Abstract
Autoinflammatory diseases (ADs) refer to a group of disorders of the innate immune system, mainly monogenic, marked by episodes of systemic inflammation. Aseptic meningitis is a rare neurological manifestation of ADs characterized by meningeal inflammation, negative routine cultures in the cerebrospinal fluid and identical signs and symptoms of bacterial meningitis. Herein, the aim of this review article is to describe the association between aseptic meningitis and ADs, especially in patients with familial Mediterranean fever (FMF) and chronic infantile neurological cutaneous articular (CINCA) syndrome. We will discuss the emerging role of proinflammatory cytokines, such as interleukin 1 (IL-1), interleukin 6 (IL-6) and tumor necrosis factor-alpha (TNF-α), in the pathogenesis of aseptic meningitis in ADs, and will explore recent treatment developments, such as the use of biological agents.
Highlights
BackgroundAutoinflammatory diseases (ADs) are systemic disorders of the innate immune system, characterized by repeated episodes of inflammation, without the presence of autoantibodies or reactive T cells
An overactive innate immune system plays a key role in the pathogenesis of these disorders through the increased production and activity of interleukin 1β (IL-1β) and interleukin 6 (IL-6) [1]
This mutation induces an overexpression of IL-1β, leading to episodic fever associated with organ-specific inflammatory symptoms
Summary
Autoinflammatory diseases (ADs) are systemic disorders of the innate immune system, characterized by repeated episodes of inflammation, without the presence of autoantibodies or reactive T cells. FMF: familial Mediterranean fever, MKD: mevalonate kinase deficiency, TRAPS: tumor necrosis factor receptor-associated periodic syndrome, CINCA: chronic infantile neurological cutaneous articular syndrome, FCAS: familial cold autoinflammatory syndrome, MWS: Muckle–Wells syndrome, BS: Blau syndrome, DIRA: deficiency of interleukin 1 receptor antagonist, MS: Majeed syndrome, PAPA: pyogenic arthritis pyoderma gangrenosum and cystic acne syndrome, NLRP: NACHT domain-, leucine-rich repeat- and pyrin domain-containing protein, AD: autosomal dominant, AR: autosomal recessive [5]. CAPS are a group of ADs transmitted by autosomal dominant inheritance caused by mutations in the NLRP3 gene, encoding cryopyrin, an inflammasome protein that directly activates IL-1β This mutation induces an overexpression of IL-1β, leading to episodic fever associated with organ-specific inflammatory symptoms. There are no approved biologic disease-modifying antirheumatic drugs for aseptic meningitis in ADs
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
