scholarly journals Tofacitinib in the treatment of skin and musculoskeletal involvement in patients with systemic sclerosis, evaluated by ultrasound

2021 ◽  
Author(s):  
Rositsa Valerieva Karalilova ◽  
Zguro Anastasov Batalov ◽  
Tanya Lyubomirova Sapundzhieva ◽  
Marco Matucci-Cerinic ◽  
Anastas Zgurov Batalov
Keyword(s):  
Systemic Sclerosis ◽  
Treatment Options ◽  
Unmet Need ◽  
Skin Thickness ◽  
Digital Ulcers ◽  
Internal Organs ◽  
Percent Improvement ◽  
Significant Difference ◽  
Modified Rodnan Skin Score ◽  
Musculoskeletal Involvement

AbstractSystemic sclerosis (SSc) is a rare autoimmune connective tissue disease characterized by fibrosis of the skin and internal organs, autoimmunity-driven damage and vasculopathy. The current approved disease-modifying treatments have limited efficacy, and treatment is guided toward alleviating organ complications. Thus, there is an unmet need for discovering new effective treatment options. There is recent evidence that the JAK/STAT signaling pathway is markedly activated in SSc patients. To assess the efficacy and safety of tofacitinib (TOF) on skin and musculoskeletal involvement as compared to methotrexate (MTX) in systemic sclerosis (SSc). In this 52-week pilot study, 66 patients with SSc were enrolled: 33 patients received 5 mg of oral TOF twice a day; 33 received 10 mg of MTX weekly. The proportion of dcSSc and lcSSc patients was similar (dcSSc: 42% TOF group and 36% MTX group; lcSSc: 58% TOF group and 64% MTX group). The primary outcome was the change in the modified Rodnan skin score (mRSS). Secondary outcomes included ultrasound (US) skin thickness and musculoskeletal involvement (US10SSc score). Digital ulcers (DUs) and adverse events (AEs) were documented through the treatment. Both groups had similar characteristics and medians on the outcome measures at baseline. At week 52, the TOF median mRSS was significantly lower than the MTX (p < 0.001) with a mean reduction of 13 points versus MTX 2.57. The mean percent improvement in the TOF group was 44% higher than in the MTX group. TOF median US skin thickness was significantly lower than MTX (p < 0.001), with a mean reduction of 0.31 mm versus 0.075 mm in the MTX group. The US10SSc median score was significantly lower in the TOF group (p = 0.002); mean reduction of 10.21 versus 5.27 in the MTX group. Healing of DUs with no new occurrences was observed in the TOF group. There was no significant difference between the groups in the number of AEs from baseline to week 52. TOF showed greater efficacy than MTX in reducing mRSS, skin thickness and musculoskeletal involvement in SSc and a satisfactory safety profile.

scholarly journals Systemic sclerosis and localized scleroderma: Where are we now?

2021 ◽  
Vol 97 (3) ◽  
pp. 129-143
Author(s):  
Gábor Bali ◽  
◽  
Bernadett Hidvégi ◽  
Miklós Sárdy
Keyword(s):  
Systemic Sclerosis ◽  
Cannabinoid Receptor ◽  
Treatment Options ◽  
Immunosuppressive Treatment ◽  
Treatment Modalities ◽  
Localized Scleroderma ◽  
Digital Ulcers ◽  
Internal Organs ◽  
Cannabinoid Receptor 2 ◽  
Pulmonary Arterial

Systemic sclerosis is a collagen vascular disease which could lead to fibrosis of the skin and internal organs. It is characterized by a high disease burden and mortality. The 2013 classification criteria system helps to recognize the disease early, which allows prompt intervention and a better survival. In the past few year the recognition of the association between the presence of RNA polimerase III antibodies and malignancies was important. Treatment options of SSc have been improved. Early, intensive immunosuppressive treatment (cyclophosphamide, mycophenolate mofetil) of interstitial lung disease inhibits progression. There are several treatment modalities for the treatment of pulmonary arterial hypertension and digital ulcers (endothelin receptor antagonists, phosphodiesterase 5 inhibitors, prostaglandin analogues). Antifibrotic drugs (nintedanib, riociguate) are also available among former immunosuppressive and vasoactive agents. Lenabasum is a new promising selective cannabinoid receptor 2 agonist, which inhibits inflammation and fibrosis. Localized scleroderma presents a diverse clinical picture. The disease leads to fibrosis of the skin but it is not accompanied with serious inner organ involvement typically seen in systemic sclerosis. Capillaroscopy abnormalities, sclerodactyly and SSc specific antibodies are absent in localized scleroderma . The 2017 EDF guideline formulates a very useful classification, checkup and treatment recommendations.

scholarly journals FRI0228 TOFACITINIB IN THE TREATMENT OF SKIN AND MUSCULOSKELETAL INVOLVEMENT IN ADULT PATIENTS WITH EARLY SYSTEMIC SCLEROSIS, EVALUATED BY ULTRASOUND

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 697-697
Author(s):  
R. Karalilova ◽  
T. Sapundzhieva ◽  
Z. Batalov ◽  
M. Matucci-Cerinic ◽  
A. Batalov
Keyword(s):  
Systemic Sclerosis ◽  
Adverse Events ◽  
Outcome Measures ◽  
Controlled Trial ◽  
Pilot Trial ◽  
Skin Thickness ◽  
Open Label ◽  
Safety And Efficacy ◽  
Significant Difference ◽  
Musculoskeletal Involvement

Background:The whole management of systemic sclerosis (SSc) remains to be defined while trials mainly focus on the treatment of different organ involvement and disease-modifying treatments are still not available.Objectives:To assess the safety and efficacy of tofacitinib (TOF) treatment on skin and musculoskeletal involvement as compared to methotrexate (MTX) treatment in patients with early SSc.Methods:In this 52-week, prospective, investigator-initiated, open-label, single-centre study, 66 patients with SSc were enrolled. Thirty-three patients received 5mg of oral TOF twice a day; and thirty-three received 7.5-10 mg of MTX weekly. The primary outcome measures were: skin fibrosis improvement at week 26, assessed by the reduction in skin thickness - evaluated by the modified Rodnan skin score (mRSS) and the ultrasound (US) measured skin thickness; improvement in the musculoskeletal involvement, assessed by the reduction in the joint and tendon score (US10SSc score); and adverse events from baseline to week 26. The dynamics in the outcome measures within each group were examined through Wilcoxon tests and between-group comparisons were performed through Mann-Whitney U and Chi-square tests.Results:At baseline, both groups of patients had similar median scores with no significant differences on all measures: mRSS (p = 0.589), US measured skin thickness (p = 0.822), and US10SSc score (p = 0.918). At week 26, significant differences were observed between the two treatment groups as the TOF treated patients showed a greater reduction in mRSS and musculoskeletal manifestations. In the TOF group, the median mRSS score decreased by 50% from 24 to 12 (IQR = 7.50) versus a smaller decrease of 8.70% in the MTX group, from 23 to 21 (IQR = 8.00), p < 0.001. The median US measured skin thickness in the TOF treated patients decreased by 12.87% from 1.71 to 1.49 (IQR =0.31) versus a decrease of 4.73%, from 1.69 to 1.61 (IQR =0.52) in the MTX group, p = 0.040. The US10SSc median score in the TOF group decreased by 56.25% from 16 to 7 (IQR = 6.50) versus a decrease of 12.5% in MTX group from 16 to 14 (IQR =10.50), p < 0.001. There was no significant difference between the groups in the number of adverse events from baseline to week 26. No cases of herpes zoster and deep vein thrombosis were observed in the TOF group.Conclusion:The data show that in early SSc TOF may lead to a significant improvement of skin thickness, measured with the mRSS and US, and of the musculoskeletal involvement, measured by the US10SSc score. TOF has also shown a satisfactory safety profile.References:[1]Elhai M, Boubaya M, Distler O, et al. Outcomes of patients with systemic sclerosis treated with rituximab in contemporary practice: a prospective cohort study. Ann Rheum Dis. 2019 Jul;78(7):979-987.[2]Khanna D, Denton CP, Lin CJF, et al. Safety and efficacy of subcutaneous tocilizumab in systemic sclerosis: results from the open-label period of a phase II randomised controlled trial (faSScinate). Ann Rheum Dis. 2018;77:212-220.[3]Gordon JK, Martyanov V, Franks JM, et al. Belimumab for the Treatment of Early Diffuse Systemic Sclerosis: Results of a Randomized, Double-Blind, Placebo-Controlled, Pilot Trial. Arthritis Rheumatol. 2018 Feb;70(2):308-316.Figure 1.Panel A - Dynamics in mRSS between baseline and week 26 in the MTX group;Panel B- Dynamics in mRSS between baseline and week 26 in the TOF group.Figure 2.Panel A - Dynamics in skin thickness between baseline and week 26 in the MTX group; Panel B - Dynamics in skin thickness between baseline and week 26 in the TOF group.Disclosure of Interests:Rositsa Karalilova: None declared, Tanya Sapundzhieva: None declared, Zguro Batalov: None declared, Marco Matucci-Cerinic Grant/research support from: Actelion, MSD, Bristol-Myers Squibb, Speakers bureau: Acetelion, Lilly, Boehringer Ingelheim, Anastas Batalov: None declared

scholarly journals Reassessing the Role of the Active TGF-β1 as a Biomarker in Systemic Sclerosis: Association of Serum Levels with Clinical Manifestations

2016 ◽  
Vol 2016 ◽  
pp. 1-6 ◽  
Author(s):  
Andréa Tavares Dantas ◽  
Sayonara Maria Calado Gonçalves ◽  
Anderson Rodrigues de Almeida ◽  
Rafaela Silva Guimarães Gonçalves ◽  
Maria Clara Pinheiro Duarte Sampaio ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Clinical Manifestations ◽  
Serum Levels ◽  
Vascular Involvement ◽  
Digital Ulcers ◽  
Serum Samples ◽  
Peripheral Blood Mononuclear ◽  
Pbmc Culture ◽  
Significant Difference ◽  
Skin Biopsies

Objective. To determine active TGF-β1 (aTGF-β1) levels in serum, skin, and peripheral blood mononuclear cell (PBMC) culture supernatants and to understand their associations with clinical parameters in systemic sclerosis (SSc) patients.Methods. We evaluated serum samples from 56 SSc patients and 24 healthy controls (HC). In 20 SSc patients, we quantified spontaneous or anti-CD3/CD28 stimulated production of aTGF-β1 by PBMC. The aTGF-β1 levels were measured by ELISA. Skin biopsies were obtained from 13 SSc patients and six HC, and TGFB1 expression was analyzed by RT-PCR.Results. TGF-β1 serum levels were significantly higher in SSc patients than in HC (p< 0.0001). Patients with increased TGF-β1 serum levels were more likely to have diffuse subset (p= 0.02), digital ulcers (p= 0.02), lung fibrosis (p< 0.0001), positive antitopoisomerase I (p= 0.03), and higher modified Rodnan score (p= 0.046). Most of our culture supernatant samples had undetectable levels of TGF-β1. No significant difference in TGFB1 expression was observed in the SSc skin compared with HC skin.Conclusion. Raised active TGF-β1 serum levels and their association with clinical manifestations in scleroderma patients suggest that this cytokine could be a marker of fibrotic and vascular involvement in SSc.

scholarly journals AB0566 NAILFOLD EXTENT OF REDUCED CAPILLARY DENSITY IS ASSOCIATED WITH DIGITAL ULCERS AND WITH AN INCREASED RISK OF DIGITAL ULCERS IN SYSTEMIC SCLEROSIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1580.2-1580
Author(s):  
R. De Angelis ◽  
F. Salaffi
Keyword(s):  
Systemic Sclerosis ◽  
Laboratory Data ◽  
Quantitative Measure ◽  
Capillary Density ◽  
Optical Probe ◽  
Typical Feature ◽  
Slight Reduction ◽  
Digital Ulcers ◽  
Increased Risk ◽  
Significant Difference

Background:A growing evidence supports the role of microvasculopathy as a primary pathogenic event in systemic sclerosis (SSc). The most commonly used imaging technique to identify microangiopathy in SSc is high magnification videocapillaroscopy (NVC), and reduced capillary density and/or capillary loss, which is a typical feature of “scleroderma microangiopathy”, easily identified by NVC, has been associated with digital ulcers (DUs). Different approaches have been proposed to measure capillary density or capillary loss. Some of these were qualitative methods, others semi-quantitative, others only concerned a limited nailfold area, without ever evaluating the overall density, which is more suitable for quantitative estimate.Objectives:To assess the association between the extent of different values of nailfold capillary density and the presence of DUs and to identify the risk of developing DUs, based on quantitative parameters.Methods:The study involved 54 SSc selected patients (47 women and 7 men, mean age 59.5 years, 50 with limited and 4 with diffuse). The study population came from an ongoing database, that includes clinical and laboratory data of patients with definite SSc. A videocapillaroscope (VideoCap® 3.0, DS Medica, Milan, Italy) with a 200x optical probe was used. During examination, eight fingers (fingers 2–5 of each hand), 4 fields per finger, according to the standard literature were assessed. For each patient, a total of 32 images were collected, then classified as having either “normal”, “non-specific” or the “scleroderma pattern” (SP). Capillary density was defined as the number of capillaries/mm in the distal row, regardless of its shape and morphology. Avascular areas were defined by the absence of loops within a width/area extending over more than 500 microns. For each patient, the SP images were further graded with no/slight reduction of the capillary density (7-9 loops/mm) (NOR), with a well-defined reduction of capillary density (6-4 loops/mm) (RED) and with loss of capillaries (<4) plus avascular areas (AA). Then, the overall percentages were calculated (the number with SP, the number with NOR, with RED and with AA, respect to 32), thus obtaining the quantitative measures. All data were analysed using the MedCalc® version 18.6; 64-bit (MedCalc Software, Mariakerke, Belgium).Results:A total of 1728 images were analyzed. Patients with DUs were 16/54 (29.6%). All patients had a SP, but only five patients showed a SP along the entire nailfold. A comparison between patients with or without DUs showed a significant difference both for the overall extent of AA (p=0.032), and particularly for the overall extent of RED (p<0.001). No significant difference was found regarding the overall extent of the SP (p=0.085). Factor significantly associated with DUs in multivariate analysis was the overall extent of RED (p=0.0286). The ROC curve was very effective at discriminating the capillary feature able to distinguish patients with DUs from patients without DUs. The discriminatory power of the overall extent of RED was very good, with an AUC of 0.948 (95 % CI 0.852 ± 0.990). Then, we calculated the cut-off values of the overall extent of RED for presence/absence of DUs with the highest combination of sensitivity and specificity. The resulting cut-off value (Yourden index of 0.825) was >68.7 (sensitivity 92.31 %; specificity 90.24 %) with a LR+ of 9.46.Conclusion:Our data strongly support that the capillary density between 4 and 6 loops/mm is the best capillaroscopic quantitative measure associated with DUs and able to discriminate the probability of having DUs. If all SSc-specific antibodies and/or other laboratory/clinical parameters are not yet available, the overall capillary density can allow physicians to assess SSc patients easily, regarding DUs and risk for developing DUs.Disclosure of Interests:None declared

History of skin thickness assessment and the Rodnan skin thickness scoring method in systemic sclerosis

2019 ◽  
Vol 4 (2) ◽  
pp. 83-88 ◽  
Author(s):  
Thomas A Medsger ◽  
Thomas G Benedek
Keyword(s):  
Systemic Sclerosis ◽  
Gold Standard ◽  
Observational Studies ◽  
Skin Thickness ◽  
Scoring Method ◽  
Laboratory Techniques ◽  
Examination Method ◽  
History Of ◽  
Modified Rodnan Skin Score ◽  
Simple Bedside

The pathology of skin involvement in systemic sclerosis (or scleroderma) was first described in detail in 1892. In this article, we trace the history of cutaneous scleroderma and the evolution of thinking of scholars who have addressed this topic. We focus on skin histopathologic abnormalities and both clinical and laboratory techniques proposed for quantifying skin thickening and mobility. We examine the development of the simple bedside physical examination method of Dr Gerald Rodnan, first published in the 1970s and subsequently modified by others in the early 1990s (modified Rodnan skin score). This method has been found to be the only completely validated technique for assessing skin thickness in systemic sclerosis. Now nearly 50 years later, the modified Rodnan skin thickness scoring system remains the gold standard for use in both systemic sclerosis clinical trials and observational studies.

Management of Raynaud’s phenomenon in systemic sclerosis—a practical approach

2019 ◽  
Vol 4 (2) ◽  
pp. 102-110 ◽  
Author(s):  
Andreu Fernández-Codina ◽  
Esperanza Cañas-Ruano ◽  
Janet E Pope
Keyword(s):  
Systemic Sclerosis ◽  
Raynaud’S Phenomenon ◽  
Treatment Options ◽  
Raynaud's Phenomenon ◽  
Practical Approach ◽  
Digital Ulcers ◽  
Channel Blockers ◽  
Tissue Ischemia ◽  
Multiple Variables ◽  
Third Line

Raynaud’s phenomenon is nearly universal in systemic sclerosis. Vasculopathy is part of systemic sclerosis. Raynaud’s phenomenon can cause of complications and impairment, especially when tissue ischemia and digital ulcers develop. There are many treatment options for Raynaud’s phenomenon in systemic sclerosis often with sparse data and few robust studies comparing the different treatment options. Recommendations from guidelines usually include calcium channel blockers as first-line pharmacological treatment. In the clinical setting, multiple variables such as financial factors, geography where access to medications varies, and patient factors, baseline hypotension, can influence the treatment for Raynaud’s phenomenon and digital ulcers. Prostacyclins and PDE-5 inhibitors are reserved for more severe Raynaud’s phenomenon or healing of digital ulcers. Prevention of digital ulcers may also include endothelin receptor blocker (bosentan) in some countries. Other treatments had less consensus. Algorithms developed by systemic sclerosis experts might be helpful in deciding which treatment to choose for each setting, using a step-wise strategy, which intends to complement guidelines. This review focuses on a practical approach to the treatment of Raynaud’s phenomenon and digital ulcers in systemic sclerosis, based on algorithms designed by systemic sclerosis experts using consensus, and we review the evidence that supports treatment from initial to second and third-line options.

scholarly journals Evaluation of thyroid volume in patients with systemic sclerosis; what did we find?

2021 ◽  
Author(s):  
Suade BADAK ◽  
Bozkurt GÜLEK ◽  
Esra KAYACAN ERDOĞAN ◽  
Hülya BİNOKAY ◽  
Eren ERKEN
Keyword(s):  
Systemic Sclerosis ◽  
Severity Score ◽  
Thyroid Dysfunction ◽  
Disease Duration ◽  
Thyroid Volume ◽  
Significant Negative Correlation ◽  
Skin Thickness ◽  
Cross Sectional ◽  
Skin Score ◽  
Modified Rodnan Skin Score

Introduction: Systemic sclerosis is a multisystemic disease. Thyroid involvement in systemic sclerosis is an issue that can be ignored. Our study aimed to evaluate the decreased thyroid volume in SSc. Also, we aimed to show the relationship between patients’ thyroid volume and severity score, clinical and laboratory parameters. Method: This was a single-center, cross-sectional study. Eighty-eight patients were included in the study. A radiologist evaluated patients’ thyroid volumes by ultrasonography. Demographic and clinical characteristics of the patients were recorded. Skin thickness was evaluated by the modified Rodnan skin score and the disease severity by the Medsger severity score. Findings were analyzed statistically. Results: Thyroid volume was in the atrophic range in 53.4% of the patients. There was a significant negative correlation between thyroid volume and mRSS, MSS, and disease duration. Logistic regression analysis showed that modified Rodnan skin score and disease duration were risk factors for thyroid atrophy. Conclusions: Many studies point out that thyroid autoantibodies are a cause of thyroid dysfunction in patients with SSc. However, in most of these studies, thyroid volume was not evaluated. As a result of our study, we saw that the major cause of thyroid dysfunction in our SSc patients was thyroid atrophy. Also, we observed that thyroid atrophy was more common in patients with ILD. We would like to draw attention to the fact that thyroid dysfunction and volume changes increase with the disease’s duration and severity in systemic sclerosis.

scholarly journals Photoacoustic and High-Frequency Ultrasound Imaging of Systemic Sclerosis Patients

2020 ◽  
Author(s):  
Brigit E. Kersten ◽  
Khalid Daoudi ◽  
Cornelia H van den Ende ◽  
Frank H van den Hoogen ◽  
Chris L. de Korte ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Oxygen Saturation ◽  
High Frequency ◽  
Skin Thickness ◽  
Healthy Individuals ◽  
High Frequency Ultrasound ◽  
The Third ◽  
Significant Difference ◽  
Primary Raynaud’S Phenomenon ◽  
Frequency Ultrasound

Abstract Introduction: Systemic sclerosis starts with an early phase characterized by Raynauds phenomenon, puffy fingers/hands, autoantibodies and a scleroderma nailfold­microscopic pattern. Alterations in the nailfoldmicroscopic pattern are not evident in all early SSc patients. Photoacoustics(PA) and high-frequency ultrasound (HFUS) could fulfill this need. The former can measure oxygen saturation while the latter can measure skin thickening. We hypothesize that photoacoustics and high-frequency ultrasound can distinguish (early) SSc patients from individuals with primary Raynaud's phenomenon (PRP) by measuring oxygenation of the fingertip and skin thickening.Methods: We compared measurements of the third finger in (early)SSc patients to healthy and PRP individuals. The level of oxygenation and skin thickness were compared between groups. Nailfoldcapillaroscopy was performed on all subjects.Results: Thirty-one adult subjects participated in this study: twelve patients with SSc, 5 patients with early SSc, 5 volunteers with PR and 9 healthy controls. We found a significant difference in oxygen saturation between (early) SSc patients (80.8% ± 8.1 and 77,9% ± 10.5 ) and individuals with PRP (93.9% ± 1.1). Measurements of skin thickening showed a significant difference in (early) SSc patients compared to individuals with PRP (0.48 ± 0.06 mm and 0.51 ±0.16 mm vs. 0.27 ± 0.01 mm). There was no significant difference between healthy and PRP individuals in oxygenation or skin thickening.Conclusion: Photoacoustic and high-frequency ultrasound can distinguish between (early)SSc, PRP and healthy individuals in both oxygenation and skin thickening.

scholarly journals PATIENTS WITH SYSTEMIC SCLEROSIS ASSOCIATING OTHER AUTOIMMUNE DISEASES HAVE A MILDER DISEASE COURSE

2015 ◽  
Vol 24 (2) ◽  
pp. 114-117
Author(s):  
Alina Soare ◽  
◽  
Simona Pintilie ◽  
Ana Maria Gherghe ◽  
Alexandra Radu ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Autoimmune Diseases ◽  
Disease Duration ◽  
Control Group ◽  
Digital Ulcers ◽  
Chi Square ◽  
Study Enrollment ◽  
Chi Square Test ◽  
Modified Rodnan Skin Score

Background and objectives. Systemic sclerosis (SSc) is a multisystem connective tissue disease (CTD), being one of the most heterogeneous diseases of the spectrum of CTDs. It may associate other autoimmune diseases (AIDs), therefore in this study we aimed to evaluate the prevalence of other AIDs in a cohort of patients with SSc and to evaluate their prognosis in comparison to patients with SSc without this association. Patients and methods. We performed a retrospective study in patients with SSc satisfying the ACR 1980 or the ACR/EULAR 2013 criteria, who were evaluated between January 2005 and May 2014 in our SSc center. These patients were investigated according to the EUSTAR recommendations, including modified Rodnan Skin Score (mRSS), lung function tests (LFT), echocardiography etc. As a control group for evaluating the prevalence of the AIDs we used all patients with Rheumatoid Arthritis (RA) visiting our clinic between January-December 2005. Data of all RA patients were collected from the electronic database of the hospital. All AIDs mentioned in the discharge diagnoses were recorded. Between-group comparisons were made with the chi-square test for nominal variables and with the independent-sample t-test for numeric variables. Results. 144 patients with SSc were included: 88.8% females, 66.6% with the limited cutaneous subset of disease (lcSSc), mean age 53.9 ± 12.8 years, mean disease duration at study enrollment 5.1±10.5 years and a mean follow-up time of 3.7 ± 2.6 years. Prevalence of the AID in the SSc cohort was 19.4%, patients with lcSSc being more prone to associate other AID (p = 0.001). We noted a tendency towards less interstitial lung disease (p = 0.056) and less digital ulcers (p = 0.081) in patients with SSc and AIDs. Comparing skin involvement for each year of follow-up in patients with SSc with and without and AIDs we observed that the first tended to have lower and more stable values of the mRSS. Conclusion. Patients with SSc and AIDs tend to have a better outcome than the ones without this association. mRSS remains stable across the years in patients with SSc and AID, with lower values than the SSc patients.

scholarly journals Endothelin Receptor Antagonists for the Treatment of Raynaud's Phenomenon and Digital Ulcers in Systemic Sclerosis

2011 ◽  
Vol 2011 ◽  
pp. 1-7 ◽  
Author(s):  
Kait Arefiev ◽  
David F. Fiorentino ◽  
Lorinda Chung
Keyword(s):  
Systemic Sclerosis ◽  
Case Reports ◽  
Raynaud’S Phenomenon ◽  
Raynaud's Phenomenon ◽  
Digital Ulcers ◽  
Endothelin Receptor Antagonists ◽  
Endothelin Receptor ◽  
Significant Morbidity ◽  
Receptor Antagonists ◽  
Internal Organs

Systemic sclerosis is a connective tissue disease characterized by fibrosis of the skin, internal organs, and widespread vasculopathy. Raynaud's phenomenon and digital ulcers are vascular manifestations of this disease and cause significant morbidity. Current treatments are only moderately effective in reducing the severity of Raynaud's in a portion of patients and typically do not lead to substantial benefit in terms of the healing or prevention of digital ulcers. Several studies have evaluated the efficacy of targeting the vasoconstrictor endothelin-1 for the treatment of systemic sclerosis-associated vascular disease. The purpose of this paper is to summarize the published studies and case reports evaluating the efficacy of endothelin receptor antagonists in the treatment of Raynaud's phenomenon and digital ulcers associated with systemic sclerosis.

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