Inflammation and autoimmunity in patients anti-neutrophil nuclear antibodies positive

Two patients, female, with overlap syndrome AIH/PSC and pANNA positive, was placed at two extremities of the bimodal curve that characterised the AIH I° behaviour.Therefore they show difference of age (V.L. 76 years and E.G. 8 years) and different prognosis and therapy answer: the disease of the young patient has an aggressive outcome and fails the treatment more commonly.The more important theory to explain this different features was the “molecular footprint hypothesis” that considers decisive the type of HLA II°. The two patients considered have the same HLADR3. The serological analysis and the recent pathogenically orientations show that the local motives regulate the features of disease: in the child the inflammatory component prevails and maintains a chronic granulomatous aggression, whereas in the old patient the non specific autoimmune IgG prevails and drives a fibrosis evolution and immunosuppressive therapy response.The common positive pANNA can be expression of common chronic auto-inflammatory granulomatous origin.