Masaki Shimizu1, Mao Mizuta2, Nami Okamoto3, Takahiro Yasumi4, Naomi Iwata5, Hiroaki Umebayashi6, Yuka Okura7, Noriko Kinjo8, Tomohiro Kubota9, Yasuo Nakagishi10, Kenichi Nishimura11, Mariko Mohri12, Masato Yashiro13, Junko Yasumura14, Hiroyuki Wakiguchi15, Masaaki Mori12. 1. Department of Pediatrics, Graduate School of Medical Sciences, Kanazawa University, 13-1 Takaramachi, Kanazawa, 920-8641, Japan. shimizum@staff.kanazawa-u.ac.jp. 2. Department of Pediatrics, Graduate School of Medical Sciences, Kanazawa University, 13-1 Takaramachi, Kanazawa, 920-8641, Japan. 3. Department of Pediatrics, Osaka Medical College, Takatsuki, Japan. 4. Department of Pediatrics, Kyoto University Graduate School of Medicine, Kyoto, Japan. 5. Department of Immunology and Infectious Diseases, Aichi Children's Health and Medical Center, Obu, Japan. 6. Department of Rheumatics, Miyagi Children's Hospital, Sendai, Japan. 7. Department of Pediatrics, KKR Sapporo Medical Center, Sapporo, Japan. 8. Department of Pediatrics, Faculty of Medicine, University of the Ryukyus, Nakagami-gun, Japan. 9. Department of Pediatrics, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan. 10. Department of Pediatric Rheumatology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Japan. 11. Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Japan. 12. Department of Lifetime Clinical Immunology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan. 13. Department of Pediatrics, Okayama University Hospital, Okayama, Japan. 14. Department of Pediatrics, Hiroshima University Graduate School of Biomedical & Health Sciences, Hiroshima, Japan. 15. Department of Pediatrics, Yamaguchi University Graduate School of Medicine, Ube, Japan.
Abstract
BACKGROUND: This study aimed to determine the influence of tocilizumab (TCZ) in modifying the clinical and laboratory features of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (s-JIA). Furthermore, we assessed the performance of the 2016 MAS classification criteria for patients with s-JIA-associated MAS while treated with TCZ. METHODS: A panel of 15 pediatric rheumatologists conducted a combination of expert consensus and analysis of real patient data. Clinical and laboratory features of s-JIA-associated MAS in 12 TCZ-treated patients and 18 untreated patients were evaluated. Possible MAS was defined as having characteristic laboratory features but lack of clinical features of MAS, or atypical MAS, or early treatment that prevented full-blown MAS. RESULTS: Clinically, the TCZ-treated patients with s-JIA-associated MAS were less likely febrile and had significantly lower ferritin, triglyceride, and CRP levels than the untreated patients with s-JIA-associated MAS. Other laboratory features of MAS including lower platelet counts and lower fibrinogen were more pronounced in TCZ-treated patients. The TCZ-treated patients with s-JIA-associated MAS were less likely to be classified as MAS based on the MAS classification criteria (25% vs 83.3%, p < 0.01). This is ascribed to the absence of fever or insufficient ferritin elevation, compared with the untreated patients. CONCLUSION: TCZ could modify the clinical and laboratory features of s-JIA-associated MAS. When evaluating the s-JIA patients while treated with TCZ, it is not applicable to use MAS classification criteria. Care must be taken to not underdiagnose MAS based on the MAS classification criteria.
BACKGROUND: This study aimed to determine the influence of tocilizumab (TCZ) in modifying the clinical and laboratory features of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (s-JIA). Furthermore, we assessed the performance of the 2016 MAS classification criteria for patients with s-JIA-associated MAS while treated with TCZ. METHODS: A panel of 15 pediatric rheumatologists conducted a combination of expert consensus and analysis of real patient data. Clinical and laboratory features of s-JIA-associated MAS in 12 TCZ-treated patients and 18 untreated patients were evaluated. Possible MAS was defined as having characteristic laboratory features but lack of clinical features of MAS, or atypical MAS, or early treatment that prevented full-blown MAS. RESULTS: Clinically, the TCZ-treated patients with s-JIA-associated MAS were less likely febrile and had significantly lower ferritin, triglyceride, and CRP levels than the untreated patients with s-JIA-associated MAS. Other laboratory features of MAS including lower platelet counts and lower fibrinogen were more pronounced in TCZ-treated patients. The TCZ-treated patients with s-JIA-associated MAS were less likely to be classified as MAS based on the MAS classification criteria (25% vs 83.3%, p < 0.01). This is ascribed to the absence of fever or insufficient ferritin elevation, compared with the untreated patients. CONCLUSION: TCZ could modify the clinical and laboratory features of s-JIA-associated MAS. When evaluating the s-JIApatients while treated with TCZ, it is not applicable to use MAS classification criteria. Care must be taken to not underdiagnose MAS based on the MAS classification criteria.
Authors: Punnoth Poonkuzhi Naseef; Muhammed Elayadeth-Meethal; K T Mohammed Salim; A Anjana; C Muhas; K Abdul Vajid; Mohamed Saheer Kuruniyan Journal: Saudi J Biol Sci Date: 2021-12-13 Impact factor: 4.052