What are the guideline sources, guidelines and references for Systemic sclerosis ?
Guideline sources The following summarized guidelines for the evaluation and management of systemic sclerosis (SSc) are prepared by our editorial team based on guidelines from the American College of Rheumatology (ACR 2023), the European League Against Rheumatism (EULAR 2022; 2017), the European Society of Cardiology (ESC/ERS 2022), the Canadian Cardiovascular Society (CCS/CTS 2020), the European Society for Vascular Medicine (ESVM 2017), and the French Society of Pulmonology (SPLF 2017). 1 2 3 4 5 6 7 8 9 10 10 11 Definition SSc is a systemic autoimmune disease characterized by diffuse fibrosis and vasculopathy of the skin and internal organs. 8 Epidemiology SSc is caused by a complex interplay of environmental factors that trigger an abnormal immune response in genetically predisposed individuals. 10 Pathophysiology The overall incidence and prevalence of SSc in the United States are 5.6 cases per 100,000 person-years and 13.5-18.4 cases per 100,000 persons, respectively. 9 Disease course https://web.pathway.md/diseases/recbfaLA9MujaNbUC 1/5 6/24/23, 3:14 PM Systemic sclerosis Pathway The resultant vascular injury and endothelial dysfunction lead to vasoconstriction, excessive deposition of extracellular matrix and collagen, hypertrophy of intimal and medial layers of small blood vessels, and endothelial cell apoptosis, resulting in clinical manifestations of Raynaud's phenomenon, digital ulcers, pulmonary arterial hypertension, renal failure, obstructive vasculopathy, and fibrosis of the skin and internal organs. 10 Prognosis and risk of recurrence The estimated 5-year and 10-year cumulative survival from the time of diagnosis is 74.9% and 62.5%, respectively. 11 Pathway Systemic sclerosis Chronic management Guidelines 1. Diagnostic investigations Screening for pulmonary hypertension: As per ESC 2022 guidelines, obtain an annual assessment of the risk of having pulmonary arterial hypertension in patients with SSc. B Consider obtaining an annual assessment of the risk of pulmonary arterial hypertension in patients with connective tissue disease with overlap features of SSc. B Show 5 more As per CCS 2020 guidelines, obtain annual echocardiography and measurement of lung diffusing capacity for CO to screen for pulmonary hypertension in all patients with SSc. B Autoantibody titers: consider obtaining antibodies specific for SSc (anti-centromere, anti- topoisomerase-1, anti-U3RNP) in patients with suspected pulmonary involvement or in case of detection of antinuclear antibodies, in the absence of a formal diagnosis. E Screening for chronic infections: screen for HBV infection in all patients eligible for treatment with conventional synthetic, biological, or targeted synthetic DMARDs, immunosuppressants, or corticosteroids (according to dose and duration). B Show 3 more 2. Medical management Management of skin disease: consider methotrexate for the treatment of skin manifestations in early diffuse SSc. B https://web.pathway.md/diseases/recbfaLA9MujaNbUC 2/5 6/24/23, 3:14 PM Systemic sclerosis Pathway Management of Raynaud's phenomenon: Consider dihydropiridine-type calcium antagonists, usually oral nifedipine, as first-line therapy for SSc-associated Raynaud's phenomenon. B Consider intravenous iloprost for severe SSc-associated Raynaud's phenomenon. B Updated evidence: BRASS In adult patients with systemic sclerosis-associated Raynaud's phenomenon, botulinum toxin was not superior to placebo with respect to reduction in the median number of daily Raynaud's phenomenon attacks at 4 weeks postinjection. Patricia Senet et al. Arthritis Rheumatol. 2023 Mar. Management of digital ulcerations: As per ESVM 2017 guidelines, consider performing surgery in patients with SSc-related digital ulceration. C As per EULAR 2017 guidelines, consider phosphodiesterase type 5 inhibitors to treat digital ulcerations in patients with SSc. B Show 2 more Management of pulmonary hypertension: Consider endothelin receptor antagonists, phosphodiesterase type 5 inhibitors or riociguat to treat SSc-associated pulmonary arterial hypertension. C Consider IV epoprostenol in patients with severe SSc-related pulmonary arterial hypertension. B Management of interstitial lung disease: consider cyclophosphamide in patients with SSc- related ILD, in particular for patients with progressive disease. B Management of gastrointestinal disease: administer PPIs for SSc-related gastro-esophageal reflux disease and for the prevention of esophageal ulcers and strictures. B Show 2 more Management of scleroderma renal crisis: Initiate ACEIs immediately in patients with scleroderma renal crisis. B Monitor BP and renal function carefully in patients with SSc being treated with corticosteroids. B 3. Therapeutic procedures Hematopoietic stem cell transplantation: consider HSCT for the treatment of selected patients with rapidly progressive SSc who are at risk of organ failure. B https://web.pathway.md/diseases/recbfaLA9MujaNbUC 3/5 6/24/23, 3:14 PM Systemic sclerosis Pathway 4. Preventative measures Routine immunizations: consider offering high-dose or adjuvanted influenza vaccination, rather than regular-dose influenza vaccination, in 65 years old patients with rheumatic or musculoskeletal diseases and in 18-65 years old patients with rheumatic or musculoskeletal diseases on immunosuppressive medications. C Show 17 more Prophylaxis for Pneumocystis jirovecii pneumonia: consider administering prophylaxis against P. jirovecii pneumonia in patients initiating high-dose corticosteroids, especially in combination with immunosuppressants and depending on the risk-benefit ratio. C References 1. Kowal-Bielecka O, Fransen J, Avouac J et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017 Aug;76 8 1327 1339. Open 2. George E Fragoulis, Elena Nikiphorou, Mrinalini Dey et al. 2022 EULAR recommendations for screening and prophylaxis of chronic and opportunistic infections in adults with autoimmune inflammatory rheumatic diseases. Ann Rheum Dis. 2022 Nov 3;ard-2022 223335. Open 3. Anne R Bass, Eliza Chakravarty, Elie A Akl et al. 2022 American College of Rheumatology Guideline for Vaccinations in Patients With Rheumatic and Musculoskeletal Diseases. Arthritis Rheumatol. 2023 Jan 4. Online ahead of print. Open 4. Belch J, Carlizza A, Carpentier PH et al. ESVM guidelines - the diagnosis and management of Raynaud's phenomenon. Vasa. 2017 Oct;46 6 413 423. Open 5. V Cottin, B Crestani, J Cadranel et al. French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis 2017 update. Full-length version. Rev Mal Respir. 2017 Oct;34 8 900 968. Open 6. Marc Humbert, Gabor Kovacs, Marius M Hoeper et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022 Oct 11;43 38 3618 3731. Open 7. Naushad Hirani, Nathan W Brunner, Ali Kapasi et al. Canadian Cardiovascular Society/Canadian Thoracic Society Position Statement on Pulmonary Hypertension. Can J Cardiol. 2020 Jul;36 7 977 992. Open 8. Denton CP, Khanna D. Systemic sclerosis. Lancet. 2017 Oct 7;390 10103 1685 1699. Open 9. Furst DE, Fernandes AW, Iorga SR et al. Epidemiology of systemic sclerosis in a large US managed care population. J Rheumatol. 2012 Apr;39 4 784 6. Open 10. Viswanath V, Phiske MM, Gopalani VV. Systemic sclerosis: current concepts in pathogenesis and therapeutic aspects of dermatological manifestations. Indian J Dermatol. 2013 Jul;58 4 255 68. Open 11. Rubio-Rivas M, Royo C, Simeon CP et al. Mortality and survival in systemic sclerosis: systematic review and meta-analysis. Semin Arthritis Rheum. 2014 Oct;44 2 208 19. Open 12. R Knobler, P Moinzadeh, N Hunzelmann et al. European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1 localized scleroderma, systemic sclerosis and overlap syndromes. J Eur Acad Dermatol Venereol. 2017 Sep;31 9 1401 1424. Open https://web.pathway.md/diseases/recbfaLA9MujaNbUC 4/5 6/24/23, 3:14 PM Systemic sclerosis Pathway 13. Anand Padmanabhan, Laura Connelly-Smith, Nicole Aqui et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The Eighth Special Issue. J Clin Apher. 2019 Jun;34 3 171 354. Open 14. Patricia Senet, Herv Maillard, Elisabeth Diot et al. Efficacy and Safety of Botulinum Toxin in Adults with Raynaud's Phenomenon Secondary to Systemic Sclerosis: A Multicenter, Randomized, Double-Blind, Placebo-Controlled Study. Arthritis Rheumatol. 2023 Mar;75 3 459 467. 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