Araştırma Makalesi
PDF Zotero Mendeley EndNote BibTex Kaynak Göster

Çocuklarda sistemik lupus eritematozusun klinik özellikleri ve uzun dönem sonuçları

Yıl 2021, Cilt 9, Sayı 2, 78 - 83, 31.08.2021
https://doi.org/10.21765/pprjournal.982781

Öz

Amaç: Sistemik lupus eritematozus (SLE), otoantikorların varlığı ve çoklu organ sistemi tutulumu ile karakterize, yaygın bir multisistemik otoimmün hastalıktır. Bu çalışmanın amacı, çocuklarda SLE'nin klinik belirtilerini, laboratuvar bulguları, klinik seyrini ve prognozunu tanımlamaktır.

Yöntemler: 18 yaşından önce tanı almış SLE'li hastaları retrospektif olarak incelendi.. Klinik ve laboratuvar verileri, ilk kabulden çalışmanın başlamasına kadar geçen sürede hastaların klinik ve laboratuvar verileri kaydedildi.

Bulgular: Otuz beş çocuk ve adölesan SLE (cSLE) hastanın %85.7'si kadındı. Ortalama hastalık başlangıç ​​yaşı 12 (4-17 arası) yıl ve medyan takip süresi 5 (1-14) yıldı. En sık görülen klinik özellik artrit (%65.1) idi, bunu konstitusyonel semptomlar (%48.6), malar döküntü (%31.4), ışığa duyarlılık (%5.7), alopesi (%5.7) ve oral ülserler (%5.7) izliyordu. Böbrek tutulumu hastaların 4/5'ini (%80) oluşturuyordu. Hematüri ve proteinüri en sık başvuru bulgusuydu (sırasıyla %48.5 ve %45.7). 27 hastaya böbrek biyopsisi yapıldı. WHO sınıflamasına göre: 1 hastada sınıf V, 10 hastada sınıf IV, 4 hastada sınıf III ve 12 hastada sınıf II nefrit vardı. Çocukların %20'sinde nörolojik semptomlar gelişti. Takip süresi boyunca bir hasta öldü. Son takipte hiçbir hastada böbrek yetmezliği gelişmedi, ancak 4'ünde (%11.4) proteinüri devam etti. Hastaların sekel dışında tüm nörolojik bulguları remisyondadır.

Sonuç: Hastalarımızda klinik sonuç olumluydu. Böbrek tutulumu yaygındır, ancak en azından kısa vadede son dönem böbrek hastalığına ilerleme nadirdir.

Kaynakça

  • 1. Malleson PN, Fung MY, Rosenberg AM. The incidence of pediatric rheumatic disease: results from the Canadian pediatric Rheumatology Association Disease Registry. J Rheumatol 1996;23(11):1981-7.
  • 2. Levy DM, Kamphuis S. Systemic lupus erythematosus in children and adolescents. Pediatr Clin North Am. 2012;59(2):345-64.
  • 3. Pan L, Lu MP, Wang JH, et al. Immunological pathogenesis and treatment of systemic lupus erythematosus. World J Pediatr. 2020;16(1):19-30.
  • 4. Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of the systemic lupus erythematosus. Artrhritis Rheumatol 1982; 25(11):1271-7.
  • 5. Charras A, Smith E, Hedrich CM. Systemic Lupus Erythematosus in Children and Young People. Curr Rheumatol Rep. 2021;23 (3):20.
  • 6. Klein-Gitelman M. Pediatric lupus versus adult lupus role of the laboratory. Clin Appl Immunol Rev.2004;4:333–350.
  • 7. Brunner HI, Gladman DD, Ibanez D, et al. Difference in disease features between childhood-onset and adult-onset systemic lupus erythematosus. Arthritis Rheum 2008;58(2):556–62.
  • 8. Wang LC, Yang YH, Lu MY, et al. Retrospective analysis of mortality and morbidity of pediatric systemic lupus erythematosus in the past two decades. J Microbiol Immunol Infect 2003;36:203–8.
  • 9. Oni L, Wright RD, Marks S, et al. Kidney outcomes for children with lupus nephritis. Pediatr Nephrol. 2021; 36(3):1377-85.
  • 10. Hill GS, Delahousse M, Nochy D, et al. Class IV-S versus class IV-G lupus nephritis: clinical and morphologic differences suggesting different pathogenesis. Kidney Int 2005;68(5):2288–97.
  • 11. Srivastava P, Abujam B, Misra R, et al. Outcome of lupus nephritis in childhood onset SLE in North and Central India: single-centre experience over 25 years. Lupus. 2016;25(5):547-57.
  • 12. Sibbitt, W.L, Brandt, J.R, Johnson C.R, et al. The incidence and prevalence of neuropsychiatric syndromes in pediatric onset systemic lupus erythematosus. The Journal of Rheumatology 2002; 29(7):1536-42.
  • 13. Spinosa MJ, Bandeira M, Liberalesso PB, et al. Clinical, laboratory and neuroimage findings in juvenile systemic lupus erythematosus presenting involvement of the nervous system. Arq Neuropsiquiatr 2007;65(2B):433–9.
  • 14. Greenwood DL, Gitlits VM, Alderuccio F, et al. Autoantibodies in neuropsychiatric lupus.Autoimmunity 2002;35(2):79–86.
  • 15. Akca UK, Ayaz NA. Comorbidities of antiphospholipid syndrome and systemic lupus erythematosus in children. Curr Rheumatol Rep. 2020;22(6):21.
  • 16. Steens SC, Bosma GP, Steup-Beekman GM, et al. . Association between microscopic brain damage as indicated by magnetization transfer imaging and anticardiolipin antibodies in neuropsychiatric lupus. Arthritis Res Ther 2006; 8(2): R38.
  • 17. Singh S, Gupta MK, Ahluwalia J, et al. Neuropsychiatric manifestations and antiphospholipid antibodies in pediatric onset lupus: 14 years of experience from a tertiary center of North India. Rheumatol Int. 2009;29(12):1455-61.
  • 18. Zuniga Zambrano YC, Guevara Ramos JD, Penagos Vargas NE, et al. Risk factors for neuropsychiatric manifestations in children with systemic lupus erythematosus: case- control study. Pediatr Neurol. 2014;51(3):403-9. 
  • 19. Avcin T, Benseler SM, Tyrrell PN, et al. A follow-up study of antiphospholipid antibodies and associated neuropsychiatric manifestation in 137 children with systemic lupus erythematosus. Arthritis Rheum. 2008;59(2):206-13.
  • 20. Miettunen PM, Ortiz-Alvarez RE, Petty RE, et al. Gender and ethnic origin have no effect on longterm of childhood-onset systemic lupus erythematosus. J Rheumatol. 2004; 31(8):1650-4.
  • 21. Wang LC, Yang YH, Lu MY, et al. Retrospective analysis of mortality and morbidity of pediatric systemic lupus erythematosus in the past two decades. J Microbiol Immunol Infect. 2003; 36(3):203-8.
  • 22. Singh S, Abujam B, Gupta A, et al. Childhood lupus nephritis in a developing country-24 years’ single-center experience from North India. Lupus 2015;24(6):641–7.

Clinical characteristics and long-term outcomes of systemic lupus erythematosus in children

Yıl 2021, Cilt 9, Sayı 2, 78 - 83, 31.08.2021
https://doi.org/10.21765/pprjournal.982781

Öz

Background: Systemic lupus erythematosus (SLE) is a common multisystemic autoimmune disease characterized by the presence of autoantibodies and multiorgan system involvement. The aim of this study was to describe the presenting clinical manifestations, laboratuary findings, clinical course and prognosis of SLE in children.

Methods: We performed a retrospective study patients with SLE, diagnosed before the age of 18 years. Clinical and laboratory data were collected from initial admission to study initiation.

Results: Thirty-five children and adolescents SLE (cSLE) were recorded, 85.7% of female. The median age at disease onset was 12(range 4-17) years, and median follow up duration was 5 (1-14) years. The most common clinical feature was arthritis (65.1%), followed by constitutional symptoms (48.6%), malar rash (31.4%), photosensitivity (5.7%), alopecia (5.7%) and oral ulcers (5.7%). Renal involvement accounted 4/5 of the patients (80%). Hematuria and proteinuria were the most frequent presenting findings (48.5% and 45.7% respectively). Renal biopsy was performed in 27 patients. According to WHO classification: 1 patient had classV nephritis, 10 had class IV, 4 had class III and 12 had class II nephritis. 20% of children developed neurologic symptoms. One patient died during the follow-up period. At the last follow up none of the patients had renal failure but, proteinuria persisted in 4 of them (11.4%). All neurological findings of patients are in remission apart from sequelae.

Conclusion: Clinical outcome was favorable in our patients. Renal involvement is common but progression to end stage kidney disease, at least in the short term, is rare.

Kaynakça

  • 1. Malleson PN, Fung MY, Rosenberg AM. The incidence of pediatric rheumatic disease: results from the Canadian pediatric Rheumatology Association Disease Registry. J Rheumatol 1996;23(11):1981-7.
  • 2. Levy DM, Kamphuis S. Systemic lupus erythematosus in children and adolescents. Pediatr Clin North Am. 2012;59(2):345-64.
  • 3. Pan L, Lu MP, Wang JH, et al. Immunological pathogenesis and treatment of systemic lupus erythematosus. World J Pediatr. 2020;16(1):19-30.
  • 4. Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of the systemic lupus erythematosus. Artrhritis Rheumatol 1982; 25(11):1271-7.
  • 5. Charras A, Smith E, Hedrich CM. Systemic Lupus Erythematosus in Children and Young People. Curr Rheumatol Rep. 2021;23 (3):20.
  • 6. Klein-Gitelman M. Pediatric lupus versus adult lupus role of the laboratory. Clin Appl Immunol Rev.2004;4:333–350.
  • 7. Brunner HI, Gladman DD, Ibanez D, et al. Difference in disease features between childhood-onset and adult-onset systemic lupus erythematosus. Arthritis Rheum 2008;58(2):556–62.
  • 8. Wang LC, Yang YH, Lu MY, et al. Retrospective analysis of mortality and morbidity of pediatric systemic lupus erythematosus in the past two decades. J Microbiol Immunol Infect 2003;36:203–8.
  • 9. Oni L, Wright RD, Marks S, et al. Kidney outcomes for children with lupus nephritis. Pediatr Nephrol. 2021; 36(3):1377-85.
  • 10. Hill GS, Delahousse M, Nochy D, et al. Class IV-S versus class IV-G lupus nephritis: clinical and morphologic differences suggesting different pathogenesis. Kidney Int 2005;68(5):2288–97.
  • 11. Srivastava P, Abujam B, Misra R, et al. Outcome of lupus nephritis in childhood onset SLE in North and Central India: single-centre experience over 25 years. Lupus. 2016;25(5):547-57.
  • 12. Sibbitt, W.L, Brandt, J.R, Johnson C.R, et al. The incidence and prevalence of neuropsychiatric syndromes in pediatric onset systemic lupus erythematosus. The Journal of Rheumatology 2002; 29(7):1536-42.
  • 13. Spinosa MJ, Bandeira M, Liberalesso PB, et al. Clinical, laboratory and neuroimage findings in juvenile systemic lupus erythematosus presenting involvement of the nervous system. Arq Neuropsiquiatr 2007;65(2B):433–9.
  • 14. Greenwood DL, Gitlits VM, Alderuccio F, et al. Autoantibodies in neuropsychiatric lupus.Autoimmunity 2002;35(2):79–86.
  • 15. Akca UK, Ayaz NA. Comorbidities of antiphospholipid syndrome and systemic lupus erythematosus in children. Curr Rheumatol Rep. 2020;22(6):21.
  • 16. Steens SC, Bosma GP, Steup-Beekman GM, et al. . Association between microscopic brain damage as indicated by magnetization transfer imaging and anticardiolipin antibodies in neuropsychiatric lupus. Arthritis Res Ther 2006; 8(2): R38.
  • 17. Singh S, Gupta MK, Ahluwalia J, et al. Neuropsychiatric manifestations and antiphospholipid antibodies in pediatric onset lupus: 14 years of experience from a tertiary center of North India. Rheumatol Int. 2009;29(12):1455-61.
  • 18. Zuniga Zambrano YC, Guevara Ramos JD, Penagos Vargas NE, et al. Risk factors for neuropsychiatric manifestations in children with systemic lupus erythematosus: case- control study. Pediatr Neurol. 2014;51(3):403-9. 
  • 19. Avcin T, Benseler SM, Tyrrell PN, et al. A follow-up study of antiphospholipid antibodies and associated neuropsychiatric manifestation in 137 children with systemic lupus erythematosus. Arthritis Rheum. 2008;59(2):206-13.
  • 20. Miettunen PM, Ortiz-Alvarez RE, Petty RE, et al. Gender and ethnic origin have no effect on longterm of childhood-onset systemic lupus erythematosus. J Rheumatol. 2004; 31(8):1650-4.
  • 21. Wang LC, Yang YH, Lu MY, et al. Retrospective analysis of mortality and morbidity of pediatric systemic lupus erythematosus in the past two decades. J Microbiol Immunol Infect. 2003; 36(3):203-8.
  • 22. Singh S, Abujam B, Gupta A, et al. Childhood lupus nephritis in a developing country-24 years’ single-center experience from North India. Lupus 2015;24(6):641–7.

Ayrıntılar

Birincil Dil İngilizce
Konular Sağlık Bilimleri ve Hizmetleri
Bölüm Orjinal Araştırma Makaleleri
Yazarlar

Elif ÇELİKEL (Sorumlu Yazar)
Ankara Şehir Hastanesi
0000-0003-0129-4410
Türkiye


Zahide EKİCİ TEKİN
Ankara Şehir Hastanesi
0000-0002-9254-667X
Türkiye


Fatma AYDIN
Ankara Şehir Hastanesi
0000-0003-0306-7473
Türkiye


Tuba KURT
Ankara Şehir Hastanesi
0000-0003-3711-8347
Türkiye


Melike KAPLAN
Ankara Şehir Hastanesi
0000-0002-8012-2774
Türkiye


Cüneyt KARAGÖL
Ankara Şehir Hastanesi
0000-0002-2987-1980
Türkiye


Müge SEZER
Ankara Şehir Hastanesi
0000-0002-9254-9935
Türkiye


Nilüfer TEKGÖZ
Ankara Şehir Hastanesi
0000-0002-0223-5448
Türkiye


Serkan COŞKUN
Ankara Şehir Hastanesi
0000-0003-2568-9329
Türkiye


Banu ACAR
Ankara Şehir Hastanesi
0000-0002-1808-3655
Türkiye


Nilgün ÇAKAR
ANKARA UNIVERSITY, ANKARA FACULTY OF MEDICINE
0000-0002-1853-0101
Türkiye

Destekleyen Kurum Yok
Proje Numarası Yok
Yayımlanma Tarihi 31 Ağustos 2021
Kabul Tarihi 16 Ağustos 2021
Yayınlandığı Sayı Yıl 2021, Cilt 9, Sayı 2

Kaynak Göster

Vancouver Çelikel E. , Ekici Tekin Z. , Aydın F. , Kurt T. , Kaplan M. , Karagöl C. , Sezer M. , Tekgöz N. , Coşkun S. , Acar B. , Çakar N. Clinical characteristics and long-term outcomes of systemic lupus erythematosus in children. Pediatric Practice and Research. 2021; 9(2): 78-83.