Background/Purpose: 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). One study form North America revealed that there is more skeletal muscle involvement, more severe cardiac disease, whereas improved survival compared with adult onset pSSc cases (1). Recently, one multicenter European study revealed that juvenile-onset PSSc showed scleroderma pattern on the capilleroscopy more frequently than that observed in adult-onset pSSc (2). As there would be also effects of ethnic differences, we aimed to assess clinical differences between the two forms of pSSc of pediatric and adult rheumatology centers of a tertiary center, in Turkey.

Methods: Adult onset patients were defined as those who were registered and followed as ‘scleroderma’ at the departments of adult and pediatric rheumatology at Cerrahpasa Medical Faculty, Istanbul, between 2005 and 2017. Only those with at least 2 follow-up visits were included in the study. Patients’s charts were re-evaluated retrospectively for demographic and clinic characteristics.

Results: There were 140 patients with scleroderma diagnosis in the adult outpatient clinic records, and 51 in the pediatric clinic records. Of these patients, 3 adults and 25 (49 %) pediatric patients had localized scleroderma (p<0.001).  We studied the remaining patients (adults: n=137, juvenile: n =26) who had systemic pattern.

As shown in Table, 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 juvenile and adult onset groups. 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, on the other hand, the use of vasodilators was more frequent among adults.

Conclusion: 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, et al. J Rheumatol. 2006.

2)      Ingegnoli F, et al. Microvasc Res. 2015.
 

Table. Demographic and clinic characteristics of the patients