Juvenile idiopathic arthritis: Old disease, new tactics

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Applied Evidence

Juvenile idiopathic arthritis: Old disease, new tactics

J Fam Pract. 2019 March;68(2):E8-E13

By

Tobe Momah, MD

Linda Ray, MD

Beyond NSAIDs and disease-modifying antirheumatic drugs are now biologic agents and anti-interleukin drugs that can augment therapy.

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PRACTICE RECOMMENDATIONS

› Pair the findings of your clinical exam with the results of imaging and laboratory testing to make the diagnosis of juvenile idiopathic arthritis (JIA), as it is a diagnosis of exclusion. B

› Individualize treatment based on where the patient falls in the JIA disease spectrum to increase the likelihood that medical therapy will be effective. A

› Consider treating diagnosed JIA with an available biologic agent, which can provide a long asymptomatic period. B

Strength of recommendation (SOR)

A Good-quality patient-oriented evidence
B Inconsistent or limited-quality patient-oriented evidence
C Consensus, usual practice, opinion, disease-oriented evidence, case series

References

1. Adriano LS, de França Fonteles MM, de Fátima Menezes Azevedo M, et al. Medication adherence in patients with juvenile idiopathic arthritis. Rev Bras Reumatol Engl Ed. 2017;57:23-29.

2. Akioka S. A better understanding of juvenile idiopathic arthritis with classification criteria. Nihon Rinsho Meneki Gakkai Kaishi. 2016;39:513-521.

3. Thierry S, Fautrel B, Lemelle I, Guillemin F. Prevalence and incidence of juvenile idiopathic arthritis: a systematic review. Joint Bone Spine. 2014;81:112-117.

4. Petty RE, Laxer RM, Lindsley CB, et al. Pediatric Rheumatology. Philadelphia, PA: Elsevier; 2016:188-201.e6.

5. Scott C, Brice N. Juvenile idiopathic arthritis–an update on its diagnosis and management. S Afr Med J. 2015;105:1077.

6. Giancane G, Consolaro A, Lanni S, et al. Juvenile idiopathic arthritis: diagnosis and treatment. Rheumatol Ther. 2016;3:187-207.

7. Criteria for the classification of juvenile rheumatoid arthritis. Bull Rheum Dis. 1972;23:712-719.

8. Wood PHN: Special meeting on nomenclature and classification of arthritis in children. In: Munthe E, ed. The Care of Rheumatic Children. Basel, Switzerland: EULAR Publishers; 1978:47-50.

9. Petty RE, Southwood TR, Baum J, et al. Revision of the proposed classification criteria for juvenile idiopathic arthritis: Durban, 1997. J Rheumatol. 1998;25:1991-1994.

10. Petty RE, Southwood TR, Manners P, et al; International League of Associations for Rheumatology. International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001. J Rheumatol. 2004;31:390-392.

11. Basra HAS, Humphries PD. Juvenile idiopathic arthritis: what is the utility of ultrasound? Br J Radiol. 2017;90:20160920.

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13. Fujikawa S, Okuni M. A nationwide surveillance study of rheumatic diseases among Japanese children. Acta Pediatric Jpn. 1997:39:242-244.

14. Ozen S, Karaaslan Y, Ozdemir O, et al. Prevalence of juvenile chronic arthritis and familial Mediterranean fever in Turkey: a field study. J Rheumatol. 1998;25:2445-2449.

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16. Moe N, Rygg M. Epidemiology of juvenile chronic arthritis in northern Norway; a ten-year retrospective study. Clin Exp Rheumatol. 1998;16:99-101.

17. Abramowicz S, Kim S, Prahalad S, et al. Juvenile arthritis: current concepts in terminology, etiopathogenesis, diagnosis, and management. Int J Oral Maxillofac Surg. 2016;45:801-812.

18. Prahalad S, Shear ES, Thompson SD, et al. Increased prevalence of familial autoimmunity in simplex and multiplex families with juvenile rheumatoid arthritis. Arthritis Rheum. 2002;46:1851-1856.

19. Gonzalez B, Larrañaga C, León O, et al. Parvovirus B19 may have a role in the pathogenesis of juvenile idiopathic arthritis. J Rheumatol. 2007;34:1336-1340.

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21. Zhou J, Ding Y, Zhang Y, et al. CD3+CD56+ natural killer T cell activity in children with different forms of juvenile idiopathic arthritis and the influence of etanercept treatment on polyarticular subgroup. Clin Immunol. 2016;176:1-11.

22. Shoop-Worrall SJW, Verstappen SMM, Baildam E, et al. How common is clinically inactive disease in a prospective cohort of patients with juvenile idiopathic arthritis? The importance of definition. Ann Rheum Dis. 2017;0:1-8.

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25. Horneff G, Klein A, Ganser G, et al. Protocols on classification, monitoring and therapy in children’s rheumatology (PRO-KIND): results of the working group polyarticular juvenile idiopathic arthritis. Pediatr Rheumatol Online J. 2017;15:78.

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Juvenile idiopathic arthritis (JIA) is a clinically heterogeneous group of arthritides that are characterized by onset before 16 years of age and defined in part as lasting ≥6 weeks.¹ Significantly, the etiology of JIA is unknown, making it a diagnosis of exclusion.²

The most common autoimmune condition of childhood, JIA has a prevalence of 3.8 to 400 affected children for every 100,000 people.^3,4 As the leading cause of musculoskeletal disability in children,⁵ and comprising 7 categories of disease, JIA must be managed with appropriate initial and ongoing intervention.

The amalgam of care that a JIA patient requires—medical, social, physical, psychological—calls for a primary care physician’s expert ability to collaborate and coordinate with medical specialists and subspecialists, including rheumatology, ophthalmology, social work, physical and occupational therapy, and psychology. The goal? As this article describes, the goal is to provide prompt diagnosis, suitable and effective intervention, and continuity of care. (JIA is a lifelong disease, in many cases.)

How JIA is classifiedfor diagnosis and treatment

JIA comprises 7 categories, or classes.⁶ The scheme devised by the International League of Associations for Rheumatology (ILAR), now widely accepted, classifies JIA on the basis of clinical and biochemical markers that aid detection and treatment of the disorder, as well as research. (See “How efforts to classify JIA have caused confusion.”^7-10) The ILAR classes (TABLE¹¹) are:

enthesitis-related arthritis (ERA)
extended oligo-articular JIA (eoJIA), which involves ≤4 joints
juvenile psoriatic arthritis (jPsA)
rheumatoid factor (RF)-positive polyarticular JIA (RF+ pJIA)
RF-negative polyarticular JIA (RF– pJIA)
systemic-onset JIA (sJIA)
undifferentiated JIA, which, generally, involves ≥4 joints.

SIDEBAR
How efforts to classiy JIA have caused confusion^7-10

Various classifications of juvenile arthritis have been proposed and used over the past 3 decades. First was the American College of Rheumatology’s 1972 criteria for juvenile rheumatoid arthritis⁷; next came the European League against Rheumatism (EULAR) criteria for juvenile chronic arthritis, developed in 1977.⁸ Being contemporaneous, the 2 classifications led to a complicated, dichotomous definition of JIA among clinicians and researchers.

As a result of this disarray, the 1997 Durban, South Africa, meeting of the Pediatric Standing Committee of the International League of Associations for Rheumatology (ILAR)⁹ proposed that juvenile idiopathic arthritis be adopted as the umbrella term for the misunderstood terms juvenile rheumatoid arthritis and juvenile chronic arthritis. The intent of including “idiopathic” in the term was to acknowledge that the cause of these diseases was (and is still) unknown.

The novel classification proposed by the Pediatric Standing Committee was followed, in 2001, by an ILAR task force meeting in Edmonton, Alberta, Canada, on the classification of childhood arthritis. The outcome was a recommendation to add exclusion and inclusion criteria, to make all classes of JIA mutually exclusive.¹⁰ Most recently, as discussed in the body of this article, updated ILAR guidelines on JIA classification emphasize 1) heterogeneity among the 7 disease subtypes and 2) the fact that overlapping and exclusive features exist from class to class.

Updated guidelines regarding the 7 ILAR classes of JIA emphasize heterogeneity among disease subtypes, with overlapping and exclusive features noted from class to class.¹¹

Extended oligo-articular JIA (27%-56%), pJIA (13%-35%), sJIA (4%-17%), and ERA,(3%-11%) are the most common JIA subtypes,¹²with age of onset and sex predilection differing according to JIA class.¹¹ The disease occurs more often in girls than in boys,¹¹and the predisposition is higher among Whites and Asians. The incidence of JIA (all classes taken together, for every 100,000 people) is: in Japan, 10 to 15 cases¹³; in Turkey, 64 cases¹⁴; in Norway, 65 cases¹⁵; and in the United States and Canada, taken together, 10 to 15 cases.¹⁶

What causes JIA?

The etiology of JIA remains unclear. It is known that the disease involves inflammation of the synovium and destruction of hard and soft tissues in joints.¹⁷It has been postulated, therefore, that a combination of genetic, environmental, and immunogenic mechanisms might be responsible for JIA.

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