Вопросы Классификации, Клиническая Картина И Базисная Терапия Ювенильной Склеродермии

Osminina, M.K.; Геппе, Н.А.

doi:10.14412/1995-4484-2015-214-219

Cited by 4 publications

(1 citation statement)

References 15 publications

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“…ЮССД достаточно редкое заболевание. Известно, что склеродермия с системными проявлениями встречается в 5-10 раз реже ограниченной формы (когда имеет место поражение только кожи) [3]. Заболеваемость ограниченной системной склеродермии у детей, по данным разных авторов, колеблется от 1 случая на 100 тыс.…”

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The Case of the Debut of Systemic Scleroderma in a Patient Aged 10

Burlutskaya¹,

Savelyeva²,

Статова³

2018

Kuban. nauсh. med. vestnik

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Aim. To show the diversity and severity of clinical symptoms, a large range of diagnostic search, as well as difficulties in therapy in a 10-year-old patient with systemic manifestations of juvenile scleroderma.Materials and methods. A retrospective analysis of the anamnetic data, the course of the disease, laboratory and instrumental data, as well as the ongoing therapy of a 10-year-old patient with juvenile systemic scleroderma was carried out.Results. The patient was diagnosed with multiple typical sclerodermic foci of irregular shape, with different stages of lesion (edema, induration and atrophy). Joint damage was noted in the form of pseudoarthritis interphalangeal joints of hands with the formation of sclerodactily and right ankle joint. As a result of computed tomography of chest, signs of interstitial lung disease by the type of fibrous alveolitis have been revealed. After verification of the diagnosis, the patient was prescribed basic therapy: methylprednisolone and cyclophosphane.Conclusion. A clinical case of the debut of juvenile systemic scleroderma with rapidly progressive, widespread skin lesions, involvement of the musculoskeletal system and the development of fibrous alveolitis is presented. Due to intensive and long-term pathogenetic therapy (glucocorticoids, cyclophosphamide), the patient's condition was stabilized and the progression of interstitial lung disease was stopped.

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Section: âведениеunclassified

The Case of the Debut of Systemic Scleroderma in a Patient Aged 10

Burlutskaya¹,

Savelyeva²,

Статова³

2018

Kuban. nauсh. med. vestnik

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Clinical Case: Rare Course of Juvenile Scleroderma in Residents of Donetsk Region

Koniushevska,

Parkhomenko,

Vaizer

et al. 2022

EUMJ

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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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