Evaluation of the Efficacy and Side Effects of Interleukin-1 Inhibitor Therapy in Adult Still's Disease

Abstract

Adult Still's disease (ASD) is a multigenic autoinflammatory disease. Interleukin (IL)-1 inhibitors can be used in treatment-resistant ASD. We aimed to present our experience with the efficacy and side effects of IL-1 inhibitors in ASD. The records of 24 patients who were treated in the rheumatology clinic of our hospital between January 2016 and March 2024 with a diagnosis of ASD were retrospectively analyzed. There were 22 patients who received anakinra and 5 patients who received kanakinumab. Three of the patients receiving kanakinumab had previously been treated with anakinra. The mean age at diagnosis was 29.7 (16.5-75.3) years. Sixteen of the patients were female. The median follow-up time of patients who received anakinra was 13.5 (0.8-92.5) months. There were statistically significant differences in fever, sore throat, rash, arthritis, arthralgia, modified Pouchot score, ferritin, erythrocyte sedimentation rate (ESR) and C-reactive protein between baseline anakinra, month 3 and the last visit (p<0.001, p<0.001, p<0.001, p<0.001, p<0.001, p=0.001, p<0.001, p<0.001, p<0.001 and p<0.001, respectively). The mean follow-up time of patients receiving canakinumab was 70.2±46.7 months. There was a statistically significant difference between the rash, modified Pouchot score and ESR at baseline, month 3 and the last outpatient visit (p=0.015, p=0.015 and p=0.029, respectively). Allergic skin rash in 1 patient, recurrent urinary tract infections in 1 patient, leukopenia and neutropenia in 1 patient were observed with anakinra. No side effects were observed with kanakinumab. IL-1 inhibitors can be considered as first-line biologic treatment in patients with ASD who do not respond to conventional treatment.

Keywords

• Anakinra.
• Adult Still disease
• Canakinumab

Erişkin Still Hastalığında İnterlökin-1 İnhibitörü Tedavisinin Etkinlik ve Yan Etkilerinin Değerlendirilmesi

Abstract

Erişkin Still hastalığı (ESH) multigenik otoinflamatuar bir hastalıktır. İnterlökin (IL)-1 inhibitörleri tedaviye dirençli ESH’de kullanılabilir. Çalışmamızda ESH’de IL-1 inhibitörlerinin etkinlik ve yan etkileri ile ilgili gerçek yaşam deneyimimizi sunmayı amaçladık. Hastanemiz romatoloji kliniğinde Ocak 2016-Mart 2024 tarihleri arasında ESH tanısı ile takip edilen 24 hastanın dosyası retrospektif olarak incelendi. Anakinra alan 22 hasta ve kanakinumab alan 5 hasta mevcuttu. Kanakinumab alan hastalardan 3 tanesi daha önce anakinra tedavisi almıştı. Çalışmaya dahil edilen hastaların ortalama tanı yaşı 29,7 (16,5-75,3) yıldı. Hastaların 16’sı kadındı. Anakinra alan hastaların ortanca takip süresi 13,5 (0,8-92,5) ay saptandı. Hastaların anakinra başlangıç, 3. ay ve son vizitleri arasında ateş, boğaz ağrısı, döküntü, artrit, artralji, modifiye Pouchot skoru, ferritin, sedimentasyon ve C-reaktif protein parametrelerinde istatistiksel açıdan anlamlı fark mevcuttu (p<0,001, p<0,001, p<0,001, p<0,001, p=0,001, p<0,001, p<0,001, p<0,001 ve p<0,001, sırasıyla). Kanakinumab alan hastaların ortalama takip süresi 70,2±46,7 aydı. Kanakinumab başlangıcı, 3.ay ve son poliklinik vizitindeki döküntü, modifiye Pouchot skoru ve sedimentasyon değerleri arasında istatistiksel açıdan anlamlı fark saptandı (p=0,015, p=0,015 ve p=0,029, sırasıyla). Anakinra tedavisi ile 1 hastada alerjik cilt döküntüsü, 1 hastada tekrarlayan idrar yolu enfeksiyonu, 1 hastada lökopeni ve nötropeni saptandı. Kanakinumab tedavisi ile yan etki görülmedi. IL-1 inhibitörleri geleneksel tedaviye yanıt vermeyen ESH hastalarında ilk basamak biyolojik ajan olarak düşünülebilir.

Keywords

• Anakinra
• Erişkin Still Hastalığı
• Kanakinumab.

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