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Update of EULAR recommendations for the treatment of systemic sclerosis.
Kowal-Bielecka O, Fransen J, Avouac J, Becker M, Kulak A, Allanore Y, Distler O, Clements P, Cutolo M, Czirjak L, Damjanov N, Del Galdo F, Denton CP, Distler JHW, Foeldvari I, Figelstone K, Frerix M, Furst DE, Guiducci S, Hunzelmann N, Khanna D, Matucci-Cerinic M, Herrick AL, van den Hoogen F, van Laar JM, Riemekasten G, Silver R, Smith V, Sulli A, Tarner I, Tyndall A, Welling J, Wigley F, Valentini G, Walker UA, Zulian F, Müller-Ladner U; EUSTAR Coauthors. Kowal-Bielecka O, et al. Ann Rheum Dis. 2017 Aug;76(8):1327-1339. doi: 10.1136/annrheumdis-2016-209909. Epub 2016 Nov 9. Ann Rheum Dis. 2017. PMID: 27941129 Free article.
The procedure resulted in 16 recommendations being developed (instead of 14 in 2009) that address treatment of several SSc-related organ complications: Raynaud's phenomenon (RP), digital ulcers (DUs), pulmonary arterial hypertension (PAH), skin and lung disease, scleroderma
The procedure resulted in 16 recommendations being developed (instead of 14 in 2009) that address treatment of several SSc-related organ com …
Corticosteroids and the risk of scleroderma renal crisis: a systematic review.
Trang G, Steele R, Baron M, Hudson M. Trang G, et al. Rheumatol Int. 2012 Mar;32(3):645-53. doi: 10.1007/s00296-010-1697-6. Epub 2010 Dec 4. Rheumatol Int. 2012. PMID: 21132302 Review.
Scleroderma renal crisis (SRC) has been associated with the use of corticosteroids (CS) in retrospective studies. ...
Scleroderma renal crisis (SRC) has been associated with the use of corticosteroids (CS) in retrospective studies. ...
Angiotensin-converting enzyme inhibitors prior to scleroderma renal crisis in systemic sclerosis: A systematic review and meta-analysis.
Xiong A, Cao Y, Xiang Q, Song Z, Zhang Y, Zhou S, Qiang Y, Chen H, Hu Z, Cui H, Luo J, Wang Y, Yang Y, Yang M, Shuai S. Xiong A, et al. J Clin Pharm Ther. 2022 Jun;47(6):722-731. doi: 10.1111/jcpt.13621. Epub 2022 Mar 1. J Clin Pharm Ther. 2022. PMID: 35233779 Review.
WHAT IS KNOWN AND OBJECTIVE: Angiotensin-converting enzyme inhibitors (ACEIs) are widely used in the treatment of scleroderma renal crisis (SRC), and their use prior to the onset of SRC in patients with systemic sclerosis (SSc) has received wide attention in …
WHAT IS KNOWN AND OBJECTIVE: Angiotensin-converting enzyme inhibitors (ACEIs) are widely used in the treatment of scleroderma rena
Studying the Role of C5-Inhibition Therapy in Scleroderma Renal Crisis-Induced Thrombotic Microangiopathy - A Review of Literature.
Farrukh L, Steen VD, Shapiro L, Mehta S. Farrukh L, et al. Semin Arthritis Rheum. 2023 Dec;63:152256. doi: 10.1016/j.semarthrit.2023.152256. Epub 2023 Aug 22. Semin Arthritis Rheum. 2023. PMID: 37689027 Review.
BACKGROUND: The pathogenesis of scleroderma renal crisis (SRC) remains poorly understood but a growing body of evidence suggests that activation of the complement system may be involved in the disease. ...METHODS: A literature search was conducted from incept …
BACKGROUND: The pathogenesis of scleroderma renal crisis (SRC) remains poorly understood but a growing body of evidence …
Survival after lung transplantation in systemic sclerosis. A systematic review.
Khan IY, Singer LG, de Perrot M, Granton JT, Keshavjee S, Chau C, Kron A, Johnson SR. Khan IY, et al. Respir Med. 2013 Dec;107(12):2081-7. doi: 10.1016/j.rmed.2013.09.015. Epub 2013 Sep 26. Respir Med. 2013. PMID: 24113572 Free article. Review.
Causes of death included graft failure, infection, cardiac events, hemorrhagic stroke, respiratory failure, malignancy, pulmonary hypertension, complications of bronchiolitis obliterans syndrome, anesthetic complication, and scleroderma renal crisis. There we …
Causes of death included graft failure, infection, cardiac events, hemorrhagic stroke, respiratory failure, malignancy, pulmonary hypertensi …
Improvement with time of vascular outcomes in systemic sclerosis: a systematic review and meta-analysis study.
Hughes M, Zanatta E, Sandler RD, Avouac J, Allanore Y. Hughes M, et al. Rheumatology (Oxford). 2022 Jul 6;61(7):2755-2769. doi: 10.1093/rheumatology/keab850. Rheumatology (Oxford). 2022. PMID: 34791057
METHODS: We performed a systematic review and meta-analysis of the literature in PubMed 1950-2019 related to SSc-digital ulcers (DUs), pulmonary artery hypertension (PAH) and scleroderma renal crisis (SRC). We included full-text articles and extracted study c …
METHODS: We performed a systematic review and meta-analysis of the literature in PubMed 1950-2019 related to SSc-digital ulcers (DUs), pulmo …
Glucocorticoids in systemic sclerosis: weighing the benefits and risks - a systematic review.
Iudici M, van der Goes MC, Valentini G, Bijlsma JW. Iudici M, et al. Clin Exp Rheumatol. 2013 Mar-Apr;31(2 Suppl 76):157-65. Epub 2013 Jul 23. Clin Exp Rheumatol. 2013. PMID: 23910618 Review.
Most adverse events recorded were infections. Twenty-three cases of scleroderma renal crisis (SRC) have been reported, mainly in patients with early diffuse disease (n=10) or with anti-thymocyte treatment (n=10). ...
Most adverse events recorded were infections. Twenty-three cases of scleroderma renal crisis (SRC) have been reported, …
Risk of scleroderma according to the type of immune checkpoint inhibitors.
Terrier B, Humbert S, Preta LH, Delage L, Razanamahery J, Laurent-Roussel S, Mestiri R, Beaudeau L, Legendre P, Goupil F, Hadjadj J, Stolzenberg MC, Treluyer JM, Westeel V, Valnet-Rabier MB, Wislez M, Mouthon L, Chouchana L. Terrier B, et al. Autoimmun Rev. 2020 Aug;19(8):102596. doi: 10.1016/j.autrev.2020.102596. Epub 2020 Jun 12. Autoimmun Rev. 2020. PMID: 32540450
RESULTS: We identified two cases with underlying limited cutaneous SSc who presented a dramatic increase in the skin thickening following pembrolizumab, associated with scleroderma renal crisis in one case. In the literature, four cases of scleroderma and fou …
RESULTS: We identified two cases with underlying limited cutaneous SSc who presented a dramatic increase in the skin thickening following pe …
Survival and prognosis factors in systemic sclerosis: data of a French multicenter cohort, systematic review, and meta-analysis of the literature.
Pokeerbux MR, Giovannelli J, Dauchet L, Mouthon L, Agard C, Lega JC, Allanore Y, Jego P, Bienvenu B, Berthier S, Mekinian A, Hachulla E, Launay D. Pokeerbux MR, et al. Arthritis Res Ther. 2019 Apr 3;21(1):86. doi: 10.1186/s13075-019-1867-1. Arthritis Res Ther. 2019. PMID: 30944015 Free PMC article.

Overall SMR was 5.73 (95% CI 4.68-6.94). Age at diagnosis > 60 years, diffuse cutaneous SSc, scleroderma renal crisis, dyspnea, 6-min walking distance (6MWD), forced vital capacity < 70%, diffusing capacity of the lungs for carbon monoxide < 70%, pul

Overall SMR was 5.73 (95% CI 4.68-6.94). Age at diagnosis > 60 years, diffuse cutaneous SSc, scleroderma renal crisis
The 15% rule in scleroderma: the frequency of severe organ complications in systemic sclerosis. A systematic review.
Muangchan C; Canadian Scleroderma Research Group; Baron M, Pope J. Muangchan C, et al. J Rheumatol. 2013 Sep;40(9):1545-56. doi: 10.3899/jrheum.121380. Epub 2013 Jul 15. J Rheumatol. 2013. PMID: 23858045 Review.
Many severe complications in SSc occur about 15% of the time, including cardiac involvement (15%, 95% CI 6-24), diastolic dysfunction (16%, 95% CI 14-17), estimated pulmonary artery pressure > 40 mm Hg (18%, 95% CI 14-21), pulmonary arterial hypertension by right heart cathete …
Many severe complications in SSc occur about 15% of the time, including cardiac involvement (15%, 95% CI 6-24), diastolic dysfunction (16%, …