Juvenile Scleroderma Research

This page was written by Shelley Ensz and has not yet been medically edited. See disclaimer.

Overview
Congenital Juvenile Scleroderma
Complications

Nailfold Capillaroscopy
Prognosis and Mortality
Treatments

Overview

When any form of scleroderma (either localized or systemic) occurs in children, it is also called Childhood Scleroderma or Juvenile Scleroderma.

Congential Juvenile Scleroderma

Congenital localized scleroderma (CLS). CLS is a rare and probably underestimated condition in neonates. CLS should be included in the differential diagnosis of infants with cutaneous erythematous fibrotic lesions to avoid functional and aesthetic sequelae and to allow prompt therapy. PubMed. J Pediatr. 2006 Aug;149(2):248-51. (Also see: Linear Scleroderma )

Complications

Health-related quality of life (HRQOL) in children and adolescents with juvenile localized scleroderma (JLS). JLS patients have some impairment in skin disease-specific HRQOL when compared with a healthy reference population, but not as severe as that seen in Atopic Dermatitis patients. Overall HRQOL in this JLS cohort was as good as healthy controls, a reassuring finding for patients, families and healthcare providers. N. M. Orzechowski. Rheumatology. March 31, 2009.

Evaluation of Heart Rhythm Variability and Arrhythmia in Children with Systemic and Localized Scleroderma. The most frequent cardiac arrhythmias in both types of scleroderma in children were of supraventricular origin, whereas ventricular arrhythmias did not occur very often. J. Wozniak. J Rheumatol Dec 1 2008. (Also see: Cardiac Involvement)

Nailfold Capillaroscopy in Juvenile Scleroderma

Inflammatory myopathies in childhood: correlation between nailfold capillaroscopy findings and clinical and laboratory data. Nailfold capillaroscopy is an important tool for the diagnosis and follow-up of patients with rheumatic diseases, in particular dermatomyositis and scleroderma. A relationship has been observed in adults between improved capillaroscopic findings and reduced disease activity. PubMed. J. Pediatr (Rio J). 2006 January/February;82(1):40-45. (Also see: Dermatomyositis and Nailfold Capillaroscopy)

Prognosis and Mortality in Juvenile Scleroderma

Mortality Outcomes in Pediatric Rheumatology Conditions. In the first systematic study of mortality rates in pediatric rheumatology the mortality rate for new patients seen in the U.S. during the 1990s was not increased in relation to the general population. The mortality rate was significantly increased in SLE, other connective tissue diseases, malignancies and systemic JRA. Philip J. Hashkes 254/254 ACR 2007. (Also see: Systemic Scleroderma: Prognosis and Mortality)

Systemic sclerosis in childhood: clinical and immunologic features of 153 patients in an international database. This study provides information on the largest collection of patients with juvenile SSc ever reported. Juvenile SSc appears to be less severe than in adults because children have less internal organ involvement, a less specific autoantibody profile, and a better long-term outcome. PubMed. Arthritis Rheum. 2006 Dec;54(12):3971-8.

Juvenile onset systemic sclerosis: a single center experience of 23 cases from Asia. DSSc and LSSc in childhood have a clinical presentation similar to adult patients, with cardiopulmonary involvement being the major predictor of outcome. The short-term prognosis of JSSc is good. PubMed. Clin Rheumatol. 2006 Dec 19.

Childhood Onset Systemic Sclerosis (SSc): Classification, Clinical and Serologic Features, and Survival in Comparison with Adult Onset Disease. Patients with juvenile onset SSc more frequently have an overlap syndrome with PM-DM, higher frequency of skeletal muscle involvement, serum anti-PM-Scl and anti-U1RNP antibody, fatal cardiac disease, and improved survival compared with adult onset SSc cases. PubMed. J Rheumatol. 2006 Apr 1.

Juvenile Systemic Sclerosis: A Follow-up Study of Eight Patients. Our results suggest that in Juvenile Systemic Sclerosis (jSSc), Raynaud's phenomenon is more severe, whereas internal organ manifestations and the frequency of autoantibodies are far less pronounced than in adult-onset SSc. Also, the survival rate and final outcome of patients with jSSc appear to be better than those in patients with adult-onset SSc. PubMed. Ann N Y Acad Sci. 2005 Jun;1051:229-34.

Treatments for Juvenile Scleroderma

High Frequency Ultrasound (US) in Juvenile Localised Scleroderma (JLS). US seems to be a sensitive tool to evaluate skin modifications in JLS and correlate with clinical evaluation. Further studies are needed to better correlate clinical stage of lesions and morphological US images. F. Falcini. (THU0429) EULAR 2009.

Evaluation of methotrexate and corticosteroids for the treatment of localized scleroderma (morphea) in children. These data suggest that systemic corticosteroids and methotrexate in combination are beneficial and well tolerated in the treatment of children with LS. Long-term monitoring is mandatory. IngentaConnect. Br J Dermatol. 2006 Nov;155(5):1013-20. (Also see: Morphea)

Improving Hand Function in Childhood Scleroderma using Continuous Passive Motion (CPM). Hand/wrist function in a highly motivated adolescent with localized scleroderma improved with the addition of CPM to her therapeutic regimen. Kimberly A. Wesdock. 1963/662 ACR 2006. (Also see: Sclerodactyly)

Treatment of Pediatric Localized Scleroderma with Methotrexate. Methotrexate appears to be a safe and effective therapy for pediatric Localized Scleroderma. S. J Rheumatol 2006; 33:609–14. (Also see: Localized Scleroderma)

Systemic and localized scleroderma in children: current and future treatment options. The juvenile forms of systemic sclerosis and localized scleroderma are important conditions in children because of the clinical severity and substantial mortality of systemic scleroderma and the major growth defects associated with childhood-onset localized disease even if the active disease itself is self-limited. For localized disease, anti-inflammatories, vitamin D analogs, and UV irradiation have been investigated. PubMed. Paediatr Drugs. 2006;8(2):85-97.