Diagnostic Considerations
Arthritis or arthritis-like symptoms may be present in many conditions. Leukemia is the single most important disease that is mistaken for juvenile idiopathic arthritis (JIA).
Leukemia
Patients with acute lymphocytic leukemia can present with joint pain and arthritis. Expansion of lymphoblasts in bone metaphyses results in pain, which is typically severe and may awaken the child from sleep. Thrombocytopenia is rare in children with JIA; its presence suggests the possibility of leukemia. Lymphocytosis is also uncharacteristic of JIA and likewise raises the possibility of leukemia, particularly when neutropenia is present.
Additional differential diagnoses
Postinfectious arthritis typically affects large joints. This syndrome is clinically indistinguishable from the early phase of JIA, particularly because onset of JIA may be triggered by viral infections; a duration of longer than 6 weeks eventually differentiates JIA.
Acute joint inflammation from JIA needs to be differentiated from septic arthritis, because the 2 disorders may produce a similar clinical picture, with warmth, acute pain, and severely painful range of motion. Although polyarticular symmetrical involvement and distinctive clinical features help to differentiate JIA from pyogenic arthritis, joint aspiration and joint fluid analysis may be indicated.
Thrombocytopenia may be observed in patients with systemic lupus erythematosus (SLE) who present with arthritis, as well as in those with marrow-occupying malignancies (eg, acute lymphocytic leukemia). Thrombocytosis reflects an inflammatory state and often mirrors inflammatory markers in JIA.
Severe joint pain raises the possibility of acute rheumatic fever (also suggested by migratory, but not additive, arthritis with fevers), malignancy with bone marrow–occupying cancers (eg, neuroblastoma, acute lymphocytic leukemia), septic arthritis, and osteomyelitis.
Weight loss without diarrhea is rarely observed in individuals with active JIA but is sometimes associated with anorexia in those with systemic disease. Weight loss is also found in individuals with malignancy, such as acute lymphocytic leukemia or inflammatory bowel disease.
Monoarticular arthritis in a hip is highly unusual in JIA. For isolated hip arthritis, consider Legg-Calvé-Perthes disease, toxic synovitis of the hip, septic arthritis, osteomyelitis, or, in an older child, slipped capital femoral epiphysis or chondrolysis of the hip.
A history of illness in pets and enteritis indicates that the patient might be suffering from reactive arthritis. A history of travel to an endemic area with exposure to ticks suggests the possibility of Lyme disease.
GI symptoms, microcytic anemia, and elevated inflammatory markers raise the possibility of inflammatory bowel disease.
Differential Diagnoses
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Lyme Disease
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Patient with active polyarticular arthritis. Note swelling (effusions) of all proximal interphalangeal (PIP) joints in addition to boney overgrowth. Also note lack of distal interphalangeal joint (DIP) involvement. The patient has interosseus muscle wasting (observed on the dorsum of the hands), and subluxation and ulnar deviation of the wrists are present. Image courtesy of Barry L. Myones, MD.
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Wrist radiographs of the patient with active polyarticular arthritis shown in Media file 2. Note severe loss of cartilage in the intercarpal spaces and the radiocarpal space of the right wrist. A large erosion is present in the articular surface of the ulnar epiphysis. The view of the left wrist shows boney ankylosis involving the lateral 4 carpal bones with sparing of the pisiform. Erosions are present in the distal radius and ulna. Almost a loss of cartilage has occurred between the radius and ulna and the carpus. Narrowing of the carpal/metacarpal joints is present. Image courtesy of Barry L. Myones, MD.
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Close-up of the proximal interphalangeal (PIP) effusions in the patient with active polyarthritis shown in Media files 2 and 3. Synovial thickening and effusion, as well as boney overgrowth, are present at the PIP joints bilaterally. Image courtesy of Barry L. Myones, MD.
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Patient with inactive polyarticular arthritis. Long-term sequelae of polyarticular disease includes joint subluxation (note both wrists and thumbs), joint contractures (at proximal interphalangeal joints [PIPs] and distal interphalangeal joints [DIPs]), boney overgrowth (at all PIPs), and finger deformities (eg, swan-neck or boutonniere deformities). Image courtesy of Barry L. Myones, MD.
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Hand and wrist radiographs of the patient with inactive polyarticular arthritis shown in Media file 5. Long-term sequelae of polyarticular disease includes periarticular osteopenia, generalized increase in the size of epiphyses, accelerated bone age, narrowed joint spaces (especially at the fourth and fifth proximal interphalangeal joints [PIPs] bilaterally), boutonniere deformities (at left third and fourth interphalangeal joints), and medial subluxation of the first metacarpophalangeal joints (MCPs) bilaterally. Flattening and erosion of the radial carpal articular surface is present in both wrists. Mild narrowing of the joint spaces exists at the carpometacarpal joints and intercarpal rows bilaterally, with sclerotic change of the intercarpal row (right > left). The trapezium and trapezoid may be fused bilaterally. Image courtesy of Barry L. Myones, MD.
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Sequelae of chronic anterior uveitis. Note the posterior synechiae (weblike attachments of the pupillary margin to the anterior lens capsule) of the right eye secondary to chronic anterior uveitis. This patient has a positive antinuclear antibodies (ANAs) and initially had a pauciarticular course of her arthritis. She now has polyarticular involvement but no active uveitis. Image courtesy of Carlos A. Gonzales, MD.
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One set of suggested algorithms for the treatment of patients with juvenile arthritis. This should not be considered dogmatic because treatment is not standardized and remains empiric and, at times, controversial.
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Systemic juvenile idiopathic arthritis (JIA) rash.
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Child with pericardial effusion due to systemic onset juvenile idiopathic arthritis (JIA).
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Flexion and extension views of C-spine in child with poorly controlled polyarticular juvenile idiopathic arthritis (JIA).
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Temporal-mandibular joint (TMJ) MRI postgadolinium infusion. Abnormal increased uptake indicative of synovitis in child with polyarticular juvenile idiopathic arthritis (JIA).
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Eighteen-month-old girl with arthritis in her right knee. Note the flexion contracture of that knee.
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Ankylosis in the cervical spine at several levels due to long-standing juvenile rheumatoid arthritis (also known as juvenile idiopathic arthritis).
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Widespread osteopenia, carpal crowding (due to cartilage loss), and several erosions affecting the carpal bones and metacarpal heads in particular in a child with advanced juvenile rheumatoid arthritis (also known as juvenile idiopathic arthritis).
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(A) T2-weighted MRI shows high signal in both hips, which may be due to hip effusions or synovitis. High signal intensity in the left femoral head indicates avascular necrosis. (B) Coronal fat-saturated gadolinium-enhanced T1-weighted MRI shows bilateral enhancement in the hips. This indicated bilateral active synovitis, which is most pronounced on the right. Because the image was obtained with fat saturation, the hyperintensity in both hips is pathologic, reflecting an inflamed pannus.
Tables
Classification |
ACR(1977) |
ILAR (1997) |
Nomenclature |
Juvenile rheumatoid arthritis |
Juvenile idiopathic arthritis |
Minimum duration |
≥6 wk |
≥6 wk |
Age at onset |
< 16 y |
< 16 y |
≤ 4 joints in first 6 mo after presentation |
Pauciarticular juvenile rheumatoid arthritis |
Oligoarticular juvenile idiopathic arthritis: (A) Persistent < 4 joints for course of disease; (B) Extended >4 joints after 6 mo |
>4 joints in first 6 mo after presentation |
Polyarticular juvenile rheumatoid arthritis |
Polyarticular juvenile idiopathic arthritis-rheumatoid factor negative Polyarticular juvenile arthritis-rheumatoid factor positive |
Fever, rash, arthritis |
Systemic juvenile rheumatoid arthritis |
Systemic juvenile idiopathic arthritis |
Other categories included |
Exclusion of other forms |
Psoriatic juvenile idiopathic arthritis Enthesitis-related arthritis Undifferentiated: (A) Fits no other category; (B) Fits more than 1 category |
Inclusion of psoriatic arthritis, inflammatory bowel disease, juvenile ankylosing spondylitis |
No |
Yes |
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- Overview
- Presentation
- DDx
- Workup
- Treatment
- Approach Considerations
- History of Arthritis in 4 or Fewer Joints
- History of Arthritis in 5 or More Joints
- Active Sacroiliac Arthritis
- Systemic Arthritis with Active Systemic Features and without Active Arthritis
- Systemic Arthritis with Active Arthritis and without Active Systemic Features
- Hospital Admission
- Exercise and Other Nonpharmacologic Therapy
- Surgical Treatment
- Treatment of Macrophage Activation Syndrome
- Treatment of Uveitis
- Diet and Activity
- Consultations
- Long-Term Monitoring
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- Medication
- Questions & Answers
- Media Gallery
- Tables
- References
