Hints for Genetic and Clinical Differentiation of Adult-Onset Monogenic Autoinflammatory Diseases

Figure 5

Diagnostic flow chart for main autoinflammatory diseases displaying recurrent fevers and gastrointestinal (a), serosal (b), and neurological (c) manifestations. List of abbreviations: FMF—familial Mediterranean fever; PFAPA—periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis; BD—Behçet’s disease; CAPS—cryopyrin-associated periodic syndrome; AOSD—adult-onset Still’s disease; NLRC4—nucleotide-binding domain, leucine-rich repeat, and caspase recruitment domain-containing 4; ADA2—adenosine deaminase 2.

(a)

(b)

(c)