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Awareness weeks guide

World Scleroderma Day

World Scleroderma Day

29 June 2024

On June 29, the world’s scleroderma community comes together to recognize World Scleroderma Day.  On this day in 1940, internationally renowned Swiss painter Paul Klee died. He had scleroderma and his artwork was widely influenced by his experience with the condition. Scleroderma, or systemic sclerosis, is a chronic connective tissue condition generally classified as one of the autoimmune rheumatic diseases. The word “scleroderma” comes from two Greek words: “sclero” meaning hard, and “derma” meaning skin. Hardening of the skin is one of the most visible manifestations of the condition. Scleroderma is a condition where symptoms may be visible, as is the case when the skin is affected, or the symptoms may be invisible, as when internal organs are affected. Scleroderma Australia

UpToDate

Diagnosis and manifestations

General

Treatment

Cochrane Library

Guidelines

Patient resources

Articles

Diagnosis

Mental health

Research

Treatment

E-books

E-journals

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Articles

Diagnosis

Can machine learning assist in systemic sclerosis diagnosis and management? A scoping review
This scoping review aims to summarize the existing literature on how machine learning can be used to impact systemic sclerosis diagnosis, management, and treatment. Journal of scleroderma and related disorders 24 May 2024

Use of exercise tests in screening for pulmonary arterial hypertension in systemic sclerosis: A systematic literature review
Exercise tests are infrequently used for screening for pulmonary arterial hypertension (PAH)  in systemic sclerosis (SSc) but can predict the presence of PAH. More data are required to establish which tests are most effective. Journal of scleroderma and related disorders 2 October 2023

Systemic sclerosis-associated interstitial lung disease: Diagnostic approaches and challenges
Interstitial lung disease (ILD) is a leading cause of both morbidity and mortality in systemic sclerosis (SSc). Radiographic lung abnormalities on high-resolution computed tomography (HRCT) imaging may be identified in 75–90% of those with SSc, while clinically significant ILD occurs in up to 40%. Early detection is important as early treatment in those with progressive ILD may improve outcomes. Appropriately risk-stratifying systemic sclerosis-associated ILD (SSc-ILD) is important in identifying those at highest risk of progression. This article summarises recent advances in SSc-ILD, particularly recommendations for screening, defining disease progression and monitoring. Revista Colombiana de Reumatología (English Edition)  2 April 2024

The role of lung biopsy for diagnosis and prognosis of interstitial lung disease in systemic sclerosis: a systematic literature review
The data from this systematic literature review (SLR) clearly show the paucity and heterogeneity of the studies reporting lung biopsy in systemic sclerosis interstitial lung disease (SSc-ILD). Moreover, they highlight the need for further research to address whether the lung biopsy can be helpful to refine prognostic prediction and guide therapeutic choices. Respiratory research 23 March 2024

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Mental health

Within-person fluctuations of fatigue in patients with a clinical diagnosis of systemic sclerosis and its relationship with mood, pain, sleep and physical activity
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, these results indicate that integrating activities with positive impact on mood into fatigue treatment strategies might reduce the frequency of fatigue fluctuations. Journal of scleroderma and related disorders 2 May 2024

The association of resilience and positive mental health in systemic sclerosis: A Scleroderma Patient-centered Intervention Network (SPIN) cohort cross-sectional study
Highlights

  • The authors examined resilience among people with systemic sclerosis.
  • Participants were grouped in 5 classes based on patient-reported outcome patterns.
  • High disease severity was linked to less resilience and worse patient outcomes.
  • A distinct class reported higher resilience despite high disease severity.
  • Resilience characteristics of this subgroup require further investigation.

Journal of psychosomatic research 11 March 2024

Psychosomatic symptoms in patients with limited scleroderma
Patients with limited scleroderma have a wider range of psychosomatic symptoms. The predominant characteristics of psychosomatic changes are somatization, obsessive-compulsive disorders, depression, and anxiety. International journal of medical sciences 29 May 2024

Low cognitive functioning and depressive symptoms in patients with rheumatoid arthritis and systemic sclerosis: a clinical study
Patients with Rheumatoid Arthritis (RA) and Systemic Sclerosis (SSc)encountered cognitive dysfunction, which partially pertains to depressive symptoms. Of note, the severity of cognitive dysfunction in many cases exceeded that of mild neurocognitive disorder due to Alzheimer’s disease (MiND). BMC psychiatry 18 July 2023

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Research

Patients’ perspectives on systemic sclerosis-related Raynaud's phenomenon in the feet: A qualitative study from the OMERACT Foot and Ankle Working Group
This large qualitative study exploring the experiences of patients with Raynaud's phenomenon (RP) systemic sclerosis (SSc-RP), in the feet identified several key domains of high importance to patients. SSc-RP is common in the feet, presents in several patterns, and impacts multiple aspects of patients’ lives. These findings indicate where future foot-specific interventions for RP could be targeted. Findings from this study improve understanding of what domains are important to patients with SSc-RP affecting the feet and will contribute to the development of a core outcome set for foot and ankle disorders in rheumatic and musculoskeletal diseases. Seminars in arthritis and rheumatism 2 February 2024

Multiple serum biomarkers associate with mortality and interstitial lung disease progression in systemic sclerosis
Multiple biomarkers, especially VCAM-1, E-selectin, SP-D and CXCL4, provide prognostic utility beyond that of established risk factors for patients with SSc. Rhemaology 15 February 2024

Preliminary nomogram model for predicting irreversible organ damage of patients with systemic sclerosis
The authors included commonly used clinical indicators. According to the nomogram, the probability of irreversible organ damage can be calculated and high-risk patients can be identified. Rhemaology 6 February 2024

Proteomic biomarkers for survival in systemic sclerosis-associated pulmonary hypertension
The prognosis of SSc-PH-ILD patients is poor. This proteomic approach found 7 plasma proteins (involved in haemostasis and fibrosis pathways) associated with survival. These potential biomarkers may be good candidates to prognostic enrichment. Respiratory research 7 November 2023

Sleep quality and clinical association with sleep disturbance in systemic sclerosis
Around half of the SSc patients reported poor sleep quality, and the significantly associated factors were digital ulcers and dyspepsia. The Pittsburgh Sleep Quality Index (PSQI) scores positively correlated with overall pain as evaluated by VAS. With early assessment and treatment of digital ulcers, stomach symptoms, and pain control, sleep problems might be reduced among SSc patients. BMC rheumatology 21 July 2023

The association between air pollution and the severity at diagnosis and progression of systemic sclerosis-associated interstitial lung disease: results from the retrospective ScleroPol study
these findings suggest that high levels of O3 exposure are associated with more severe SSc-associated ILD at diagnosis, and progression at 24 months. Respiratory research 8 June 2024

Clinical characteristics and survival of pulmonary arterial hypertension with or without interstitial lung disease in systemic sclerosis
The prevalence of concurrent pulmonary arterial hypertension (PAH) with or without interstitial lung disease (ILD) in the Australian Scleroderma Cohort Study (ASCS) is 7%, with poorer survival in those patients with PAH-ILD compared to ILD or SSc alone. The presence of PAH confers a poorer overall prognosis than even extensive ILD; however, further data are required to better understand the clinical outcomes of this high-risk patient group. Arthritis research and therapy 12 May 2023

Distinct Scleroderma Autoantibody profiles stratify patients for cancer risk at Scleroderma onset and during the disease course
These data suggest that five distinct scleroderma immune responses, alone or in combination, may be useful tools to stratify the risk of cancer for scleroderma patients. Further study examining cancer risk in autoantibody subgroups relative to the general population is warranted. Arthritis and rheumatology  24 July 2023

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Treatment

Australian rheumatologists' perception of autologous haemopoietic stem cell transplantation for the treatment of systemic sclerosis: a cross-sectional survey
In this national survey of rheumatologists, Autologous haemopoietic stem cell transplantation (AHSCT) is considered an accepted therapy. However, concern about toxicity remains a potential barrier to patient referral. Access, studies to refine patient selection and development of AHSCT protocols that improve safety were identified as key areas of need. Internal medicine journal 31 May 2024

Successful long-term systemic sclerosis treatment by high-frequent low-dose B cell-depleting therapy
Systemic sclerosis (SSc) was effectively and safely treated with low-dose rituximab (RTX) quarterly. randomized controlled trials (RCTs) are warranted to validate the advantage of continuous B cell depletion by quarterly low-dose RTX administration compared to other treatment intervals. Journal of autoimmunity 23 May 2024

Rituximab retention rate in systemic sclerosis: a long term real-life multicenter study
Rituximab is a safe and effective treatment in SSc: clinical response emerged as the primary reason for rituximab discontinuation, and AEs had a limited impact on treatment persistence. The identification of specific disease features associated with a response to rituximab will be useful in the management of SSc-patients. Rheumatology 15 May 2024

Outcomes of patients with Diffuse Systemic Sclerosis Eligible for Autologous Stem Cell Transplantation treated with conventional therapy
Autologous stem cell transplant (ASCT)  patients meeting Autologous Stem Cell Transplantation International Scleroderma (ASTIS) and/or Scleroderma: Cyclophosphamide Or Transplantation (SCOT) inclusion criteria who were not treated with ASCT have similar better event-free survival (EFS)   at 4 years as patients receiving ASCT and EFS than those receiving cyclophosphamide in the ASTIS and SCOT trials. This may reflect confounders unable to be controlled for, including survivor bias, but may also reflect improved standard of care for diffuse cutaneous systemic sclerosis (dcSSc) over time. Arthritis & rheumatology 1 April 2024

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E-books

This is just a sample of the e-books the library subscribes to – you will need your library login

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E-Journals

A sample of the journals the library subscribes to – you will need your library login

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