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

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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.

12. Weiss J, Ilowite NT. Juvenile idiopathic arthritis. Pediatr Clin North Am. 2005;52:413-442, vi.

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.

15. Aoust L, Rossi-Semerano L, Koné-PauL I, et al. Time to diagnosis in juvenile idiopathic arthritis: a French perspective. Orphanet J Rare Dis. 2017;12:43.

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.

20. Prakken B, Albani S, Martini A. Juvenile idiopathic arthritis. Lancet. 2011;377:2138-2149.

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.

23. Nordal EB, Zak M, Berntson L, et al. Juvenile Arthritis Disease Activity Score (JADAS) based on CRP; validity and predictive ability in a Nordic population-based setting. Pediatr Rheumatol Online J. 2011;9(suppl 1):155.

24. Swart JF, Dijkhuizen EHP, Wulffraat NM, et al. Clinical Juvenile Arthritis Disease Activity Score proves to be a useful tool in treat-to-target therapy in juvenile idiopathic arthritis. Ann Rheum Dis. 2018;77:336-342.

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.

26. Shoop-Worrall SJW, Verstappen SMM, McDonagh JE, et al. Long‐term outcomes following achievement of clinically inactive disease in juvenile idiopathic arthritis. Arthritis Rheumatol. 2018;70:1519-1529.

27. Ahn SS, Yoo BW, Jung SM, et al. In-hospital mortality in febrile lupus patients based on 2016 EULAR/ACR/PRINTO classification criteria for macrophage activation syndrome. Sem Arthritis Rheum. 2017;.47:216-221.

28. Yokota S, Mori M, Imagawa T, et al. Proposal for juvenile idiopathic arthritis guidance on diagnosis and treatment for primary care pediatricians and nonpediatric rheumatologists (2007). Mod Rheumatol. 2007;17:353-363.

29. Barut K, Adrovic A, Şahin S, et al. Juvenile idiopathic arthritis. Balkan Med J. 2017;34:90-101.

30. Colebatch-Bourn AN, Edwards CJ, et al. EULAR-PReS points to consider for the use of imaging in the diagnosis and management of juvenile idiopathic arthritis in clinical practice. Ann Rheum Dis. 2015;74:1946-1957.

31. Blazina Š, Markelj G, AvramoviČ MZ, et al. Management of juvenile idiopathic arthritis: a clinical guide. Pediatr Drugs. 2016;18:397-412.

32. Santos MJ, Conde M, Mourão AF, et al. 2016 update of the Portuguese recommendations for the use of biologic therapies in children and adolescents with juvenile idiopathic arthritis. Acta Rheumatol Port. 2016;41:194-212.

33. Steinbrocker 0, Traeger CH, Batterman RC. Therapeutic criteria in rheumatoid arthritis. JAMA. 1949;140:659-662.

34. Oen K, Malleson PN, Cabral D, et al. Disease course and outcome of juvenile rheumatoid arthritis in a multicenter cohort. J Rheumatol. 2002;29:1989-1999.

For example, there is an increased frequency of autoimmune diseases among JIA patients.¹⁸ There are also reports documenting an increased rate of infection, including with enteric pathogens, parvovirus B,¹⁹ rubella, mumps, hepatitis B, Epstein-Barr virus, mycoplasma, and chlamydia.¹⁹ Stress and trauma have also been implicated.¹²

The T-lymphocyte percentage is increased in the synovial fluid of JIA patients, although that percentage varies from subtype to subtype.²⁰ This elevation results in an increase in the number of macrophages, which are induced by secreted cytokines to produce interleukin (IL)-1, IL-6, and tumor necrosis factor alpha (TNF-a). This activity of cellular immunity leads to joint destruction.²¹

Clinical features

The most common signs and symptoms of JIA are arthralgias (39%), arthritis (25%), fever (18%), limping (9%), rash (8%), abdominal pain (1.3%), and uveitis (1.3%).¹⁵ Forty percent of JIA patients are reported to have temporomandibular joint involvement at some point in their life; mandibular asymmetry secondary to condylar resorption and remodeling¹⁷ is the most common presenting complaint—not arthralgia or pain, as would be expected.

Most JIA patients (52%) first present to the emergency department; another 42% present to the office of a general medical practitioner.¹⁵On average, 3 visits to a physician, over the course of approximately 3 months, are made before a definitive diagnosis (usually by a pediatric rheumatologist) is made.¹⁵

Pertinent questions to ask a patient who has a confirmed diagnosis of JIA include the nature, severity, and duration of morning stiffness and pain, as well as any encumbering factors to regular functioning at home or school.²² Different scoring charts can be used to determine the extent of pain and disability, including the Juvenile Arthritis Disease Activity Score (JADAS)²³and the clinical JADAS (cJADAS),²⁴ which measure minimal disease activity²⁵ and clinically inactive disease²⁶ cutoffs.

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