Clinical and laboratory profile of juvenile-onset systemic sclerosis in a Brazilian cohort

Abstract

To characterize the clinical and laboratory profile of juvenile-onset compared to adult-onset systemic sclerosis in a large Brazilian cohort. Methods: Retrospective analysis of a cohort of 1016 systemic sclerosis patients followed at the Scleroderma Outpatient Clinic from two referral university centers in Brazil. Patients were classified as systemic sclerosis according to the 1980 American College of Rhaumatology (ACR) criteria. Juvenile-onset systemic sclerosis was defined if age at onset was <16 years. Results: Thirty-one (3.1%) patients were classified as juvenile-onset systemic sclerosis. These patients were predominantly females (90.3%), Caucasians (71.0%), and presented diffuse systemic sclerosis (51.6%), with mean age at onset of 12.71 years. Compared to the adult-onset patients, juvenile onset was associated with diffuse systemic sclerosis (p < 0.001), calcinosis (p < 0.001), myositis (p = 0.050), and lower frequency of interstitial lung disease (p = 0.050), pulmonary hypertension (p = 0.035), and esophageal (p = 0.005) involvement. Conclusion: Juvenile-onset systemic sclerosis characterized a distinct clinical pattern in this large series of systemic sclerosis patients, since it was predominantly associated with diffuse systemic sclerosis without significant organ involvement.

Calcinosis Cutis in Juvenile Systemic Sclerosis

“…Oral video capillaroscopy can be developed into a reliable technique for detecting oral microcirculation abnormalities [9]. The 2013 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) classification criteria for systemic sclerosis still maintains its effectiveness in diagnosis and monitoring disease prognosis [10][11][12][13]. This patient was started on the tablet nifedipine (60 mg), a CCB, to treat the monophasic Raynaud's phenomenon.…”

Childhood rheumatic diseases: bites not only the joint, but also the heart

“…Juvenile systemic sclerosis (Juvenile SSc) accounts for approximately 1.2%-10% of overall systemic sclerosis cases [ 5 , 26 , 27 ]. The prevalence of cardiac diseases is 6–17% compared to 15–20% in adults [ 5 , 28 ].…”

“…Cardiac involvement in juvenile SSc is more common in the limited cutaneous (lcSSc) subset (Table 1 ) [ 29 ]. At the same time, lcSSc is less common in children [ 26 , 27 ]. Arrhythmias are the most common cardiac feature of Juvenile SSc.…”

“…The frequency of conduction abnormalities decrease with age [ 30 ]. Pulmonary hypertension is seen in 5–8.6% of children with SSc which is lower compared to the adults [ 26 , 29 , 31 , 55 ]. Similar to adults, it is a significant contributor to mortality.…”

Joint contractures responsive to immunosuppressive therapy in a girl with childhood‐onset systemic sclerosis double‐seropositive for rare anti‐nucleolar autoantibodies: a case report

“…Diffuse cutaneous SSc (dcSSc) develops with equal frequency in boys and girls younger than 8 years old, whereas girls outnumber boys 3 to 1 for disease onset occurring in children older than 8 years [ 9 ]. Childhood-onset SSc, as compared with adulthood-onset SSc, is predominantly associated with dcSSc, calcinosis ( p < 0.001), myositis ( p = 0.050), and lower frequencies of internal organ involvement, such as interstitial lung disease (ILD, p = 0.050), pulmonary hypertension ( p = 0.035), and esophageal involvement ( p = 0.005) [ 10 ]. Anti-nuclear antibodies (ANAs) are frequently detected in patients with childhood-onset SSc as well as in those with adult-onset SSc.…”