Essential Pharmacist Counseling For Systemic Juvenile Idiopathic Arthritis Treatment and Complications

Essential Pharmacist Counseling For Systemic Juvenile Idiopathic Arthritis Treatment and Complications

Systemic Juvenile Idiopathic Arthritis Treatment and ComplicationsIt’s tough enough for adults to learn they have arthritis, imagine the challenge when it strikes a 2-year-old child in the form of systemic juvenile idiopathic arthritis. These young patients and their parents must first learn to manage systemic illness, then continue to deal with the symptoms as the arthritis develops and becomes a chronic, potentially disabling condition.

Pharmacists are key professionals for parents because frequent contact when dispensing medications creates the perfect opportunity to quickly check in and see how the child is doing. This also puts you in the best position to educate about the symptoms of life-threatening complications and to offer desperately needed encouragement.

Incidence and Symptoms of Systemic Juvenile Idiopathic Arthritis

Incidence and prevalence rates for juvenile idiopathic arthritis (JIA) are quite divergent due to confounding factors such as the source cohort, geography, and changing classification systems. While the estimated range in the United States is 4 to 14 cases per 100,000, one county in Minnesota reported two years when JIA spiked from their norm of 10 per 100,000 to 51 per 100,000.1 In Europe, incidence rates vary from 1.6 to 23 and prevalence from 3.8 to 400 per 100,000.2

Systemic juvenile idiopathic arthritis (SJIA) is one of six sub-types of JIA. Even though it’s less prevalent than most other types—SJIA represents about 4 to 15 percent of all cases of JIA—the systemic type is associated with significantly more morbidity and mortality.3 Systemic JIA typically arises between 0 to 5 years of age, but its peak age of onset is 2 years and technically it can occur up to the age of 16 years.

The systemic type of juvenile arthritis presents with a triad of symptoms—fever, transient rash, and arthritis—but the specific diagnostic criteria include:

  • Arthritis in at least one joint for at least six weeks
  • Fever lasting at least two weeks and occurring daily for at least three days—marked by a pattern of high spikes occurring once or twice daily at about the same time each day
  • One or more of the following must be present:
    • Evanescent rash—usually on trunk and extremities, salmon colored, nonpruritic, and macular—occasionally very pruritic and resistant to antihistamines
    • Generalized lymphadenopathy
    • Hepatomegaly
    • Splenomegaly
    • Serositis – pericarditis is most common

Children may develop anemia and about 20 percent have abdominal pain. While systemic symptoms resolve over several months, the arthritis often persists into adulthood.

Systemic Juvenile Idiopathic Arthritis Treatment Options

Treatment for SJIA focuses on controlling inflammation, relieving symptoms, and preventing disease- and treatment-related morbidities. It’s often hard to treat and persistent disease puts children at risk for growth impairment, joint damage, and functional limitations. It’s interesting to note that the inflammatory process associated with SJIA may be different from the inflammation underlying other types of juvenile idiopathic arthritis, which can impact the efficacy of standard agents.

Treatment is based on symptom and disease severity and may include any of the following:

  • Nonsteroidal anti-inflammatory drug monotherapy – Initial treatment for mild-to-moderate, non-disabling symptoms. Aspirin is no longer the first choice; commonly used NSAIDs include naproxen, ibuprofen, diclofenac and indomethacin.
  • Biologic agents – For moderate-to-severe disease and when initial symptoms include serositis and/or polyarthritis. Agents that inhibit interleukin-1 or interleukin-6 such as anakinra, canakinumab or tocilizumab are more effective than methotrexate due to differences in underlying mechanisms.4 Biologic DMARDs are added to the regimen for patients with refractory disease who were initially treated with an NSAID plus glucocorticoid.
  • Glucocorticoids – Along with anti-IL-1 or IL-6 agents for serious symptoms—add glucocorticoids for ongoing polyarthritis, fever, and for refractory arthritis.

Emerging evidence suggests there’s a window of opportunity for treating SJIA. As children grow older, chronic disease may become more resistant, while early and aggressive treatment using anti-IL-1 agents can improve the prognosis.5

Counsel Parents to Be Alert for Serious Complications

Children with early SJIA need frequent monitoring due to the risk for medication toxicity and unstable disease that can rapidly escalate. In other words, pharmacists need to step up their efforts to ensure adherence to medical therapy. Also be sure to encourage parents to keep all doctors’ appointments and to follow through on blood tests that are ordered. Beyond your usual medication counseling, it’s vital to educate parents about potentially serious complications associated with SJIA.

Macrophage activation syndrome (MAS) – Roughly 10 percent of children with SJIA develop MAS; subclinical symptoms occur in up to one-third of patients. MAS is a life-threatening emergency, with mortality rates as high as 20 to 30 percent. It’s caused by an uncontrolled immune response with excessive activation and proliferation of T lymphocytes and macrophages, which leads to liver dysfunction, cytopenia, and coagulopathy. Parents should be warned that onset can be acute and instructed to seek immediate medical attention if they see these symptoms:

  • Continuous high fever
  • Lethargy
  • Confusion
  • Mood changes
  • Headaches

Pulmonary disease – Children with SJIA have a significantly higher risk for pulmonary artery hypertension, interstitial lung disease, and alveolar proteinosis, according to a study published in the May 2013 issue of Arthritis Care and Research.6 A large percentage of patients with pulmonary complications also developed MAS. Advise parents to call the doctor if their child experiences:

  • Dyspnea on exertion
  • Shortness of breath
  • Clubbing – deformed finger or toe nails
  • Cough
  • Chest pain

Pharmacist Support for Parents Yields Better Outcomes for Children

Over the long term, pharmacists can continue to support parents by reaching out when refills are dispensed. These parents need all the help they can get, so ask how their child is doing and whether they’ve seen any changes in pain and functioning. Remind them that early treatment is key so they should never take a wait-and-see approach should symptoms worsen. Also be sure to follow up when prescriptions are filled. Keeping in touch will improve treatment adherence, which promotes a healthier life and better prognosis for these young patients.

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Show 6 footnotes

  1. “Juvenile Idiopathic Arthritis in Olmsted County, Minnesota, 1960-2013,” January 2016,
  2. “Prevalence and Incidence of Juvenile Idiopathic Arthritis: A Systematic Review,” March 2014,
  3. “2013 Update of the 2011 American College of Rheumatology Recommendations for the Treatment of Juvenile Idiopathic Arthritis,” October 2013,
  4. “Systemic Juvenile Idiopathic Arthritis: Treatment,” August 2016,
  5. “Molecular Analysis Reveals Potential Treatment Window for Systemic Juvenile Idiopathic Arthritis,” January 2013,
  6. “Pulmonary Hypertension and Other Potentially Fatal Pulmonary Complications in Systemic Juvenile Idiopathic Arthritis,” May 2013,

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