What Is New - Myositis and Connective Tissue Disease (other than SLE)
Take a look at our 'What is new' highlights in Myositis and Contective Tissue Disease (other than SLE)!
May 2023
"Predicting lymphoma in Sjögren's syndrome and the pathogenetic role of parotid microenvironment through precise parotid swelling recording"
Why WIN?
Parotid swelling is a well-known risk factor for the development of lymphoma in Sjögren‘s Syndrome (SS). From this study of a very large cohort of SS-related lymphoma patients, we now know that short-lasting episodic swelling does not produce a significantly higher risk, unlike persistent and frequent swelling (> 2 months). This observation contributes to a better stratification of risk in SS patients, thus improving clinical surveillance.
Do you perform periodic ultrasound scan of salivary glands in Sjogren’s patients for early detection of suspected lymphoma?
Yes / No
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Link to original article: https://academic.oup.com/rheumatology/article/62/4/1586/6692297?login=false
April 2023
"Rituximab versus intravenous cyclophosphamide in patients with connective tissue disease-associated interstitial lung disease in the UK (RECITAL): a double-blind, double-dummy, randomised, controlled, phase 2b trial"
Why WIN?
Both cyclophosphamide and rituximab are employed to treat severe and progressive ILD in patients with CTDs. However, the evidence of their efficacy, especially for rituximab, is scarce. Although superiority of rituximab to cyclophosphamide was not demonstrated, both treatments induced comparable improvement of PFT, clinical parameters and quality of life at 48 weeks.
What do you usually use as 1st-line agent to treat patients with non-scleroderma related CTD-ILD?
- Mycophenolate mofetil
- Cyclophosphamide
- Rituximab
- Something else
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March 2023
"Damage Trajectories in Systemic Sclerosis Using Group-Based Trajectory Modeling"
Why WIN?
It is of common knowledge that patients with systemic sclerosis differ widely, ranging from very mild to rapidly progressive disease. This registry-based study was able to identify three subsets of patients with distinct trajectory in terms of damage accrual and mortality. Baseline risk factors for worse or better disease evolution may be helpful to establish intensity of treatment at diagnosis.
Aggressive immunosuppressive treatment should be initiated at diagnosis in SSc patients with poor prognostic factors even when no major organ involvement is present.
Agree / Disagree
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February 2023
" Characterizing the autoantibody repertoire in systemic sclerosis following myeloablative haematopoietic stem cell transplantation "
Why WINS?
Haematopoietic stem cell transplantation (HSCT) is a promising therapy for severe diffuse SSc however little is understood about its potential in reshaping abnormal immune system in autoimmune diseases. This study shows that HSCT favorably ages the autoantibody repertoire, which remains virtually unchanged in patients treated with cyclophosphamide and also identifies new markers that can be used to monitor transplanted SSc patients. Significant differences in autoantibody targets were identified in a subset of HSCT-treated subjects, particularly antigen targets such as soluble cytokines and chemokines, and cell surface receptors which may be useful to identify targets for future therapeutic interventions.
Interactive question
Do you think HSCT is a promising therapy for rebooting B cell repertoire and ultimately leading to antigen-specific tolerance? yes/no
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Jan 2023
" Continued treatment with nintedanib in patients with systemic sclerosis-associated interstitial lung disease: data from SENSCIS-ON "
Why WINS?
The efficacy and safety of nintedanib in patients with SSc-ILD were investigated in the SENSCIS trial, in which patients were randomised to receive nintedanib or placebo until the last patient had reached week 52 but for a maximum of 100 weeks. Over 52 weeks, nintedanib reduced the rate of decline in FVC (mL/year) by 44% compared with placebo. The results of SENSCIS-ON, a open-label extension study show that the safety profile of nintedanib over longer term use was consistent with that seen in the SENSCIS trial and that the change in FVC over 52 weeks of the open-label extension was similar to that seen in patients who received nintedanib in SENSCIS. These findings suggest that nintedanib can be used over the long term to slow the progression of SSc-ILD and so improve patient outcomes
Interactive question
Do you think nintedanib is an effective option for slowing the progression of SSc-ILD?
And if yes, do you use it in monotherapy or combined with MMF?
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December 2022
"Effectiveness and safety of tocilizumab in patients with systemic sclerosis: a propensity score matched controlled observational study of the EUSTAR cohort"
Why WINS?
In the phase II faSScinate trial, a trend for improving skin fibrosis over placebo was found. Exploratory analysis revealed a possible stabilisation of FVC. The phase III focuSSced study confirmed the trend on skin fibrosis without reaching statistical significance. Stabilization of lung fibrosis, with FVC as a key secondary endpoint and additional HRCT quantification, was observed. These data have resulted in the approval of tocilizumab for SSc-associated interstitial lung disease (SSc-ILD) by the FDA however, little is known about the use of Tocilizumab in a broader SSc population. This study assesses the safety and effectiveness of tocilizumab in a real-life setting using the European Scleroderma Trial and Research (EUSTAR) database. No significant effectiveness of tocilizumab was shown in this broader, multicentre, propensity score matched, controlled observational, heterogeneous, non-enriched real-life SSc population from the large European Scleroderma Trial and Research registry. This study adds important information from real-life to the existing RCTs with tocilizumab by generating hypothesis that should be confirmed in a prospective RCT with broader SSc population
Interactive question
What is your opinion on the use of TCZ in patients with SSc-ILD?
Share your response with us using the hashtag #WhatIsNew and tag us @EMEUNET
