Abstract
The objectives of this study are to assess the frequency of chronic arthritis and compare the clinical and laboratory features in a large population of childhood-onset systemic lupus erythematosus (cSLE) and adult-onset (aSLE) patients. This historical study evaluated 336 cSLE and 1830 aSLE patients. Chronic arthritis was defined as synovitis of at least 6 weeks of duration. Rhupus was characterised as the association of SLE and chronic inflammatory arthritis with erosion and positive rheumatoid factor. Jaccoud’s arthropathy is a non-erosive subluxation leading to severe deformity of the hands and feet. Data were compared using Student’s t test or the Mann-Whitney test for continuous variables. For categorical variables, differences were assessed by Fisher’s exact test and Pearson chi-square. Frequencies of chronic arthritis were similar in cSLE and aSLE (2.4 vs. 3.8 %, p = 0.261). The median time from disease onset to appearance of chronic arthritis was shorter in cSLE (0 vs. 10 years, p < 0.001), and the median of age at chronic arthritis diagnosis was [10.8 (4.2–14.6) vs. 40 (21–67), p < 0.001]. The children presented with more chronic polyarthritis than the adults (75 vs. 32 %, p = 0.024), a higher median number of joints with arthritis [8.5 (1–18) vs. 3 (1–9), p = 0.017] and a higher number of joints with limitation [1.5(0–24) vs. 0(0–4), p = 0.004]. The chronic arthritis diagnosis frequencies of hepatomegaly (25 vs. 0 %, p = 0.009), splenomegaly (25 vs. 0 %, p = 0.009), pericarditis (25 vs. 0 %, p = 0.009), nephritis (37 vs. 3 % , p = 0.006), haematuria (37 vs. 1.4 %, p = 0.002), lupus anticoagulant (40 vs. 1.6 %, p = 0.012), anticardiolipin IgM (40 vs. 1.5 %, p = 0.012) and median Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) [10.5(1–20) vs. 6(4–16), p = 0.029] were higher in cSLE. Frequency of rhupus, (12 vs. 17 %, p = 1.0), Jaccoud’s arthropathy (0 vs. 17 %, p = 0.343) and treatments were similar in cSLE and aSLE. We determined that chronic arthritis in SLE has distinct features in children, with very early onset, polyarticular involvement and association with active disease. We further demonstrated in this series that a proportion of chronic arthritis involvement in SLE is manifested as rhupus and Jaccoud’s arthropathy.
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References
Tarr T, Dérfalvi B, Győri N, Szántó A, Siminszky Z, Malik A, Szabó AJ, Szegedi G, Zeher M (2015) Similarities and differences between pediatric and adult patients with systemic lupus erythematosus. Lupus 24:796–803
Cavalcante EG, Aikawa NE, Lozano RG, Lotito AP, Jesus AA, Silva CA (2011) Chronic polyarthritis as the first manifestation of juvenile systemic lupus erythematosus patients. Lupus 20:960–964
Richter Cohen M, Steiner G, Smolen JS, Isenberg DA (1998) Erosive arthritis in systemic lupus erythematosus: analysis of a distinct clinical and serological subset. Br J Rheumatol 37:421–424
Tani C, D’Aniello D, Delle Sedie A, Carli L, Cagnoni M, Possemato N, Carbone M, Della Rossa A, Riente L, Baldini C, Talarico R, Caramella D, Bombardieri S, Mosca M (2013) Rhupus syndrome: assessment of its prevalence and its clinical and instrumental characteristics in a prospective cohort of 103 SLE patients. Autoimmun Rev 12:537–541
Ziaee V, Moradinejad MH, Bayat R (2013) RHUPUS Syndrome in children: a case series and literature review. Case Rep Rheumatol 819629
Unsal E, Arli AO, Akman H (2007) Rhupus arthropathy as the presenting manifestation in juvenile SLE: a case report. Pediatr Rheumatol Online J 4(5):7
Hochberg MC (1997) Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 40:1725
Pipili C, Sfritzeri A, Cholongitas E (2008) Deforming arthropathy in systemic lupus erythematosus. Eur J Intern Med 19:482–487
Gladman DD, Ibanez D, Urowitz MB (2002) Systemic lupus erythematosus disease activity index 2000. J Rheumatol 29:288–291
Fernández A, Quintana G, Rondón F, Restrepo JF, Sánchez A, Matteson EL, Iglesias A (2006) Lupus arthropathy: a case series of patients with rhupus. Clin Rheumatol 25:164–167
Ostendorf B, Scherer A, Specker C, Mödder U, Schneider M (2003) Jaccoud’s arthropathy in systemic lupus erythematosus: differentiation of deforming and erosive patterns by magnetic resonance imaging. Arthritis Rheum 48:157–65
Ball EM, Bell AL (2012) Lupus arthritis—do we have a clinically useful classification? Rheumatology 51:771–779
Vugt RM, Derksen RH, Kater L, Bijlsma JW (1998) Deforming arthropathy or lupus and rhupus hands in systemic lupus erythematosus. Ann Rheum Dis 57:540–544
Grossman JM (2009) Lupus arthritis. Best Pract Res Clin Rheumatol 23:495–506
Santiago MB, Galvão V (2008) Jaccoud arthropathy in systemic lupus erythematosus: analysis of clinical characteristics and review of the literature. Medicine (Baltimore) 87:37–44
Gormezano NW, Silva CA, Otsuzi CI, Barros DL, da Silva MA, Sallum AM, Pasoto S, Pereira RM, Bonfá E (2015) Higher prevalence and distinct features of herpes zoster infection in children than adults with systemic lupus erythematosus. Pediatr Infect Dis J
Araujo DB, Borba EF, Silva CA, Campos LM, Pereira RM, Bonfa E, Shinjo SK (2012) Alveolar hemorrhage: distinct features of juvenile and adult onset systemic lupus erythematosus. Lupus 21:872–877
Mina R, Brunner HI (2010) Pediatric lupus—are there differences in presentation, genetics, response to therapy, and damage accrual compared with adult lupus? Rheum Dis Clin North Am 36:53–80
Acknowledgments
We are grateful to Ulysses Doria-Filho for the statistical analysis. This study was supported by grants from Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq 2009/51897-5 to EB, 301805/2013-0 to RMRP and 302724/2011-7 to CAS), Federico Foundation (to EB, RMRP and CAS) and by Núcleo de Apoio à Pesquisa “Saúde da Criança e do Adolescente” da USP (NAP-CriAd) to CAS.
The brief report was read and approved by all authors.
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Gormezano, N.W.S., Silva, C.A., Aikawa, N.E. et al. Chronic arthritis in systemic lupus erythematosus: distinct features in 336 paediatric and 1830 adult patients. Clin Rheumatol 35, 227–231 (2016). https://doi.org/10.1007/s10067-015-3127-3
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DOI: https://doi.org/10.1007/s10067-015-3127-3