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Published on: May 31, 2018
Interleukin-6 Immunology in Macrophage Activation Syndrome Related to Neuroimmunological Diseases
Taichi Nomura1, Masaaki Niino2, Toshio Odani3
1Department of Neurology, National Hospital Organization Hokkaido Medical Center, Japan.
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.
Insights
Macrophage activation syndrome (MAS) can co-occur with neuroimmunological diseases like myasthenia gravis. Targeting interleukin-6 (IL-6) receptor with antibodies proved effective when standard treatments failed for these IL-6 related conditions.
Area of Science:
- Immunology
- Neurology
- Rheumatology
Background:
- Macrophage activation syndrome (MAS) is characterized by hyperinflammation driven by cytokines like interleukin-6 (IL-6).
- Interleukin-6 (IL-6) plays a significant role in the pathology of several neuroimmunological diseases.
- Comorbidity between MAS and neuroimmunological disorders is infrequently reported.
Purpose of the Study:
- To report two cases of MAS comorbid with IL-6 related neuroimmunological diseases.
- To highlight the potential role of IL-6 in the pathogenesis of these combined conditions.
- To evaluate treatment strategies for MAS and comorbid neuroimmunological diseases.
Main Methods:
- Case report analysis of two patients with MAS and co-occurring neuroimmunological diseases.
- Review of clinical presentation, laboratory findings, and treatment responses.
- Assessment of efficacy of standard immunosuppressive therapies versus IL-6 receptor blockade.
Main Results:
- Both patients presented with MAS comorbid with IL-6 related neuroimmunological diseases (myasthenia gravis and neuromyelitis optica spectrum disorders).
- Initial immunosuppressive treatments were insufficient to control symptoms.
- Administration of antibodies targeting the IL-6 receptor led to clinical stabilization in both cases.
Conclusions:
- MAS can be associated with IL-6 related neuroimmunological diseases.
- Targeting the IL-6 receptor is a potential therapeutic strategy for patients with comorbid MAS and these specific neuroimmunological conditions.
- Considering the shared IL-6 pathway is crucial for effective management of these complex cases.
