The following is a summary of “Juvenile and adult-onset scleroderma: Different clinical phenotypes,” published in the June 2023 issue of the Seminars in Arthritis and Rheumatism by Adrovic et al.

Systemic sclerosis (SSc) is an uncommon disease, with most data from adult patients. Few studies compare juvenile-onset (jSSc) and adult-onset (aSSc) patients. Researchers’ objective was to compare the clinical characteristics, treatment methods, and survival rates of jSSc and aSSc patients. A retrospective examination of pediatric and adult patients with Scl has been conducted. The databases were queried for demographic characteristics, clinical characteristics, autoantibody profiles, and treatment information. Using a Kaplan-Meier plot, an analysis of survival was conducted, and factors associated with mortality were identified using multiple regression analysis. 

About 158 adult and 58 juvenile patients with Scl were identified. For aSSc and jSSc patients, the mean age at disease onset was 37±14.7 vs. 8.8± 4.1 years, the mean age at diagnosis was 42±15.2 vs. 10.4±3.8 years, and the mean follow-up duration was 6.3 ±4.9 years vs. 6.6±  4.9 years, respectively. Significantly higher rates of interstitial lung disease (ILD) (50.9% vs. 30%, P<0.001) and systemic hypertension (17.9% vs. 0%, P = 0.009) were observed in aSSc. Patients with aSSc primarily presented with a limited cutaneous subset (74.1%), whereas patients with jSSc primarily presented with a diffuse cutaneous subset (76.7%) (P<0.001). Adults had a substantially higher mortality rate (P= 0.005). In both aSSc and jSSc patients, ILD (P= 0.03) and cardiac insufficiency (P = 0.05) were independent risk factors for mortality.

Juvenile and adult-onset Scl are uncommon conditions with distinct clinical phenotypes. In contrast to adults, pediatric rheumatologists see more children with LS than adult rheumatologists. The diffuse disease subset is the predominant form among pediatric patients, whereas the limited disease subset is the dominant form among adults. On the contrary, juvenile-onset patients have a higher survival rate than adult-onset patients.

Source: sciencedirect.com/science/article/abs/pii/S0049017223000392