Systemic Sclerosis: a Modern View on the Pathogenesis, Clinic, Diagnosis and Treatment

##plugins.themes.bootstrap3.article.sidebar##

PDF (Русский)
Published: Jul 26, 2019
DOI: https://doi.org/10.30841/2307-5112.3.2019.178568
Keywords:
systemic sclerosis, pathogenesis, classification, clinical manifestations, diagnosis, treatment,

##plugins.themes.bootstrap3.article.main##

І. Ю. Головач
Feofaniya Clinical Hospital of State Management of Affairs of Ukraine, Ukraine
Є. Д. Єгудіна
State Establishment «Dnipropetrovsk Medical Academy of Health Ministry of Ukraine», Ukraine

Abstract

Systemic sclerosis or systemic scleroderma (SSD) is an immune-mediated rheumatic disease of the connective tissue, the main clinical manifestations of which are associated with fibrosis of the skin and internal organs and vasculopathy. SSD has high morbidity and mortality. Improved understanding of the pathogenetic constructs of systemic sclerosis and the introduction of new classification criteria have improved the management of the disease and its prognosis. Additionally, treatments for specific complications have emerged and a growing evidence base supports the use of immune suppression for the treatment of skin and lung fibrosis. Modern approaches to the treatment of some manifestations of this disease, such as sclerodermic renal crisis, pulmonary arterial hypertension, Raynaud’s phenomenon, digital ulceration and gastroesophageal reflux, have been proposed. However, the burden of non-lethal complications associated with SSD is significant and it turns out to be a difficult task for recognition and management by specialists. Here, we review the clinical features of systemic sclerosis and describe the best practice approaches for its management. A literature review is presented for family doctors in order to better identify the clinical signs that foreshadow the onset of the disease. Because early diagnosis of SSD, treatment monitoring, recognition of exacerbations and lethal complications by first-stage physicians can prevent or delay significant morbidity and mortality.

##plugins.themes.bootstrap3.article.details##

How to Cite
Головач, І. Ю., & Єгудіна, Є. Д. (2019). Systemic Sclerosis: a Modern View on the Pathogenesis, Clinic, Diagnosis and Treatment. Family Medicine, (3), 7–16. https://doi.org/10.30841/2307-5112.3.2019.178568
Issue
No. 3 (2019)
Section
Distance learning
Copyright (c) 2020 І. Ю. Головач, Є. Д. Єгудіна

Creative Commons License

This work is licensed under a Creative Commons Attribution 4.0 International License.

Authors retain the copyright and grant the journal the first publication of original scientific articles under the Creative Commons Attribution 4.0 International License, which allows others to distribute work with acknowledgment of authorship and first publication in this journal.

Author Biographies

І. Ю. Головач, Feofaniya Clinical Hospital of State Management of Affairs of Ukraine

Iryna Yu. Golovach,

Head of the Center of Reumatology

Є. Д. Єгудіна, State Establishment «Dnipropetrovsk Medical Academy of Health Ministry of Ukraine»

Yelizaveta D. Yehudina,

Department of Internal Medicine 3

References

Головач И.Ю., Чипко Т.М., Корбут Н.Н. Феномен Рейно и дигитальный язвы при системной склеродермии: вопросы патофизиология и менеджмента на современном этапе // Украинский ревматологический журнал. – 2017. – № 70 (4). – С. 15–23.

Головач И.Ю., Егудина Е.Д. Особенности поражения почек при системных заболеваниях соединительной ткани // Почки. – 2018. – Т. 7, № 4. – С. 275–290.

Головач І.Ю., Єгудіна Є.Д. Алгоритми лікування системної склеродермії за умов переважного ураження шкіри та суглобів, при синдромі Рейно та дигітальних виразках згідно з сучасними рекомендаціями // Боль. Суставы. Позвоночник. – 2018. – Т. 8, № 4. – С. 150–159.

Головач І.Ю., Єгудіна Є.Д. Склеродермічна ниркова криза: питання патогенезу, клініка та сучасні підходи до лікування // Почки. – 2018. – Т. 7, № 4. – С. 298–310.

Синяченко О.В., Егудина Е.Д., Микукстс В.Я., Ермолаева М.В. Ангиопатия при системной склеродермии // Український ревматологічний журнал. – 2017. – Т. 67, № 1. – С. 5–12.

Фадеенко Г.Д., Фролова-Романюк Э.Ю. Вторичные поражения желудка в практике врача-терапевта // Мистецтво лікування. – 2006. – № 8 (34). – С. 14–18.

Єгудіна Є.Д., Ханюков О.О., Головач И.Ю., Калашникова О.С. Легенева гіпертензія, асоційована із системними захворюваннями сполучної тканини: огляд літератури з акцентом на діагностичні аспекти /Артеріальна гіпертензія. – 2018. – Т. 6, № 62. – С. 30–42.

Alastal Y., Hammad T.A., Renno A., et al. Gastrointestinal manifestations associated with systemic sclerosis: results from the nationwide inpatient sample // Ann Gastroenterol. – 2017. – Vol. 30. – P. 498–503.

Asano Y., Jinnin M., Kawaguchi Y., et al. Diagnostic criteria, severity classification and guidelines of systemic sclerosis // J Dermatol. – 2018. – Vol. 45 (6). – P. 633–691.

Avouac J, Fransen J, Walker UA, et al. EUSTAR Group Preliminary criteria for the very early diagnosis of systemic sclerosis: results of a Delphi Consensus Study from EULAR Scleroderma Trials and Research Group // Ann Rheum Dis. – 2011. – Vol. 70 (3). – P. 476–481.

Avouac J, Huscher D, Furst DE, et al. EPOSS group Expert consensus for performing right heart catheterisation for suspected pulmonary arterial hypertension in systemic sclerosis: a Delphi consensus study with cluster analysis // Ann Rheum Dis. – 2013. – Vol. 20.

Avouac J, Walker UA, Hachulla E, et al. Joint and tendon involvement predict disease progression in systemic sclerosis: a EUSTAR prospective study // Ann Rheum Dis. – 2016. – Vol. 75 (1). – P. 103–9.

Denton C.P., Korn J.H. Digital ulceration and critical digital ischaemia in scleroderma // Scleroderma Care Res. – 2003. – Vol. 1. – P. 12–16.

Desbois A.C., Cacoub P. Systemic sclerosis: An update in 2016 // Autoimmun Rev. – 2016. – Vol. 15 (5). – P. 417–26.

Herrick A.L. Management of Raynaud’s phenomenon and digital ischemia. // Curr Rheumatol Rep. – 2013. – Vol. 15 (1). – P. 303.

Jimenez S.A. Endothelial to mesenchymal transition (EndoMT) in the pathogenesis of Systemic Sclerosis-associated pulmonary fibrosis and pulmonary arterial hypertension. Myth or reality? // Matrix biology: journal of the International Society for Matrix Biology. – 2016. – Vol. 1. – P. 51–55.

Kowal-Bielecka O., Landewe R., Avouac J., et al. EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR) // Ann Rheum Dis. – 2009. – Vol. 68. – P. 620–628.

Kumánovics G., Péntek M., Bae S., et al. Assessment of skin involvement in systemic sclerosis // Rheumatology (Oxford). – 2017. – Vol. 56 (5). – P. 53–66.

Kumar S., Singh J., Rattan S., et al. Review article: pathogenesis and clinical manifestations of gastrointestinal involvement in systemic sclerosis // Aliment Pharmacol Ther. – 2017. – Vol. 45. – P. 883–898.

Magno C.H., Matos N., Costa R., Takahashi F. Incidence and prevalence of systemic sclerosis in Campo Grande, State of Mato Grosso do Sul // Brazil rev bras reumatol. – 2017. –Vol. 57 (2). – P. 107–114.

Moore O.A., Goh N., Corte T. et al. Extent of disease on high-resolution computed tomography lung is a predictor of decline and mortality in systemic sclerosis-related interstitial lung disease // Rheumatology (Oxford). – 2013. – Vol. 52 (1). – P. 155–160.

Pattanaik D., Brown M., Postlethwaite B., Arnold E. Pathogenesis of Systemic Sclerosis // Postlethwaite Front Immunol. – 2015. – Vol. 6. – P. 72.

Ranque B., Bérezné A., Le-Guern V., et al. Myopathies related to systemic sclerosis: a case-control study of associated clinical and immunological features // Scand J Rheumatol. – 2010. – Vol. 39. – P. 498–505.

Shah A.A., Wigley F.M., Hummers L.K. Telangiectases in scleroderma: a potential clinical marker of pulmonary arterial hypertension // J Rheumatol. – 2010. – Vol. 37. – P. 98–104.

Sharif R., Mayes M.D., Nicassio P.M., et al. Determinants of work disability in patients with systemic sclerosis: a longitudinal study of the GENISOS cohort // Semin Arthritis Rheum. – 2011. – Vol. 41. – P. 38–47.

Schoenfeld S.R., Castelino F.V. Interstitial Lung Disease in Scleroderma // Rheum Dis Clin North Am. – 2015. – Vol. 41 (2). – P. 237–248.

Steen V.D., Medsger T.A. Changes in causes of death in systemic sclerosis, 1972–2002 // Ann Rheum Dis. – 2007. – Vol. 66. – P. 940–944.

Tyndall A.J., Bannert B., Vonk M., et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database // Ann Rheum Dis. – 2010. – Vol. 69. – P. 1809–1815.

Valentini G., Cuomo G., Abignano G., et al. Early systemic sclerosis assessment of clinical and preclinical organ involvement in patients with different disease features // Rheumatology. – 2011. – Vol. 50. – P. 317–323.

Van den Hoogen F., Khanna D., Fransen J. et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology. European League Against Rheumatism Collaborative Initiative // Ann Rheum Dis. – 2013. – Vol. 72 (11). – P. 1747–55.

Walker U.A., Tyndall A., Czirjak L., et al. Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group database // Ann Rheum Dis. – 2007. – Vol. 66. – P. 754–763.