Adult-onset systemic autoinflammatory disorders: a clinical approach.

Area of Science:

• Immunology
• Rheumatology
• Genetics

Background:

• Autoinflammatory disorders (AIDs) are immune-mediated syndromes stemming from innate immune system dysfunction.
• AIDs can be monogenic or polygenic, with systemic forms presenting with fever and acute-phase reactant elevation.

Purpose of the Study:

• To review common adult-onset systemic autoinflammatory disorders.
• Focus on polygenic and mixed-pattern AIDs, presumed more prevalent in adults.
• Exclude organ-specific or childhood-onset AIDs.

Main Methods:

• Literature search of the Medline database.
• Categorization of AIDs into mixed-pattern, polygenic, and adult-onset monogenic types.
• Exclusion of organ-specific and childhood-onset systemic AIDs.

Main Results:

• Most adult-onset AIDs are polygenic, though late-onset monogenic forms exist (e.g., Familial Mediterranean Fever).
• Diagnosis in adults is often delayed, sometimes until complications like amyloidosis arise.
• Common presentations include fever of unknown origin and polyserositis.

Conclusions:

• Adult-onset Still's disease and Familial Mediterranean Fever are common considerations in adults with unexplained fever.
• Early exclusion of prevalent AIDs is crucial for timely diagnosis.
• Treatment options include colchicine, NSAIDs, steroids, immunosuppressants, and biologic agents targeting IL-1 and TNF.