Differences in Clinical Features Observed Between Childhood-onset Versus Adult-onset Systemic Lupus Erythematosus: A Systematic Review and Meta-analysis

Overview

Journal Medicine (Baltimore)

Specialty General Medicine

Date 2017 Sep 15

PMID 28906413

Citations 31

Authors

Pravesh Kumar Bundhun

Alka Kumari

Feng Huang

Affiliations

Soon will be listed here.

Abstract

Background: Systemic lupus erythematosus (SLE) affects people in childhood (childhood onset) or in adulthood (adult onset). Observational studies that have previously compared childhood-onset versus adult-onset SLE were often restricted to 1 ethnic group, or to a particular area, with a small sample size of patients. We aimed to systematically compare childhood-onset versus adult-onset SLE through a meta-analysis.

Methods: Electronic databases were searched for relevant publications comparing childhood-onset with adult-onset SLE. Adverse clinical features were considered as the endpoints. The Newcastle Ottawa Scale (NOS) was used to assess the methodological quality of the studies and RevMan software (version 5.3) was used to carry out this analysis whereby risk ratios (RRs) and 95% confidence intervals (95% CIs) were used as the statistical parameters.

Results: A total number of 10,261 participants (1560 participants with childhood-onset SLE and 8701 participants with adult-onset SLE) were enrolled. Results of this analysis showed that compared with childhood-onset SLE, pulmonary involvement was significantly higher with adult-onset SLE (RR: 1.51, 95% CI: 1.18-1.93; P = .001), whereas renal involvement was significantly higher with childhood-onset SLE (RR: 0.65, 95% CI: 0.55-0.77; P = .00001). Raynaud phenomenon and photosensitivity were significantly higher in adult-onset SLE (RR: 1.29, 95% CI: 1.04-1.60; P = .02) and (RR: 1.08, 95% CI: 1.01-1.17; P = .03), respectively. Malar rash significantly favored adult-onset SLE (RR: 0.84, 95% CI: 0.75-0.94; P = .002). Childhood-onset SLE was associated with significantly higher hemolytic anemia, thrombocytopenia, leukocytopenia, and lymphopenia. Seizure and ocular manifestations were significantly higher with childhood-onset SLE (RR: 0.57, 95% CI: 0.47-0.70; P = .00001) and (RR: 0.34, 95% CI: 0.21-0.55; P = .00001), respectively, whereas pleuritis was significantly higher with adult-onset SLE (RR: 1.45, 95% CI: 1.17-1.79; P = .0008). Vasculitis and fever were significantly higher with childhood-onset SLE (RR: 0.51, 95% CI: 0.36-0.74; P = .0004) and (RR: 0.78, 95% CI: 0.68-0.89; P = .0002) respectively.

Conclusion: Significant differences were observed between childhood-onset versus adult-onset SLE, showing the former to be more aggressive.

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References

Mok C, Mak A, Chu W, To C, Wong S . Long-term survival of southern Chinese patients with systemic lupus erythematosus: a prospective study of all age-groups. Medicine (Baltimore). 2005; 84(4):218-224. DOI: 10.1097/01.md.0000170022.44998.d1. View

Sassi R, Hendler J, Fagundes Piccoli G, Gasparin A, Mendonca da Silva Chakr R, Tavares Brenol J . Age of onset influences on clinical and laboratory profile of patients with systemic lupus erythematosus. Clin Rheumatol. 2016; 36(1):89-95. DOI: 10.1007/s10067-016-3478-4. View

Tucker L, Menon S, Schaller J, Isenberg D . Adult- and childhood-onset systemic lupus erythematosus: a comparison of onset, clinical features, serology, and outcome. Br J Rheumatol. 1995; 34(9):866-72. DOI: 10.1093/rheumatology/34.9.866. View

Mina R, Brunner H . Update on differences between childhood-onset and adult-onset systemic lupus erythematosus. Arthritis Res Ther. 2013; 15(4):218. PMC: 3978647. DOI: 10.1186/ar4256. View

Nikpour M, Bridge J, Richter S . A systematic review of prevalence, disease characteristics and management of systemic lupus erythematosus in Australia: identifying areas of unmet need. Intern Med J. 2014; 44(12a):1170-9. DOI: 10.1111/imj.12568. View

Al Dhanhani A, Agarwal M, Othman Y, Bakoush O . Incidence and prevalence of systemic lupus erythematosus among the native Arab population in UAE. Lupus. 2016; 26(6):664-669. DOI: 10.1177/0961203316678677. View

Gormezano N, Kern D, Pereira O, Esteves G, Sallum A, Aikawa N . Autoimmune hemolytic anemia in systemic lupus erythematosus at diagnosis: differences between pediatric and adult patients. Lupus. 2016; 26(4):426-430. DOI: 10.1177/0961203316676379. View

Papadimitraki E, Isenberg D . Childhood- and adult-onset lupus: an update of similarities and differences. Expert Rev Clin Immunol. 2010; 5(4):391-403. DOI: 10.1586/eci.09.29. View

Brinks R, Hoyer A, Weber S, Fischer-Betz R, Sander O, Richter J . Age-specific and sex-specific incidence of systemic lupus erythematosus: an estimate from cross-sectional claims data of 2.3 million people in the German statutory health insurance 2002. Lupus Sci Med. 2016; 3(1):e000181. PMC: 5133401. DOI: 10.1136/lupus-2016-000181. View

10.

Fatemi A, Matinfar M, Smiley A . Childhood versus adult-onset systemic lupus erythematosus: long-term outcome and predictors of mortality. Clin Rheumatol. 2016; 36(2):343-350. DOI: 10.1007/s10067-016-3509-1. View

11.

Sousa S, Goncalves M, Ines L, Eugenio G, Jesus D, Fernandes S . Clinical features and long-term outcomes of systemic lupus erythematosus: comparative data of childhood, adult and late-onset disease in a national register. Rheumatol Int. 2016; 36(7):955-60. DOI: 10.1007/s00296-016-3450-2. View

12.

Tu Y, Yeh K, Chen L, Yao T, Ou L, Lee W . Differences in disease features between childhood-onset and adult-onset systemic lupus erythematosus patients presenting with acute abdominal pain. Semin Arthritis Rheum. 2010; 40(5):447-54. DOI: 10.1016/j.semarthrit.2010.06.011. View

13.

Font J, Cervera R, Espinosa G, Pallares L, Ramos-Casals M, Jimenez S . Systemic lupus erythematosus (SLE) in childhood: analysis of clinical and immunological findings in 34 patients and comparison with SLE characteristics in adults. Ann Rheum Dis. 1998; 57(8):456-9. PMC: 1752720. DOI: 10.1136/ard.57.8.456. View

14.

Janwityanujit S, Totemchokchyakarn K, Verasertniyom O, Vanichapuntu M, Vatanasuk M . Age-related differences on clinical and immunological manifestations of SLE. Asian Pac J Allergy Immunol. 1995; 13(2):145-9. View

15.

Rood M, Ten Cate R, den Ouden E, Ouwerkerk F, Breedveld F, Huizinga T . Childhood-onset Systemic Lupus Erythematosus: clinical presentation and prognosis in 31 patients. Scand J Rheumatol. 1999; 28(4):222-6. DOI: 10.1080/03009749950155580. View

16.

Hoffman I, Lauwerys B, de Keyser F, Huizinga T, Isenberg D, Cebecauer L . Juvenile-onset systemic lupus erythematosus: different clinical and serological pattern than adult-onset systemic lupus erythematosus. Ann Rheum Dis. 2008; 68(3):412-5. DOI: 10.1136/ard.2008.094813. View

17.

Carreno L, Lopez-Longo F, Monteagudo I, Rodriguez-Mahou M, Bascones M, Gonzalez C . Immunological and clinical differences between juvenile and adult onset of systemic lupus erythematosus. Lupus. 1999; 8(4):287-92. DOI: 10.1191/096120399678847786. View

18.

Liberati A, Altman D, Tetzlaff J, Mulrow C, Gotzsche P, Ioannidis J . The PRISMA statement for reporting systematic reviews and meta-analyses of studies that evaluate healthcare interventions: explanation and elaboration. BMJ. 2009; 339:b2700. PMC: 2714672. DOI: 10.1136/bmj.b2700. View

19.

Tiao J, Feng R, Carr K, Okawa J, Werth V . Using the American College of Rheumatology (ACR) and Systemic Lupus International Collaborating Clinics (SLICC) criteria to determine the diagnosis of systemic lupus erythematosus (SLE) in patients with subacute cutaneous lupus erythematosus (SCLE). J Am Acad Dermatol. 2016; 74(5):862-9. PMC: 4879000. DOI: 10.1016/j.jaad.2015.12.029. View

20.

Gormezano N, Silva C, Aikawa N, Barros D, da Silva M, Otsuzi C . Chronic arthritis in systemic lupus erythematosus: distinct features in 336 paediatric and 1830 adult patients. Clin Rheumatol. 2015; 35(1):227-31. DOI: 10.1007/s10067-015-3127-3. View