Polyarticular juvenile idiopathic arthritis: Clinical manifestations, diagnosis, and complications
- Pamela F Weiss, MD, MSCE
Pamela F Weiss, MD, MSCE
- Associate Professor
- Departments of Pediatrics and Epidemiology
- Perelman School of Medicine, University of Pennsylvania
- Division of Rheumatology, Children's Hospital of Philadelphia
Polyarticular juvenile idiopathic arthritis (formerly called polyarticular-onset juvenile rheumatoid arthritis [JRA]) is a subset of juvenile idiopathic arthritis (JIA) that is defined by the presence of more than four affected joints during the first six months of illness . In the revised nomenclature, this disease, which comprises 20 to 30 percent of patients with JIA, is included in the group termed "childhood polyarthritis." (See "Classification of juvenile arthritis".)
The clinical manifestations, diagnosis, course, complications, and prognosis of polyarticular JIA are reviewed here. The treatment of polyarticular JIA is discussed separately. (See "Polyarticular juvenile idiopathic arthritis: Treatment".)
Polyarticular JIA is more frequent in females than males. There is a bimodal distribution of the age at onset. The first peak is between the ages of two and five years, and the second is between 10 and 14 years. This age distribution suggests that two or more distinct diseases may be included in this classification. The classification, epidemiology, and immunopathogenesis of JIA are reviewed in greater detail separately. (See "Classification of juvenile arthritis" and "Juvenile idiopathic arthritis: Epidemiology and immunopathogenesis".)
The clinical presentation of polyarticular JIA is varied, although it tends to fall into patterns based upon the age of onset.
Younger children — In children less than 10 years of age, polyarticular JIA often begins similarly to oligoarticular disease, with one or two joints affected . The development of the disease is often indolent until an intercurrent infection precipitates a dramatic increase in symptoms. The disease then becomes relentlessly progressive, spreading to involve five or more joints within the first six months after disease onset. Joint involvement is symmetric, with the knees, wrists, and ankles most frequently affected . There are typically periods of apparent response to therapy followed by relapses with an increasing number of involved joints. Polyarticular JIA may go unrecognized at first because of its initial indolent course. This failure to recognize the initial symptoms may make it appear that the disease had a sudden onset and rapid progression.
- Criteria for the classification of juvenile rheumatoid arthritis. Bull Rheum Dis 1972; 23:712.
- Rosenberg AM, Oen KG. Polyarticular juvenile idiopathic arthritis. In: Textbook of pediatric rheumatology, 7th ed, Petty RE, Laxer RM, Lindsley CB, Wedderburn LR (Eds), Elsevier, Philadelphia 2015. p.217.
- Ferucci ED, Majka DS, Parrish LA, et al. Antibodies against cyclic citrullinated peptide are associated with HLA-DR4 in simplex and multiplex polyarticular-onset juvenile rheumatoid arthritis. Arthritis Rheum 2005; 52:239.
- Twilt M, Mobers SM, Arends LR, et al. Temporomandibular involvement in juvenile idiopathic arthritis. J Rheumatol 2004; 31:1418.
- Avcin T, Cimaz R, Falcini F, et al. Prevalence and clinical significance of anti-cyclic citrullinated peptide antibodies in juvenile idiopathic arthritis. Ann Rheum Dis 2002; 61:608.
- Hamooda M, Fouad H, Galal N, et al. Anti-cyclic citrullinated peptide antibodies in children with Juvenile Idiopathic Arthritis. Electron Physician 2016; 8:2897.
- Naidu SH, Ostrov BE, Pellegrini VD Jr. Isolated digital swelling as the initial presentation of juvenile rheumatoid arthritis. J Hand Surg Am 1997; 22:653.
- Chen J, Veras MM, Liu C, Lin J. Methotrexate for ankylosing spondylitis. Cochrane Database Syst Rev 2013; :CD004524.
- Dougados M, van der Heijde D, Sieper J, et al. Symptomatic efficacy of etanercept and its effects on objective signs of inflammation in early nonradiographic axial spondyloarthritis: a multicenter, randomized, double-blind, placebo-controlled trial. Arthritis Rheumatol 2014; 66:2091.
- Haibel H, Rudwaleit M, Listing J, et al. Efficacy of adalimumab in the treatment of axial spondylarthritis without radiographically defined sacroiliitis: results of a twelve-week randomized, double-blind, placebo-controlled trial followed by an open-label extension up to week fifty-two. Arthritis Rheum 2008; 58:1981.
- Barkham N, Keen HI, Coates LC, et al. Clinical and imaging efficacy of infliximab in HLA-B27-Positive patients with magnetic resonance imaging-determined early sacroiliitis. Arthritis Rheum 2009; 60:946.
- Dhib M, Prieur AM, Courville S, et al. Crescentic glomerulonephritis in juvenile chronic arthritis. J Rheumatol 1996; 23:1636.
- Rosenberg AM, Yee EH, MacKenzie JW. Arthritis in childhood sarcoidosis. J Rheumatol 1983; 10:987.
- Horton DB, Sherry DD, Baldassano RN, Weiss PF. Enthesitis is an Extraintestinal Manifestation of Pediatric Inflammatory Bowel Disease. Ann Paediatr Rheumatol 2012; 1.
- Jose FA, Garnett EA, Vittinghoff E, et al. Development of extraintestinal manifestations in pediatric patients with inflammatory bowel disease. Inflamm Bowel Dis 2009; 15:63.
- Hurvitz JR, Suwairi WM, Van Hul W, et al. Mutations in the CCN gene family member WISP3 cause progressive pseudorheumatoid dysplasia. Nat Genet 1999; 23:94.
- Sen M, Cheng YH, Goldring MB, et al. WISP3-dependent regulation of type II collagen and aggrecan production in chondrocytes. Arthritis Rheum 2004; 50:488.
- El-Hallak M, Giani T, Yeniay BS, et al. Chronic minocycline-induced autoimmunity in children. J Pediatr 2008; 153:314.
- Jones OY, Spencer CH, Bowyer SL, et al. A multicenter case-control study on predictive factors distinguishing childhood leukemia from juvenile rheumatoid arthritis. Pediatrics 2006; 117:e840.
- Żuber Z, Dyduch G, Jaworek A, et al. Pachydermodactyly - a report of two cases. Reumatologia 2016; 54:136.
- Liu CY, Zhang YF. Images in clinical medicine. Pachydermoperiostosis. N Engl J Med 2014; 370:1930.
- Guzman J, Oen K, Tucker LB, et al. The outcomes of juvenile idiopathic arthritis in children managed with contemporary treatments: results from the ReACCh-Out cohort. Ann Rheum Dis 2015; 74:1854.
- Shen CC, Yeh KW, Ou LS, et al. Clinical features of children with juvenile idiopathic arthritis using the ILAR classification criteria: a community-based cohort study in Taiwan. J Microbiol Immunol Infect 2013; 46:288.
- Yamasaki Y, Takei S, Imanaka H, et al. Prediction of long-term remission of oligo/polyarticular juvenile idiopathic arthritis with S100A12 and vascular endothelial growth factor. Mod Rheumatol 2016; 26:551.
- Giancane G, Pederzoli S, Norambuena X, et al. Frequency of radiographic damage and progression in individual joints in children with juvenile idiopathic arthritis. Arthritis Care Res (Hoboken) 2014; 66:27.
- Henderson CJ, Specker BL, Sierra RI, et al. Total-body bone mineral content in non-corticosteroid-treated postpubertal females with juvenile rheumatoid arthritis: frequency of osteopenia and contributing factors. Arthritis Rheum 2000; 43:531.
- Lien G, Flatø B, Haugen M, et al. Frequency of osteopenia in adolescents with early-onset juvenile idiopathic arthritis: a long-term outcome study of one hundred five patients. Arthritis Rheum 2003; 48:2214.
- Markula-Patjas KP, Valta HL, Kerttula LI, et al. Prevalence of vertebral compression fractures and associated factors in children and adolescents with severe juvenile idiopathic arthritis. J Rheumatol 2012; 39:365.
- Weiss PF, Arabshahi B, Johnson A, et al. High prevalence of temporomandibular joint arthritis at disease onset in children with juvenile idiopathic arthritis, as detected by magnetic resonance imaging but not by ultrasound. Arthritis Rheum 2008; 58:1189.
- Arvidsson LZ, Smith HJ, Flatø B, Larheim TA. Temporomandibular joint findings in adults with long-standing juvenile idiopathic arthritis: CT and MR imaging assessment. Radiology 2010; 256:191.
- Minden K, Niewerth M, Listing J, et al. Long-term outcome in patients with juvenile idiopathic arthritis. Arthritis Rheum 2002; 46:2392.
- 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.
- 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.
- Cassidy J, Kivlin J, Lindsley C, et al. Ophthalmologic examinations in children with juvenile rheumatoid arthritis. Pediatrics 2006; 117:1843.
- Bolt IB, Cannizzaro E, Seger R, Saurenmann RK. Risk factors and longterm outcome of juvenile idiopathic arthritis-associated uveitis in Switzerland. J Rheumatol 2008; 35:703.
- CLINICAL PRESENTATION
- Younger children
- Older children and adolescents
- LABORATORY FINDINGS
- DIFFERENTIAL DIAGNOSIS
- Reactive arthritis
- Psoriatic JIA
- Enthesitis-related JIA
- Adult-type rheumatoid arthritis
- Systemic JIA
- SLE and mixed connective tissue disease
- Systemic vasculitis
- Juvenile systemic granulomatosis (Blau syndrome)
- Inflammatory bowel disease
- Epiphyseal dysplasia
- Minocycline-induced autoimmunity
- INFORMATION FOR PATIENTS