Innate versus acquired immune response in the pathogenesis of recurrent idiopathic pericarditis

Interleukin‐1 as a Common Denominator from Autoinflammatory to Autoimmune Disorders: Premises, Perils, and Perspectives

“…Idiopathic recurrent acute pericarditis (IRAP) is a troubling complication of acute pericarditis, occurring in approximately 30% of cases [ 204 ]. Recently, considering clinical and laboratory features of IRAP (absence of autoantibodies or self-antigen-specific T lymphocytes) [ 205 – 208 ] and the growing evidence about IRAP favourable response to IL-1 inhibition, it has been hypothesized that this condition can be included in the group of AIDs [ 209 – 211 ]. However, IRAP may occur in the framework of two peculiar AIDs, familial Mediterranean fever (FMF) and TNF receptor-associated periodic syndrome (TRAPS), becoming a diagnostic clue for identifying these disorders [ 22 , 59 , 212 – 214 ].…”

Monogenic Autoinflammatory Syndromes: State of the Art on Genetic, Clinical, and Therapeutic Issues

“…Serous membrane inflammation is also common, usually in the form of polyserositis [ 62 – 65 , 72 – 74 ]. Pericardial or myocardial involvement has also been reported as the only clinical manifestation of TRAPS [ 9 – 11 , 62 , 64 , 71 , 73 , 75 – 77 ].…”

Caution Should be Used in the Recognition of Adult-Onset Autoinflammatory Disorders: Facts or Fiction?

“…On the contrary, a more significant variability in terms of clinical phenotype may be observed in TRAPS (Aksentijevich et al, 2001; Dodé et al, 2002; Aganna et al, 2003; Ravet et al, 2006; Cantarini et al, 2009, 2010c,d,e,f,g; Rigante et al, 2011; Brizi et al, 2012): this heterogeneity is largely related to the wide spectrum of known TNFRSF1A mutations, which can be distinguished into high-penetrance and low-penetrance variants (Touitou et al, 2004). As in FMF, adult-onset of symptoms is usually related to low-penetrance mutations, which are associated with feeble clinical signs and a lower risk of amyloidosis (Aksentijevich et al, 2001; Dodé et al, 2002; Aganna et al, 2003).…”

“…As in FMF, adult-onset of symptoms is usually related to low-penetrance mutations, which are associated with feeble clinical signs and a lower risk of amyloidosis (Aksentijevich et al, 2001; Dodé et al, 2002; Aganna et al, 2003). In addition, TRAPS patients carrying low-penetrance TNFRSF1A variants may show atypical clinical manifestations and symptoms that mimic other AIDs and/or autoimmune diseases, such as idiopathic recurrent acute pericarditis, thus hindering differential diagnosis (Cantarini et al, 2009, 2010d,e,g, 2012a; Rigante et al, 2011). These alleles have also been described in patients with recurrent inflammatory attacks who lack the most typical TRAPS manifestations, even when the duration of fever episodes is short and might resemble FMF, and even in healthy controls (Dodé et al, 2002; Ravet et al, 2006; Cantarini et al, 2010a, 2011; Muscari et al, 2012).…”