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Systemic sclerosis sine scleroderma is more aggressive in children than in adults
Francesca Tirelli1, Elisabetta Zanatta2, Beatrice Moccaldi2
1Department of Woman's and Child's Health, University of Padua, Padua, Italy.
Insights
Pediatric systemic sclerosis sine scleroderma (ssJSSc) presents more aggressively than adult-onset ssSSc, with higher rates of vascular, respiratory, and cardiac complications, leading to a worse prognosis in children.
Area of Science:
- Rheumatology
- Pediatric Rheumatology
- Autoimmune Diseases
Background:
- Systemic sclerosis sine scleroderma (ssSSc) is a subtype of scleroderma without skin thickening.
- Understanding the differences between juvenile-onset (ssJSSc) and adult-onset ssSSc is crucial for diagnosis and management.
Purpose of the Study:
- To compare the clinical and laboratory features of pediatric ssSSc (ssJSSc) with adult-onset ssSSc.
- To identify differences in disease presentation, organ involvement, and outcomes between these two groups.
Main Methods:
- Retrospective analysis of demographic, clinical, and laboratory data from ssJSSc patients.
- Comparison with a cohort of adult patients with ssSSc.
- Patients were defined by specific criteria including internal organ involvement, positive ANA, and absence of other connective tissue diseases.
Main Results:
- Fewer females were affected in the ssJSSc group (38.9% vs 89.5%).
- ssJSSc patients showed less frequent SSc-specific capillaroscopy abnormalities but significantly higher rates of vascular, respiratory, and cardiac involvement.
- The outcome was worse in ssJSSc, with higher mortality and end-stage organ failure rates (33% vs 5.3%).
Conclusions:
- Pediatric ssSSc presents with a more aggressive disease course compared to adult-onset ssSSc.
- Children with ssSSc experience higher cardiorespiratory morbidity and mortality, indicating a poorer prognosis.
Objectives:
To compare the clinical and laboratory features of paediatric SSc sine scleroderma (ssJSSc) with adult-onset ssSSc.
Methods:
Demographic, clinical and laboratory data of ssJSSc, retrospectively retrieved from our hospital medical records, case reports from the literature and from the Pediatric Rheumatology European Society JSSc registry, were compared with the Padua cohort of adult patients with ssSSc. Patients were defined as having ssSSc if they never had skin involvement but all the following features: (i) RP and/or digital vasculopathy, (ii) positive ANA, (iii) internal organs involvement typical of scleroderma and (iv) no other defined CTD.
Results:
Eighteen juvenile and 38 adult-onset ssSSc patients, mean disease duration 5.8 and 9.7 years, respectively, entered the study. The frequency of females affected was significantly lower in ssJSSc (38.9% vs 89.5%, P < 0.0001). When compared with adults, ssJSSc displayed fewer SSc-specific capillaroscopy abnormalities (68.8% vs 94.7%, P = 0.02) while having significantly higher vascular (digital pitting scars, ulcers 35.3% vs 10.5%, P = 0.042), respiratory (50.0% vs 23.7%, P = 0.02) and cardiac (50.0% vs 2.6%, P < 0.0001) involvement. The outcome was significantly worse in ssJSSc as six patients (33%) died (n = 3) or reached an end-stage organ failure (n = 3) in comparison with only two deaths (5.3%) in the adult cohort. ACA were significantly lower in children (20.0% vs 68.4%, P = 0.001) while no difference was noted for other SSc-specific autoantibodies.
Conclusion:
Compared with adults where ssSSc generally has an indolent course, children present with aggressive disease that heralds a worse prognosis characterized by high cardiorespiratory morbidity and mortality.
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