Author: Hemalatha Srinivasalu, MD
10-year-old female presents with joint pain over knees, heels, ankles, shoulders and neck over past 2-3 months. She has joint swelling, and morning stiffness. Pain increases with physical activity, using the stairs hurts her knees. She has pain over ribcage and pain with taking deep breaths. She was an avid soccer player and has had to stop playing due to pain.
Review of systems: Positive for decreased energy, oral ulcerations, abdominal pain, nausea, dysuria, eczema and headaches.
No significant past medical history.
Family history: Maternal grandmother – juvenile idiopathic arthritis (JIA) and thyroid disorder; Maternal aunt – kidney disease and thyroid disorder; Paternal grandfather: rheumatoid arthritis. No psoriasis, inflammatory bowel disease, celiac disease or lupus in the family
Immunizations: up to date
Allergies: no known drug allergies
Medications: Ibuprofen 400 mg three times daily
Vital Signs: Normal.
General, head and neck, cardiovascular, respiratory, and neurologic systems: Normal.
Skin: No rashes. Normal nails.
Labs – Normal ESR, CRP. ANA neg, RF neg, HLA B27 neg
X-rays of ankles, hands, knees and back – all normal
What form of juvenile idiopathic arthritis (JIA) does the patient have?
- Psoriatic JIA (JPsA)
- Polyarticular JIA
- Extended oligoarticular JIA
- Enthesitis related arthritis (ERA)
4. Enthesitis related arthritis (ERA)
Explanation: JIA is classified according to the ILAR classification system (1) into 7 sub-types, each of which have a set of inclusion and exclusion criteria. Of these, enthesitis related arthritis, psoriatic arthritis and undifferentiated JIA fall under the spectrum of juvenile spondyloarthritis. ERA and PsA are mutually exclusive in this classification system. Incongruity of classification system with that of adult spondyloarthritis is confusing both in clinical practice and research. There is impetus to consider juvenile arthritis as a continuum of adult arthritis (2).
According to the ILAR criteria, the definition of ERA requires either
- Arthritis and Enthesitis; or
- Arthritis or Enthesitis plus two or more of the following: 1) sacroiliac joint tenderness and/or inflammatory spinal pain; 2) HLA-B27 positivity; 3) arthritis in a male over 6 years of age; 4) family history in ankylosing spondylitis, ERA, sacroiliitis with IBD, reactive arthritis; or acute anterior uveitis; or 5) acute anterior uveitis
Exclusions are: 1) Psoriasis or a history of psoriasis in the patient or first-degree relative; 2) Presence of RF on at least 2 occasions at least 3 months apart; 3) Systemic JIA
JPsA is defined as
- Arthritis and psoriasis; or
- Arthritis plus at least 2 of the following: 1) Dactylitis; 2) Nail pitting or onycholysis; 3) Psoriasis in a first-degree relative.
Exclusions are: 1) Arthritis in an HLA-B27 positive male after 6 years of age; 2) Ankylosing spondylitis, ERA, sacroiliitis with IBD, reactive arthritis or acute anterior uveitis, or a history of one of these disorders in a first degree relative; 3) Presence of RF on at least 2 occasions at least 3 months apart; 4) Systemic JIA
She was started on naproxen 15 mg/kg/day by mouth twice daily and physical therapy. She continued to have active arthritis and enthesitis after 6 weeks on naproxen. She was having increasing difficulty ambulating and performing activities of daily living. She was hence started on oral prednisone 2 weeks ago as bridge therapy.
What should be the next step?
- Start a TNFi
- Continue systemic corticosteroids with plan to taper over 6 months
- Corticosteroid injection of affected joints and entheses
- Start a non-biologic DMARD such as Sulfasalazine or Methotrexate
1. Start a TNFi
In 2019, ACR published guidelines for treatment of JIA (3). In children with JIA and active enthesitis despite treatment with NSAIDs, using a TNFi is conditionally recommended over methotrexate or sulfasalazine. While TNFi is preferred, a trial of methotrexate or sulfasalazine may be warranted in the following scenarios: a) contraindications to TNFi, b) mild enthesitis, and c) concomitant active peripheral polyarthritis. Use of systemic corticosteroids must be limited to less than 3 months as a bridging therapy during initiation or escalation of therapy. Bridging therapy may be of most benefit when patients have high disease activity, limited mobility, and/or significant symptoms.
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- Petty RE, Southwood TR, Manners P, Baum J, Glass DN, Goldenberg J, et al. International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001. The Journal of rheumatology. 2004;31(2):390-2.
- Nigrovic PA, Colbert RA, Holers VM, Ozen S, Ruperto N, Thompson SD, et al. Biological classification of childhood arthritis: roadmap to a molecular nomenclature. Nature reviews Rheumatology. 2021.
- Ringold S, Angeles-Han ST, Beukelman T, Lovell D, Cuello CA, Becker ML, et al. 2019 American College of Rheumatology/Arthritis Foundation Guideline for the Treatment of Juvenile Idiopathic Arthritis: Therapeutic Approaches for Non-Systemic Polyarthritis, Sacroiliitis, and Enthesitis. Arthritis & rheumatology (Hoboken, NJ). 2019;71(6):846-63.