{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T03:11:17Z","timestamp":1792725077362,"version":"4.5.0"},"reference-count":21,"publisher":"Termedia Sp. z.o.o.","issue":"6","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Reumatologia"],"published-print":{"date-parts":[[2022,1,21]]},"abstract":"<jats:sec><jats:title>Objectives<\/jats:title><jats:p>The systemic form of scleroderma (SSc) in children is a very rare disease; therefore, it is recognized relatively late, which increases the risk of complications. The aim of the study was to assess the clinical symptoms of juvenile systemic sclerosis (JSSc) in our cohort patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Material and methods<\/jats:title><jats:p>A group of (N = 22) scleroderma patients aged between 2 and 16 years were observed. Demographic data and all clinical results obtained during 16 years of observation in the clinic of rheumatic diseases of developmental age were collected and analysed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In all observed children the major JSSc criterion was found, i.e. skin thickening proximal to the metacarpal phalangeal and\/or metatarsophalangeal joints. Other symptoms are presented as follows: nailfold capillary abnormalities \u2013 100%, Raynaud\u2019s phenomenon \u2013 90.9%, sclerodactyly \u2013 27.3%, digital tip ulcers \u2013 27.3%, dysphagia \u2013 18.2%, gastroesophageal reflux \u2013 27.3% (assessed in only 10 children), arrhythmias \u2013 22.7%, heart failure \u2013 9.1%, new-onset arterial hypertension \u2013 9.1%, pulmonary fibrosis \u2013 72.7%, pulmonary arterial hypertension \u2013 9.1%, neuropathy \u2013 13.6%, carpal tunnel syndrome \u2013 4.5%, tendon friction rubs \u2013 4.5%, arthritis \u2013 22.7%, and myositis \u2013 13.6%. There were no cases of renal crisis. Decreased diffusing capacity of oxygen was confirmed in 12 patients (58.3%). The presence of antinuclear antibodies was noticed in 86.7% of patients, and among SSc selective autoantibodies: anticentromere \u2013 31.8%, anti-topoisomerase I \u2013 18.2%, anti-PM-Scl 100 or 75 \u2013 45.5%, anti-RP11, Th\/To, PCNA in total in 27.3% were presented. In 4.5% of cases, apart from the presence of anti-PM-Scl autoantibodies, positive lupus band test, reduced concentration of complement, and antiphospholipid antibodies were also found. In 59% of studied children, the body mass index was below the 25th percentile.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The presented retrospective analysis shows that the occurrence of Raynaud\u2019s phenomenon with changes in nailfold capillaroscopy is the best screening toll for the assessment of risk of JSSc. All patients of developmental age with Raynaud\u2019s phenomenon, especially in the case of the appearance of antinuclear antibodies, should be monitored with capillaroscopy regardless of other laboratory or imaging tests.<\/jats:p><\/jats:sec>","DOI":"10.5114\/reum.2021.112350","type":"journal-article","created":{"date-parts":[[2022,1,12]],"date-time":"2022-01-12T14:44:22Z","timestamp":1641998662000},"page":"367-372","source":"Crossref","is-referenced-by-count":12,"title":["Juvenile systemic sclerosis \u2013 observations of one clinical centre"],"prefix":"10.5114","volume":"59","author":[{"given":"Lidia","family":"Rutkowska-Sak","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Piotr","family":"Gietka","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Agnieszka","family":"Gazda","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beata","family":"Ko\u0142odziejczyk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"3061","published-online":{"date-parts":[[2021,12,30]]},"reference":[{"key":"ref-784028","doi-asserted-by":"publisher","DOI":"10.1177\/2397198318790494"},{"key":"ref-784029","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-018-4182-3"},{"key":"ref-784030","doi-asserted-by":"publisher","DOI":"10.1002\/art.39765.2772"},{"key":"ref-784031","doi-asserted-by":"publisher","DOI":"10.1093\/rap\/rky041"},{"key":"ref-784032","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajog.2015.07.034"},{"key":"ref-784033","doi-asserted-by":"publisher","DOI":"10.1002\/art.22551"},{"key":"ref-784034","doi-asserted-by":"publisher","DOI":"10.1002\/art.38098"},{"key":"ref-784035","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kel292"},{"key":"ref-784036","doi-asserted-by":"publisher","DOI":"10.1002\/art.34652"},{"key":"ref-784037","doi-asserted-by":"publisher","DOI":"10.1177\/2397198319898367"},{"key":"ref-784038","first-page":"1004","article-title":"Childhood onset systemic sclerosis: classification, clinical and serologic features, and survival in comparison with adult onset disease","volume":"33","author":"Scalapino K","year":"2006","unstructured":"Scalapino K, Arkachaisri T, Lucas M, et al. (2006). Childhood onset systemic sclerosis: classification, clinical and serologic features, and survival in comparison with adult onset disease J Rheumatol. 33: 1004-1013.","journal-title":"J Rheumatol"},{"key":"ref-784039","doi-asserted-by":"publisher","DOI":"10.5606\/ArchRheumatol.2018.6578"},{"key":"ref-784040","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/39.5.556"},{"key":"ref-784041","doi-asserted-by":"publisher","DOI":"10.1002\/acr.23547"},{"key":"ref-784042","doi-asserted-by":"publisher","DOI":"10.1002\/art.22207"},{"key":"ref-784043","doi-asserted-by":"publisher","DOI":"10.3109\/03009742.2011.609489"},{"key":"ref-784044","first-page":"660","article-title":"Obraz dr\u00f3g \u017c\u00f3\u0142ciowych u dzieci chorych na zapalne uk\u0142adowe choroby tkanki \u0142\u0105cznej","volume":"67","author":"Rutkowska-Sak L","year":"1992","unstructured":"Rutkowska-Sak L. (1992). Obraz dr\u00f3g \u017c\u00f3\u0142ciowych u dzieci chorych na zapalne uk\u0142adowe choroby tkanki \u0142\u0105cznej Ped Pol. 67: 660-666.","journal-title":"Ped Pol"},{"key":"ref-784045","first-page":"235","article-title":"Zmiany w uk\u0142adzie pokarmowym u dzieci chorych na zapalne uk\u0142adowe choroby tkanki \u0142\u0105cznej","volume":"70","author":"Rutkowska-Sak L","year":"1995","unstructured":"Rutkowska-Sak L, Legatowicz-Koprowska M, Ry\u017cko J, Socha J. (1995). Zmiany w uk\u0142adzie pokarmowym u dzieci chorych na zapalne uk\u0142adowe choroby tkanki \u0142\u0105cznej Ped Pol. 70: 235-241.","journal-title":"Ped Pol"},{"key":"ref-784046","doi-asserted-by":"publisher","DOI":"10.1016\/j.jad.2020.09.025"},{"key":"ref-784047","first-page":"934","article-title":"Pos1304 Juvenile systemic sclerosis (JSSC) patients with overlap characteristics do not have mild disease. Results from the jssc inception cohort. www.juvenilescleroderma.com","volume":"80","author":"Foeldvari I","year":"2021","unstructured":"Foeldvari I, Klotsche J, Kasapcopur O, et al. (2021). Pos1304 Juvenile systemic sclerosis (JSSC) patients with overlap characteristics do not have mild disease. 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