Case 1: Enthesitis related arthritis case
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, psoriasis or 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
On examination:
Normal vital signs; General, head and neck, cardiovascular, respiratory, and neurologic systems: Normal.
Skin: No rashes. Normal nails.
Musculoskeletal system:
Joint exam: Areas shaded in blue in the homunculus had signs of active arthritis
Entheseal exam: Blue dots indicate areas of active enthesitis
Investigations:
Labs – Normal ESR, CRP. ANA neg, RF neg, HLA B27 neg
X-rays of ankles, hands, knees and back – all normal
Question: What form of juvenile idiopathic arthritis does the patient have?
- Psoriatic JIA
- Polyarticular JIA
- Extended oligoarticular JIA
- Enthesitis related arthritis
4. Enthesitis related arthritis
Explanation: ILAR criteria for JIA defines enthesitis related arthritis (1) as requiring 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
She was started on naproxen, sulfasalazine 500 mg (13.8 mg/kg) by mouth twice daily and physical therapy. She continued to have active arthritis and enthesitis after 3 months on naproxen and sulfasalazine combination. She could not tolerate Methotrexate due to persistent nausea.
What should be the next step?
- Start a TNFi
- Start systemic corticosteroids with plan to taper over 6 months
- Corticosteroid injection of affected joints and entheses
- Continue same therapy for additional 3 months
1. Start a TNFi
In 2019, ACR published guidelines for treatment of JIA (2).
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 patients with c) concomitant active peripheral polyarthritis. Limited course of oral glucocorticoids for less than 3 months is recommended as bridging therapy during initiation or escalation of therapy.
References
- Petty RE, Southwood TR, Baum J, et al. Revision of the proposed classification criteria for juvenile idiopathic arthritis: Durban 1997. J Rheumatol 1998;25:1991-4.
- Ringold S, Angeles-Han ST, Beukelman T 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 Care Res (Hoboken). 2019 Jun;71(6):717-734. doi: 10.1002/acr.23870. Epub 2019 Apr 25.
