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Case Reports
. 2025 Mar 15;64(6):941-945.
doi: 10.2169/internalmedicine.3757-24. Epub 2024 Aug 28.

Interleukin-6 Immunology in Macrophage Activation Syndrome Related to Neuroimmunological Diseases

Taichi Nomura  1 , Masaaki Niino  2 , Toshio Odani  3 , Ryoji Naganuma  1 , Itaru Amino  1 , Yusei Miyazaki  1 , Sachiko Akimoto  1 , Zen-Ichi Tanei  4 , Taichi Kimura  5 , Naoya Minami  1 , Seiji Kikuchi  1
Affiliations
Case Reports

Interleukin-6 Immunology in Macrophage Activation Syndrome Related to Neuroimmunological Diseases

Taichi Nomura et al. Intern Med. .

Abstract

Macrophage activation syndrome (MAS) involves an excessive amount of acute inflammatory responses to inflammatory cytokines, particularly interleukin-6 (IL-6). IL-6 is also strongly associated with the pathophysiology of certain neuroimmunological diseases. However, there have so far been few reports of MAS being accompanied by neuroimmunological diseases. We herein report two cases of MAS comorbid with myasthenia gravis or neuromyelitis optica spectrum disorders, IL-6 related neuroimmunological diseases. Standard immunosuppressive therapies could not stabilize the symptoms in our cases until antibodies against the IL-6 receptor were administered. This finding suggests that it is important to consider the underlying pathophysiology of MAS in relation to these neuroimmunological diseases when treating affected patients.

Keywords: interleukin-6; macrophage activation syndrome; myasthenia gravis; neuromyelitis optica spectrum disorders.

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Conflict of interest statement

The authors state that they have no Conflict of Interest (COI).

Figures

Figure 1.
Figure 1.
Clinical course in Cases 1 and 2. (a) Case 1: tocilizumab administration in addition to corticosteroid therapy alleviated the fever and improved the serum ferritin level. We defined the onset of MAS as day 0. (b) Case 2: relapses of optic neuritis and myelitis in are represented by arrowheads (▼). Despite corticosteroid and immunosuppressive therapies, relapses occurred until satralizumab was administered. After the introduction of satralizumab, oral corticosteroid was discontinued without any relapse. We defined the onset of NMOSD as day 0. Tac: tacrolimus, mPSL: methylprednisolone pulse therapy, PSL: prednisolone, BT: body temperature, AZT: azathioprine, IAPP: immunoadsorption plasmapheresis, MAS: macrophage activating syndrome, EDSS: Expanded Disability Status Scale, NMOSD: neuromyelitis optica spectrum disorders
Figure 2.
Figure 2.
Magnetic resonance images and pathological findings of macrophage activation in Case 2. Magnetic resonance images of the spinal cord are shown (a, b). Abnormal swelling and a high intensity on T2-weighted images, especially from the 6th to 7th levels (arrowhead), were observed. Immunohistochemistry for CD68 in the biopsy lesion (from the 6th to 7th levels of the thoracic spinal cord) showed an accumulation of macrophages (c). Scale bar=50 μm.
See this image and copyright information in PMC

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