Juvenile systemic sclerosis – observations of one clinical centre

Abstract

ObjectivesThe 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.Material and methodsA 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.ResultsIn 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 – 100%, Raynaud’s phenomenon – 90.9%, sclerodactyly – 27.3%, digital tip ulcers – 27.3%, dysphagia – 18.2%, gastroesophageal reflux – 27.3% (assessed in only 10 children), arrhythmias – 22.7%, heart failure – 9.1%, new-onset arterial hypertension – 9.1%, pulmonary fibrosis – 72.7%, pulmonary arterial hypertension – 9.1%, neuropathy – 13.6%, carpal tunnel syndrome – 4.5%, tendon friction rubs – 4.5%, arthritis – 22.7%, and myositis – 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 – 31.8%, anti-topoisomerase I – 18.2%, anti-PM-Scl 100 or 75 – 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.ConclusionsThe presented retrospective analysis shows that the occurrence of Raynaud’s 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’s phenomenon, especially in the case of the appearance of antinuclear antibodies, should be monitored with capillaroscopy regardless of other laboratory or imaging tests.