OTHER RHEUMATIC DISEASES OF CHILDHOOD

Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease with widespread immune complex deposition that results in episodic inflammation, vasculitis, and serositis. Children are more likely than adults to present with this systemic disease; 20% of cases begin in childhood. Females are affected 4.5 times more than males. One-third of children have the erythematous butterfly rash over the bridge of the nose and cheeks; this rash may occur after exposure to sunlight. Most children develop a transient, migratory arthritis of the extremities; radiographic evidence of joint deformity and erosion is not common. Pain may be out of proportion to joint findings on examination. Proximal muscle weakness may be a result of acute illness, myositis, or the result of steroid-induced myopathy. Long-term steroids also increase the risk of avascular necrosis of the femoral head.

Systemic features of SLE may include pericarditis or endocarditis; proliferative glomerulonephritis or other renal disease; seizures, psychosis, memory deficits, headaches, or behavior changes; pulmonary hypertension and/or hypertension. Nephritis occurs in approximately 75% of children with SLE and is the main factor for determining outcome. Hematuria, proteinuria, persistent hypertension, chronic active disease, and biopsy proven diffuse proliferative glomerulonephritis are associated with a poor outcome. Ten-year survival is approximately 80%, although this number is lower in lower socioeconomic populations.

Management of SLE is symptomatic. Maintaining physical activity as much as possible, avoiding excess sunlight exposure, optimizing nutrition, and providing adequate social supports are key. For some children with open discoid lupus rash lesions, dressing changes and wound cares may be best facilitated with individualized whirlpool therapy, much like is used for burn wound cares.

NSAIDs are mainly used for arthritis and musculoskeletal conditions. Fever, dermatitis, arthritis, and serositis usually resolve quickly with low-dose steroids, whereas serologic findings may require weeks of steroid therapy. Hydroxychloroquine may be used for skin manifestations or in concert with steroids to lower the steroid dose. High-dose steroids, immunosuppressive agents, and biologic agents may be necessary for more severe disease manifestations.

Scleroderma

Systemic sclerosis is uncommon in children; linear and focal cutaneous involvement is most common in children. Girls between ages 8 and 10 years are more often affected; duration can last 7 to 9 years. Linear scleroderma presents with atrophic, erythematous skin areas, which later become fibrotic. This skin then binds to underlying subcutaneous tissues, and underlying muscle and bone also become involved. Children may have pain from these skin changes. Soft tissues can atrophy, leaving areas of asymmetry. Scleroderma en coup de sabre is a unilateral linear involvement of the face and scalp, often with loss of hair on the involved side, with loss of facial asymmetry. Systemic disease in children is uncommon. Physical therapy is necessary to prevent loss of ROM and contractures because of the cutaneous involvement. Soft tissue massage, moist heat, stretching, and ROM exercises help maximize joint mobility. Topical corticosteroids may be helpful in treating localized skin disease; systemic steroids, methotrexate, and physical therapy may alter the course of progressive disease.

 
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