Juvenile systemic sclerosis (scleroderma)
- Francesco Zulian, MD
Francesco Zulian, MD
- Chief, Pediatric Rheumatology Unit
- University of Padua, Italy
Scleroderma is a spectrum of disorders that can occur at any stage of life, although the clinical patterns of childhood sclerosis differ from that of adulthood .
The predominant form of childhood sclerosis is localized scleroderma. Juvenile systemic sclerosis (JSSc) is uncommon, but has the most significant morbidity of the sclerotic disorders.
JSSc is a chronic, multisystem, connective tissue disorder characterized by symmetrical fibrous thickening and hardening of the skin combined with fibrous changes in internal organs, such as the esophagus, intestinal tract, heart, lungs, and kidneys. The extent of skin involvement and the accompanying pattern of internal organ involvement form the basis for the classification of systemic sclerosis in children . Provisional classification criteria for JSSc using consensus-based methodologies have been developed .
This topic will review the clinical presentation, diagnosis, and management of JSSc. Localized scleroderma and the pathogenesis of systemic sclerosis are discussed in detail elsewhere. (See "Localized scleroderma in childhood" and "Pathogenesis of systemic sclerosis (scleroderma)".)
The principal types of systemic sclerosis include the following subsets in adults  (see "Overview and classification of scleroderma disorders"):
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- PATHOGENESIS AND GENETICS
- CLINICAL MANIFESTATIONS
- General laboratory findings
- Other organ involvement
- Disease progression
- Laboratory tests and pathology
- DIFFERENTIAL DIAGNOSIS
- LIMITED CUTANEOUS SYSTEMIC SCLEROSIS
- Clinical manifestations of lcSSc
- SYSTEMIC SCLEROSIS-SINE SCLERODERMA
- SIMILARITIES AND DIFFERENCES BETWEEN CHILDREN AND ADULTS WITH SYSTEMIC SCLEROSIS
- ASSESSMENT OF DISEASE ACTIVITY AND SEVERITY
- Nonpharmacologic measures
- Pharmacologic therapy
- - Raynaud phenomenon
- - Digital ulcers
- - Interstitial lung disease
- - Pulmonary arterial hypertension
- - Skin involvement
- - Renal disease
- - Musculoskeletal involvement
- - Gastrointestinal disease
- - Experimental therapy
- Disease monitoring
- SUMMARY AND RECOMMENDATIONS