Assessment of disease outcome measures in systemic sclerosis

Lafyatis, Robert; Valenzi, Eleanor

doi:10.1038/s41584-022-00803-6

Cited by 13 publications

(34 citation statements)

References 161 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Some authors recommend that ssSSc should be a separate condition to avoid misdiagnosis,4 28 while others consider ssSSc as a mild subvariant of lcSSc 8 9 12. In this scenario, several data suggested that the investigation of SSc subsets might help to better understand the disease aetiopathogenesis, and to shape the prognosis predicting the severity of organ complications 1–4. The fact that ssSSc and lcSSc share a similar clinical picture, the autoantibody profile and the peripheral microangiopathy may suggest that these subsets are strongly related, although with a different skin phenotype.…”

Section: Discussionmentioning

confidence: 99%

“…Systemic sclerosis (SSc) is a connective tissue disease affecting skin and internal organs, characterised by autoimmunity, microvascular injury and collagen deposition 1 2. In SSc, widespread skin and visceral fibrosis are associated with a reduction of quality of life, poor patients’ outcomes and increased mortality 1 3. The hallmark of the disease is the remarkable heterogeneity of clinical manifestations 3.…”

Section: Introductionmentioning

confidence: 99%

“…In SSc, widespread skin and visceral fibrosis are associated with a reduction of quality of life, poor patients’ outcomes and increased mortality 1 3. The hallmark of the disease is the remarkable heterogeneity of clinical manifestations 3. The disease is clinically classified according to the extension of skin involvement in two main subsets, limited cutaneous SSc (lcSSc) and diffuse cutaneous (dcSSc), that also include SSc-specific autoantibodies, nailfold capillaroscopic patterns and fibrosis of internal organ 2 3.…”

Section: Introductionmentioning

confidence: 99%

“…The hallmark of the disease is the remarkable heterogeneity of clinical manifestations 3. The disease is clinically classified according to the extension of skin involvement in two main subsets, limited cutaneous SSc (lcSSc) and diffuse cutaneous (dcSSc), that also include SSc-specific autoantibodies, nailfold capillaroscopic patterns and fibrosis of internal organ 2 3. The two subsets have well-recognised differences with respect to disease severity and prognosis 2 4.…”

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry ‘SPRING’ of the Italian Society for Rheumatology

et al. 2023

View full text Add to dashboard Cite

ObjectiveTo describe demographic, clinical and laboratory features of systemic sclerosis sine scleroderma (ssSSc) in a large multicentre systemic sclerosis (SSc) cohort.MethodsData involving 1808 SSc patients from Italian Systemic sclerosis PRogression INvestiGation registry were collected. The ssSSc was defined by the absence of any cutaneous sclerosis and/or puffy fingers. Clinical and serological features of ssSSc were compared with limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) subsets.ResultsAmong patients with SSc, only 61 (3.4%) were classified as having ssSSc (F/M=19/1). Time from Raynaud’s phenomenon (RP) onset to diagnosis was longer in ssSSc (3 years, IQR 1–16.5) than lcSSc (2 years, IQR 0–7), and dcSSc (1 year, IQR 0–3) (p<0.001). Clinical ssSSc phenotype was comparable to lcSSc, except for digital pitting scars (DPS) (19.7% vs 42%, p=0.01), but significantly milder than dcSSc, particularly for digital ulcers (DU) (6.6% vs 35.7%, p<0.001), oesophagus (46.2% vs 63.5%, p=0.009), lung (mean diffusion capacity for carbon monoxide 72.2±19.6 vs 62.4±22.8, p=0.009; mean forced vital capacity 105.6±21.7 vs 89.2±20.9, p<0.001) and major videocapillaroscopic alterations (late pattern 8.6% vs 47.6%, p<0.001). Moreover, in ssSSc the percentages of anticentromere and antitopoisomerase were comparable to lcSSc (40% and 18.3% vs 36.7% and 26.6%), but divergent respect to dcSSc (8.6% and 67.4%, p<0.001).ConclusionThe ssSSc is a quite rare disease variant characterised by clinico-serological features comparable to lcSSc, but significantly different from dcSSc. Overall, longer RP duration, low percentages of DPS and peripheral microvascular abnormalities, and increased anti-centromere seropositivity distinguish ssSSc. Further investigations based on national registries might provide useful insights on the actual relevance of the ssSSc within the scleroderma spectrum.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry ‘SPRING’ of the Italian Society for Rheumatology

et al. 2023

View full text Add to dashboard Cite

show abstract

“…SSc is a relative rare rheumatic disease with vascular injury and fibrosis as its main clinical features (34). According to a study from the European Alliance of Associations for Rheumatology (EULAR) Scleroderma Trials and Research (EUSTAR) database, malignancy has become one of the leading causes of death in this disease (13%) (35).…”

Section: Systemic Sclerosis (Ssc)mentioning

confidence: 99%

Malignancies in systemic rheumatic diseases: A mini review

Geng

Ye²,

Zhu³

2023

Front. Immunol.

View full text Add to dashboard Cite

There is an increased risk of malignancies in patients with many systemic rheumatic diseases, which negatively impact on their quality of life. The risk and types of malignancies can differ by the type of rheumatic diseases. Possible mechanisms linking them are dynamic and complicated, including chronic inflammation and damage in rheumatic disease, inability to clear oncogenic infections, shared etiology and some anti-rheumatic therapies. Although certain disease-modifying anti-rheumatic drugs (DMARDs) have been proved to be potentially carcinogenic, the majority of them were not associated with increased risk of most malignancies in patients with systemic rheumatic diseases.

show abstract

The role of immunosuppressive myofibroblasts in the aging process and age-related diseases

Salminen

2023

J Mol Med

View full text Add to dashboard Cite

Tissue-resident fibroblasts are mesenchymal cells which control the structural integrity of the extracellular matrix (ECM). Fibroblasts possess a remarkable plasticity to allow them to adapt to the changes in the microenvironment and thus maintain tissue homeostasis. Several stresses, also those associated with the aging process, convert quiescent fibroblasts into myofibroblasts which not only display fibrogenic properties but also act as immune regulators cooperating both with tissue-resident immune cells and those immune cells recruited into affected tissues. TGF-β cytokine and reactive oxygen species (ROS) are major inducers of myofibroblast differentiation in pathological conditions either from quiescent fibroblasts or via transdifferentiation from certain other cell types, e.g., macrophages, adipocytes, pericytes, and endothelial cells. Intriguingly, TGF-β and ROS are also important signaling mediators between immunosuppressive cells, such as MDSCs, Tregs, and M2 macrophages. It seems that in pathological states, myofibroblasts are able to interact with the immunosuppressive network. There is clear evidence that a low-grade chronic inflammatory state in aging tissues is counteracted by activation of compensatory immunosuppression. Interestingly, common enhancers of the aging process, such as oxidative stress, loss of DNA integrity, and inflammatory insults, are inducers of myofibroblasts, whereas anti-aging treatments with metformin and rapamycin suppress the differentiation of myofibroblasts and thus prevent age-related tissue fibrosis. I will examine the reciprocal interactions between myofibroblasts and immunosuppressive cells within aging tissues. It seems that the differentiation of myofibroblasts with age-related harmful stresses enhances the activity of the immunosuppressive network which promotes tissue fibrosis and degeneration in elderly individuals.

show abstract

Assessment of disease outcome measures in systemic sclerosis

Cited by 13 publications

References 161 publications

Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry ‘SPRING’ of the Italian Society for Rheumatology

Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry ‘SPRING’ of the Italian Society for Rheumatology

Malignancies in systemic rheumatic diseases: A mini review

The role of immunosuppressive myofibroblasts in the aging process and age-related diseases

Contact Info

Product

Resources

About