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

Figure 2

Diagnostic flow chart for main autoinflammatory diseases displaying recurrent fevers and mucocutaneous 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; SD—Schnitzler’s disease; AOSD—adult-onset Still’s disease; NLRP12—nucleotide-binding domain and leucine-rich repeat-containing protein 12; NLRC4—nucleotide-binding domain, leucine-rich repeat, and caspase recruitment domain-containing 4; PLAID—phospholipase C gamma 2-associated antibody deficiency and immune dysregulation; PAAND—pyrin-associated autoinflammation with neutrophilic dermatosis; PSTPiP1—proline-serine-threonine phosphatase interacting protein 1.