Heart injury in juvenile systemic sclerodermy (clinical case)

Authors

  • L.I. Omelchenko State Institution «Institute of pediatrics, obstetrics and gynecology named after academician O. lukyanova of the National academy of medical sciences of Ukraine», P. Mayborody str., 8, Kyiv, 04050, Ukraine https://orcid.org/0000-0003-2989-9278
  • O.N. Mukvich State Institution «Institute of pediatrics, obstetrics and gynecology named after academician O. lukyanova of the National academy of medical sciences of Ukraine», P. Mayborody str., 8, Kyiv, 04050, Ukraine https://orcid.org/0000-0001-6405-4997
  • O.A. Belska State Institution «Institute of pediatrics, obstetrics and gynecology named after academician O. lukyanova of the National academy of medical sciences of Ukraine», P. Mayborody str., 8, Kyiv, 04050, Ukraine
  • I.V Dudka State Institution «Institute of pediatrics, obstetrics and gynecology named after academician O. lukyanova of the National academy of medical sciences of Ukraine», P. Mayborody str., 8, Kyiv, 04050, Ukraine
  • Y.I. Klymyshyn The scientific and practical medical center of pediatric cardiology and cardiac surgery of the ministry of health of Ukraine Government institution, Yu. Illienko str., 24. Kyiv, 04050, Ukraine
  • N.M. Rudenko The scientific and practical medical center of pediatric cardiology and cardiac surgery of the ministry of health of Ukraine Government institution, Yu. Illienko str., 24. Kyiv, 04050, Ukraine
  • R.V. Kalashnikova The scientific and practical medical center of pediatric cardiology and cardiac surgery of the ministry of health of Ukraine Government institution, Yu. Illienko str., 24. Kyiv, 04050, Ukraine
  • D.L. Ismakaieva State Institution «Institute of pediatrics, obstetrics and gynecology named after academician O. lukyanova of the National academy of medical sciences of Ukraine», P. Mayborody str., 8, Kyiv, 04050, Ukraine
  • N.M. Vdovina State Institution «Institute of pediatrics, obstetrics and gynecology named after academician O. lukyanova of the National academy of medical sciences of Ukraine», P. Mayborody str., 8, Kyiv, 04050, Ukraine
  • T.A. Liudvik State Institution «Institute of pediatrics, obstetrics and gynecology named after academician O. lukyanova of the National academy of medical sciences of Ukraine», P. Mayborody str., 8, Kyiv, 04050, Ukraine

DOI:

https://doi.org/10.26641/2307-0404.2021.3.242344

Keywords:

juvenile systemic scleroderma, dilated cardiomyopathy, children

Abstract

Juvenile systemic sclerosis (JSS) has many clinical manifestations that differ from adults. Early diagnosis is problematic. The course of the disease and the severity of the prognosis depend on the involvement of internal organs in the process, first of all, the heart, lungs, kidneys. Cardiac pathology is a frequent and prognostically unfavorable target of the scleroderma process in adults, but it is rarely diagnosed in children. The aim of the work was to study the features of the clinical manifestations of systemic sclerosis in a child with severe heart disease. A polymorphism of the clinical symptoms of severe heart damage with the development of dilated cardiomyopathy in a one-year-old child with systemic sclerosis is presented. The features of the case are the early debut of systemic sclerosis in a child with a burdened hereditary history of autoimmune pathology (psoriasis in the father and grandmother), rapid progression of the autoimmune process, severe heart damage by the type of non-compact (dilated) cardiomyopathy, positive clinical dynamics when using pathogenetic therapy. Early detection of cardiovascular lesions using modern diagnostic methods, timely implementation of adequate therapy in a multidisciplinary team and regular cardiovascular screening can improve the prognosis, quality of life and reduce mortality.

References

Porter A, Mirizio E, Fritzler MJ, Brown R, Choi M, Schollaert-Fitch K, et al. Autoantibody testing in pediatric localized scleroderma (LS). Arthritis Rheumatol. 2018;70 (suppl 10). doi: https://doi.org/10.3389/fimmu.2019.00908

Wu EY, Li SC, Torok KS, Virkud Y, Fuhlbrigge R, Rabinovich CE. Baseline description of the juvenile localized scleroderma subgroup from the childhood arthritis and rheumatology research alliance legacy registry. ACR Open Rheumatol In press. 2019;1:119-24. doi: https://doi.org/10.1002/acr2.1019

Stasiulis E, Gladstone B, Boydell K, et al. Children with facial morphoea managing everyday life: a qualitative study. Br J Dermatol. 2018;179:353. doi: https://doi.org/10.1111/bjd.16449

Zulian F, Culpo R, Sperotto F, et al. Consensus-based recommendations for the management of juvenile localised scleroderma. Ann Rheum Dis. 2019;78:1019.

doi: https://doi.org/10.1136/annrheumdis-2018-214697

Hung G, Mercurio V, Hsu S, Mathai S. Shah C. Progress in Understanding, Diagnosing, and Managing Cardiac Complications of Systemic Sclerosis. Current rheumatology reports. 2019;12:68. doi: https://doi.org/10.1007/s11926-019-0867-0

Mirizio E, Marathi A, Hershey N, Ross C, Schollaert K, Salgadgo CM, et al. Identifying the signature immune phenotypes present in pediatric localized scleroderma. J Invest Dermatol. 2018;139:715-8. doi: https://doi.org/10.1016/j.jid.2018.09.025

Torok KS, Li SC, Jacobe HM, et al. Immunopathogenesis of pediatric localized scleroderma. Front Immunol. 2019;10:1-11. doi: https://doi.org/10.3389/fimmu.2019.00908

Macaubas C, Mirizio E, Schollaert-Fitch K, Mellins ED, Torok KS. Interferon gamma (IFN-γ) Subpopulations in skin homing T-cells of localized scleroderma [abstract]. Arthritis Rheumatol. 2017;69 (suppl 10). doi: https://doi.org/10.3389/fimmu.2019.00908

Kurzinski K, Zigler CK, Torok KS. Prediction of disease relapse in a cohort of juvenile localized scleroderma patients. Br J Dermatol. The British Journal of Dermatology. 2018;180(5):1183-9. doi: https://doi.org/10.1111/bjd.17312

Singhvi G, Manchanda P, Krishna Rapalli V, et al. MicroRNAs as biological regulators in skin disorders. Biomed Pharmacother. 2018;108:996-1004. doi: https://doi.org/10.2147/JPR.S221615

Li Y, Zhang J, Lei Y, et al. MicroRNA-21 in skin fibrosis: potential for diagnosis and treatment. 2017;21:633-42. doi: https://doi.org/10.1007/s40291-017-0294-8

Li SC, Li X, Pope E, Stewart K, Higgins GC, Rabinovich CE, et al. New Features for Measuring Disease Activity in Pediatric Localized Scleroderma. J Rheumatol in press. 2018;45:1680-8. doi: https://doi.org/10.3899/jrheum.171381

Schoch J, Schoch B, Davis D. Orthopedic complications of linear morphea: Implications for early interdisciplinary care. Pediatric Dermatol. 2018;35:43-6. doi: https://doi.org/10.1111/pde.13336

Chouri E, Servaas NH, Bekker CPJ, et al. Serum microRNA screening and functional studies reveal miR-483-5p as a potential driver of fibrosis in systemic sclerosis. J Autoimmun. 2018;89:162-70. doi: https://doi.org/10.1007/s00403-019-01991-0

Tenea D. The puzzle of the skin patterns. Integrative Medicine International. 2017;4:1-12. doi: https://doi.org/10.1159/000452949

Published

2021-09-30

How to Cite

1.
Omelchenko L, Mukvich O, Belska O, Dudka I, Klymyshyn Y, Rudenko N, Kalashnikova R, Ismakaieva D, Vdovina N, Liudvik T. Heart injury in juvenile systemic sclerodermy (clinical case). Med. perspekt. [Internet]. 2021Sep.30 [cited 2022Jan.26];26(3):219-27. Available from: http://journals.uran.ua/index.php/2307-0404/article/view/242344

Issue

Section

A CASE FROM PRACTICE