Case 328.

HISTORY: A 45-year-old female patient with diffuse osteoarticular pain, particularly low back pain, was referred by a rheumatologist for an updated radiologic evaluation. The patient had experienced these symptoms for many years and was diagnosed with human leukocyte antigen B27-negative spondyloarthritis approximately 11 years prior, based on findings of bilateral erosive sacroiliitis at pelvic radiography (Fig 1A) and bone scintigraphy with technetium 99m methylene diphosphonate (Fig 1B). After 3 years of treatment with a tumor necrosis factor-α inhibitor (adalimumab), which was effective for pain, the patient was lost to follow-up. At the current presentation, approximately 8 years after being lost to follow-up, the patient presented with worsening low back pain. The presence of nonobstructing kidney stones on US images confounded the underlying cause of worsening pain. The patient also experienced fatigue and depressed mood. Routine blood tests revealed a normal blood cell count, creatinine level of 0.64 mg/dL (56.58 µmol/L) (normal range, 0.30-1.1 mg/dL [26.52-97.24 mmol/L]), C-reactive protein level of 1.1 mg/dL (normal, <1 mg/dL), and vitamin D level of 21 ng/mL (52.42 nmol/L) (normal range, 30-100 ng/mL [74.88-249.60 nmol/L]). Noncontrast MRI of the thoracic and lumbar spine (Fig 2), MRI of the sacroiliac joints (Fig 3), and CT of the abdomen and pelvis (Fig 4) were performed.

Efficacy of repeated intravenous infusions of an anti-tumor necrosis factor alpha monoclonal antibody, infliximab, in persistently active, refractory juvenile idiopathic arthritis: results of an open-label prospective study.

OBJECTIVE: To evaluate the efficacy and safety of a chimeric monoclonal anti-tumor necrosis factor alpha antibody (infliximab) with methotrexate (MTX) in juvenile idiopathic arthritis (JIA) with an active polyarticular course that is not responsive to MTX. METHODS: Twenty-four young adults with long-lasting, refractory JIA were enrolled in an open, prospective, 2-year pilot study. Patients received intravenous infliximab at 3 mg/kg of body weight at weeks 0, 2, and 6 and every 8 weeks thereafter, with weekly subcutaneous MTX. RESULTS: The median duration of therapy was 9.1 months. Significant improvements were observed in the number of joints (28-joint count) with active disease (median 6 at baseline, 2 at 2 weeks, 0 at 6 months, 0 at 1 year; P < 0.05). Pain as well as patient's and physician's global assessments of disease status were assessed on 0-100-mm (0 = best; 100 = worst) visual analog scales (VAS). There were significant improvements in VAS pain scores (45 at baseline, 25 at 2 weeks, 8.5 at 6 months, 10 at 1 year; P < 0.05), patient's global assessment of disease status (50 at baseline, 22 at 2 weeks, 11.5 at 6 months, 18 at 1 year; P < 0.05), and physician's global assessment of disease status (50.5 at baseline, 22.5 at 2 weeks, 6.5 at 6 months, 10 at 1 year; P < 0.01). In addition, there were significant improvements in the erythrocyte sedimentation rate (64 mm/hour at baseline, 36 mm/hour at 2 weeks, 23.5 mm/hour at 6 months, 35 mm/hour at 1 year; P < 0.01) and C-reactive protein level (4.9 mg/dl at baseline, 2.8 mg/dl at 2 weeks, 3.1 mg/dl at 6 months, 3.2 mg/dl at 1 year; P < 0.005). The percentage of patients meeting the American College of Rheumatology 20% improvement criteria at each assessment ranged from 54.2% to 86.7%. Of the responses on the Disease Activity Score in 28 joints, 37.5-63.6% were classified as "good," 14.3-33.3% were classified as "moderate," and 18-37.5% were classified as "no response." Twelve patients (50%) had adverse events, and 5 patients (20.8%) withdrew. CONCLUSION: Infliximab plus MTX showed high effectiveness and safety in short- and medium-term treatment of long-lasting refractory JIA. A controlled multicenter clinical trial is needed.

Remission in juvenile chronic arthritis: a cohort study of 683 consecutive cases with a mean 10 year followup.

OBJECTIVE: As continuity of care in our institution allows longterm followup studies, we reviewed the files of all consecutive patients with juvenile chronic (idiopathic) arthritis (JCA) followed since 1970 to establish the frequency of remission. METHODS: Charts of all patients with JCA were reviewed. Relevant variables were entered into a customized database. The presence of remission (lack of signs of disease activity in the absence of antirheumatic therapy for at least 6 mo) during the disease course and at the last visit was assessed. RESULTS: The cohort included 683 patients, 463 females and 220 males. According to the disease onset, 420 had oligoarticular, 108 polyarticular (23 rheumatoid factor positive), and 88 systemic disease; 67 had a juvenile spondyloarthropathy (SpA). For all 4 categories the mean followup period was about 10 years. At the last visit 224 cases were in remission (32.8%). Remission rate was scarcely influenced by age at disease onset, but differed in the different disease categories. Of the total group of 683 patients, 153 (22.4%) were lost to followup (no control for at least 2 years). For all 4 categories the remission rate at the last visit was higher in patients who had been lost to followup: 42.3% versus 29.0% for systemic onset JCA, 20.8% versus 16.5% for polyarticular onset JCA, 44.7% versus 33.6% for pauciarticular onset JCA, and 66.7% versus 26.8% for juvenile SpA. The probability of attaining remission decreased in proportion to delay in entering the tertiary care center (from 35.7% to 22.8%). The rate of remission reached its peak after 5-10 years of followup, after which the trend reversed. CONCLUSION: Childhood arthritis achieved remission in only about one-third of our cases, with differences among disease categories based on the diagnosis.