From the Guidelines
The most likely consequence of impaired synthesis of interleukin-1 (IL-1) is a compromised inflammatory response and immune function, leading to increased susceptibility to infections, particularly bacterial infections, and potentially delayed wound healing. This is because IL-1 is a critical pro-inflammatory cytokine that plays a central role in initiating and regulating inflammation, particularly in response to infection and tissue injury 1. When IL-1 synthesis is impaired, the body cannot mount an adequate inflammatory response to pathogens, which may result in recurrent infections. Additionally, IL-1 is important for recruiting immune cells to injury sites and promoting tissue repair processes, so its deficiency may lead to delayed wound healing. Fever responses might also be diminished, as IL-1 is a key endogenous pyrogen that acts on the hypothalamus to increase body temperature during infection. The adaptive immune response could be affected too, as IL-1 helps activate T cells and promotes antibody production by B cells. Some key points to consider in the management of patients with impaired IL-1 synthesis include:
- Monitoring of disease activity and burden of disease, which should be done regularly depending on disease activity and severity, often requiring a multidisciplinary team 1
- Symptom control, which can be monitored with validated tools that assess disease-specific symptoms, with patient-reported outcome and quality of life assessments, and by recording missing school or work days 1
- Systemic inflammation, which should be monitored by following up inflammatory markers, including peripheral neutrophilia, CRP, and ESR, and SAA and S100 protein may be used as inflammatory markers where available 1
- Organ damage, which should be monitored based on disease manifestations and can include monitoring of hearing loss, eye disease, aseptic meningitis, CNS disease, and bone disease 1 Clinically, patients with IL-1 deficiency might present with recurrent infections, poor wound healing, and potentially reduced inflammatory responses to various stimuli, highlighting IL-1's essential role in coordinating both innate and adaptive immune responses. It is also important to note that patients with IL-1 mediated autoinflammatory diseases, such as cryopyrin-associated periodic syndromes (CAPS), tumour necrosis factor receptor-associated periodic syndrome (TRAPS), mevalonate kinase deficiency (MKD), and deficiency of the IL-1 receptor antagonist (DIRA), may have variable multiorgan involvement and require a tailored approach to management 1.
From the Research
Impaired Synthesis of Interleukin-1 (IL-1)
The impaired synthesis of interleukin-1 (IL-1) can have significant consequences on the immune response and overall health of an individual. Some key points to consider include:
- IL-1 is a crucial cytokine involved in the initiation of the immune response and plays a key role in the pathogenesis of various diseases 2, 3.
- The impaired synthesis of IL-1 can lead to a compromised immune response, making an individual more susceptible to infections and diseases 4.
- IL-1 is also involved in the regulation of systemic changes in neurologic, metabolic, hematologic, and endocrinologic systems, and its impaired synthesis can disrupt these processes 2.
- The consequences of impaired IL-1 synthesis can be far-reaching, affecting various aspects of health, including:
Potential Consequences of Impaired IL-1 Synthesis
Some potential consequences of impaired IL-1 synthesis include:
- Increased risk of infections and diseases due to a compromised immune response 4
- Disrupted regulation of systemic changes in neurologic, metabolic, hematologic, and endocrinologic systems 2
- Potential long-term effects on overall health and well-being, including increased risk of chronic diseases 5, 6
- Impaired wound healing and tissue repair due to the role of IL-1 in the regulation of inflammatory responses 3