| Types of Scleroderma: Overview |
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| Juvenile Scleroderma Prognosis |
| This page was written by Shelley Ensz and has not yet been medically edited. See disclaimer. |
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| Overview |
| When any form of scleroderma (either localized or systemic) occurs in children, it is also called Childhood Scleroderma or Juvenile Scleroderma. (Also see: What is Scleroderma? and Types of Scleroderma) |
| Prognosis: Juvenile Localized Scleroderma (Linear and Morphea) |
| Localized scleroderma, such as linear, morphea, en coup de sabre, may cause disfigurement and disability (which is why it is important to seek expert care early on). However, localized scleroderma does not reduce a person's life expectancy. (Also see: Juvenile Scleroderma Experts) |
| Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study. The insidious onset of the disease, the delay in diagnosis, the recognition of mixed subtype and the better definition of the other subtypes should influence our efforts in educating trainees and practitioners and help in developing a comprehensive classification system for this syndrome. PubMed. Rheumatology (Oxford). 2005 Dec 20. |
| Prognosis: Juvenile Systemic Sclerosis (Diffuse, Limited/CREST) |
| Systemic sclerosis (scleroderma), such as diffuse or limited/CREST, can affect the skin, blood vessels, and internal organs. In general, the survival rate is better for those who have childhood onset of systemic scleroderma (as opposed to those with adult onset) because children tend to have less internal organ involvement. (Also see: Juvenile Scleroderma Experts, Systemic Sclerosis, Systemic Sclerosis Prognosis, and Systemic Symptoms) |
| Factors affecting survival in juvenile systemic sclerosis (JSSc). All patients with JSSc and fatal outcome were affected by the diffuse form of the disease, and most of them showed a very rapid progression and early signs of internal organ involvement. This suggests that, in children, SSc may have two possible courses: a rapid development of internal organ failure leading to severe disability and eventually to death, or a slow course of the disease with lower mortality. G. Martini. Rheumatology. October 14, 2008. |
| 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. |
| 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. (Also see: Systemic Sclerosis and Types of Scleroderma) |
