Macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis on anti-interleukin-1 or -6 therapy

• Published in: Rheumatology (Oxford, England) England), 2024-09, Vol.63 (SI2), p.SI167-SI172
• Main Authors: Ulu, Kadir, Aliyev, Emil, Kılıç Könte, Elif, Tanatar, Ayşe, Türkmen, Şeyma, Doğantan, Şeyda, Kızıldağ, Zehra, Kasap Demir, Belde, Gezgin Yıldırım, Deniz, Otar Yener, Gülçin, Öztürk, Kübra, Baba, Özge, Açarı, Ceyhun, Kılbaş, Gülşah, Taşkın, Sema Nur, Haşlak, Fatih, Çağlayan, Şengül, Bağlan, Esra, Dundar, Hatice Adıgüzel, Başaran, Özge, Barut, Kenan, Karadağ, Şerife Gül, Coşkuner, Taner, Sönmez, Hafize Emine, Yüksel, Selçuk, Kalyoncu, Mukaddes, Bakkaloğlu, Sevcan A, Ünsal, Erbil, Paç Kısaarslan, Ayşenur, Bilginer, Yelda, Aktay Ayaz, Nuray, Kasapçopur, Özgür, Özen, Seza, Sözeri, Betül
• Format: Article
• Language: English
• Subjects: Adolescent; Antibodies, Monoclonal, Humanized - therapeutic use; Antirheumatic Agents - therapeutic use; Arthritis; Arthritis, Juvenile - complications; Arthritis, Juvenile - drug therapy; Biological products; Biological Products - therapeutic use; Biopharmaceuticals; Blood Sedimentation; C-reactive protein; C-Reactive Protein - analysis; C-Reactive Protein - metabolism; Cell activation; Child; Child, Preschool; Cytokines; Erythrocyte sedimentation rate; Female; Ferritin; Ferritins - blood; Humans; Interleukin 1; Interleukin-1 - antagonists & inhibitors; Interleukin-6 - antagonists & inhibitors; Interleukin-6 - blood; Macrophage Activation Syndrome - drug therapy; Macrophage Activation Syndrome - etiology; Macrophages; Male; Patients; Pediatrics; Platelet Count; Rheumatology; Splenomegaly
• ISSN: 1462-0324; 1462-0332; 1462-0332
• DOI: 10.1093/rheumatology/keae124

Abstract Objectives The aim of this study is to investigate the effect of anti-interleukin (IL)-1/-6 biologics on systemic juvenile idiopathic arthritis (sJIA)-associated macrophage activation syndrome (MAS). Methods Demographic, clinical and laboratory data of patients followed up with a diagnosis of sJIA-associated MAS assessed from sixteen paediatric rheumatology centres across the country. The clinical and laboratory features of MAS developing while on biological drugs were compared with those without this treatment. Results One hundred and sixty-two patients were included in the study. Forty-five of the MAS events were detected under the effect of anti-IL-1/-6 biologics, while the patients experiencing the remaining 155 events have not received biological treatment in the last three months. Platelet count [128 (72–232) vs 199 (130–371) 109/l], ferritin level on admission [1107 (676–2050) vs 2863 (1193–9562) ng/ml], C-reactive protein level [15.4 (2.9–56) vs 90 (32–160) mg/l], erythrocyte sedimentation rate [13 (3–36) vs 43.5 (13–77) mm/h] and fever duration [5 (4–7.5) vs 10 (7–14.3) days] were found lower in the group under the impact of anti-IL-1/-6 biologics. Among patients treated with biologics, 26.6% did not meet the published 2016 MAS classification criteria at presentation. The rates of hepatomegaly and splenomegaly were relatively lower in the canakinumab-treated group when compared with those receiving other biologicals or to patients, not on biologicals. Conclusion Anti-IL-1/-6 therapies can mask the clinical and laboratory features of MAS, and proposed guidelines for MAS classification criteria may not be met.