Pedi̇atri̇k Si̇stemi̇k Lupus Eri̇tematozus Hastalarında Düşük Hastalık Akti̇vi̇tesi̇ ve Remi̇syonun Beli̇rleyi̇ci̇leri̇

Abstract

Objective: In our study, it was aimed to investigate the effects of disease symptoms at presentation in pediatric systemic lupus erythematosus (SLE) patients on low disease activity and remission success after the 2nd year of the disease.

Method: Demographic, clinical, and laboratory data of pediatric SLE patients being followed up at our center were obtained from their electronic medical records and patient files. Disease activity at 2 years after diagnosis was measured based on SLE Disease Activity Index-2000 (SLEDAI-2K) scores. Results: In this study, 29 patients diagnosed with pediatric SLE and followed up regularly for at least 2 years were included. At 2 years following diagnosis, according to their SLE activity measurements, 14 (48.2%) patients had high disease activity status (HDAS), whereas 15 (51.7%) had low disease activity status (LDAS)-remission. There was no statistically significant difference between the initial presenting symptoms of the two groups. At 5 years following diagnosis, among 15 patients, 6 (40%) had LDAS-remission, and 9 (60%) had HDAS. The 5th-year SLEDAI-2K scores of the patients with HDAS at 2 years were significantly higher than the 5th-year scores of those with LDAS-remission at 2 years (p=0.028). It was also found that the HDAS of 8 patients who had active disease at 2 years (80%) continued at 5 years. Discussion: The results of our study showed that pediatric SLE presenting symptoms did not have a significant determining effect on low disease activity and remission at 2 years. On the other hand, low disease activity and remission observed at 2 years may be indicative of LDAS and remission at 5 years. 

Keywords

• Sistemik lupus eritamatozus
• SLEDAİ-2K
• Prognoz
• Prognosis

Determinants of Low Disease Activity And Remission in Pediatric Systemic Lupus Erythematosus Patients

Abstract

Amaç: Çalışmamızda pediatrik sistemik lupus eritematozus (SLE) hastalarında, hastalık prezentasyon bulgularının, hastalığın 2. yılındaki düşük hastalık aktivitesi ve remisyona ulaşmadaki etkilerinin incelenmesi amaçlanmıştır.
Gereç ve Yöntemler: Merkezimizde takipli olan pediatrik SLE hastalarının elektronik tıbbi kayıtları ve hasta dosyalarından demografik, klinik ve laboratuvar verileri kaydedildi. Hastalığın 2. yılındaki aktivite ölçümü SLE Hastalığı Aktivite İndeksi-2000 (SLEDAI-2K) skoru baz alınarak hesaplandı. Prezentasyon bulguları ile düşük hastalık aktivitesi ve remisyon arasında ilişki olup olmadığı istatiksel yöntemlerle incelendi.
Sonuçlar: Bu çalışmaya merkezimizde pediatrik SLE tanısı almış ve en az 2 yıl düzenli takibe gelmiş 29 hastayı dahil ettik. Tanıdan sonraki 2. yılda hastalarda SLE aktivite ölçümüne göre 14 hastada (%48.2) HDAS, 15 hastada (%51,7) LDAS-remisyon elde edilmişti. İki grup arasında başlangıçtaki prezentasyon bulguları açısından anlamlı istatiksel farklılık saptanmadı. 5. yılda 15 hastadan 6’sında (%40) LDAS-remisyon, 9’unda (%60) HDAS mevcuttu. 2. yılda HDAS’a sahip hastaların 5. yıl SLEDAI-2K skorları, 2. yılda LDAS-remisyona sahip hastaların 5. yıl SLEDAI-2K skorlarına göre anlamlı olarak yüksekti (p= 0.028). Ayrıca 2. yılda aktif olan 8 hastanın (%80) 5. yılda HDAS’ın devam ettiği gözlendi.
Tartışma: Çalışmamızda pediatrik SLE prezentasyon bulgularının hastalığın 2. yılındaki düşük hastalık aktivitesi ve remisyon üzerine belirleyici etkilerinin olmadığını gösterildi. Ayrıca 2. yılda elde edilen düşük hastalık aktivitesi ve remisyon 5. yıldaki düşük hastalık aktivitesi ve remisyonun belirleyicisi olabilir. 

Keywords

• Systemic lupus erythematosus
• SLEDAI-2K
• Prognosis

References

1. 1Klein-Gitelman MS, Beresford MW. Systemic Lupus Erythematosus, Mixed Connective Tissue Disease, and Undifferentiated Connective Tissue Disease. In: Petty RE, Lindsley CB, Laxer RM, Wedderburn L, eds. Textbook of pediatric rheumatology. 8th ed. Philadelphia : Elsevier, 2020: 295-330.
2. Malattia C, Martini A. Paediatric-onset systemic lupus erythematosus. Best Pract Res Clin Rheumatol 2013;27:351-62.
3. Hoi A, Nim HT, Koelmeyer R, Sun Y, Kao A, Gunther O, et al. Algorithm for calculating high disease activity in SLE. Rheumatology Oxford 2021;60 :4291-7.
4. Lattanzi B, Consolaro A, Solari N, Ruperto N, Martini A, Ravelli A. Measures of disease activity and damage in pediatric systemic lupus erythematosus. Arthritis Care Res 2011;63: 112-7.
5. Jesus D, Rodrigues M, Matos A, Henriques C, Pereira da Silva JA, Inês LS. Performance of SLEDAI-2K to detect a clinically meaningful change in SLE disease activity: a 36-month prospective cohort study of 334 patients. Lupus 2019;28:607-12.
6. Gladman DD, Ibanez D, Urowitz MB. Systemic lupus erythematosus disease activity index 2000. J Rheumatol 2002;29:288-91.
7. Ugarte-Gil MF, Gamboa-Cárdenas RV, Reátegui-Sokolova C, Medina-Chinchón M, Zevallos F, Elera-Fitzcarrald C, et al. Better Health-Related Quality of Life in Systemic Lupus Erythematosus Predicted by Low Disease Activity State/Remission. Arthritis Care Res 2020;72:1159-62.
8. Charras A, Smith E, Hedrich CM. Systemic Lupus Erythematosus in Children and Young People. Curr Rheumatol Rep 2021;23:20.

9. Ugarte-Gil MF, Wojdyla D, Pons-Estel GJ, Quintana R, Gómez-Puerta JA, Catoggio LJ, et al. Predictive factors of flares in systemic lupus erythematosus patients: data from a multiethnic Latin American cohort. Lupus 2018;27:536-44.
10. Aljohani R, Gladman DD, Su J, Urowitz MB. Disease evolution in late-onset and early-onset systemic lupus erythematosus. Lupus 2017;26:1190–96.
11. Levy DM, Kamphuis S. Systemic lupus erythematosus in children and adolescents. Pediatr Clin North Am 2012;59:345-64.
12. Bertsias GK, Tektonidou M, Amoura Z, Aringer M, Bajema I, Berden JH et al. EULAR recommendations for the management of systemic lupus erythematosus. Report of a Task Force of the EULAR Standing Committee for International Clinical Studies Including Therapeutics. Ann Rheum Dis 2008; 67:1771-82.
13. Dima A, Caraiola S, Delcea C, Ionescu RA, Jurcut C, Badea C. Self-reported disease severity in women with systemic lupus erythematosus. Rheumatol Int 2019;39:533-39.
14. Doria A, Amoura Z, Cervera R, Khamastha MA, Schneider M, Richter J, et al. Annual direct medical cost of active systemic lupus erythematosus in five European countries. Ann Rheum Dis 2014;73:154-60.
15. Franklyn K, Lau CS, Navarra SV, Louthrenoo W, Lateef A, Hamijoyo L, et al. Asia-Pacific Lupus Collaboration. Definition and initial validation of a Lupus Low Disease Activity State (LLDAS). Ann Rheum Dis 201;75:1615-21.
16. Zen M, Iaccarino L, Gatto M, Saccon F, Larosa M, Ghirardello A, et al. Lupus low disease activity state is associated with a decrease in damage progression in Caucasian patients with SLE, but overlaps with remission. Ann Rheum Dis 2018;77:104-10.
17. Babaoglu H, Li J, Goldman D, Magder LS, Petri M. Predictors of predominant Lupus Low Disease Activity State (LLDAS-50). Lupus 2019; 28:1648-55.
18. Golder V, Kandane-Rathnayake R, Hoi AY, Huq M, Louthrenoo W, An Y, et al. Asia-Pacific Lupus Collaboration. Frequency and predictors of the lupus low disease activity state in a multi-national and multi-ethnic cohort. Arthritis Res Ther 2016;18:260-9.
19. Hao Y, Oon S, Ji L, Gao D, Fan Y, Geng Y, et al. Determinants and protective associations of the lupus low disease activity state in a prospective Chinese cohort. Clin Rheumatol 2022;41:357-66.
20. Koelmeyer R, Nim HT, Nikpour M, Sun YB, Kao A, Guenther O, et al. High disease activity status suggests more severe disease and damage accrual in systemic lupus erythematosus. Lupus Sci Med 2020;4:372-80.