Management Challenges in the Early Systemic Sclerosis Population: Navigating Side Effects of Therapeutics and Approach to Diarrhea

2020 ◽  
pp. 381-388
Author(s):  
Tracy M. Frech ◽  
Jeanmarie Mayer ◽  
Silvia Bellando-Randone
Keyword(s):  
Systemic Sclerosis ◽  
Side Effects ◽  
Early Systemic Sclerosis

Tocilizumab Prevents Progression of Early Systemic Sclerosis Associated Interstitial Lung Disease

2021 ◽  
Author(s):  
David Roofeh ◽  
Celia J F Lin ◽  
Jonathan Goldin ◽  
Grace Hyun Kim ◽  
Daniel E Furst ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Early Systemic Sclerosis

scholarly journals SAT0330 NEW IMMUNOMODULATORY COMBINATION THERAPIES IN PATIENTS WITH SYSTEMIC SCLEROSIS: A RETROSPECTIVE CROSS-SECTIONAL STUDY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1110.1-1111
Author(s):  
J. Qiao ◽  
S. X. Zhang ◽  
T. T. Zhang ◽  
J. Zhang ◽  
M. T. Qiu ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Side Effects ◽  
Lymphocyte Subsets ◽  
Cross Sectional Study ◽  
Peripheral Lymphocyte ◽  
High Morbidity ◽  
Combination Therapies ◽  
Sectional Study ◽  
Cross Sectional ◽  
Long Term Treatment

Background:Systemic sclerosis (scleroderma, SSc) is a rare complex connective tissue disease associated with high mortality and high morbidity1. Active SSc are typically treated with immunosuppressants, which may create a variety of severe side-effects, especially for long-term treatment2. As the pathogenesis of SSc is still a matter of debate, growing evidences have focused on the immune disorders3. However, the quantitative status of lymphocyte subsets in SSc patients are unclear and effects of immunomodulatory combination therapies (avoiding side-effects of conventional therapy) on the lymphocyte subsets are unknown.Objectives:To investigate the quantitative status of peripheral lymphocyte subpopulations and CD4+T subsets in SSc patients for the exploration of SSc pathogenesis and evaluate the effects of new immunomodulatory combination therapies on those cells.Methods:From July 2014 to December 2019, total 166 patients with SSc and 206 healthy controls (HCs) were enrolled in this study, in which, 79 follow-up patients received immunomodulatory drugs (IMiDs) such as low-dose interleukin-2, rapamycin, metformin, retinoic acid and coenzyme Q10. The absolute numbers of T, B, NK, CD4+T, CD8+T, Th1, Th2, Th17 and Tregs in peripheral blood of these subjects were detected by flow cytometry combined with standard absolute counting beads.Results:Patients with SSc had lower absolute counts of total T, NK, Th2, Th17 and Tregs as compared with those of HCs (P<0.05) (Figure 1). After immunomodulatory combination treatments, there were increases in a various of peripheral lymphocyte subsets such as T, B and CD8+T (P< 0.05). Moreover, the increased level of Tregs was much more dramatical than those of other lymphocyte subsets, resulting in the decrease ratios of Teffs/Tregs such as Th1/Tregs and Th2/Tregs and rebuilding immunologic equilibrium (Figure 2).Conclusion:This cross-sectional study clarified the abnormal status of lymphocyte subsets in SSc patients, suggesting lymphocyte subsets, especially Tregs, might be relevant and play a crucial role in the pathogenesis of SSc, thus providing a potential therapeutic target for SSc patients. Immunomodulatory combination therapies effectively increase the level of Tregs as well as other lymphocytes to some degree and maintain the immunologic equilibrium, which may help for SSc patients’ symptom remission.References:[1]Denton CP, Khanna D. Systemic sclerosis. Lancet 2017;390(10103):1685-99. doi: 10.1016/S0140-6736(17)30933-9 [published Online First: 2017/04/18][2]Winthrop KL, Weinblatt ME, Bathon J, et al. Unmet need in rheumatology: reports from the Targeted Therapies meeting 2019. Ann Rheum Dis 2020;79(1):88-93. doi: 10.1136/annrheumdis-2019-216151 [published Online First: 2019/10/31][3]Skaug B, Khanna D, Swindell WR, et al. Global skin gene expression analysis of early diffuse cutaneous systemic sclerosis shows a prominent innate and adaptive inflammatory profile. Ann Rheum Dis 2019 doi: 10.1136/annrheumdis-2019-215894 [published Online First: 2019/11/27]Acknowledgments :None.Disclosure of Interests:None declared

scholarly journals Early systemic sclerosis: assessment of clinical and pre-clinical organ involvement in patients with different disease features

Rheumatology ◽  
2010 ◽  
Vol 50 (2) ◽  
pp. 317-323 ◽  
Author(s):  
G. Valentini ◽  
G. Cuomo ◽  
G. Abignano ◽  
A. Petrillo ◽  
S. Vettori ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Organ Involvement ◽  
Early Systemic Sclerosis

FRI0249 Pulmonary arterial hypertension in very early systemic sclerosis:

2013 ◽  
Vol 71 (Suppl 3) ◽  
pp. 398.2-398
Author(s):  
J. Sanchez ◽  
S. Jordan ◽  
J. Distler ◽  
B. Maurer ◽  
D. Huscher ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pulmonary Arterial ◽  
Early Systemic Sclerosis

scholarly journals New Autoantibody Specificities in Systemic Sclerosis and Very Early Systemic Sclerosis

Antibodies ◽  
2021 ◽  
Vol 10 (2) ◽  
pp. 12
Author(s):  
Roberto Lande ◽  
Raffaella Palazzo ◽  
Anna Mennella ◽  
Immacolata Pietraforte ◽  
Marius Cadar ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Early Diagnosis ◽  
Antibody Response ◽  
Allelic Variant ◽  
The Other ◽  
Autoantibody Response ◽  
Autoimmune Condition ◽  
Early Systemic Sclerosis ◽  
Systemic Sclerosis Patient

Chemokine (C-X-C motif) ligand 4 (CXCL4) is a biomarker of unfavorable prognosis in Systemic Sclerosis (SSc), a potentially severe autoimmune condition, characterized by vasculitis, fibrosis and interferon (IFN)-I-signature. We recently reported that autoantibodies to CXCL4 circulate in SSc patients and correlate with IFN-α. Here, we used shorter versions of CXCL4 and CXCL4-L1, the CXCL4 non-allelic variant, to search for autoantibodies exclusively reacting to one or the other CXCL4 form. Moreover, to address whether anti-CXCL4/CXCL4-L1 antibodies were present before SSc onset and predicted SSc-progression, we longitudinally studied two VEDOSS (Very Early Diagnosis of Systemic Sclerosis) patient cohorts, separating SSc-progressors from SSc-non-progressors. We found that anti-CXCL4-specific autoantibodies were present in both SSc and VEDOSS patients (both SSc-progressors and SSc-non-progressors). Anti-CXCL4-L1-specific autoantibodies were especially detected in long-standing SSc (lsSSc). Anti-CXCL4/CXCL4-L1 antibodies correlated with IFN-α and with specific SSc-skin features but only in lsSSc and not in early SSc (eaSSc) or VEDOSS. Thus, a broader antibody response, with reactivity spreading to CXCL4-L1, is characteristic of lsSSc. The early anti-CXCL4 autoantibody response seems qualitatively different from, and likely less pathogenic than, that observed in advanced SSc. Lastly, we confirm that anti-CXCL4 autoantibodies are SSc-biomarkers and uncover that also CXCL4-L1 becomes an autoantigen in lsSSc.

scholarly journals Early systemic sclerosis: short-term disease evolution and factors predicting the development of new manifestations of organ involvement

2012 ◽  
Vol 14 (4) ◽  
pp. R188 ◽  
Author(s):  
Gabriele Valentini ◽  
Serena Vettori ◽  
Giovanna Cuomo ◽  
Michele Iudici ◽  
Virginia D'Abrosca ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Organ Involvement ◽  
Short Term ◽  
Disease Evolution ◽  
Early Systemic Sclerosis

Classification of Japanese patients with mild/early systemic sclerosis (SSc) by the 2013 ACR/EULAR classification criteria for SSc

2016 ◽  
Vol 27 (4) ◽  
pp. 614-617 ◽  
Author(s):  
Yuka Ikawa ◽  
Yasuhito Hamaguchi ◽  
Naoki Mugii ◽  
Takashi Matsushita ◽  
Kazuhiko Takehara
Keyword(s):  
Systemic Sclerosis ◽  
Japanese Patients ◽  
Classification Criteria ◽  
Early Systemic Sclerosis

Incidence and predictors of interstitial lung disease (ILD) in Thai patients with early systemic sclerosis: Inception cohort study

2015 ◽  
Vol 26 (4) ◽  
pp. 588-593 ◽  
Author(s):  
Suparaporn Wangkaew ◽  
Juntima Euathrongchit ◽  
Pittaporn Wattanawittawas ◽  
Nuntana Kasitanon ◽  
Worawit Louthrenoo
Keyword(s):  
Cohort Study ◽  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Inception Cohort ◽  
Early Systemic Sclerosis

AB0231 Esophageal involvement in very early systemic sclerosis (SSC)

2013 ◽  
Vol 71 (Suppl 3) ◽  
pp. 650.14-650
Author(s):  
G. Lepri ◽  
S. Bellando Randone ◽  
G. Serena ◽  
I. Giani ◽  
G. Carnesecchi ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Esophageal Involvement ◽  
Early Systemic Sclerosis

Up-regulation of CC chemokine ligand 20 and its receptor CCR6 in the lesional skin of early systemic sclerosis

2011 ◽  
Vol 21 (5) ◽  
pp. 731-736 ◽  
Author(s):  
Juan Tao ◽  
Lin Li ◽  
Zhijian Tan ◽  
Yan Li ◽  
Jing Yang ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Lesional Skin ◽  
Cc Chemokine ◽  
Chemokine Ligand ◽  
Early Systemic Sclerosis ◽  
Cc Chemokine Ligand 20
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