from January to March 2022. Data were collected on demographics, symptoms that may impact dietary intake, nutrition priorities and preferred nutrition education models. Data are mean ± standard deviation or number (%).

Results: Of 322 eligible patients, 156 (48%) participated (63 ± 12 years, 86% female, body mass index 27 ± 7 kg/m²). Most patients experienced gastrointestinal conditions (n = 123/155; 79%), which occurred daily in 26% (n = 40/155) of patients. A third of patients (n = 48/156; 31%) reported diet manipulation for symptom management. Recent weight loss was common (n = 36/154; 23% of patients). Less than a third of patients had seen a dietitian (n = 45; 29%), while 69% of patients (n = 107) desired dietetic consultancy. The preferred methods of consultation were written resources and face-to-face, respectively, and systemic sclerosis symptom management (n = 100; 64%) and losing weight (n = 53; 34%) were the most desired education topics reported.

Conclusion: Gastrointestinal conditions are common and occur frequently in patients with systemic sclerosis. Patients want to engage with dietetics services to better manage symptoms via face-to-face consultations and written resources. These results will inform future dietetic service delivery.

Objectives: To summarize the published evidence in the literature on the use of intravenous immunoglobulin in gastrointestinal tract involvement in systemic sclerosis patients and report the experience of our department.

Methods: A systematic literature review was performed; and a literature search was conducted in MEDLINE and Embase until 1/5/2024, using the participants, intervention, comparator and outcomes framework. Only full-text articles involving systemic sclerosis adults, submitted to intravenous immunoglobulin (at least one administration) to treat primary gastrointestinal tract manifestations. The outcome was the University of California at Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0 score to evaluate response to treatment. Two reviewers performed the assessment of data extraction and synthesis, independently.

Results: Four papers (two case reports and two retrospective studies) out of 35 references were included. In addition, we added two systemic sclerosis patients from our department in this review. In 25 systemic sclerosis patients, with various gastrointestinal tract manifestations, the intravenous immunoglobulin therapy was found to improve digestive tract symptoms in SSc patients, as shown by the decrease in the scores of University of California at Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0. No adverse events were reported, except for one case of low-grade fever post-administration.

Conclusion: The results from this systematic literature review based on case series suggest that intravenous immunoglobulin may improve gastrointestinal tract symptoms assessed by the University of California at Los Angeles Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0 scale, with only minor reported adverse events, suggestive of an acceptable safety profile. We believe that this systematic literature review will contribute to shed light on the efficacy and safety aspects of intravenous immunoglobulin treatment in the management of gastrointestinal tract symptoms; and multicenter randomized placebo-controlled trials are urgently needed to foster progress in this field.

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.

Introduction: Pulmonary arterial hypertension and left ventricular diastolic dysfunction are associated with significant morbidity and mortality in systemic sclerosis. N-terminal pro-brain natriuretic peptide has been proposed as part of composite screening algorithms for pulmonary arterial hypertension. Our aim was to assess the prevalence of pulmonary hypertension and diastolic dysfunction, and evaluate their association with serum N-terminal pro-brain natriuretic peptide in systemic sclerosis patients.

Methods: Patients with systemic sclerosis were prospectively enrolled to undergo N-terminal pro-brain natriuretic peptide testing and transthoracic echocardiography at a tertiary Australian centre from January to October 2022. We collected demographic and transthoracic echocardiography variables including pulmonary hypertension estimated by tricuspid regurgitant velocity and diastolic dysfunction assessed by the ASE/EACVI 2016 guidelines. Pearson's correlation coefficient was used to evaluate association between N-terminal pro-brain natriuretic peptide and echocardiographic parameters.

Results: Sixty-one patients were enrolled (median age = 62 years (interquartile range = 55-69 years); 84% female). Two-thirds of patients had limited systemic sclerosis (40/61). Five patients (8%) had high likelihood of pulmonary hypertension by transthoracic echocardiography. Seven patients (11%) had diastolic dysfunction; however, seven patients (11%) had indeterminate diastology. Six patients underwent right heart catheterisation, with five patients diagnosed with pulmonary hypertension. N-terminal pro-brain natriuretic peptide in patients with pulmonary hypertension or diastolic dysfunction was significantly higher (median = 207 and 226 pg/mL, respectively) compared to patients without either condition (median = 69 pg/mL, p = 0.01). N-terminal pro-brain natriuretic peptide showed a statistically significant although limited correlation with estimated pulmonary pressures measured by tricuspid regurgitant velocity (r = 0.44, p = 0.002) and left ventricular filling pressures (r = 0.27, p = 0.04).

Conclusion: Pulmonary hypertension and diastolic dysfunction are both observed in systemic sclerosis. N-terminal pro-brain natriuretic peptide is associated with both conditions; however, it cannot distinguish between the two disease processes. Right heart catheterisation may be required to make this distinction.

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.

Objective: Coronary artery calcification assessed on thoracic computed tomography represents the calcific component of established coronary artery disease, is a biomarker of total atheromatous plaque burden and predicts mortality. Systemic sclerosis is a pro-inflammatory condition, and inflammation is also a driver of coronary artery disease. We assessed coronary artery calcification prevalence, mortality risk and potential clinical impact on primary prevention in a cohort of patients with systemic sclerosis, differentiated by clinical phenotype including the presence of interstitial lung disease and pulmonary arterial hypertension.

Methods: Retrospective analysis of 258 computed tomographies in systemic sclerosis patients from three prospectively maintained clinical and research databases at a single tertiary rheumatology/pulmonary hypertension (PH) service between March 2007 and September 2020 (mean age = 65 ± 12, 14% male). Co-morbidities, statin prescription and all-cause mortality were recorded. Patients were subtyped according to underlying systemic sclerosis complications. Computed tomographies were re-reviewed for coronary artery calcification; severity was graded using a 4-point scale per vessel and summed for total coronary artery calcification score. The impact of reporting coronary artery calcification was assessed against pre-existing statin prescriptions.

Results: Coronary artery calcification was present in 58% (149/258). Coronary artery calcification was more prevalent in systemic sclerosis-pulmonary arterial hypertension than in systemic sclerosis subgroups with interstitial lung disease or without pulmonary arterial hypertension, controlling for age, sex, co-morbidities and smoking status (71%; χ ²(13) = 81.4; p < 0.001). The presence and severity of coronary artery calcification were associated with increased risk of mortality independently of age and co-morbidities (hazard ratio = 2.8; 95% confidence interval = 1.2-6.6; p = 0.018). The 'number needed to report' coronary artery calcification presence to potentially impact management was 3.

Conclusions: Coronary artery calcification is common in systemic sclerosis. Coronary artery calcification predicts mortality independently of age and confounding co-morbidities which suggests this finding has clinical relevance and is a potential target for screening and therapeutic intervention.

Background: Acute heart failure in patients with prosthetic heart valves is a complex problem with clinical and therapeutic challenges. Systemic sclerosis is a chronic autoimmune disease frequently associated with valvular abnormalities. The association between systemic sclerosis and acute heart failure in patients with prosthetic heart valves remains understudied.

Methods: Prosthetic valve patients were extracted from the National Inpatient Sample Database. Baseline patient demographics, comorbidities, and known acute heart failure risk factors were collected from the database using International Classification of Diseases, 10th Revision codes. Patients were subsequently stratified by the diagnosis of systemic sclerosis. The primary outcome was acute heart failure. while secondary outcome included pulmonary outcomes. Univariate and multivariate logistic regression analyses were performed. 1:1 matching was performed to verify our findings.

Results: Among 188,615 patients, 235 patients had systemic sclerosis. Systemic sclerosis patients had higher rates of acute heart failure relative to non-systemic sclerosis patients (28.5% vs 22.6%). On multivariate analysis, systemic sclerosis was associated with increased acute heart failure (adjusted OR: 1.38 (1.02-1.85), p = 0.036). After matching, systemic sclerosis was still associated with an increased incidence of acute heart failure (OR: 1.94 (1.25-3.03), p = 0.003). On subgroup analysis, patients with CREST syndrome did not show significantly increased acute heart failure (OR: 1.44 (0.84-2.47), p = 0.184). Patients with systemic sclerosis also showed a significantly higher rate of acute respiratory failure compared to non-systemic sclerosis patients (20.9% vs 13.7%, p = 0.001).

Conclusion: Systemic sclerosis may increase the risk for acute heart failure in patients with prosthetic valves. Closer monitoring for heart failure symptoms should be considered in systemic sclerosis patients with prosthetic valves.

Objectives: There is an under recognition of juvenile-onset localized scleroderma and its extracutaneous manifestations leading to delay in systemic treatment. Our study aims to address this gap by describing the demographics, presentation, associated family history, concurrent autoimmune disease, extracutaneous manifestations, laboratory evaluation, treatment, and course of disease in juvenile-onset localized scleroderma patients enrolled in the National Registry for Childhood Onset Scleroderma.

Methods: Participants for this study were derived from the National Registry for Childhood Onset Scleroderma and included 341 patients with juvenile-onset localized scleroderma. Demographic, and prospectively collected outcome measures, such as the Localized Scleroderma Cutaneous Assessment Tool, physical exam findings, laboratory values, and patient-reported outcomes were reviewed.

Results: Most patients were female (71%), Caucasian (94%), had a linear subtype (56%), and had the onset of disease at age 7.5 (±4.2) years, and diagnosis 1.9 (±2.6) years after symptom onset. Most patients experienced at least one extracutaneous manifestation (70%), most commonly musculoskeletal (57%), followed by neurological (46%), and ophthalmological (11%). Those with musculoskeletal extracutaneous manifestation have significantly abnormal inflammatory and antibody laboratory values. Of patients with 1-year follow-up, a majority were treated with systemic therapy and globally improved with significant reduction in both modified Localized Scleroderma Skin Index (p < 0.001) and Localized Scleroderma Damage Index (p = 0.001).

Conclusion: The study highlights need for earlier recognition of juvenile-onset localized scleroderma after demonstrating the delay in diagnosis and frequent extracutaneous manifestations with significant disease burden in a juvenile-onset localized scleroderma cohort. The benefits of systemic treatment and full extracutaneous manifestation screening in juvenile-onset localized scleroderma is supported.

Digital ischemia can be a painful complication of Raynaud's phenomenon or systemic sclerosis, which is caused by narrowing of blood vessels in the toes and hands. Epoprostenol is a potent vasodilator that may be used to treat digital ischemia in this patient population. Our institution provides epoprostenol infusion using two different administration techniques: a 30-h continuous infusion option and a 5-day intermittent 6-h infusion. In this retrospective chart review, we compared two administration techniques of intravenous epoprostenol administered to patients with digital ischemia. The primary outcome was to compare the efficacy of intravenous epoprostenol 30-h continuous infusion versus 5-day intermittent infusion, as defined by the presence of treatment failure. Between June 2019 and June 2020, 72 adult patient encounters met the inclusion criteria (intermittent: n = 20; continuous: n = 52). The primary outcome did not achieve a statistically significant difference between the two groups: intermittent 20% versus continuous 33.3% p = 0.390, odds ratio = 0.57 (95% confidence interval = 0.17-1.90). Adverse reactions were documented in 28% of patients across both treatment groups, and there was no difference detected when treatment groups were compared (25% vs 28.8%). Patients who received the 5-day infusion experienced a significantly longer average length of stay, with a mean of 8.9 days versus 3 days for those treated with the continuous 30-h infusion (p < 0.05; 95% confidence interval = 2.15-9.47). This study determined that the efficacy and safety profiles of the two administration techniques may not be comparable. Each protocol offers advantages over the other, and selection should be guided by patient history and risk factors to optimize management.

Background: Morphea-like tattoo reactions are rare phenomena, with few cases reported in the literature. We present a case of a morphea-like tattoo reaction and a literature review of such reactions for comparison.

Case description: A 38-year-old woman with known history of systemic sclerosis presented with abnormal healing and skin thickening over a red tattoo. Histopathological examination revealed sclerosing dermatitis, consistent with morphea-like tattoo reaction. Treatment included topical clobetasol and oral mycophenolate mofetil.

Methods: A literature search of PubMed and EMBASE was performed in August 2023 for known morphea-like tattoo reactions. No time or language filters were applied.

Results: A total of six articles were included. Case reports of morphea-like tattoo reactions in patients with no significant past medical history comprise five articles. One review article notes that red tattoo ink with and without cinnabar is associated with adverse skin reactions.

Conclusions: Morphea-like tattoo reactions can be triggered by ingredients of tattoo ink, possibly due to local hypersensitivity or the Koebner phenomenon. We encourage high clinical suspicion for morphea-like tattoo reactions when a patient with known history of connective tissue disease presents with skin changes around a tattoo.