Treatment of Rheumatic Diseases

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Chapter 148 Treatment of Rheumatic Diseases

Esi Morgan DeWitt, Laura E. Schanberg, C. Egla Rabinovich

The rheumatic diseases of childhood are complex chronic illnesses that present management challenges to both primary care and subspecialty providers. Optimal disease management requires family-centered care delivered by a multidisciplinary team of health care professionals providing medical, psychologic, social, and school support. Rheumatologic conditions, such as juvenile idiopathic arthritis (JIA) and systemic lupus erythematosus (SLE), most often follow a disease course marked by flares and periods of remission, but some children have unremitting disease. Treatment is aimed at achieving and maintaining clinical remission while minimizing medication toxicities. Disease management includes surveillance for potential complications of disease, such as inflammatory eye disease in JIA and early nephritis in SLE.

The goals of treatment are to avert disability, maximize the physical function and quality of life of affected children, relieve discomfort, prevent or reduce organ damage, and avoid or limit drug toxicity. Nonpharmacologic therapy is an important adjunct to medical management of rheumatic diseases. A key predictor of long-term outcome consists of early recognition and referral to a rheumatology team experienced in the specialized care of children with rheumatic diseases. Significant differences in outcome are seen at 10 yr after onset in patients with JIA depending on whether referral to a pediatric rheumatology center was accomplished within 6 mo of onset.

Pediatric Rheumatology Teams and Primary Care Physicians

The multidisciplinary pediatric rheumatology team (Table 148-1) offers coordinated services for children and their families. General principles of treatment include: early recognition of signs and symptoms of rheumatic disease with timely referral to rheumatology for prompt initiation of treatment; monitoring for disease complications and adverse effects of treatment; coordination of subspecialty care and rehabilitation services with communication of clinical information; and child and family–centered chronic illness care, including self-management support, alliance with community resources, partnership with schools, resources for dealing with the financial burdens of disease, and connection with advocacy groups. Planning for transition to adult care providers needs to start in adolescence. Central to effective care is partnership with the primary care provider, who helps coordinate care, monitor compliance with treatment plans, ensure appropriate immunization, monitor for medication toxicities, and identify disease exacerbations and concomitant infections. Communication between the primary care provider and subspecialty team permits timely intervention when needed.

Table 148-1 MULTIDISCIPLINARY TREATMENT OF RHEUMATIC DISEASES IN CHILDHOOD

Accurate diagnosis and education of family
Pediatric rheumatologist
Pediatrician

Nurse:

• Disease-related education
• Medication administration (injection teaching)
• Safety monitoring

Social worker:

• Facilitation of school services
• Resource identification (community, government, financial, advocacy groups)

Physical medicine and rehabilitation
Physical therapy:

• Addressing deficits in joint or muscle mobility, limb length discrepancies, gait abnormalities, weakness

Occupational therapy:

• Splinting to reduce joint contractures/deformities, lessen stress on joints, adaptive devices for activities of daily living

Consultant team
Ophthalmology:

• Eye screening for uveitis (see Table 149-4)
• Screening for medication-related ocular toxicity (hydroxychloroquine, glucocorticoids)

Nephrology Orthopedics Dermatology Gastroenterology Physical and psychosocial growth and development
Nutrition:

• Addressing undernourishment from systemic illness, obesity/overnourishment due to glucocorticoids

School integration:

• Individualized Educational Plan (IEP) or 504 plan

Peer group relationships Individual and/or family counseling Coordination of care Involvement of patient and family as active team members Communication among health care providers Involvement of school (school nurse) and community (social worker) resources

Therapeutics

A key principle of pharmacologic management of rheumatic diseases is that early disease control, striving for induction of remission, leads to less tissue and organ damage with improved short- and long-term outcomes. Medications are chosen from broad therapeutic classes on the basis of diagnosis, disease severity, anthropometrics, and adverse effect profile. Many of therapeutics used do not have U.S. Food and Drug Administration (FDA) indications for pediatric rheumatic diseases. The evidence base may be limited to case series, uncontrolled studies, or extrapolation from use in adults. The exception is JIA, for which there is a growing body of randomized control trial evidence, particularly for newer therapeutics. Therapeutic agents used for treatment of childhood rheumatic diseases (Table 148-2) have various mechanisms of action, but all suppress inflammation. Both biologic and nonbiologic disease-modifying antirheumatic drugs (DMARDs) directly affect the immune system. DMARDs should be prescribed by specialists. Live vaccines are contraindicated in patients taking immunosuppressive glucocorticoids or DMARDs. A negative test result for tuberculosis (purified protein derivative [PPD]) should be verified and the patient’s immunization status updated, if possible, before such treatment is initiated. Killed vaccines are not contraindicated, and annual injectable influenza vaccine is recommended in children with rheumatic diseases.

Table 148-2 THERAPEUTICS FOR CHILDHOOD RHEUMATIC DISEASES*

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Nonsteroidal Anti-Inflammatory Drugs

Nonsteroidal anti-inflammatory drugs (NSAIDs) are prescribed to decrease both pain symptoms and the acute and chronic inflammation associated with arthritis, pleuritis, pericarditis, uveitis, and cutaneous vasculitis, but they are not disease modifying. NSAID anti-inflammatory effects require regular administration at adequate doses based on weight (mg/kg) or body surface area (mg/m2), for longer periods than needed for analgesia alone. The mean time to achieve anti-inflammatory effect in JIA is 4-6 wk of consistent administration. NSAIDs work primarily by inhibiting the enzyme cyclo-oxygenase (COX), which is critical in the production of prostaglandins, a family of substances that promote inflammation. Two types of COX receptors have been demonstrated; selective COX-2 inhibitors (such as celecoxib and meloxicam) inhibit receptors responsible for promoting inflammation with potential for fewer gastrointestinal (GI) adverse effects. Clinical trials in children with JIA found celecoxib and meloxicam to be similar, in effectiveness and tolerability, to the nonselective NSAID naproxen.

The most frequent adverse effects of NSAIDs in children are nausea, decreased appetite, and abdominal pain. Gastritis or gastric or duodenal ulceration occurs less frequently in children. Less common adverse effects, occurring in ≤ 5% of children undergoing long-term NSAID therapy, include mood change, concentration difficulty that can simulate attention deficit disorder, sleepiness, irritability, headache, tinnitus, alopecia, anemia, elevated liver enzyme values, proteinuria, and hematuria. Certain agents (indomethacin) have a higher risk of toxicity than others (ibuprofen); naproxen has an intermediate risk. These NSAID-associated adverse effects reverse quickly once the medication is stopped. Several NSAID-specific adverse reactions may also occur. Ibuprofen can induce aseptic meningitis, primarily affecting patients with lupus. Naproxen is more likely than other NSAIDs to cause a unique skin reaction called pseudoporphyria, which is characterized by small hypopigmented depressed scars occurring in areas of minor skin trauma, such as fingernail scratches, or after small spontaneous vesicular lesions. Pseudoporphyria is more likely to occur in fair-skinned individuals and on sun-exposed areas. If pseudoporphyria develops, the inciting NSAID should be discontinued because scars can persist for years or be permanent. NSAIDs should be used cautiously in patients with dermatomyositis or systemic vasculitis because of an increased frequency of GI ulceration with these disorders. Salicylates have been supplanted by other NSAIDs owing to the relative frequency of salicylate hepatotoxicity and these agents’ association with Reye syndrome.

The response to NSAIDs varies greatly among individual patients, but overall, 40-60% of children with JIA experience improvement in their arthritis with NSAID therapy. Patients may try several different NSAIDs for 6-wk trials before finding one that demonstrates clinical benefit. NSAIDs with longer half-lives or sustained-release formulations allow for once- or twice-daily dosing and improve compliance. Laboratory monitoring for toxicity includes a complete blood count (CBC), serum creatinine, liver function tests (LFTs), and urinalysis every 6-12 mo, though guidelines for frequency of testing are not established.

Nonbiologic Disease-Modifying Antirheumatic Drugs

Methotrexate

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