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Background/Aims Systemic sclerosis (SSc) is characterised by endothelial dysfunction and vasculopathy, which may lead to venous thrombosis. Here, we report four cases of extensive venous thrombosis of the upper limbs and right atrium associated with implantable venous access devices (port-a-cath) in patients with a diagnosis of SSc, who presented to our specialist centre between 2018 and 2022. Methods We retrospectively reviewed four patients with SSc and port-a-cathassociated thrombosis who presented to the Department of Rheumatology, Royal Free Hospital NHS Trust between 2018 and 2022. All patients are diagnosed with systemic sclerosis according to the 2013 ACR/EULAR classification criteria. Results Three patients were diagnosed with a port-a-cath-associated thrombosis in 2022, and one in 2018. Two patients had limited cutaneous SSc with positive anti-centromere antibodies, and 2 had diffuse subset with anti-U3RNP antibodies. All patients had a right-sided port-a-cath that had been in-situ for at least 3 years. Two patients were diagnosed with right atrium thrombus (measuring 2.2 and 3cm respectively), one patient with an internal jugular vein and right subclavian thrombosis, and one with a left subclavian thrombosis. None had a history of previous thromboembolic event. A full thrombophilia screen was negative in 2 patients, and is pending in the others. Of note, 2 patients had COVID-19 infection within the 3 months prior the thrombotic event. 1 patient had tocilizumab administered through the line, 1 rituximab and IVIG, the other 2 had prostanoids only. Conclusion We described four recent cases of port-a-cath-associated thrombosis of the upper limbs and right atrium in SSc patients with no previous history of thrombosis. This highlights the increased risk of thrombosis related to long term indwelling catheters in SSc and demonstrates the potential interplay between covid microvasculopathy and the associated thrombotic risks reported with both ACA and antiU3RNP antibodies in SSc. We note that from previous reports the relative lower risk of adverse outcomes in SSc patient receiving parenteral nutrition. Further research into frequency of port-a-cath-related thrombosis in SSc patients is warranted, especially with use of prostanoids, and adequate screening and non-invasive follow up might be needed to avoid life-threatening thromboembolic complications. (Table Presented).
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Background: Evaluation of skin is central to both clinical practice and trials in systemic sclerosis (SSc). This is generally done with the modified Rodnan Skin Score (mRSS). Remote consultations are now widely implemented in response to the COVID-19 pandemic, which has inevitably limited evaluation of skin. To monitor skin during this pandemic and to further explore ways to assess skin, we developed the PASTUL (Patient self-Assessment of Skin Thickness in Upper Limb) questionnaire. Objectives: This study evaluated feasibility and validity of PASTUL in SSc. Methods: The PASTUL questionnaire uses a simple self-assessed grading of skin as normal, mild, moderate, or severely thickened at eight sites of upper limb corresponding to mRSS. Assessed grades were converted to an integer scale [0, 1, 2, 3]. Detailed instructions for patients were provided. Scleroderma Skin PRO (SSPRO) and Scleroderma Health Assessment Disability Index (SHAQ-DI) were also completed. For comparison, physician assessed mRSS was performed in a subgroup of patients. Construct validity was evaluated by examining the correlation between PASTUL, mRSS, SSPRO and SHAQ-DI using Pearson's correlation coefficient. Content validity was evaluated by scoring relevance, clarity and practical difficulty. Test-retest reliability was estimated using intraclass correlation coefficient (ICC). Results: 130 patients were invited of which 104 (80%) completed the questionnaires. The mRSS was undertaken in 78 patients (n=42, 54% limited cutaneous SSc (lcSSc)). The PASTUL was completed by patients (86%) or by a partner/ friend (14%). Mean PASTUL score was 11 (SD 7), mean HAQ-DI 1.41 (SD 0.77) and mean SSPRO 48.3 (SD 27.0). PASTUL strongly correlated with total SSPRO and SSPRO subdomain physical limitations (r=0.60 and 0.62, respectively) (Figure 1A). Correlations between PASTUL and mRSS and mRSS upper limbs were moderate (r=0.56 and 0.58, respectively) (Figure 1B). An overview of all correlations is provided in Table 1. Correlation between PASTUL and mRSS was stronger in lcSSc compared to diffuse cutaneous SSc patients (r=0.53 vs 0.43) and when assessed by a partner/friend compared to patients themselves (r=0.90 vs 0.54). The PASTUL demonstrated excellent test-retest reliability (ICC of 0.93, p<0.001) and good content validity. Conclusion: The significant correlation of PASTUL scores with total SSPRO and physical limitation scores and moderate correlation with mRSS support the potential of PASTUL for remote evaluation of skin thickness in virtual clinical settings. Future studies may explore sensitivity to change and utility in clinical trials.
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Background/AimsEvaluation of skin is central to both clinical practice and trials insystemic sclerosis (SSc). This is generally done with the modifiedRodnan Skin Score (mRSS). Remote consultations are now widelyimplemented in response to the COVID-19 pandemic, which hasinevitably limited evaluation of skin. To monitor skin during thispandemic and to further explore ways to assess skin, we developedthe PASTUL (Patient self-Assessment of Skin Thickness in UpperLimb) questionnaire. The aim of this study was to evaluate feasibilityand validity of the PASTUL in SSc.MethodsThe PASTUL questionnaire specifies a simple grading of skin asnormal, mild, moderate, or severely thickened at eight sites of upperlimb corresponding to mRSS. Assessed grades were converted to aninteger scale [0, 1,2,3]. Detailed instructions for patients wereprovided. Scleroderma Skin PRO (SSPRO) and Scleroderma HealthAssessment Disability Index (SHAQ-DI) were also completed. ThemRSS was done in a selection of patients. Construct validity wasevaluated by examining the correlation between PASTUL, mRSS, SSPRO and SHAQ-DI using Pearson's correlation coefficient. Contentvalidity was evaluated by scoring relevance, clarity and practicaldifficulty. Test-retest reliability was estimated using intraclass correlation coefficient (ICC).ResultsIn total, 107 patients were invited of which 83 (77.6%) completed thequestionnaires. The mRSS was undertaken in 61 patients. ThePASTUL was completed by patients (83.1%) or by a partner/friend(16.9%). Mean PASTUL score was 11 (SD 6), mean HAQ-DI 1.47 (SD0.76) and mean SSPRO 49.8 (SD 26.6). PASTUL and SSPRO physicallimitations correlated strongly (0.62, p < 0.001). Correlations betweenPASTUL and total SSPRO and mRSS upper limbs were moderate toweak (0.59, 0.50 and 0.32 respectively). Correlation between PASTULand mRSS was stronger in lcSSc compared to dcSSc patients (0.61 vs0.29) and when assessed by a partner/friend compared to patientsthemselves (0.98 vs 0.45). The PASTUL demonstrated excellent testretest reliability (ICC of 0.92) and good content validity. ConclusionModerate and significant correlations of PASTUL scores with totalSSPRO, physical limitation scores and mRSS support the usefulnessof PASTUL as an outcome measure and indicates it's potential for usein virtual clinical settings.
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At the time of writing of this article, there have been over 110 million cases and 2.4 million deaths worldwide since the start of the Coronavirus Disease 2019 (COVID-19) pandemic, postponing millions of non-urgent surgeries. Existing literature explores the complexities of rationing medical care. However, implications of non-urgent surgery postponement during the COVID-19 pandemic have not yet been analyzed within the context of the four pillars of medical ethics. The objective of this review is to discuss the ethics of elective surgery cancellation during the COVID-19 pandemic in relation to beneficence, non-maleficence, justice, and autonomy. This review hypothesizes that a more equitable decision-making algorithm can be formulated by analyzing the ethical dilemmas of elective surgical care during the pandemic through the lens of these four pillars. This paper's analysis shows that non-urgent surgeries treat conditions that can become urgent if left untreated. Postponement of these surgeries can cause cumulative harm downstream. An improved algorithm can address these issues of beneficence by weighing local pandemic stressors within predictive algorithms to appropriately increase surgeries. Additionally, the potential harms of performing non-urgent surgeries extend beyond the patient. Non-maleficence is maintained through using enhanced screening protocols and modifying surgical techniques to reduce risks to patients and clinicians. This model proposes a system to transfer patients from areas of high to low burden, addressing the challenge of justice by considering facility burden rather than value judgments concerning the nature of a particular surgery, such as cosmetic surgeries. Autonomy can be respected by giving patients the option to cancel or postpone non-urgent surgeries. However, in the context of limited resources in a global pandemic, autonomy is not absolute. Non-urgent surgeries can ethically be postponed in opposition to the patient's preference. The proposed algorithm attempts to uphold the four principles of medical ethics in rationing non-urgent surgical care by building upon existing decision models, using additional measures of resource burden and surgical safety to increase health care access and decrease long-term harm as much as possible. The next global health crisis will undoubtedly present its own unique challenges. This model may serve as a comprehensive starting point in determining future guidelines for non-urgent surgical care.