Juvenile Arthritis - AAFP Home · 2020-07-07 · arthritis: recommendations for the medical therapy...

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1 Juvenile Arthritis Sarah Merrill, MD ACTIVITY DISCLAIMER The material presented here is being made available by the American Academy of Family Physicians for educational purposes only. Please note that medical information is constantly changing; the information contained in this activity was accurate at the time of publication. This material is not intended to represent the only, nor necessarily best, methods or procedures appropriate for the medical situations discussed. Rather, it is intended to present an approach, view, statement, or opinion of the faculty, which may be helpful to others who face similar situations. The AAFP disclaims any and all liability for injury or other damages resulting to any individual using this material and for all claims that might arise out of the use of the techniques demonstrated therein by such individuals, whether these claims shall be asserted by a physician or any other person. Physicians may care to check specific details such as drug doses and contraindications, etc., in standard sources prior to clinical application. This material might contain recommendations/guidelines developed by other organizations. Please note that although these guidelines might be included, this does not necessarily imply the endorsement by the AAFP.

Transcript of Juvenile Arthritis - AAFP Home · 2020-07-07 · arthritis: recommendations for the medical therapy...

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Juvenile Arthritis

Sarah Merrill, MD

ACTIVITY DISCLAIMERThe material presented here is being made available by the American Academy of Family Physicians for educational purposes only. Please note that medical information is constantly changing; the information contained in this activity was accurate at the time of publication. This material is not intended to represent the only, nor necessarily best, methods or procedures appropriate for the medical situations discussed. Rather, it is intended to present an approach, view, statement, or opinion of the faculty, which may be helpful to others who face similar situations.

The AAFP disclaims any and all liability for injury or other damages resulting to any individual using this material and for all claims that might arise out of the use of the techniques demonstrated therein by such individuals, whether these claims shall be asserted by a physician or any other person. Physicians may care to check specific details such as drug doses and contraindications, etc., in standard sources prior to clinical application. This material might contain recommendations/guidelines developed by other organizations. Please note that although these guidelines might be included, this does not necessarily imply the endorsement by the AAFP.

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DISCLOSUREIt is the policy of the AAFP that all individuals in a position to control content disclose any relationships with commercial interests upon nomination/invitation of participation. Disclosure documents are reviewed for potential conflict of interest (COI), and if identified, conflicts are resolved prior to confirmation of participation. Only those participants who had no conflict of interest or who agreed to an identified resolution process prior to their participation were involved in this CME activity.

All individuals in a position to control content for this session have indicated they have no relevant financial relationships to disclose.

The content of my material/presentation in this CME activity will not include discussion of unapproved or investigational uses of products or devices.

Sarah Merrill, MDAssistant Professor, Department of Family Medicine and Public Health, University of California, San Diego School of Medicine

Dr. Merrill is a board-certified family physician who specializes in sports medicine. In addition to diagnosing and treating injuries associated with athletics, she provides primary care for patients of all ages, including preventive care and treatment of acute and chronic diseases. An avid yoga practitioner and certified yoga instructor, she has a special interest in rehabilitating individuals who have yoga injuries. She also enjoys performing ultrasound-guided diagnostics and procedures. Recently, she published two chapters in the clinical reference text The 5-Minute Sports Medicine Consult, 3rd Edition.Dr. Merrill instructs students, residents, and fellows at UC San Diego School of Medicine, is an assistant program director for the UCSD Family Medicine Residency Program, and is the medical director of UC San Diego Health’s Scripps Ranch Family Medicine Center. She also provides care throughout the San Diego community and for many sporting events, serving as the team physician for the University City High School and Scripps Ranch High School football teams; medical director for the California State Games; medical team captain for San Diego Rock ‘n’ Roll Marathon; and event physician for the BMX World Championships. Dr. Merrill completed a sports medicine fellowship at UC San Diego School of Medicine, where she also completed a residency in family medicine with an additional certification in integrative medicine. She earned her medical degree from Loyola University Chicago Stritch School of Medicine in Illinois. She is a member of numerous professional associations, including the American Medical Society for Sports Medicine (AMSSM), the American Medical Association (AMA), and the AAFP.

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Learning Objectives1. Describe diagnostic principles, appropriate pre-referral

evaluation, and red flags in pediatric rheumatologic diseases.

2. Discuss treatment options, including side effects of common medications and what contraindications to immunizations exist for patients in active treatment.

3. Define the subtypes of Juvenile Idiopathic Arthritis (formerly Juvenile Rheumatoid Arthritis) and identify common signs and symptoms of each subtype.

Audience Engagement SystemStep 1 Step 2 Step 3

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• Variety of autoimmune and inflammatory diseases that affect the joints of children 18 years and younger3

Juvenile Arthritis: Definition

• Genetic and environmental factors3, 4

• Stress

• Trauma

• Gut microbiome

• History of infections4

Pathogenesis

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• “Group of inflammatory disorders that begins before the 18th birthday and persists for at least 6 weeks with other known conditions excluded”13

Juvenile Idiopathic Arthritis

Juvenile Idiopathic Arthritis: Categories

JIA

Systemic JIA RF+ JIAEnthesitis/spondylitis 

related JIAEarly onset ANA+ JIA Other JIA Unclassified JIA

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Systemic JIAFever of unknown origin for > 3 days and recurring for at 

least 2 weeks

Major criteria: erythematous rash and/or arthritis

Minor criteria: generalized LAD/hepatomegaly/splenomegaly, serositis; arthralgia without arthritis; leukocytosis with neutrophila

Need fever + 2 major or 1 major and 2 minor 

Systemic JIA

• Leukocytosis, hypochromic microcytic anemia, thrombocytosis, elevated acute phase reactants4,13

• ANA and RF negative4,13

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Systemic JIA

• Salmon colored rash on trunk and proximal extremities

• Can be polyarticular in both small or large joints

Poll Question #1

Which of the following is not required for the diagnosis of systemic juvenile idiopathic arthritis?A. +ANAB. Fever of unknown originC. ArthralgiaD. A and CE. A and B

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+Rheumatoid Factor JIA

• Arthritis > 6 weeks and 2 +RF tests or +CCP13

+Rheumatoid Factor JIA

• Worst prognosis• Female predominance• Late onset• Symmetric and progressive• Predominantly affects wrists and small

joints hands and feet13

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Enthesitis/spondylitis-related JIA

Peripheral arthritis and enthesitis

Arthritis or enthesitis + >/=3 months of inflammatory back pain and sacroiliitis on xrays

Athritis or enthesitis + two of the following

•SIJ TTP

•Inflammatory back pain

•+HLA‐B27 antigen 

•Acute symptomatic anterior uveitis

•H/o SpA in 1st degree relative

Early onset ANA+ JIA

Arthritis >/= 6 weeksEarly onset (< 6 

years)

Two +ANA at least 3 months apart (titer > 

1/160)

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Other JIA

• Arthritis > 6 weeks

• Does not fit criteria for previous categories

Unclassified JIA

• Arthritis > 6 weeks

• Fits > 1 previous disorder

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Poll Question #2A 13 year female with a 8 week history of joint pain and swelling in bilateral wrist and toes would most likely have the following lab values?A. -ANAB. -RF and -ANAC. +RF and +CCPD. +HLA-B27E. Type O blood

Work Up

• No antibody panels unless positive ANA and evidence of rheum disease!2

• Initial lab testing: ANA, CBC, RF, ESR and CRP1

• Initial imaging: MRI or ultrasound preferred8

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Complications

• Uveitis

• Increased risk of developing CVD4,11

• Increased systolic and diastolic pressures11

• Increased aortic stiffness11

Complications

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• Sleep and psychosocial factors

Complications

Complications

• Growth retardation

• Macrophage activation syndrome

• Multi-organ insufficiency

• Osteoporosis

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Treatment

• Involves multidisciplinary team4, 9

– PCP– Pediatric rheumatologic– Ophthalmologist– Pediatric psychiatrist– Physical therapist– Sports med/ortho

Treatment

• NSAIDs

• DMARDs

• Corticosteroids

• Biologics

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Treatment

• Monitor pain, sleep and psychosocial indicators for both parents and patients6

Prognosis

• FHx disease, early ankle or hip joint involvement, erosions on xray and higher number of joints affected are poor prognostic indicators4, 8

• Remission rate increased and rate of joint damage decreases with early initiation of treatment4

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Follow Up

• Psychosocial monitoring

• Cardiac screening

• Eye screening

Poll Question 4You are evaluating a 3 year old female with fever of unknown origin daily for 2 weeks and joint swelling. You suspect JIA, although are still waiting on lab results and imaging. Which is the most appropriate next step?A. Refer to sports medicine/orthoB. Discuss patient’s sleep habits with patientC. Refer to ophthalmology D. Order EKGE. Order bone marrow biopsy

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Practice Recommendations• JIA is a broad diagnosis of exclusion and does not necessarily

require arthritis/arthralgia• Practitioners need to be aware of presentations in order to start work

ups quickly – better outcomes are associated with early treatment• Initial lab work should include ANA, RF, CBC, ESR, CRP, HLA-B27

and imaging of affected joints• Inclusion of multidisciplinary team is key early in diagnosis and

treatment• Uveitis is the primary extra-articular complication of JIA and needs to

be screened frequently• NSAIDs and DMARDs are first line therapies

Questions

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Resources1. Ali Y. Rheumatologic Tests: A Primer for Family Physicians. American family physician. 2018;98(3):164-170.2. American College of Rheumatolgy. Choosing Wisely: Don’t order antibody panels unless positive antinuclear antibodies (ANA) and evidence of rheumatic disease.

Choosing Wisely https://www.aafp.org/afp/recommendations/viewRecommendation.htm?recommendationId=151. Accessed June 2019.3. Arthritis Foundation. Pediatric Rheumatic Diseases: What are Pediatric Rheumatic Diseases? 2018; https://www.arthritis.org/about-arthritis/types/pediatric-rheumatic-

diseases. Accessed June, 2019.4. Barut, et al. Juvenile Idiopathic Arthritis. Balkan Med J. 2017 Mar, 34(2): 90-101.5. Beukelman T, Patkar NM, Saag KG, et al. 2011 American College of Rheumatology recommendations for the treatment of juvenile idiopathic arthritis: initiation and

safety monitoring of therapeutic agents for the treatment of arthritis and systemic features. Arthritis care & research. 2011;63(4):465-482.6. Bromberg MH, Gil KM, Schanberg LE. Daily sleep quality and mood as predictors of pain in children with juvenile polyarticular arthritis. Health psychology : official

journal of the Division of Health Psychology, American Psychological Association. 2012;31(2):202-209.7. Cassidy J, Kivlin J, Lindsley C, Nocton J. Ophthalmologic Examinations in Children With Juvenile Rheumatoid Arthritis. Pediatrics. 2006;117(5):1843-1845.8. Cellucci T, Guzman J, Petty RE, et al. Management of Juvenile Idiopathic Arthritis 2015: A Position Statement from the Pediatric Committee of the Canadian

Rheumatology Association. The Journal of rheumatology. 2016;43(10):1773-1776.9. Chausset A, Gominon AL, Montmaneix N, et al. Why we need a process on breaking news of Juvenile Idiopathic Arthritis: a mixed methods study. Pediatric

rheumatology online journal. 2016;14(1):31.10. Clark, Sarah, et al. Juvenile idiopathic arthritis-associated uveitis. Pediatri Rheumatol Online J. 2016; 14:27.11. Coulson, Elizabeth, et al. Cardiovascular risk in juvenile idiopathic arthritis. Rheumatology, Vol 52: 7. 07/2013: 1163-1171.12. Hersh AO, Salimian PK, Weitzman ER. Using Patient-Reported Outcome Measures to Capture the Patient's Voice in Research and Care of Juvenile Idiopathic

Arthritis. Rheumatic diseases clinics of North America. 2016;42(2):333-346.13. Martini, et al. Toward new classification criteria for juvenile idiopathic arthritis: first steps, pediatric rheumatology international trials organization international

consensus. Journal of Rheumatology 2019, 46:2.14. Ringold S, Weiss PF, Beukelman T, et al. 2013 update of the 2011 American College of Rheumatology recommendations for the treatment of juvenile idiopathic

arthritis: recommendations for the medical therapy of children with systemic juvenile idiopathic arthritis and tuberculosis screening among children receiving biologic medications. Arthritis and rheumatism. 2013;65(10):2499-2512.

15. Seid M, Huang B, Niehaus S, Brunner HI, Lovell DJ. Determinants of health-related quality of life in children newly diagnosed with Juvenile Idiopathic Arthritis. Arthritis care & research. 2014;66(2):263-269.

16. CME Training Session Needs Survey. In. Leawood KS: AAFP, 2016.17. Rodriguez-Garcia A. The importance of an ophthalmologic evaluation in patients with juvenile idiopathic arthritis. Reumatologia clinica. 2015; 11(3):133-138.18. Junnila JL, Cartwright VW. Chronic musculoskeletal pain in children: part II. Rheumatic causes. American family physicians. 2006; 74 (2): 293-300.19. Garcia-Carrasco M et al. Efficacy of thalomide in systemic onset juvenile rheumatoid arthritis. Joint Bone Spine 2017; 75(2): 500-503. Available

https://doi.org/10.1016/j.jbspin.2006.12.004

Contact Information

Sarah Merrill, MD

UC San Diego Health Systems

[email protected]