Araştırma Makalesi
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Early characteristics of patients with systemic juvenile idiopathic arthritis and differences with adult-onset Still's disease

Yıl 2023, Cilt: 11 Sayı: 3, 158 - 163, 15.10.2023

Öz

Aim: The purpose of this study was to evaluate the demographic characteristics, early clinical and laboratory findings and treatment approaches in patients with systemic juvenile idiopathic arthritis (sJIA). In addition, it was aimed to discuss the differences of patients with sJIA from adult-onset Still's disease (AOSD).
Methods: Patient data were collected from two tertiary hospital rheumatology centers. Pediatric patients diagnosed with sJIA according to ILAR criteria between 2015 and 2022 and adult patients diagnosed with AOSD according to Yamaguchi criteria between 2016 and 2022 were included in the study. Demographic, clinical and laboratory findings were recorded from patient files.
Results: The median age at diagnosis of 63 sJIA patients included in the study was 6.4 years. Fever (n=63, 100%), arthritis (n=53, 84.1%), skin rash (n=50, 79.4%), hepatosplenomegaly (n=42, 66.7%), and lymphadenopathy (n=24, 38.1%) were commonly observed. The monocyclic pattern was the most frequently observed disease pattern (n=39, 61.9%). The mean leukocyte count was 158306604/mm3, while the mean erythrocyte sedimentation rate was 75.927.3 mm/hour. Methotrexate (n=21, 33.3%) and cyclosporine (n=9, 14.3%) were the most frequently preferred immunosuppressive agent in combination with corticosteroids. Among biological agents, canakinumab was used in 16 patients, etanercept in 11, infliximab in 10, tocilizumab in 9 and anakinra in 9 patients. Remission was achieved in 59 (98.3%) patients within the study group. To compare with sJIA patients, 39 AOSD patients were included in the study. Arthritis and hepatosplenomegaly were more common in sJIA (p<0.001). Duration of fever, frequency of lymphadenopathy, skin rash and serositis were similar in both groups. Ferritin and CRP levels were significantly higher in AOSD (p=0.021 and p<0.001, respectively). Monocyclic pattern was more common in sJIA and chronic pattern was more common in AOSD (p=0.005). The duration of oral steroid and synthetic DMARD treatment was significantly longer in AOSD (p<0.001 and p=0.017, respectively).
Conclusion: sJIA is a complex and multifaceted autoinflammatory disease characterized by a range of symptoms including fever, rash, and arthritis. Although it has similar characteristics to AOSD, AOSD patients have longer treatment durations.

Kaynakça

  • 1. Petty RE, Southwood TR, Manners P, et al. International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001. J Rheumatol. 2004;31(2):390-392.
  • 2. Lee J, Schneider R. Systemic juvenile idiopathic arthritis. Pediatr Clin North Am 2018;65(4):691‐709.
  • 3. Efthimiou P, Kontzias A, Hur P et al. Adult-onset Still's disease in focus: Clinical manifestations, diagnosis, treatment, and unmet needs in the era of targeted therapies. Semin Arthritis Rheum 2021;51(4):858-874.
  • 4. Kishida D, Ichikawa T, Takamatsu R et al. Clinical characteristics and treatment of elderly onset adult-onset Still's disease. Sci Rep 2022;12(1):6787.
  • 5. Yamaguchi M, Ohta A, Tsunematsu T. et al. Preliminary criteria for classification of adult Still's disease. J Rheumatol 1992;19:424–30.
  • 6. Wallace CA, Giannini EH, Huang B et al. American College of Rheumatology provisional criteria for defining clinical inactive disease in select categories of juvenile idiopathic arthritis. Arthritis Care Res (Hoboken) 2011;63(7):929‐936.
  • 7. Bahabri S, Al-Sewairi W, Al-Mazyad A et al. Juvenile rheumatoid arthritis: the Saudi experience. Ann Saudi Med 1997;17(4):413–8.
  • 8. Saurenmann RK, Rose JB, Tyrrell P et al. Epidemiology of juvenile idiopathic arthritis in a multiethnic cohort: ethnicity as a risk factor. Arthritis Rheum 2007;56(6):1974–84.
  • 9. Minoia F, Davì S, Horne AnnaCarin et al. Clinical features, treat‐ ment, and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a multinational, multicenter study of 362 patients. Arthritis Rheumatol. 2014;66(11):3160‐3169.
  • 10. Sağ E, Uzunoğlu B, Bal F et al. Systemic onset juvenile idiopathic arthritis: a single center experience. Turk J Pediatr 2019;61(6):852-858.
  • 11. C. Iliou, C. Papagoras, N. Tsifetaki et al. Adult-onset Still's disease: clinical, serological and therapeutic considerations. Clin Exp Rheumatol 2013; 31 (1): 47-52.
  • 12. Feist E, Mitrovic S, Fautrel B. Mechanisms, biomarkers and targets for adult-onset Still's disease. Nat. Rev. Rheumatol 2018;14:603–618.
  • 13. Vastert SJ, Jamilloux Y, Quartier P et al. Anakinra in children and adults with Still's disease. Rheumatology (Oxford). 2019 ;58(Suppl 6):vi9-vi22.

Sistemik juvenil idiyopatik artritli hastaların erken dönem özellikleri ve erişkin başlangıçlı Still hastalığı ile farklılıkları

Yıl 2023, Cilt: 11 Sayı: 3, 158 - 163, 15.10.2023

Öz

Amaç: Bu çalışmanın amacı sistemik juvenil idiyopatik artrit (sJIA) hastalarının demografik özelliklerini, erken dönem klinik ve laboratuvar bulgularını ve tedavi yaklaşımlarını değerlendirmektir. Ayrıca hastalarımızın erişkin başlangıçlı Still hastalığından farklılıklarının tartışılması amaçlandı.
Yöntemler: Hasta verileri iki üçüncü basamak hastane romatoloji merkezinden toplandı. Çalışmaya 2015-2022 yılları arasında ILAR kriterlerine göre sJIA tanısı alan çocuk hastalar ve 2016-2022 yılları arasında Yamaguchi kriterlerine göre erişkin başlangıçlı Still hastalığı tanısı alan erişkin hastalar dahil edildi. Demografik, klinik ve laboratuvar bulguları hasta dosyalarından kaydedildi.
Bulgular: Çalışmaya dahil edilen 63 sJIA hastasının tanı anındaki ortanca yaşı 6,4 yıldı. Ateş (n=63, %100), artrit (n=53, %84,1), deri döküntüsü (n=50, %79,4), hepatosplenomegali (n=42, %66,7) ve lenfadenopati (n=24, %38,1) yaygın olarak gözlendi. Monosiklik patern en sık gözlenen hastalık paterniydi (n=39, %61,9). Ortalama lökosit sayısı 158306604/mm3, ortalama eritrosit sedimentasyon hızı ise 75,927,3 mm/saat idi. Metotreksat (n=21, %33,3) ve siklosporin (n=9, %14,3) kortikosteroidlerle birlikte en sık tercih edilen immünsüpresif ilaçlardı. Biyolojik tedavi kapsamında hastaların 16'sında canakinumab, 11'inde etanersept, 10'unda infliximab, 9'unda tocilizumab ve 9'unda anakinra kullanıldı. Çalışma grubundaki 59 (%98,3) hastada remisyon sağlandı. sJIA hastaları ile karşılaştırmak amacıyla 39 AOSD hastası çalışmaya dahil edildi. Artrit ve hepatosplenomegali sJİA'da daha sık görüldü (p<0,001). Ateşin süresi, lenfadenopati sıklığı, deri döküntüsü ve serozit her iki grupta da benzerdi. AOSD'de ferritin ve CRP düzeyleri anlamlı derecede yüksekti (sırasıyla p=0,021 ve p<0,001). Monosiklik patern sJIA'da, kronik patern ise AOSD'de daha sıktı (p=0,005). AOSD'de oral steroid ve sentetik DMARD tedavisinin süresi anlamlı olarak daha uzundu (sırasıyla p<0,001 ve p=0,017).
Sonuç: sJIA, ateş, döküntü ve artrit gibi bir dizi semptomla karakterize karmaşık ve çok yönlü bir otoinflamatuar hastalıktır. AOSD ile benzer özelliklere sahip olsa da AOSD hastalarının daha uzun süreli tedaviye ihtiyacı vardır.
Anahtar kelimeler: Biyolojik ilaçlar, ateş, jüvenil idiyopatik artrit, erişkin başlangıçlı Still hastalığı

Kaynakça

  • 1. Petty RE, Southwood TR, Manners P, et al. International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001. J Rheumatol. 2004;31(2):390-392.
  • 2. Lee J, Schneider R. Systemic juvenile idiopathic arthritis. Pediatr Clin North Am 2018;65(4):691‐709.
  • 3. Efthimiou P, Kontzias A, Hur P et al. Adult-onset Still's disease in focus: Clinical manifestations, diagnosis, treatment, and unmet needs in the era of targeted therapies. Semin Arthritis Rheum 2021;51(4):858-874.
  • 4. Kishida D, Ichikawa T, Takamatsu R et al. Clinical characteristics and treatment of elderly onset adult-onset Still's disease. Sci Rep 2022;12(1):6787.
  • 5. Yamaguchi M, Ohta A, Tsunematsu T. et al. Preliminary criteria for classification of adult Still's disease. J Rheumatol 1992;19:424–30.
  • 6. Wallace CA, Giannini EH, Huang B et al. American College of Rheumatology provisional criteria for defining clinical inactive disease in select categories of juvenile idiopathic arthritis. Arthritis Care Res (Hoboken) 2011;63(7):929‐936.
  • 7. Bahabri S, Al-Sewairi W, Al-Mazyad A et al. Juvenile rheumatoid arthritis: the Saudi experience. Ann Saudi Med 1997;17(4):413–8.
  • 8. Saurenmann RK, Rose JB, Tyrrell P et al. Epidemiology of juvenile idiopathic arthritis in a multiethnic cohort: ethnicity as a risk factor. Arthritis Rheum 2007;56(6):1974–84.
  • 9. Minoia F, Davì S, Horne AnnaCarin et al. Clinical features, treat‐ ment, and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a multinational, multicenter study of 362 patients. Arthritis Rheumatol. 2014;66(11):3160‐3169.
  • 10. Sağ E, Uzunoğlu B, Bal F et al. Systemic onset juvenile idiopathic arthritis: a single center experience. Turk J Pediatr 2019;61(6):852-858.
  • 11. C. Iliou, C. Papagoras, N. Tsifetaki et al. Adult-onset Still's disease: clinical, serological and therapeutic considerations. Clin Exp Rheumatol 2013; 31 (1): 47-52.
  • 12. Feist E, Mitrovic S, Fautrel B. Mechanisms, biomarkers and targets for adult-onset Still's disease. Nat. Rev. Rheumatol 2018;14:603–618.
  • 13. Vastert SJ, Jamilloux Y, Quartier P et al. Anakinra in children and adults with Still's disease. Rheumatology (Oxford). 2019 ;58(Suppl 6):vi9-vi22.
Toplam 13 adet kaynakça vardır.

Ayrıntılar

Birincil Dil Türkçe
Konular Çocuk Romatolojisi
Bölüm Orjinal Araştırma Makaleleri
Yazarlar

Nilüfer Tekgöz 0000-0002-2235-4489

Merve Cansu Polat 0000-0003-3279-8435

Merve Sungur Özgünen 0000-0002-7441-3946

Emre Tekgöz 0000-0002-0866-1503

Seda Çolak 0000-0002-5703-6739

Elif Çelikel 0000-0003-0129-4410

Fatma Aydın 0000-0003-0306-7473

Müge Sezer 0000-0002-9254-9935

Sedat Yılmaz 0000-0002-9567-9452

Banu Acar 0000-0002-1808-3655

Yayımlanma Tarihi 15 Ekim 2023
Kabul Tarihi 23 Eylül 2023
Yayımlandığı Sayı Yıl 2023 Cilt: 11 Sayı: 3

Kaynak Göster

Vancouver Tekgöz N, Polat MC, Sungur Özgünen M, Tekgöz E, Çolak S, Çelikel E, Aydın F, Sezer M, Yılmaz S, Acar B. Sistemik juvenil idiyopatik artritli hastaların erken dönem özellikleri ve erişkin başlangıçlı Still hastalığı ile farklılıkları. pediatr pract res. 2023;11(3):158-63.