Evolution of Mixed Connective Tissue Disease in 7 Years Old Child: Clinical Case

Background. Mixed connective tissue disease (Sharp syndrome) is the rare chronic autoimmune pathology combining various features of systemic lupus erythematosus, systemic scleroderma, rheumatoid arthritis, dermatomyositis and high antibody titer to nuclear ribonucleoprotein. The mixed connective tis...

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Main Authors: Tamara P. Makarova, Khakim M. Vakhitov, Dina R. Sabirova, Dinara I. Sadykova, Liliya R. Khusnutdinova, Natalya N. Firsova, Anna A. Kucheryavaya
Format: Article
Language:English
Published: "Paediatrician" Publishers LLC 2020-08-01
Series:Voprosy Sovremennoj Pediatrii
Subjects:
Online Access:https://vsp.spr-journal.ru/jour/article/view/2426
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spelling doaj-a8f6e174a7854cf7ac76a8ff7778d1a72021-07-28T21:15:47Zeng"Paediatrician" Publishers LLC Voprosy Sovremennoj Pediatrii1682-55271682-55352020-08-0119321421910.15690/vsp.v19i3.21171885Evolution of Mixed Connective Tissue Disease in 7 Years Old Child: Clinical CaseTamara P. Makarova0Khakim M. Vakhitov1Dina R. Sabirova2Dinara I. Sadykova3Liliya R. Khusnutdinova4Natalya N. Firsova5Anna A. Kucheryavaya6Kazan State Medical UniversityKazan State Medical UniversityKazan State Medical UniversityKazan State Medical UniversityKazan State Medical UniversityChildren’s Republican Clinical HospitalChildren’s Republican Clinical HospitalBackground. Mixed connective tissue disease (Sharp syndrome) is the rare chronic autoimmune pathology combining various features of systemic lupus erythematosus, systemic scleroderma, rheumatoid arthritis, dermatomyositis and high antibody titer to nuclear ribonucleoprotein. The mixed connective tissue disease may evolve into other systemic diseases over time. Description of any cases of mixed connective tissue disease and its evolution in Russian patients has not been published previously.Clinical Case Description. The results of observations of the child with clinical and immunological signs of the mixed connective tissue disease followed by the progression of systemic scleroderma symptoms and development of Sjogren's syndrome in the short period of time are presented in the article. Improvement (such as pain attenuation, increase in volume of movements in affected joints, decrease of Raynaud syndrome manifestations duration) was observed on treatment (methotrexate 10 mg/week with subsequent addition of prednisolone 0.75 mg/kg/day).Conclusion. Timely diagnostics of clinical signs of the systemic diseases debut is crucial for correct patient routing and for achieving of disease improvement.https://vsp.spr-journal.ru/jour/article/view/2426childrenmixed connective tissue diseasesharp syndromeoverlap syndromeraynaud syndromearthritis
collection DOAJ
language English
format Article
sources DOAJ
author Tamara P. Makarova
Khakim M. Vakhitov
Dina R. Sabirova
Dinara I. Sadykova
Liliya R. Khusnutdinova
Natalya N. Firsova
Anna A. Kucheryavaya
spellingShingle Tamara P. Makarova
Khakim M. Vakhitov
Dina R. Sabirova
Dinara I. Sadykova
Liliya R. Khusnutdinova
Natalya N. Firsova
Anna A. Kucheryavaya
Evolution of Mixed Connective Tissue Disease in 7 Years Old Child: Clinical Case
Voprosy Sovremennoj Pediatrii
children
mixed connective tissue disease
sharp syndrome
overlap syndrome
raynaud syndrome
arthritis
author_facet Tamara P. Makarova
Khakim M. Vakhitov
Dina R. Sabirova
Dinara I. Sadykova
Liliya R. Khusnutdinova
Natalya N. Firsova
Anna A. Kucheryavaya
author_sort Tamara P. Makarova
title Evolution of Mixed Connective Tissue Disease in 7 Years Old Child: Clinical Case
title_short Evolution of Mixed Connective Tissue Disease in 7 Years Old Child: Clinical Case
title_full Evolution of Mixed Connective Tissue Disease in 7 Years Old Child: Clinical Case
title_fullStr Evolution of Mixed Connective Tissue Disease in 7 Years Old Child: Clinical Case
title_full_unstemmed Evolution of Mixed Connective Tissue Disease in 7 Years Old Child: Clinical Case
title_sort evolution of mixed connective tissue disease in 7 years old child: clinical case
publisher "Paediatrician" Publishers LLC
series Voprosy Sovremennoj Pediatrii
issn 1682-5527
1682-5535
publishDate 2020-08-01
description Background. Mixed connective tissue disease (Sharp syndrome) is the rare chronic autoimmune pathology combining various features of systemic lupus erythematosus, systemic scleroderma, rheumatoid arthritis, dermatomyositis and high antibody titer to nuclear ribonucleoprotein. The mixed connective tissue disease may evolve into other systemic diseases over time. Description of any cases of mixed connective tissue disease and its evolution in Russian patients has not been published previously.Clinical Case Description. The results of observations of the child with clinical and immunological signs of the mixed connective tissue disease followed by the progression of systemic scleroderma symptoms and development of Sjogren's syndrome in the short period of time are presented in the article. Improvement (such as pain attenuation, increase in volume of movements in affected joints, decrease of Raynaud syndrome manifestations duration) was observed on treatment (methotrexate 10 mg/week with subsequent addition of prednisolone 0.75 mg/kg/day).Conclusion. Timely diagnostics of clinical signs of the systemic diseases debut is crucial for correct patient routing and for achieving of disease improvement.
topic children
mixed connective tissue disease
sharp syndrome
overlap syndrome
raynaud syndrome
arthritis
url https://vsp.spr-journal.ru/jour/article/view/2426
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