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 [2-14]. Series employing the more inclusive Vancouver criteria (see 'Diagnosis' below) suggest that psJIA represents 8 to 20 percent of JIA [2,5,6,15]. By contrast, psoriasis occurs in only approximately 0.5 to 1 percent of all children, rising to 2 to 3 percent in adulthood [16-18]. 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 [2,5,19]. 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 [5,20]. Several features do distinguish these children from those with nonpsoriatic JIA, 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 [6,21-23]. 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 [3,5,20,24].
- Nigrovic PA. Juvenile psoriatic arthritis: bathwater or baby? J Rheumatol 2009; 36:1861.
- Southwood TR, Petty RE, Malleson PN, et al. Psoriatic arthritis in children. Arthritis Rheum 1989; 32:1007.
- Truckenbrodt H, Häfner R. [Psoriatic arthritis in childhood. A comparison with subgroups of chronic juvenile arthritis]. Z Rheumatol 1990; 49:88.
- Koó E, Balogh Z, Gömör B. Juvenile psoriatic arthritis. Clin Rheumatol 1991; 10:245.
- Stoll ML, Zurakowski D, Nigrovic LE, et al. Patients with juvenile psoriatic arthritis comprise two distinct populations. Arthritis Rheum 2006; 54:3564.
- Flatø B, Lien G, Smerdel-Ramoya A, Vinje O. Juvenile psoriatic arthritis: longterm outcome and differentiation from other subtypes of juvenile idiopathic arthritis. J Rheumatol 2009; 36:642.
- Thomas E, Barrett JH, Donn RP, et al. Subtyping of juvenile idiopathic arthritis using latent class analysis. British Paediatric Rheumatology Group. Arthritis Rheum 2000; 43:1496.
- Hofer MF, Mouy R, Prieur AM. Juvenile idiopathic arthritides evaluated prospectively in a single center according to the Durban criteria. J Rheumatol 2001; 28:1083.
- Krumrey-Langkammerer M, Häfner R. Evaluation of the ILAR criteria for juvenile idiopathic arthritis. J Rheumatol 2001; 28:2544.
- Berntson L, Fasth A, Andersson-Gäre B, et al. Construct validity of ILAR and EULAR criteria in juvenile idiopathic arthritis: a population based incidence study from the Nordic countries. International League of Associations for Rheumatology. European League Against Rheumatism. J Rheumatol 2001; 28:2737.
- Merino R, de Inocencio J, García-Consuegra J. Evaluation of revised International League of Associations for Rheumatology classification criteria for juvenile idiopathic arthritis in Spanish children (Edmonton 2001). J Rheumatol 2005; 32:559.
- Saurenmann RK, Levin AV, Feldman BM, et al. Prevalence, risk factors, and outcome of uveitis in juvenile idiopathic arthritis: a long-term followup study. Arthritis Rheum 2007; 56:647.
- Arguedas O, Fasth A, Andersson-Gäre B, Porras O. Juvenile chronic arthritis in urban San José, Costa Rica: a 2 year prospective study. J Rheumatol 1998; 25:1844.
- Danner S, Sordet C, Terzic J, et al. Epidemiology of juvenile idiopathic arthritis in Alsace, France. J Rheumatol 2006; 33:1377.
- Oen K, Fast M, Postl B. Epidemiology of juvenile rheumatoid arthritis in Manitoba, Canada, 1975-92: cycles in incidence. J Rheumatol 1995; 22:745.
- Ferrándiz C, Bordas X, García-Patos V, et al. Prevalence of psoriasis in Spain (Epiderma Project: phase I). J Eur Acad Dermatol Venereol 2001; 15:20.
- Gelfand JM, Weinstein R, Porter SB, et al. Prevalence and treatment of psoriasis in the United Kingdom: a population-based study. Arch Dermatol 2005; 141:1537.
- Kurd SK, Gelfand JM. The prevalence of previously diagnosed and undiagnosed psoriasis in US adults: results from NHANES 2003-2004. J Am Acad Dermatol 2009; 60:218.
- Stoll ML, Nigrovic PA. Subpopulations within juvenile psoriatic arthritis: a review of the literature. Clin Dev Immunol 2006; 13:377.
- Shore A, Ansell BM. Juvenile psoriatic arthritis--an analysis of 60 cases. J Pediatr 1982; 100:529.
- Huemer C, Malleson PN, Cabral DA, et al. Patterns of joint involvement at onset differentiate oligoarticular juvenile psoriatic arthritis from pauciarticular juvenile rheumatoid arthritis. J Rheumatol 2002; 29:1531.
- Stoll ML, Nigrovic PA, Gotte AC, Punaro M. Clinical comparison of early-onset psoriatic and non-psoriatic oligoarticular juvenile idiopathic arthritis. Clin Exp Rheumatol 2011; 29:582.
- Petty RE. Juvenile psoriatic arthritis, or juvenile arthritis with psoriasis? Clin Exp Rheumatol 1994; 12 Suppl 10:S55.
- Hamilton ML, Gladman DD, Shore A, et al. Juvenile psoriatic arthritis and HLA antigens. Ann Rheum Dis 1990; 49:694.
- Roberton DM, Cabral DA, Malleson PN, Petty RE. Juvenile psoriatic arthritis: followup and evaluation of diagnostic criteria. J Rheumatol 1996; 23:166.
- Helliwell PS, Hetthen J, Sokoll K, et al. Joint symmetry in early and late rheumatoid and psoriatic arthritis: comparison with a mathematical model. Arthritis Rheum 2000; 43:865.
- Ansell B, Beeson M, Hall P, et al. HLA and juvenile psoriatic arthritis. Br J Rheumatol 1993; 32:836.
- Helliwell PS, Hickling P, Wright V. Do the radiological changes of classic ankylosing spondylitis differ from the changes found in the spondylitis associated with inflammatory bowel disease, psoriasis, and reactive arthritis? Ann Rheum Dis 1998; 57:135.
- D'Agostino MA, Said-Nahal R, Hacquard-Bouder C, et al. Assessment of peripheral enthesitis in the spondylarthropathies by ultrasonography combined with power Doppler: a cross-sectional study. Arthritis Rheum 2003; 48:523.
- Healy PJ, Helliwell PS. Measuring clinical enthesitis in psoriatic arthritis: assessment of existing measures and development of an instrument specific to psoriatic arthritis. Arthritis Rheum 2008; 59:686.
- Kamel M, Eid H, Mansour R. Ultrasound detection of heel enthesitis: a comparison with magnetic resonance imaging. J Rheumatol 2003; 30:774.
- Tuttle KS, Vargas SO, Callahan MJ, et al. Enthesitis as a component of dactylitis in psoriatic juvenile idiopathic arthritis: histology of an established clinical entity. Pediatr Rheumatol Online J 2015; 13:7.
- Olivieri I, Barozzi L, Favaro L, et al. Dactylitis in patients with seronegative spondylarthropathy. Assessment by ultrasonography and magnetic resonance imaging. Arthritis Rheum 1996; 39:1524.
- Olivieri I, Salvarani C, Cantini F, et al. Fast spin echo-T2-weighted sequences with fat saturation in dactylitis of spondylarthritis. No evidence of entheseal involvement of the flexor digitorum tendons. Arthritis Rheum 2002; 46:2964.
- Lalande Champetier de Ribes T, Margarit-Coll N, Sans N, et al. [Ultrasound features of entesopathy in patients with psoriatic dactylitis]. J Radiol 2006; 87:639.
- Healy PJ, Groves C, Chandramohan M, Helliwell PS. MRI changes in psoriatic dactylitis--extent of pathology, relationship to tenderness and correlation with clinical indices. Rheumatology (Oxford) 2008; 47:92.
- Padula A, Belsito F, Barozzi L, et al. Isolated tenosynovitis associated with psoriasis triggered by physical injury. Clin Exp Rheumatol 1999; 17:103.
- Butbul YA, Tyrrell PN, Schneider R, et al. Comparison of patients with juvenile psoriatic arthritis and nonpsoriatic juvenile idiopathic arthritis: how different are they? J Rheumatol 2009; 36:2033.
- Lambert JR, Ansell BM, Stephenson E, Wright V. Psoriatic arthritis in childhood. Clin Rheum Dis 1976; 2:339.
- Sills EM. Psoriatic arthritis in childhood. Johns Hopkins Med J 1980; 146:49.
- Morris A, Rogers M, Fischer G, Williams K. Childhood psoriasis: a clinical review of 1262 cases. Pediatr Dermatol 2001; 18:188.
- Farber EM, Carlsen RA. Psoriasis in childhood. Calif Med 1966; 105:415.
- Kumar B, Jain R, Sandhu K, et al. Epidemiology of childhood psoriasis: a study of 419 patients from northern India. Int J Dermatol 2004; 43:654.
- Al-Mutairi N, Manchanda Y, Nour-Eldin O. Nail changes in childhood psoriasis: a study from Kuwait. Pediatr Dermatol 2007; 24:7.
- Scarpa R, Soscia E, Peluso R, et al. Nail and distal interphalangeal joint in psoriatic arthritis. J Rheumatol 2006; 33:1315.
- Tan AL, Benjamin M, Toumi H, et al. The relationship between the extensor tendon enthesis and the nail in distal interphalangeal joint disease in psoriatic arthritis--a high-resolution MRI and histological study. Rheumatology (Oxford) 2007; 46:253.
- Heiligenhaus A, Niewerth M, Ganser G, et al. Prevalence and complications of uveitis in juvenile idiopathic arthritis in a population-based nation-wide study in Germany: suggested modification of the current screening guidelines. Rheumatology (Oxford) 2007; 46:1015.
- Cabral DA, Petty RE, Malleson PN, et al. Visual prognosis in children with chronic anterior uveitis and arthritis. J Rheumatol 1994; 21:2370.
- Stoll ML, Lio P, Sundel RP, Nigrovic PA. Comparison of Vancouver and International League of Associations for rheumatology classification criteria for juvenile psoriatic arthritis. Arthritis Rheum 2008; 59:51.
- Paiva ES, Macaluso DC, Edwards A, Rosenbaum JT. Characterisation of uveitis in patients with psoriatic arthritis. Ann Rheum Dis 2000; 59:67.
- Häfner R, Michels H. Psoriatic arthritis in children. Curr Opin Rheumatol 1996; 8:467.
- WRIGHT V. Rheumatism and psoriasis: a re-evaluation. Am J Med 1959; 27:454.
- Nordstrom BL, Mines D, Gu Y, et al. Risk of malignancy in children with juvenile idiopathic arthritis not treated with biologic agents. Arthritis Care Res (Hoboken) 2012; 64:1357.
- Lee EY, Sundel RP, Kim S, et al. MRI findings of juvenile psoriatic arthritis. Skeletal Radiol 2008; 37:987.
- Calabro JJ. Psoriatic arthritis in children. Arthritis Rheum 1977; 20 Suppl:415.
- Wesołowska H. Clinical course of psoriatic arthropathy in children. Mater Med Pol 1985; 17:185.
- Petty RE, Southwood TR, Manners P, et al. International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001. J Rheumatol 2004; 31:390.
- Cabral DA, Tucker LB. Malignancies in children who initially present with rheumatic complaints. J Pediatr 1999; 134:53.
- CLINICAL MANIFESTATIONS
- Arthritis of the spine and sacroiliac joints
- Skin and nail disease
- Other systemic manifestations
- LABORATORY FINDINGS
- RADIOLOGIC STUDIES
- Diagnostic criteria
- DIFFERENTIAL DIAGNOSIS
- INFORMATION FOR PATIENTS