Contributed by Richard K. Vehe, MD
Patterns of subspecialty referral are a known barrier to care for pediatric patients with arthritis.1 Here we describe a young girl who awoke one morning unable to stand and for whom the path to diagnosis and treatment for arthritis took 6 weeks.
A 16-month-old girl presented to her pediatrician with an inability to stand since that morning. Her examination revealed a lack of 5 degrees of extension of her knee, limited due to pain. The complete blood count was normal, and the C-reactive protein level was 14 mg/L (upper limit of normal: 8 mg/L). The patient was diagnosed with probable transient synovitis of the knee. At 1-week follow-up with an associate, the patient was still unable to bear weight. The knee was noted to be swollen, slightly warm, and restricted in motion by pain. The patient was referred to orthopaedics to rule out septic arthritis.
The orthopaedist saw the patient the next day and felt that the examination was not suggestive of septic arthritis. Radiographs from the lumbar spine to the feet were normal. At follow-up with an associate a week later, the patient still could not bear weight, and the mother mentioned possible finger swelling, but the finger exam was reassuring. An MRI from pelvis to feet was completed under general anesthesia, and visualized asymmetry of fluid in the knees was not felt by the orthopaedist to be significant.
A pediatric orthopaedic opinion was then sought. The orthopaedist hospitalized the patient and consulted pediatric rheumatology. The pediatric rheumatologist noted characteristic signs of arthritis in multiple joints, including swelling, warmth, and loss of range of motion due to pain. After laboratory tests were drawn and a NSAID was prescribed, the patient was quickly discharged.
Management and Outcome
At her 1-week follow-up in the Explorer Clinic with the pediatric rheumatologist, the patient was noted to still have 16 joints with active arthritis. Review of the hospital laboratory work showed that the C-reactive protein level had further increased to 40 mg/L, the erythrocyte sedimentation rate was 80 mm/hr, and signs of inflammatory thrombocytosis and anemia were present. An anti-nuclear antibody test was positive, and a rheumatoid factor test was negative. Collectively these findings suggested polyarticular juvenile idiopathic arthritis (JIA), which was confirmed once the disease duration extended past 6 weeks.
Treatment with methotrexate and subsequently adalimumab, in addition to the NSAID, brought the arthritis completely under control. Now 4 years old, the patient continues to do well, with normal growth and development. Slit-lamp eye examinations to screen for uveitis, a known complication of JIA, have been consistently negative.
The diagnostic journey for JIA is often circuitous. The initial examinations for this patient were diagnostic of arthritis, and the initial diagnostic considerations were appropriate. However, arthritis symptoms and signs can vary over the course of the day, and this may prevent full recognition of the extent of involvement. Such delays are commonplace. In one typical retrospective study of 152 pediatric patients with JIA, the median interval from symptom onset to first pediatric rheumatology assessment was 20 weeks2, and most of the children had been referred to multiple secondary care specialties and many had undergone potentially avoidable procedures before the confirmation of JIA by a pediatric rheumatologist. Early involvement of a pediatric rheumatologist, by phone or referral, can help.
1. Henrickson M. Policy challenges for the pediatric rheumatology workforce: Part I. Education and economics. Pediatr Rheumatol Online J. 2011;9:23.
2. Foster HE, Eltringham MS, Kay LJ, et al. Delay in access to appropriate care for children presenting with musculoskeletal symptoms and ultimately diagnosed with juvenile idiopathic arthritis. Arthritis Rheum. 2007;57:921-927.
Rheumatic diseases affect about 300,000 children in the United States. However, identifying the signs of arthritis and the types of diagnostic input needed can be challenging.Continue reading