Publication of the article:
«Bulletin of problems biology and medicine», 2021 Issue 4, 162,
SYSTEMIC SCLERODERMIA. LITERATURE REVIEW
About the author:
Koniushevska A. A., Parkhomenko T. A., Sydorenko N. V., Vaizer N. V., Balychevtseva I. V.
Heading:
LITERATURE REVIEWS
Type of article:
Scentific article
Annotation:
The article is devoted to the current state of the problem of systemic scleroderma in children. A historical insight into the concept of the disease and its definitions is made. A literary review of the modern concept of epidemiology, etiology, pathogenetic variants, immunogenetic markers and predictors of variants of the course, classification, course, clinic of systemic scleroderma in children is presented. . It is emphasized that the early symptoms of Juvenile Systemic Sclerodermia may be nonspecific – unexplained fever, fatigue, arthralgia, myalgia, muscle weakness, unexplained abdominal pain, Raynaud’s phenomenon, which creates difficulties in diagnosing the disease. The modern criteria for the diagnosis of systemic scleroderma and its clinical variants are presented. The latest data on the possibility of treating the disease are highlighted in detail: immunosuppressive, anti-inflammatory, antifibrоtic, vascular therapy, as well as local, symptomatic therapy and rehabilitation methods. Particular attention is paid to the latest international experience in the use of genetically engineered biological drugs in the treatment of systemic scleroderma in children – tocilizumab, abatacept and genetically engineered monoclonal antibody to the B-lymphocyte antigen CD20 – rituximab. The prospects for the use of the tyrosine kinase inhibitor – imatinib mesylate, abituzumab, fresolimumab, as well as the role of autologous stem cell transplantation are discussed. It is emphasized that the earliest possible diagnosis and initiation of treatment at an early stage of the disease can prevent the development of fibrosis and complications, severe angiopathy, visceropathy, which determine the prognosis. It is advisable to prescribe an aggressive therapy in the early stages of development of Juvenile Systemic Sclerodermia to prevent the progression of the pathological process. It is concluded that Systemic Sclerodermia is a serious pathology, especially in children and requires further in-depth study of this problem, optimization of the diagnostic algorithm and individual approach to treatment.
Tags:
systemic scleroderma, children, diagnosis, treatment.
Bibliography:
- Kiseleva LP, Savvo VM, Zajceva EN. Juvenil’naja sklerodermija – mnogolikost’ kozhnyh projavlenij. Dermatologіja ta venerologіja. 2014;2(64):45-52. [in Ukrainian].
- Anan’eva LP, Aleksandrova EN. Autoantitela pri sistemnoj sklerodermii: spektr, klinicheskie associacii i prognosticheskoe znachenie. Nauch-praktich revmatol. 2016;54(1):86-99. DOI: 10.14412/1995-4484-2016-86-99. [in Russian].
- Baranov AA, Alekseeva EI, redaktory. Klinicheskie rekomendacii dlja pediatrov. Detskaja revmatologija. Revmaticheskie bolezni u detej. Moskva: Sojuz pediatrov Rossii; 2016. 144 s. [in Russian].
- Marushko TV, Taranenko TV. Juvenіl’na sistemna sklerodermіja: optimіzacіja dіagnostiki ta lіkuvannja na suchasnomu etapі. V: VoronenkoYuV, redaktor. Zb. nauk. prac. spіvrobіt. NMAPO іmenі P. L. Shupika; 2017; Kyiv:TOV “7BC”; 2017. s. 378-83. [in Ukrainian].
- Burluckaja AV, Savel’eva NV, Statova AV. Sluchaj debjuta sistemnoj sklerodermii u pacientki 10 let. Kubanskij nauchnyj medicinskij vestnik. 2018;25(3):162-6. DOI: 10.25207/1608-6228-2018-25-3-162-166. [in Russian].
- Capusan I. Scleroderma or scleroedema? Comments on the case published in 1753 by Dr. Carlo Curzio of Naples. G Ital Dermatol Minerva Dermatol. 1971;46(1):40-7.
- Aslan А, Koturoglu G, Sozeri B, Kurugol Z. Systemic Scleroderma in Childhood: A Case Report Arch Rheumatol. 2014;29(3):219-23 DOI: 10.5606/ArchRheumatol.2014.3684.
- Mikuksts VJa, Sinjachenko OV, Chernyshova OE. Juvenil’naja sistemnaja sklerodermija i jekologija (obzor literatury i sobstvennye issledovanija). Zdorov’e rebenka. 2015;2(1):63-7. DOI: 10.22141/2224-0551.12.1.2017. 95028. [in Ukrainian].
- Petty RE, Laxer RM, Lindsley CB, Wedderburn LR, (eds). Textbook of Pediatric Rheumatology. 7th ed. Elsevier, Philadelphia; 2016. 384 p.
- Svіncіc’kij AS. Suchasnі zasadi dіagnostiki ta lіkuvannja sistemnoї sklerodermії. Kardіologіja, Revmatologіja, Kardіohіrurgіja. 2014;6(37):57- 9. [in Ukrainian].
- Zulian F. Systemic manifestations in localized scleroderma. Current Rheumatology Reports. 2004;6(6):417-24.
- Alekseeva EI, Valieva SI, Bzarova TM, Isaeva KB, Denisova RV, Slepcova TV, et al. Opyt primenenija rituksimaba u bol’nogo juvenil’noj sklerodermiej. Vopr. sovr. pediatrii. 2012;1(3):131-7. [in Russian].
- Osminina MK, Geppe NA. Voprosy klassifikacii, klinicheskaja kartina i bazisnaja terapija juvenil’noj sklerodermii. Nauch-prakt revmatol. 2015;53(2):214-9. DOI: 10.14412/1995-4484-2015-214-219.
- Oshljans’ka OA, Arcimovich AG, Pіljugіna BV, Nadtochіj TG. Dosvіd zastomuvannja tocilizumabu u hvorih na sistemnu sklerodermіju u dіtej. Ukr Ravm Zhurnal. 2020;3(81):70-80. DOI: 10.32471/rheumatology.2707-6970. 81.15535. [in Ukrainian].
- Anan’eva LP. Rannjaja sistemnaja sklerodermija – sovremennyj algoritm diagnostiki (lekcija). Nauch-praktich revmatol. 2012;51(2):87-93. [in Russian].
- Bajgabulova MS, Abdikadirova ZhB, Bulakbaeva AM, Dauletkeldi A, Makenbaj A, Bajsultanova AE. Osobennosti klinicheskih variantov sklerodermii u detej. Vestnik KazNMU. 2014;4:88-9. Dostupno: www.kaznmu.kz. [in Russian].
- Barnes J, Mayes MD. Epidemiology of systemic sclerosis: incidence, prevalence, survival, risk factors, malignancy, and environmental triggers. Curr Opin Rheumatol. 2012;24(2):165-70. DOI: 10.1097/BOR.0b013e 32834ff2e8.
- Anan’eva LP, Starovojtova MN, Shabanova SSh. Osnovnye itogi kooperacii mezhdu FGBNU NIIR im. V.A. Nasonovoj i nauchnymi centrami stran Evropy v ramkah EUSTAR (EULAR Scleroderma Trail and Research group) po probleme «Sistemnaja sklerodermija». Nauch-prakt revmatol. 2014;52(6):682-8. DOI: org/10.14412/1995-4484-2014-682-688. [in Russian].
- Mukvіch OM. Juvenіl’na sistemna sklerodermіja: dіagnostika, lіkuvannja, profіlaktika. Chastina 1. Zdorov’ja Ukraїni (Med. gazeta). 2021;1(57):30-2. Dostupno: www.health-ua.com. [in Ukrainian].
- Podlianova EI. Sistemnaja sklerodermija u detej: dva klinicheskih sluchaja. Vіsnik problem bіologії і medicini. 2015;2.3(120):216-21. [in Ukrainian].
- Marushko TV. Juvenіl’na sistemna sklerodermіja. Zdorov’ja Ukraїni (Med. gazeta). 2019;1(48):42-4. Dostupno: www.health-ua.com. [in Ukrainian].
- Herrick AL, Ennis H, Bhushan M, Silman AJ, Baildam EM. Incidence of childhood linear scleroderma and systemic sclerosis in the UK and Irelands. Arthritis Care Res (Hoboken). 2010;62(2):213-8. DOI: 10.1002/acr.20070.
- Gaikwad RP, Samir MS. Juvenile systemic sclerosis in 11-year-old male child responding to methotrexate. Indian J. Paediatr. Dermatol. 2016;17:156-8.
- Murdaca G, Contatore M, Gulli R, Mandich P, Puppo F.Genetic factors and systemic sclerosis. Autoimmun Rev. 2016;15(5):427-32. DOI: 10.1016/j. autrev.2016.01.016.
- Marie I, Gehanno JF. Environmental risk factors of systemic sclerosis. Semin Immunopathol. 2015;37(5):463-73. DOI: 10.1007/ s00281- 015-0507-3.
- Wei P, Yang Y, Guo X. Identification of an association of TNFAIP3 polymorphisms with matrix metalloproteinase expression in fibroblasts in an integrative study of systemic sclerosis-associated genetic and environmental factors. Arthritis Rheumatol. 2016;68(3):749-60. DOI: 10.1002/art.39476.
- Rubio-Rivas M, Moreno R, Corbella X. Occupational and environmental scleroderma. Systematic review and meta-analysis. Clin. Rheumatol. 2017;14(1):223-9. DOI: 10.1007/s10067-016-3533-1.
- Anan’eva LP. Novye klassifikacionnye kriterii sistemnoj sklerodermii (lekcija). Nauch-prakt revmatol. 2013;51(5):539-44. DOI: org/10.14412/ 1995-4484-2013-1546. [in Russian].
- Martini G, Foelvari I, Russo R, Cuttica R, Eberhard A, Ravelli A, et al. Systemic sclerosis in childhood: clinical and immunologic features of 153 patients. Arthritis Rheum. 2006;54(12): 3971-8. DOI: 10.1002/art.22207.
- Wirz EG, Jaeger VK, Allanore Y, Riemekasten G, Hachulla E, Distler O, et al. Incidence and predictors of cutaneous manifestations during the early course of systemic sclerosis: a 10-year longitudinal study from the EUSTAR database. Ann Rheum Dis. 2016;75:1285-92.
- Mukvіch OM. Juvenіl’na sistemna sklerodermіja: dіagnostika, lіkuvannja, profіlaktika. Chastina 2. Zdorov’ja Ukraїni (Med. gazeta); 2021;2 (58):32-4. Dostupno: www.health-ua.com. [in Ukrainian].
- Zlatanovic M, Tadic M, Celic V, Ivanovic B, Stevanovic A, Damjanov N. Cardiac mechanics and heart rate variability in patients with systemic sclerosis: the association that should not miss. Rheumatol Int. 2017 Jan;37(1):49-57. DOI: 10.1007/s00296-016-3618-9.
- Alekperov RT. Sindrom Rejno kak mul’tidisciplinarnaja problema. Al’manah klinicheskoj mediciny. 2014;35:94-100. [in Russian].
- Sinjachenko OV, Egudina ED, Mikuksts VJa, Ermolaeva MV, Keting EV, Djadyk EA. Angiopatija pri sistemnoj sklerodermii. Ukr revmatol zhurnal. 2017;67(1):5-11. [in Ukrainian].
- Golovach IJu, Chipko TM, Korbu NN. Fenomen Rejno, digital’nye jazvy i kriticheskaja ishemija pri sistemnoj sklerodermii: sovremennye vzgljady na problemu i menedzhment. Kardіologіja, Revmatologіja, Kardіohіrurgіja. 2017;3(52):39-41. [in Ukrainian].
- Hughes M, Herrick AL. Digital ulcers in systemic sclerosis. Rheumatology (Oxford). 2017;56(1):14-25. DOI:10.1093/rheumatology/kew047.
- Faludi R, Kö lto G, Bartos B, Csima G, Czirják L, Komócsi A. Five-year follow-up of left ventricular diastolic function in systemic sclerosis patients: determinants of mortality and disease progression. Semin Arthritis Rheum. 2014 Oct;44(2):220-7. DOI: 10.1016/j. semarthrit.2014.04.001.
- Saad EO, Anan’eva LP, Alekperov RT, Novikova DS, Korsakova JuO, Volkov AV. Strukturnye i funkcional’nye izmenenija po dannym jehokardiografii bol’nyh cistemnoj sklerodermiej i variabel’nost’ ritma serdca. Nauch-prakt revmatol. 2017;55(1):32-6. DOI: org/10.14412/1995-4484-2017-32-36. [in Russian].
- Giacomelli R, Liakouli V, Berardicurti O, Ruscitti P, Di Benedetto P, Carubbi F, et al. Interstitial lung disease in systemic sclerosis: current and future treatment. Rheumatol Int. 2017;6(1):142-8. DOI: 10.1007/s00296-016-3636-7.
- Denton CP, Hughes M, Gak N, Vila J, Buch MH, Chakravarty K, et al. Ong on behalf of the BSR and BHPR Standards, Guidelines and Audit Working Group. BSR and GHPR guideline for the treatment of systemic sclerosis. Rheumatology (Oxford). 2016;55:1906-10. DOI: 10.1093/rheumatology/ kew224.
- Anan’eva LP. Diagnostika i lechenie intersticial’nogo porazhenija legkih pri sistemnoj sklerodermii. Sovr revmatol. 2018;12(2):12-21. DOI: 10/14412/1996-7012-2018-2-12-21. [in Russian].
- Anan’eva LP, Koneva OA, Desinova OV. Vlijanie rituksimaba na projavlenija aktivnosti i legochnuju funkciju u bol’nyh sistemnoj sklerodermiej: ocenka posle goda nabljudenija. Nauch-prakt revmatol. 2019;57(3):265-73. [in Russian].
- Vojcehovskij VV, Goborov ND, Pogrebnaja MV, Gorjacheva SA, Fomina OP, Prihod’ko OB, et al. Porazhenie legkih pri sistemnoj sklerodermii. Bulletin Physiology and Pathology of Respiration. 2018;1(70):83-92. DOI: 10.12737/article_5c12717d12a437.30758301. [in Russian].
- Castellvi I, Simeon-Aznar CP, Sarmiento M, Fortuna A, Mayos M, Geli C, et.al. Association between nailfold capillaroscopy findings and pulmonary function tests in patients with systemic sclerosis. J Rheumatol. 2015 Feb 1;42(2):222-7. DOI: 10.3899/jrheum.140276.
- Kljaus NA, Simakova MA, Masljanskij AL, Moiseeva OM. Poisk klinicheskih prediktorov legochnoj gipertenzii u pacientov s sistemnoj sklerodermiej. Nauch-prakt revmatol. 2018;56(5):586-590. DOI: 10.14412/ 1995-4484-2018-586-590. [in Russian].
- Penin IN, Masljanskij AL, Novikova AN, Ilivanova EP, Konradi AO, Mazurov VI. Osobennosti porazhenija mikrocirkuljatornogo rusla pri sistemnoj sklerodermii. Transljacionnaja medicina. 2016;3(2):54-60. [in Russian].
- Karateev AE, Movsisjan MM, Anan’eva LP, Radenska-Lopovok SG. Patologija pishhevoda pri sistemnoj sklerodermii: dannye klinikojendoskopicheskogo obsledovanija. Nauch-prakt revmatol. 2012;50(1):54-9. DOI: https://doi.org/10.14412/1995-4484-2012-505. [in Russian].
- Avouac J, Fransen J, Walker UA, Riccieri V, Smith V, Muller C, et al. 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;70(3):476-81. DOI: http://dx.doi. org/10.1136/ard.2010.136929.
- Van den Hoogen F, Khanna D, Fransen J. 2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative. Ann Rheum Dis. 2013;72(11):1747-55. DOI: 10.1136/annrheumdis-2013-204424.
- Alhajeri H, Hudson M, Fritzler M. 2013 ACR/EULAR Classification Criteria for Systemic Sclerosis Out-perform the 1980 Criteria. Data from the Canadian Scleroderma Research Group. Arthritis Care Res (Hoboken). 2015 Apr;67(4):582-7. DOI: 10.1002/acr.22451.
- Koneva OA, Ovsjannikova OB, Starovojtova MN, Cherjomuhina EO, Aleksandrova EN, Anan’eva LP. Opredelenie chuvstvitel’nosti novyh kriteriev sistemnoj sklerodermii na rossijskoj populjacii pacientov. Nauch-prakt revmatol. 2015;53(4):361-6 DOI: 10.14412/1995-4484- 2015-361-366. [in Russian].
- Jordan S, Maurer B, Toniolo M, Michel B, Distler O. Performance of the new ACR/EULAR classification criteria for systemic sclerosis in clinical practice.Rheumatology (Oxford). 2015;54(8):1454-8. DOI: 10.1093/ rheumatology/keu530.
- Starovojtova MN, Desinova OV, Koneva OA, Ovsjannikova OB, Alekperov RT, Cherkasova MV, et al. Profil’ autoantitel pri sistemnoj sklerodermii. Nauch-prakt revmatol. 2016;54(4):418-23. DOI: org/10.14412/1995-4484-2016-418-423. [in Russian].
- Hamaguchi Y. Autoantibody profiles in systemic sclerosis: Predictive value for clinical evaluation and prognosis. J Dermatol. 2010;37:42- 53. DOI: 10.1111/j.1346-8138.2009.00762.x.
- Mierau R, Moinzadeh P, Riemekasten G, Melchers I, Meurer M, Reichenberger F, et al. Frequency of disease-associated and other nuclear autoantibodies in patients of the German network for systemic scleroderma: correlation with characteristic clinical features. Arthritis Res Ther. 2011;13(5):R172. DOI: 10.1186/ar3495.
- Salazar GA, Assassi S, Wigley F, Hummers L, Varga J, Hinchclif M, et al. Antinuclear antibody-negative systemic sclerosis. Semin Arthritis Rheum. 2015 Jun;44(6):680-6. DOI: 10.1016/j.semarthrit.2014.11.006.
- Hudson M, Satoh M, Chan JY, Tatibouet S, Mehra S, Baron M, et al. Canadian Scleroderma Research Group. Prevalence and clinical profiles of «autoantibody-negative» systemic sclerosis subjects. Clin Exp Rheumatol. 2014;32(86):127-32. DOI: 32(6 Suppl 86):S127-32.
- Tanahashi K, Sugiura K, Muro Y, Akiyama M. Disappearance of circulating autoantibodies to RNA polymerase III in a patient with systemic sclerosis successfully treated with corticosteroid and methotrexate. J Eur Acad Dermatol Venereol. 2015 Jul;29(7):1453-4. DOI: 10.1111/ jdv.12512.
- Silva I, Teixeira A, Oliveira J, Almeida I, Almeida R, Águas A, et al. Endothelial dysfunction and nailfold videocapillaroscopy pattern as predictors of digital ulcers in systemic sclerosis: a cohort study and review of the literature. Clinic Rev Allerg Immunol. 2015;49:240-52.
- Kowal-Bielecka O, Fransen J, Avouac J. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017;76(8):1327-133. DOI: 10.1136/annrheumdis-2016-209909.
- Fernández-Codina A, Walker KM, Pope JE, Scleroderma Algorithm Group. Treatment algorithms for systemic sclerosis according to experts. Arthritis Rheumatol. 2018;70:1820-182. DOI: 10.1002/art.40560.
- Foeldvari I. Update on the Systemic Treatment of Pediatric Localized Scleroderma. Pediatric Drugs. 2019;21:461-7. DOI.org/10.1007/ s40272-019-00363-5.
- Anan’eva LP. Sovremennaja terapija intersticial’nyh pnevmonij, associirovannyh s sistemnoj sklerodermiej. Nauch-prakt revmatol. 2020;58(5):520-31. DOI: 10.47360/1995-4484-2020-520-531. [in Russian].
- Khanna D, Denton CP, Jahreis A. Safety and efficacy of subcutaneous tocilizumab in adults with systemic sclerosis (faCCcinate): a phase 2, randomized, controlled trail. Lancet. 2016 Jun 25;387(10038):2630-40. DOI: 10.1016/S0140-6736(16)00232-4.
- Khanna D, Denton CP, Jahreis A, van Laar JM, Frech TM, Anderson ME, 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;77:212-20. DOI: org/10.1136/ annrheumdis-2017-211682.
- Lythgoe H, Baildam E, Beresford MW, Cleary G, McCann LJ, Pain CE. Tocilizumab as a potentialtherapeutic option for children with severe, refractory juvenile localized scleroderma. Rheumatology (Oxford). 2018;57:398-401.
- Koneva OA, Desinova OV, Anan’eva LP, Kovaleva NV. Znachenie komp’juternoj tomografii vysokogo razreshenija v ocenke jeffektivnosti rituksimaba u pacientov s intersticial’nym porazheniem legkih pri sistemnoj sklerodermii. Nauchno-prakticheskaja revmatologija. 2018;56(5):591-9. DOI: 10.14412/1995-4484-2018-591-599. [in Russian].
- Ovsjannikova OB, Anan’eva LP, Koneva OA, Garzanova LA, Shajahmetova RU, Desinova OV, et al. Transplantacija autologichnyh gemopojeticheskih stvolovyh kletok v lechenii sistemnoj sklerodermii. Chast’ 1. Klinicheskie aspekty. Sovremennaja revmatologija. 2020;14(4):91-7. DOI: 10.14412/ 1996-7012-2020-4-91-97. [in Russian].
- Jacob B, Vaan Laar JM. Stem cell transplantation – all scientific questions answered? Ann Rheum Dis. 2019;78(2):A33.
- Host L, Nikpour M, Calderone A, Cannell P, Roddy J. Autologous stem cell transplantation in systemic sclerosis: a systemic review. Clin Exp Rheumatol. 2017;35(106):198-207.
- Sullivan KM, Goldmuntz EA, Keyes-Elstein L. Myeloablative Autologous Stem-Cell Transplantation for Severe Scleroderma. N Engl J Med. 2018;378:35.
- Herrick AL, Pan X, Peytrignet S, Lunt M, Hesselstrand R, Mouthon L, et.al. Treatment outcome in early diffuse cutaneous systemic sclerosis: the European Scleroderma Observational Study (ESOS). Ann Rheum Dis. 2017;76:1207. DOI: 10.1136/annrheumdis-2016-210503.
Publication of the article:
«Bulletin of problems biology and medicine» Issue 4 (162), 2021 year, 29-39 pages, index UDK 616.5-004-092(048.8)
DOI:
10.29254/2077-4214-2021-4-162-29-39