Psoriatic juvenile idiopathic arthritis: Pathogenesis, clinical manifestations, and diagnosis
- Peter A Nigrovic, MD
Peter A Nigrovic, MD
- Associate Professor of Medicine
- Harvard Medical School
Psoriatic juvenile idiopathic arthritis (psJIA), or alternately juvenile psoriatic arthritis (JPsA), is a condition that can range widely in presentation and severity. Frank cutaneous psoriasis is not always evident, and the extent of articular involvement may vary from mild enthesitis (inflammation of sites at which ligaments, tendons, and other fibrous structures insert into bone) to polyarticular involvement of multiple axial (spine and sacroiliac joints) and peripheral joints.
The epidemiology, clinical manifestations, and diagnosis of psJIA are discussed here. The treatment and prognosis of psJIA, the pathogenesis of psoriatic arthritis, and psoriatic arthritis in adults are discussed separately. (See "Psoriatic juvenile idiopathic arthritis: Management and prognosis" and "Clinical manifestations and diagnosis of psoriatic arthritis" and "Treatment of psoriatic arthritis" and "Pathogenesis of psoriatic arthritis".)
The prevalence of psJIA is not known with certainty, and there appears to be considerable geographic variation. A psoriatic diathesis is evident in children with JIA at a far higher rate than in the general pediatric population . PsJIA represents approximately 7 percent (range: 0 to 11 percent) of all patients with JIA in series that include only patients with frank psoriasis or using International League of Associations for Rheumatology (ILAR) criteria [2-15]. Series employing the more inclusive Vancouver criteria (see 'Diagnosis' below) suggest that psJIA represents 8 to 20 percent of JIA [2,5,6,16]. By contrast, psoriasis occurs in only approximately 0.5 to 1 percent of all children, rising to 2 to 3 percent in adulthood [17-19]. The incidence and prevalence of psJIA among children with psoriasis is not defined.
In the pediatric population, the age at onset of psJIA is bimodal [2,5,15,20]. A first peak (mainly in girls) occurs during the preschool years and bears clinical similarity to early-onset oligoarticular JIA. The second peak is seen during middle to late childhood and resembles adult psoriatic arthritis. PsJIA is very uncommon before the age of one year. (See 'Clinical manifestations' below.)
psJIA is clinically diverse. Inflammation may involve only one joint or many, with or without involvement of the sacroiliac joints, spine, or peripheral entheses. Some of this variability in psJIA as a whole is attributable to divergent presentations in younger and older children. Younger children, presenting before the age of five to six years, are clinically similar in many ways to early-onset oligoarticular JIA. They are more likely to be female and to have a positive antinuclear antibody (ANA) titer [5,15,21]. Several features do distinguish these children from those with non-psJIA, including dactylitis (a sausage-like swelling of individual digits) and a tendency of the arthritis to involve the wrists and small joints of the hands and feet. Some patients progress to more widespread joint involvement [6,22-24]. Older children with psJIA, typically adolescents, exhibit a gender ratio closer to 1:1 and resemble adults with psoriatic arthritis. They tend to develop enthesitis and spinal or sacroiliac disease [3,5,15,21,25].To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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- CLINICAL MANIFESTATIONS
- Arthritis of the spine and sacroiliac joints
- Skin and nail disease
- Other systemic manifestations
- LABORATORY FINDINGS
- RADIOLOGIC STUDIES
- Diagnostic criteria
- DIFFERENTIAL DIAGNOSIS
- SOCIETY GUIDELINE LINKS
- INFORMATION FOR PATIENTS