Systemic autoinflammatory disease in adults

Systemic autoinflammatory disorders comprise an expanding group of rare conditions. They are mediated by dysfunction the innate immune system and share a core phenotypic manifestations including recurrent attacks fever, cutaneous signs, chest or abdominal pain, lymphadenopathy, vasculopathy, musculoskeletal symptoms. Diagnosis is often established in childhood, but growing number adult patients being recognized with systemic disorders, adult-onset disease. In this review, we provide concise update on pathophysiology, clinical presentation, diagnostic approach emphasis patient population. Despite recent advances genetic testing, diagnosis disease based thorough knowledge phenotype. Becoming acquainted features these may assist developing high index suspicion for presenting unexplained episodes fever inflammation.