CLINICAL CASE: RARE COURSE OF JUVENILE SCLERODERMA IN RESIDENTS OF DONETSK REGION

Keywords: systemic scleroderma, children, clinic, diagnosis, treatment

Abstract

The article presents a clinical case of a rare onset and a special clinical course of juvenile scleroderma. A clinical case of a child who was born and lives in the ecologically unfavorable industrial Donetsk region is described.

The literature review of influence of unfavorable environment on morbidity and features of autoimmune pathology course in patients of Donetsk region is offered. In particular, the literature indicates that negative environmental factors lead to an increase in the progression of systemic scleroderma; over the past 20 years, there has been a tendency to increased mortality of children suffering from it, which is associated with deteriorating environmental conditions in industrial regions. The growing incidence and prevalence of scleroderma, the variety of clinical manifestations, and difficulties in early diagnosis of the disease make it important to study the options for the course of this pathology in children and adolescents in the early stages of the disease.

A feature of this clinical case was the onset of juvenile scleroderma with severe convulsive syndrome at the age of 8 years, which required Finlepsin at a dose of 200 mg daily. Further course was also uncharacteristic: within 2 years, there was a linear indentation in the forehead on the right side; the patient was diagnosed with linear "saber-shaped" limited scleroderma, Parry–Romberg facial hemiatrophy syndrome, and further developed manifestations of systemic involvement, lesions of internal organs: pneumofibrosis and scleroderma esophagitis. The management and observation of the patient are presented here. It was emphasized that early aggressive intervention led to the prevention of severe organ pathology and death.

The described clinical case expands the knowledge of physicians on the clinical polymorphism of the onset and course of the disease, which allows faster and more accurate identification of the disease, timely and adequate therapy, and will lead to earlier stabilization and remission of the disease.

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References

Sinjachenko OV, Petrenko EA, Naumenko NV. [Zavisimost' techenija revmatoidnogo artrita ot jekologii atmosfery regionov prozhivanija]. Bol'. Sustavy. Pozvonochnik. 2011;4(04):

Sinjachenko OV [Bolezni sustavov i jekologija]. Novosti mediciny i farmacei. 2012;12(422). http://www.mif-ua.com›archive›article.

Shapira Y, Agmon-Levin N, Shoenfeld Y. Geoepidemiology of autoimmune rheumatic diseases. Nat. Rev. Rheumatol2010;6(8):468-76.

Toboіn GJ, Youinou P, Saraux A. The environment, geo-epidemiology, and autoimmune disease: Rheumatoid arthritis.2010;35(1):10-4.

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.

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.

Rubio-Rivas M, Moreno R, Corbella X. Occupational and environmental scleroderma. Systematic review and meta-analysis. Clin. Rheumatol. 2017;14(1):223-9.

Anan'eva LP. [New classification criteria for systemic scleroderma (lecture)]. Nauch-prakt revmatol. 2013;51(5):539–44.

Zulian F, Athreya BH, Laxer R, et al. Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. Rheumatology. 2006;45(5):614-20.

Trenkmann M, Brock M, Gay S. Epigenetics in rheumatoid arthritis. Clin. Rev. Allergy Immunol. 2010;39 (1):10-9.

Adams DD, Knight JG, Ebringer A. Autoimmune diseases: Solution of the environmental, immunological and genetic components with principles for immunotherapy and transplantation. Autoimmun. Rev.2010;9 (8):525-30. Epub 2010 Jan 18.

Marie I, Gehanno JF. Environmental risk factors of systemic sclerosis. Semin Immunopathol. 2015;37(5):463-73..

York MR. Novel insights on the role of the innate immune system in systemic sclerosis. Expert Rev Clin Immunol. 2011;7(4):481-9.

Kerneis S, Bolle PY, Grais RF, et al. Mortality trends in systemic sclerosis in France and USA, 1980-1998: an age-period-cohort analysis. Eur J Epidemiol. 2010;25(1):55-61.

Kiseleva LP, Savvo VM, Zajceva EN. [Juvenil'naja sklerodermija - mnogolikost' kozhnyh projavlenij]. Dermatologіja ta venerologіja. 2014;2(64):45-52.

Anan'eva LP, Aleksandrova EN. [Autoantitela pri sistemnoj sklerodermii: spektr, klinicheskie associacii i prognosticheskoe znachenie]. Nauch-praktich revmatol. 2016;54(1):86-99.

Baranov AA, Alekseeva EI. Detskaja revmatologija. Revmaticheskie bolezni u detej. Klinicheskie rekomendacii dlja pediatrov. [Pediatric rheumatology. Rheumatic diseases in children. Clinical guidelines for pediatricians.]. Moskva: Sojuz pediatrov Rossii Publ., 2016. 144 p.

Marushko TV, Taranenko TV. [Juvenіl'na sistemna sklerodermіja: optimіzacіja dіagnostiki ta lіkuvannja na suchasnomu etapі]. Zb. nauk. prac. spіvrobіt. NMAPO іmenі P. L. Shupika, vipusk 28; Kyiv:TOV "7BC" Publ., 2017, pp. 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.

Burluckaja AV, Savel'eva NV, Statova AV. [Sluchaj debjuta sistemnoj sklerodermii u pacientki 10 let. Kubanskij nauchnyj medicinskij vestnik]. 2018;25(3):162-6.

Alekseeva EI, Valieva SI, Bzarova TM, Isaeva KB, Denisova RV, Slepcova TV, i dr. [Opyt primenenija rituksimaba u bol'nogo juvenil'noj sklerodermiej]. Vopr. sovr. pediatrii. 2012;1(3):131-7.

Marushko TV. [Juvenіl'na sistemna sklerodermіja]. Zdorov'ja Ukraїni. 2019;1(48):42-4. www.health-ua.com.

Mukvіch OM. [Juvenіl'na sistemna sklerodermіja: dіagnostika, lіkuvannja, profіlaktika. Chastina 1] Zdorov'ja Ukraїni.2021;1(57):30-2. www.health-ua.com.

Osminina MK, Geppe NA. [Voprosy klassifikacii, klinicheskaja kartina i bazisnaja terapija juvenil'noj sklerodermii]. Nauch-prakt revmatol. 2015;53(2):214-9.

Bajgabulova MS, Abdikadirova ZhB, Bulakbaeva AM, Dauletkeldi A, Makenbaj A, Bajsultanova AE. [Osobennosti klinicheskih variantov sklerodermii u detej]. Vestnik KazNMU. 2014;4:88-9. 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.

Published
2022-03-29
How to Cite
1.
Alla A. Koniushevska, Тatiana A. Parkhomenko, Natalia V. Vaizer, Olga V. Tymoshyna, Maria V. Kuzevanova. CLINICAL CASE: RARE COURSE OF JUVENILE SCLERODERMA IN RESIDENTS OF DONETSK REGION. East Ukr Med J [Internet]. 2022Mar.29 [cited 2024Mar.28];10(1):17-24. Available from: https://eumj.med.sumdu.edu.ua/index.php/journal/article/view/229