Response of Bone Marrow Edema and Parotitis to Canakinumab in a Woman with Neonatal-Onset Multisystem Inflammatory Disease: A Case Report
by Akira Nagita1*, Naoko Yomogida1, Yuya Kodama1, Masayuki Kodama2, Toshitaka Taniguchi3, Hidetoshi Takada4
1Department of Pediatrics, Mizushima Central Hospital, Kurashiki City, Okayama, Japan
2Department of Orthopedics, Mizushima Central Hospital, Kurashiki City, Okayama, Japan
3Department of Radiology, Mizushima Central Hospital, Kurashiki City, Okayama, Japan
4Department of Child Health, Faculty of Medicine, University of Tsukuba, Tsukuba City, Ibaraki, Japan
*Corresponding author: Akira Nagita, Department of Pediatrics, Mizushima Central Hospital, 4-5 Mizushimaaoba-cho, Kurashiki City, Okayama, Japan
Received Date: 21 October 2023
Accepted Date: 26 October 2023
Published Date: 30 October 2023
Citation: Nagita A, Yomogida N, Kodama Y, Kodama M, Taniguchi T, et al (2023) Response of Bone Marrow Edema and Parotitis to Canakinumab in a Woman with Neonatal-Onset Multisystem Inflammatory Disease: A Case Report. Ann Case Report. 8: 1492. https://doi.org/10.29011/2574-7754.101492
Background: A patient is 50-year-old woman developed pyrexia, conjunctival hyperemia, and urticarial rash at the age of 2 months. In addition to these symptoms, multiple arthralgia, abdominal pain, and aseptic meningitis recurred. Mental developmental regression, sensorial deafness, ulnar side deformities in both hands, and dry mouth also gradually progressed. Marked increases in neutrophil counts and serum C-reactive protein levels persisted. She was clinically diagnosed with neonatal-onset multisystem inflammatory disease at the age of 44 years.
Methods: She underwent whole-exome sequencing analysis of the NLRP3 gene. A point mutation was determined in c.1060G>A.
Results: After the administration of anti-interleukin-1β antibody, her symptoms and acute-phase inflammatory markers markedly improved. Arthralgia after cold exposure and dysphagia persisted; however, they were reduced by improvements in lifestyle factors. Parotid salivary smears continued to show high neutrophil counts and some monocytes, whereas the evidence of bone marrow edema, present before treatment, was no longer observed on magnetic resonance imaging scans.
Conclusions: The latter finding suggests that interleukin-1β play the main role in developing bone marrow edema. To date, no studies have investigated bone marrow edema and salivary neutrophils in patients with neonatal-onset multisystem inflammatory disease.
Keywords: Neonatal-Onset Multisystem Inflammatory Disease; Bone Marrow Edema; Salivary Neutrophil; Interleukin1β; Canakinumab.