AB0764 Comparison of disease characteristics in patients with juvenile-onset and adult-onset progressive systemic sclerosis. (12th June 2018)

• Record Type: Journal Article
• Authors: Karatemiz, G.; Androvic, A.; Esatoglu, S.N.; Sahin, S.; Barut, K.; Hatemi, G.; Hamuryudan, V.; Kasapcopur, O.; Seyahi, E.
• Is Part Of: Annals of the rheumatic diseases. Volume 77(2018)Supplement 2
• Page Start: 1517
• Page End: 1518
• Publication Date: 2018-06-12
• Subjects: Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2018-eular.2087
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : Background: Progressive systemic sclerosis (PSSc) has been known to affect mainly adults of 30–50 years of age. Juvenile –onset has been reported to be rare and studies comparing clinical differences between juvenile-onset and adult onset form have been limited. 1 These studies were coming from European and North American countries. 1–2 As there would be also effects of ethnic differences, we aimed to assess clinical differences between the two forms of pSSc of paediatric and adult rheumatology centres of a tertiary centre, in Turkey. Methods: Adult onset patients were defined as those who were registered and followed as 'scleroderma' at the departments of adult and paediatric rheumatology at Cerrahpasa Medical Faculty, Istanbul, between 2005 and 2017. Only those with at least 2 follow-up visits were included. Patients's charts were re-evaluated retrospectively. Results: There were 140 patients with scleroderma in the adult outpatient clinic records and 51 in the paediatric clinic records. Of these patients, 3 (2%) adults and 25 (49%) paediatric patients had localised scleroderma (p<0.001). We studied the remaining patients (adults: n=137, juvenile: n=26) who had systemic pattern. Male/female ratio, median follow-up duration, familial history of chronic inflammatory diseases and the frequency of sclerodactyly, digital ulcers, Raynaud phenomenon, arrhythmia/heart failure and gastrointestinal involvement were similar between two groups (table 1). The frequency of interstitial lung disease, pulmonary artery hypertension, and serum ANA positivity were significantly more common in the adult onset group. Whereas joint and muscle involvements were significantly more common among juvenile onset patients. DMARD use was significantly more common in the juvenile group while the use of vasodilators was more frequent among adults1 Conclusions: Our results are online with previous reports: juvenile onset patients seem to have a milder form of disease. Major organ involvement as defined interstitial lung disease and pulmonary artery hypertension was more common among adult onset patients. On the other hand, as expected, joint involvement and myopathy were major causes of morbidity in the juvenile group. Contrary to that previously reported, cardiac involvement was not common in the juvenile group. References: [1] Scalapino K, J Rheumatol. 2006. [2] Ingegnoli F, Microvasc Res. 2015. Disclosure of Interest: None declared